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The Bone & Joint Journal
Vol. 106-B, Issue 1 | Pages 93 - 98
1 Jan 2024
Broida SE Tsoi KM Rose PS Ferguson PC Griffin AM Wunder JS Houdek MT

Aims

The sacroiliac joint (SIJ) is the only mechanical connection between the axial skeleton and lower limbs. Following iliosacral resection, there is debate on whether reconstruction of the joint is necessary. There is a paucity of data comparing the outcomes of patients undergoing reconstruction and those who are not formally reconstructed.

Methods

A total of 60 patients (25 females, 35 males; mean age 39 years (SD 18)) undergoing iliosacral resection were reviewed. Most resections were performed for primary malignant tumours (n = 54; 90%). The mean follow-up for surviving patients was nine years (2 to 19).


The Bone & Joint Journal
Vol. 105-B, Issue 7 | Pages 808 - 814
1 Jul 2023
Gundavda MK Lazarides AL Burke ZDC Focaccia M Griffin AM Tsoi KM Ferguson PC Wunder JS

Aims

The preoperative grading of chondrosarcomas of bone that accurately predicts surgical management is difficult for surgeons, radiologists, and pathologists. There are often discrepancies in grade between the initial biopsy and the final histology. Recent advances in the use of imaging methods have shown promise in the ability to predict the final grade. The most important clinical distinction is between grade 1 chondrosarcomas, which are amenable to curettage, and resection-grade chondrosarcomas (grade 2 and 3) which require en bloc resection. The aim of this study was to evaluate the use of a Radiological Aggressiveness Score (RAS) to predict the grade of primary chondrosarcomas in long bones and thus to guide management.

Methods

A total of 113 patients with a primary chondrosarcoma of a long bone presenting between January 2001 and December 2021 were identified on retrospective review of a single oncology centre’s prospectively collected database. The nine-parameter RAS included variables from radiographs and MRI scans. The best cut-off of parameters to predict the final grade of chondrosarcoma after resection was determined using a receiver operating characteristic curve (ROC), and this was correlated with the biopsy grade.


The Bone & Joint Journal
Vol. 105-B, Issue 5 | Pages 559 - 567
1 May 2023
Aoude A Nikomarov D Perera JR Ibe IK Griffin AM Tsoi KM Ferguson PC Wunder JS

Aims

Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs.

Methods

A total of 354 consecutive patients with a GCTB underwent joint salvage by curettage and reconstruction with bone graft and/or cement or en bloc resection. Patient, tumour, and treatment factors were analyzed for their impact on LR. Patients treated with denosumab were excluded.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 102 - 102
1 Dec 2022
Gundavda M Lazarides A Burke Z Griffin A Tsoi K Ferguson P Wunder JS
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Cartilage lesions vary in the spectrum from benign enchondromas to highly malignant dedifferentiated chondrosarcomas. From the treatment perspective, enchondromas are observed, Grade 1 chondrosarcomas are curetted like aggressive benign tumors, and rest are resected like other sarcomas. Although biopsy for tissue diagnosis is the gold standard for diagnosis and grade determination in chondrosarcoma, tumor heterogeneity limits the grading in patients following a biopsy. In the absence of definite pre-treatment grading, a surgeon is therefore often in a dilemma when deciding the best treatment option. Radiology has identified aggressive features and aggressiveness scores have been used to try and grade these tumors based on the imaging characteristics but there have been very few published reports with a uniform group and large number of cases to derive a consistent scoring and correlation.

The authors asked these study questions :(1) Does Radiology Aggressiveness and its Score correlate with the grade of chondrosarcoma? (2) Can a cut off Radiology Agressiveness Score value be used to guide the clinician and add value to needle biopsy information in offering histological grade dependent management?

A retrospective analysis of patients with long bone extremity intraosseous primary chondrosarcomas were correlated with the final histology grade for the operated patients and Radiological parameters with 9 parameters identified a priori and from published literature (radiology aggressiveness scores - RAS) were evaluated and tabulated. 137 patients were identified and 2 patients were eliminated for prior surgical intervention. All patients had tissue diagnosis available and pre-treatment local radiology investigations (radiographs and/or CT scans and MRI scans) to define the RAS parameters.

Spearman correlation has indicated that there was a significant positive association between RAS and final histology grading of long bone primary intraosseous chondrosarcomas. We expect higher RAS values will provide grading information in patients with inconclusive pre-surgery biopsy to tumor grades and aid in correct grade dependant surgical management of the lesion. Prediction of dedifferentiated chondrosarcoma from higher RAS will be attempted and a correlation to obtain a RAS cut off, although this may be challenging to achieve due to the overlap of features across the intermediate grade, high grade and dedifferentiated grades.

Radiology Aggressiveness correlates with the histologic grade in long bone extremity primary chondrosarcomas and the correlation of radiology and biopsy can aid in treatment planning by guiding us towards a low-grade neoplasm which may be dealt with intralesional extended curettage or high-grade lesion which need to be resected. Standalone RAS may not solve the grading dilemma of primary long bone intraosseous chondrosarcomas as the need for tissue diagnosis for confirming atypical cartilaginous neoplasm cannot be eliminated, however in the event of a needle biopsy grade or inconclusive open biopsy it may guide us towards a correlational diagnosis along with radiology and pathology for grade based management of the chondrosarcoma.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 94 - 94
1 Dec 2022
Lazarides A Novak R Burke Z Gundavda M Ghert M Rose P Houdek M Wunder JS Ferguson P Griffin A Tsoi K
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Radiation induced sarcoma of bone is a rare but challenging disease process associated with a poor prognosis. To date, series are limited by small patient numbers; data to inform prognosis and the optimal management for these patients is needed. We hypothesized that patients with radiation-induced pelvic bone sarcomas would have worse surgical, oncologic, and functional outcomes than patients diagnosed with primary pelvic bone sarcomas

This was a multi-institution, comparative cohort analysis. A retrospective chart review was performed of all patients diagnosed with a radiation-induced pelvic and sacral bone sarcoma between January 1st, 1985 and January 1st, 2020 (defined as a histologically confirmed bone sarcoma of the pelvis in a previously irradiated field with a minimum 3-year interval between radiation and sarcoma diagnosis). We also identified a comparison group including all patients diagnosed with a primary pelvic osteosarcoma/spindle cell sarcoma of bone (i.e. eligible for osteosarcoma-type chemotherapy) during the same time interval. The primary outcome measure was disease-free and overall survival.

We identified 85 patients with primary osteosarcoma of the pelvis (POP) and 39 patients with confirmed radiation induced sarcoma of the bony pelvis (RISB) undergoing surgical resection. Patients with RISB were older than patients with POP (50.5 years vs. 36.5 years, p67.7% of patients with POP underwent limb salvage as compared to 77% of patients with RISB; the type of surgery was not different between groups (p=.0.24). There was no difference in the rate of margin positive surgery for RISB vs. POP (21.1% vs. 14.1%, p=0.16). For patients undergoing surgical resection, the rate of surgical complications was high, with more RISB patients experiencing complications (79.5%) than POP patients (64.7%); this approached statistical significance (p=0.09).

15.4% of patients with RISB died perioperative period (within 90 days of surgery) as compared to 3.5% of patients with POP (p= 0.02). For patients undergoing surgical resection, 5-year OS was significantly worse for patients with RISB vs. POP (27.3% vs. 47.7%, p=0.02). When considering only patients without metastatic disease at presentation, a significant difference in 5-year survival remains for patients with RISB vs. POP (28.6% vs. 50%, p=0.03) was a trend towards poorer 5-year DFS for patients with RISB vs. POP (30% vs. 47.5%), though this did not achieve statistical significance (p=0.09).

POP and RISB represent challenging disease processes and the oncologic outcomes are similarly poor between the two; however, the disease course for patients with RISB appears to be worse overall. While surgery can result in a favorable outcome for a small subset of patients, surgical treatment is fraught with complications.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 103 - 103
1 Dec 2022
Lazarides A Burke Z Gundavda M Griffin A Tsoi K Wunder JS Ferguson P
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Wide resection, with or without adjuvant therapy, is the mainstay of treatment for soft tissue sarcoma of the extremities. The surgical treatment of soft tissue sarcoma can portend a prolonged course of recovery from a functional perspective. However, data to inform the expected course of recovery following sarcoma surgery is lacking. The purpose of this study was to identify time to maximal functional improvement following sarcoma resection and to identify factors that delay the expected course of recovery.

A retrospective chart review was performed of all patients undergoing surgical treatment of a soft tissue sarcoma of the extremities between January 1st, 1985 and November 15, 2020 with a minimum of 1 follow up. The primary outcome measure was time to maximal functional improvement, defined as failure to demonstrate improvement on two consecutive follow up appointments, as defined by the functional outcome measures of Toronto Extremity Salvage Score (TESS) and Musculoskeletal Tumor Society (MSTS) Score or by achieving 90% of maximum outcome score.

We identified 1188 patients who underwent surgical resection of a soft tissue sarcoma of the extremities. Patients typically achieved a return to their baseline level of function by 1 year and achieved “maximal” functional recovery by 2 year's time postoperatively.

Patient and tumor factors that were associated with worse functional outcome scores and a delayed return to maximal functional improvement included older age (p=0.007), female sex (p-0.004), larger tumor size (p < 0 .001), deep tumor location (p < 0 .001), pelvic location (p < 0 .001), higher tumor grade (p < 0 .001). Treatment factors that were associated with worse functional outcome scores and a delayed return to maximal functional improvement included use of radiation therapy (p < 0 .001), perioperative complications (p < 0 .001), positive margin status (p < 0 .001) and return of disease, locally or systemically (p < 0 .001).

Most patients will recover their baseline function by 1 year and achieve “maximal” recovery by 2 years’ time following surgical resection for soft tissue sarcoma of the extremities. Several patient, tumor and treatment factors should be used to counsel patients as to a delayed course of recovery.


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 97 - 97
1 Dec 2022
Burke Z Lazarides A Gundavda M Griffin A Tsoi K Ferguson P Wunder JS
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Traditional staging systems for high grade osteosarcoma (Enneking, MSTS) are based largely on gross surgical margins and were developed before the widespread use of neoadjuvant chemotherapy. It is now well known that both microscopic margins and chemotherapy are predictors of local recurrence. However, neither of these variables are used in the traditional surgical staging and the precise safe margin distance is debated. Recently, a novel staging system utilizing a 2mm margin cutoff and incorporating precent necrosis was proposed and demonstrated improved prognostic value for local recurrence free survival (LRFS) when compared to the MSTS staging system. This staging system has not been validated beyond the original patient cohort. We propose to analyze this staging system in a cohort of patients with high-grade osteosarcoma, as well as evaluate the ability of additional variables to predict the risk of local recurrence and overall survival.

A retrospective review of a prospectively collected database of all sarcoma patients between 1985 and 2020 at a tertiary sarcoma care center was performed. All patients with high-grade osteosarcoma receiving neo-adjuvant chemotherapy and with no evidence of metastatic disease on presentation were isolated and analyzed. A minimum of two year follow up was used for surviving patients. A total of 225 patients were identified meeting these criteria. Univariate analysis was performed to evaluate variable that were associated with LRFS. Multivariate analysis is used to further analyze factors associated with LRFS on univariate analysis.

There were 20 patients (8.9%) who had locally recurrent disease. Five-year LRFS was significantly different for patients with surgical margins 2mm or less (77.6% v. 93.3%; p=0.006) and those with a central tumor location (67.9 v. 94.4; <0.001). A four-tiered staging system using 2mm surgical margins and a percent necrosis of 90% of greater was also a significant predictor of 5-year LRFS (p=0.019) in this cohort. Notably, percent necrosis in isolation was not a predictor of LRFS in this cohort (p=0.875). Tumor size, gender, and type of surgery (amputation v. limb salvage) were also analyzed and not associated with LRFS. The MSTS surgical margin staging system did not significantly stratify groups (0.066).

A 2mm surgical margin cutoff was predictive of 5-year LRFS in this cohort of patients with localized high-grade osteosarcoma and a combination of a 2mm margin and percent necrosis outperformed the prognostic value of the traditional MSTS staging system. Utilization of this system may improve the ability of surgeons to stage thier patients. Additional variables may increase the value of this system and further validation is required.


Bone & Joint Research
Vol. 10, Issue 9 | Pages 602 - 610
24 Sep 2021
Tsoi KM Gokgoz N Darville-O'Quinn P Prochazka P Malekoltojari A Griffin AM Ferguson PC Wunder JS Andrulis IL

Aims

Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients.

Methods

Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).


The Bone & Joint Journal
Vol. 103-B, Issue 2 | Pages 391 - 397
1 Feb 2021
Houdek MT Wunder JS Abdel MP Griffin AM Hevesi M Rose PS Ferguson PC Lewallen DG

Aims

Hip reconstruction after resection of a periacetabular chondrosarcoma is complex and associated with a high rate of complications. Previous reports have compared no reconstruction with historical techniques that are no longer used. The aim of this study was to compare the results of tantalum acetabular reconstruction to both historical techniques and no reconstruction.

Methods

We reviewed 66 patients (45 males and 21 females) with a mean age of 53 years (24 to 81) who had undergone acetabular resection for chondrosarcoma. A total of 36 patients (54%) underwent acetabular reconstruction, most commonly with a saddle prosthesis (n = 13; 36%) or a tantalum total hip arthroplasty (THA) (n = 10; 28%). Mean follow-up was nine years (SD 4).


The Bone & Joint Journal
Vol. 103-B, Issue 1 | Pages 184 - 191
1 Jan 2021
Perrin DL Visgauss JD Wilson DA Griffin AM Abdul Razak AR Ferguson PC Wunder JS

Aims

Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up.

Methods

Patients with GCTB of the limb considered high-risk for unsuccessful joint salvage, due to minimal periarticular and subchondral bone, large soft tissue mass, or pathological fracture, were treated with Denosumab followed by extended intralesional curettage with the goal of preserving the joint surface. Patients were followed for local recurrence, metastasis, and secondary sarcoma.


The Bone & Joint Journal
Vol. 102-B, Issue 12 | Pages 1752 - 1759
1 Dec 2020
Tsuda Y Tsoi K Stevenson JD Laitinen M Ferguson PC Wunder JS Griffin AM van de Sande MAJ van Praag V Leithner A Fujiwara T Yasunaga H Matsui H Parry MC Jeys LM

Aims

Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma.

Methods

The study population consisted of 391 patients from two international sarcoma centres (development cohort) who had undergone definitive surgery for a localized high-grade (histological grade II or III) conventional primary central chondrosarcoma or dedifferentiated chondrosarcoma. Disease progression captured the first event of either metastasis or local recurrence. An independent cohort of 221 patients from three additional hospitals was used for external validation. Two nomograms were internally and externally validated for discrimination (c-index) and calibration plot.


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_8 | Pages 38 - 38
1 Aug 2020
Mattei J Alshaygy I Basile G Griffin A Wunder JS Ferguson P
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Sarcomas generally metastasize to the lung, while extra-pulmonary metastases are rare. However, they may occur more frequently in certain histological sub-types. Bone metastases from bone and soft tissue sarcomas account for a significant number of extra-pulmonary disease. Resection of lung metastases is widely accepted as therapeutic option to improve the survival of oligometastatic patients but there is currently no literature supporting curative surgical management of sarcoma bone metastases. Most are treated on a case-by-case basis, following multidisciplinary tumour boards recommendations. One study reported some success in controlling bone metastases using radiofrequency ablation. Our goal was to assess the impact of curative resection of bone metastases from soft tissue and bone sarcomas on oncologic outcomes.

Extensive review of literature was done to evaluate epidemiological and outcomes of bone metastases in sarcoma. We examined our prospective database for all cases of bone metastases from sarcoma treated with surgical resection between 1990 and 2016. Epidemiology, pathology, metastatic status upon diagnosis, type of secondary relapses and their treatments were recorded.

Overall survival and disease-free survival were calculated and compared to literature.

Thirty-five patients were included (18 men, 17 women) with a mean age of 46 years. Fifteen were soft tissue (STS) and 20 were bone (BS) sarcomas. Most STS were fibrosarcomas, leiomyosarcomas or UPS while chondrosarcomas and osteosarcomas were the most frequent BS. Nine (60%) STS were grade 3, 4 (27%) grade 2 and one grade 1 (3%). Eight (23%) were metastatic upon diagnosis (6 lungs, 3 bone). Treatment of the primary tumour included wide excision with reconstruction and (neo)-adjuvant therapies as required. Margins were negative in 32 cases and micro-positive in 3 cases. Amputation occurred in 6 (17%) cases. Primary lung metastases were treated by thoracotomy and primary bone metastases by wide excision. First relapse occurred in bone in 19 cases (54%), lungs and bone in 7 cases, 5 in lungs and 4 in soft-tissues. Lung metastases were treated by thoracotomy and chemotherapy in 3 cases, chemotherapy alone in the remaining cases. Bone metastases were treated by wide resection-reconstruction in 24 cases, extensive curettage in 4. Soft tissue relapses were re-excised in 4 patients. Two amputations were required. All margins were negative except for the 4 treated by curettage. Fourteen second relapses occurred in bone, 7 were radically-excised and 2 curetted.

At last follow-up, 6 patients were alive (overall survival of 17%), with a mean survival of 57 months, a median overall survival of 42.5 months and a median disease-free survival (DFS) of 17 months. Overall survival was 17%, compared to an 11% 10-year survival previously reported in metastatic sarcomas. Median disease-free survival was better in this study, compared to 10 months in literature, so as median OS (42.5 months vs 15). Three patients were alive with no evidence of disease. DFS, OS and median survival seemed to be improved by bone metastases wide excision and even if several recurrences occur, curative surgery with adjuvant therapies should be considered.


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_8 | Pages 4 - 4
1 Aug 2020
Basile G Alshaygy I Mattei J Griffin A Ferguson P Wunder JS
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Lymph node metastasis are a rare occurrence in soft tissue sarcomas of the extremity, arising in less than 5% of patients. Few studies have evaluated the prognosis and survival of patients with a lymph node metastasis. Early reports compared lymph node involvement to lung metastasis, while others suggested a slightly better outcome. The purpose of this study was to evaluate the impact of lymph node metastasis on patient survival and to investigate the histologic and clinical features associated with lymph node involvement.

A retrospective review was done of the prospectively collected soft tissue sarcoma database at our institution. Two thousand forty-five patients had surgery for soft tissue sarcoma of an extremity between January 1986 and August 2017. Included patients either presented with a synchronous lymph node metastasis or were diagnosed with a lymph node metastasis after their initial treatment. Demographic, treatment, and outcome data for patients with lymph node involvement were obtained from the clinical and radiographic records.

Lymph node metastases were identified as palpable adenopathy by physical examination and were further characterized on cross-sectional imaging by computed tomography (CT) or magnetic resonance imaging (MRI) scans. All cases were confirmed by pathologic examination of biopsy specimens. A pathologist with expertise in sarcoma determined the histologic type and graded tumors as 1, 2, or 3.

One hundred eighteen patients with a mean age of 55.7 (SD=18.9) were included in our study. Seventy-two (61.3%) out of 119 patients were male. Thirty six patients (57.1%) had lymph node involvement at diagnosis. The mean follow-up from the date of the first surgery was 56.3 months. The most common histological diagnoses were Malignant fibrous histiocytoma (35) and liposarcoma (12). Ninety eight patients (89%) underwent surgical treatment of the lymph node metastasis while 21 (17.6%) were treated with chemotherapy and/or radiation therapy. The mean survival was 52.6 months (range 1–307).

Our results suggest that patients with a lymph node metastasis have a better prognosis than previously described. Their overall survival is superior to patients diagnosed with lung metastasis. A signifant proportion of patients may expect long term survival after surgical excision of lymph node metastasis. Furthermore, our study also indicates that different histological subtypes such as liposarcoma or malignant peripheral nerve sheath tumor (MPNST) may also be responsible for lymph node metastasis. Additional studies to further improve the treatment of soft tissue sarcoma nodal metastasis are warranted.


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_8 | Pages 5 - 5
1 Aug 2020
Alshaygy I Mattei J Basile G Griffin A Ferguson P Wunder JS
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Dermatofibrosarcoma protuberans (DFSP) is a rare, monoclonal dermal neoplasm. DFSP is known to be locally aggressive and infiltrative, but with a very low systemic recurrence risk. It is reported to be associated with high local recurrence rates following surgical excision. Positive or marginal resection margins can lead to a high risk of local recurrence. The objective was to determine the oncologic outcome for DFSP treated at our institution.

We reviewed our prospectively collected database for all DFSP treated at our unit between 1990 and 2016. Patients were included whether or not they had excision prior to referral (“whoops” procedure). Those with fibrosarcomatous degeneration at presentation to our unit or less than 1 year of follow-up were excluded. The goal of surgery was a negative margin with a minimum margin of 2 cm where possible. Patients were followed up after surgery to monitor complications, recurrence, transformation and/or metastasis.

139 patients with a mean age of 42.7 (SD=14.1) were included. Mean follow-up was 56 months.101 patients had prior “whoops” surgery before referral. 14 patients were also treated with radiotherapy (13 preoperatively, 1 postoperatively). Following surgery, 6 patients had positive margins, 4 underwent radiation treatment while the other 2 had no further treatment. One patient who presented to our unit with a local recurrence developed a further local recurrence, which demonstrated fibrosarcomatous degeneration at the time of resection (1/139, 0.7%). 1 other patient developed a lesion at another site.

The recurrence rate in our DFSP cohort is significantly lower than previous reports. Wide margin resection following oncologic principles can result in a very low recurrence rate. After the initial recovery phase, these patients do not require ongoing, frequent follow-up. Future studies should look at if closer margins can also produce similar treatment outcome.


The Bone & Joint Journal
Vol. 99-B, Issue 7 | Pages 973 - 978
1 Jul 2017
Gupta S Kafchinski LA Gundle KR Saidi K Griffin AM Wunder JS Ferguson PC

Aims

Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes.

Patients and Methods

A total of 46 patients underwent reconstruction with an intercalary allograft between 1989 and 2014. The patients had a mean age of 32.8 years (14 to 77). The most common diagnoses were osteosarcoma (n = 16) and chondrosarcoma (n = 9). The location of the tumours was in the femur in 21, the tibia in 16 and the humerus in nine. Function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and the Toronto Extremity Salvage Score (TESS). The survival of the graft and the overall survival were assessed using the Kaplan-Meier method.


The Bone & Joint Journal
Vol. 97-B, Issue 4 | Pages 550 - 557
1 Apr 2015
Mollon B Lee A Busse JW Griffin AM Ferguson PC Wunder JS Theodoropoulos J

Pigmented villonodular synovitis (PVNS) is a rare proliferative process of the synovium which most commonly affects the knee and occurs in either a localised (LPVNS) or a diffuse form (DPVNS). The effect of different methods of surgical synovectomy and adjuvant radiotherapy on the rate of recurrence is unclear. We conducted a systematic review and identified 35 observational studies in English which reported the use of surgical synovectomy to treat PVNS of the knee.

A meta-analysis included 630 patients, 137 (21.8%) of whom had a recurrence after synovectomy. For patients with DPVNS, low-quality evidence found that the rate of recurrence was reduced by both open synovectomy (odds ration (OR) = 0.47; 95% CI 0.25 to 0.90; p = 0.024) and combined open and arthroscopic synovectomy (OR = 0.19, 95% CI = 0.06 to 0.58; p = 0.003) compared with arthroscopic surgery. Very low-quality evidence found that the rate of recurrence of DPVNS was reduced by peri-operative radiotherapy (OR = 0.31, 95% CI 0.14 to 0.70; p = 0.01). Very low-quality evidence suggested that the rate of recurrence of LPVNS was not related to the surgical approach.

This meta-analysis suggests that open synovectomy or synovectomy combined with peri-operative radiotherapy for DPVNS is associated with a reduced rate of recurrence. Large long-term prospective multicentre observational studies, with a focus on both rate of recurrence and function, are required to confirm these findings.

Cite this article: Bone Joint J 2015;97-B:550–7.


The Bone & Joint Journal
Vol. 95-B, Issue 8 | Pages 1144 - 1148
1 Aug 2013
Sternheim A Saidi K Lochab J O’Donnell PW Eward WC Griffin A Wunder JS Ferguson P

We investigated the clinical outcome of internal fixation for pathological fracture of the femur after primary excision of a soft-tissue sarcoma that had been treated with adjuvant radiotherapy.

A review of our database identified 22 radiation-induced fractures of the femur in 22 patients (seven men, 15 women). We noted the mechanism of injury, fracture pattern and any complications after internal fixation, including nonunion, hardware failure, secondary fracture or deep infection.

The mean age of the patients at primary excision of the tumour was 58.3 years (39 to 86). The mean time from primary excision to fracture was 73.2 months (2 to 195). The mean follow-up after fracture fixation was 65.9 months (12 to 205). Complications occurred in 19 patients (86%). Nonunion developed in 18 patients (82%), of whom 11 had a radiological nonunion at 12 months, five a nonunion and hardware failure and two an infected nonunion. One patient developed a second radiation-associated fracture of the femur after internal fixation and union of the initial fracture. A total of 13 patients (59%) underwent 24 revision operations.

Internal fixation of a pathological fracture of the femur after radiotherapy for a soft-tissue sarcoma has an extremely high rate of complication and requires specialist attention.

Cite this article: Bone Joint J 2013;95-B:1144–8.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVIII | Pages 141 - 141
1 Sep 2012
Biau DJ Ferguson P Chung P Turcotte R Isler M Riad S Griffin AM Catton C O'Sullivan B Wunder JS
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Purpose

Due to the aging population, an increasing proportion of elderly patients with soft tissue sarcoma are presenting to cancer centers. This population appears to have a worse prognosis but the reasons for this has not been studied in depth. The purpose of this study is to examine the effect of age on the outcome of patients with extremity and trunk soft tissue sarcoma.

Method

This is a multicenter study including 2071 patients with median age at operation of 57 years (1st quartile–3rd quartile: 42–70). The endpoints considered were local recurrence and metastasis with death as a competing event. Cox proportional hazards models were used to estimate hazard ratios across the age ranges with and without adjustment for known confounding factors.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVIII | Pages 208 - 208
1 Sep 2012
Weiss KR Biau DJ Bhumbra R Griffin AM Ferguson P Wunder JS
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Purpose

Ewings sarcoma (EWS) is the second most common primary malignancy of bone. Five-year survival ranges between 50 and 70% for patients who present with localized disease. EWS occurs in both axial and appendicular locations. The prognostic importance of anatomic location (axial vs. appendicular) is unclear. The purpose of this study is to report a single institution experience in the management of patients with EWS, and to investigate anatomic location as a prognostic factor.

Method

After ethics board approval, we searched our prospective sarcoma database for all EWS patients treated between 1989 and 2007. Sixty-seven patients with adequate follow-up were identified and records were reviewed. Axial bone EWS was defined as originating from the spine, sacrum, pelvis, scapula, clavicle, or rib cage. Appendicular bone EWS was defined as disease from any bone in the extremities. Axial soft tissue EWS was defined as originating in the head, neck, or torso. Appendicular soft tissue EWS originated from the extremeties. Sixty-six (99%) patients received chemotherapy. Definitive local treatment was by surgical resection whenever possible (n=44). Radiation therapy (RT) was used for unresectable lesions (n=10) or to treat positive margins (n=10). The primary outcome measure was overall survival (OS) measured from the date of diagnosis to the date of death or last follow-up and estimated using the Kaplan-Meier method. Univariate and multivariate analyses were performed. Besides anatomic location, the variables of tumor size, metastases at diagnosis, treatment with radiation, and surgical margins were studied.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXVIII | Pages 144 - 144
1 Sep 2012
Biau DJ Ferguson P Chung P Riad S Griffin AM Catton C O'Sullivan B Wunder JS
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Purpose

The main predictors in the literature of local control for patients operated on for a soft tissue sarcoma are age, local presentation status, depth, grade, size, surgical margins and radiation. However, due to the competing effect of death (patients who die are withdrawn from the risk of local recurrence), the influence of these predictors on the cumulative probabilities may have been misinterpreted so far. The objective of the study was to interpret the influence of known predictors of local recurrence in a competing risks setting.

Method

This single center study included 1519 patients operated on for a localized soft tissue sarcoma of the extremity or trunk. Cox models were used to estimate the cause specific hazard of known predictors on local recurrence. Cumulative incidences were estimated in a competing risks scenario.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_IV | Pages 573 - 573
1 Nov 2011
Ferguson P Fallis CE Griffin AM Deheshi B Wunder JS
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Purpose: Patients are often referred to tertiary care centers after unplanned excision of soft tissue sarcomas. In situations where the tumour is small and superficial, the situation can often easily be salvaged by re-excision of the tumour bed. However, if the original tumour is large, deep to fascia or directly adjacent to bone or neurovascular structures, the salvage procedure often becomes more complex and morbid. The purpose of this study is to evaluate the effect of unplanned excision of “high-risk” soft tissue sarcomas on patient outcome.

Method: We reviewed our prospectively collected sarcoma database from 1989 to 2006. Patients who underwent definitive resection of a soft tissue sarcoma at our centre were included. Patients were divided into 2 groups based on whether or not they had undergone initial unplanned resection of their tumour prior to referral to our centre for definitive management. Low risk patients who had tumours that were less than 5 cm in diameter, superficial to fascia, and not overlying bone or neurovascular structures were excluded.

Results: A total of 1034 patients met inclusion criteria. Of these, 385 (37%) patients had undergone an unplanned excision prior to referral, while 649 (63%) patients were referred to our centre with an intact tumour without prior unplanned excision. There was a higher percentage of high grade (61% vs. 50%) and deep tumours (88% vs. 65%) in the unplanned excision group, but the mean tumour diameter was smaller in the unplanned excision group (5.9 cm) compared to the control group (10.6 cm). There was no difference between the groups in terms of rate of amputation, necessity for flaps for coverage, and local recurrence-free survival. Complications were more common in the control group (34%) than the unplanned excision group (20%, p< 0.0005, Chi-square). 5-year overall (p< 0.00005, log rank) and metastasis-free (p< 0.00005, log rank) survival were higher in the unplanned excision group. There was no difference in TESS, MSTS87 or MSTS93 functional outcome scores between the groups.

Conclusion: Patients referred to a tertiary sarcoma centre after unplanned resection of a soft tissue sarcoma can still be salvaged with appropriate multidisciplinary care. Patients referred after unplanned excision appear to fare at least as well as those initially resected at our centre, and fare better in terms of some outcomes studied. The majority of this difference can likely be explained by significant differences in important prognostic factors such as grade, size and depth between the unplanned excision group and control group initially managed at our centre. Patients referred after unplanned excisions appear not to have as dismal an outcome as has been previously reported.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_IV | Pages 571 - 571
1 Nov 2011
Weiss KR Bhumbra R Al-Juhani W Griffin A Deheshi B Ferguson P Bell R Wunder JS
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Purpose: Impending and pathologic fractures of the humerus, usually due to metastatic disease, are associated with significant pain, morbidity, loss of function, and diminished quality of life. Several methods of stabilization have been described. Here we report the outcome of fixation using intramedullary poly methyl methacrylate (bone cement) and non-locking plates.

Method: A retrospective review was undertaken which included all patients treated at a tertiary musculoskeletal oncology referral center from February, 1989 to October, 2009. Patients who underwent surgical management of an impending or pathologic fracture of the humerus were included. All patients were treated using the following technique: Vascular tumors were embolized pre-operatively. Following gross tumor removal through curettage, antibiotic bone cement was placed into the humeral canal and bone defect. If there was a fracture, the bone ends were held in place as the cement cured. The humerus was stabilized using non-locking plates fixed with screws inserted through the bone and hardened bone/cement composite. Ideally, plates spanned the osseous defect by at least 2 cortical diameters and often the entire length of the bone.

Results: Clinical records were available for 67 patients who underwent the above procedure. There were 44 males and 23 females with an average age of 62.2 years. In 76% of patients there was a pathologic fracture at presentation, while in 24% it was impending. The most common histology was myeloma (21%), followed by renal (20%) and lung adenocarcinoma (20%). Forty-nine patients (73%) had one plate, 16 (24%) had two plates, one patient had three plates, and one had four plates. Complications occurred in 14 (21%) cases, and eight (12%) required reoperation of the humerus. The most common cause for reoperation was disease progression (six of eight). There were two nerve palsies, one deep infection, and one hardware failure. Interestingly, the single hardware failure occurred in a patient whose pain relief and functional status improved to the point that he fractured his construct while hammering with the affected arm in a home improvement project.

Conclusion: Intralesional tumor resection and stabilization of impending and pathologic fractures of the humerus with the described technique has several attributes. Most importantly, it provides immediate, absolute rigidity of the upper extremity and enables early pain relief and return of function without the need for osseous union. Radiation has no negative effects on the construct. The patient’s local disease burden is reduced, thus helping to alleviate tumor-related pain and slow local disease progression. Finally, this technique is user-friendly and cost-effective as it does not require any special equipment or devices that are not available to community orthopaedic surgeons. This technique provides a durable option for the treatment of impending and pathologic humerus fractures.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_IV | Pages 573 - 573
1 Nov 2011
Friedmann D Gefen A Turcotte RE Wunder JS Roberge D Ferguson P O’Sullivan B Catton C Freeman C Deheshi B Griffin A Riad S Wong C
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Purpose: Lymphoedema is a serious potential complication of the management of extremity soft tissue sarcoma (STS) about which relatively little is known. We aimed to evaluate the incidence of lymphoedema, its severity and associated risk factors following limb salvage for extremity STS.

Method: Lymphoedema severity (EORTC/RTOG) was recorded prospectively in two databases of soft tissue sarcoma patients. Patient’s demographics, tumor characteristics, surgical procedures, radiotherapy dosage, complications and functional outcomes (MSTS, TESS) were also prospectively collected. Charts were also retrospectively abstracted for body mass index (BMI) and medical comorbidities.

Results: 289 patients had sufficient data for analysis (158 male). Mean age was 53 (range 16–88). Mean BMI was 27.4 (range: 15.8–52.1). 209 had lower extremity tumors and 80, upper. Mean tumor size was 8.1 cm (range 1.0–35.6 cm). 77 had no adjuvant radiation, 180 had 50 Gy and 32, 66 Gy. The incidence of lymphoedema was found to be 28.7% (58 mild, 22 moderate, 3 severe). Mean MSTS score was 32 (range: 11–35) and TESS was 89.4 (range: 32.4–100). We grouped cases with lymphoedema grade 0–1 and 2–3. Univariate analysis found significant correlations between the severity of lymphedema and tumor size ≥5 cm (p=0.011), deep location (no patient with a superficial tumor had severe lymphoedema, p=0.001), and radiation dosage 50 vs 66 Gy (p=0.021) but not between upper vs lower extremity (p=0.06).

Conclusion: 9% of STS studied developped significant post-treatment lymphoedema. Large, deep tumors and necessity for 66 Gys were most at risk. This group could be targeted for prophylatic intervention.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_IV | Pages 572 - 572
1 Nov 2011
Bhumbra R Griffin A Weiss KR Al-Juhani W Deheshi B Wunder JS Ferguson P
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Purpose: Massive endoprostheses have become the mainstay of treatment for reconstruction after resection of primary bone tumours. The Kotz Modular Femoral Tibial Replacement (KMFTR, Kotz prosthesis, Stryker Inc.) system has been one of the most widely utilized uncemented modular systems. Although this prosthesis has excellent bone ingrowth characteristics and a low aseptic loosening rate, we have identified a significant incidence of mechanical failure and breakage of the prosthesis. The purpose of this investigation is to review the outcomes after prosthetic revision for a broken Kotz prosthesis.

Method: A retrospective review was undertaken of our institutional database from the years 1989, when we first utilized the Kotz prosthesis, until present. We identified all patients who had undergone a revision of the prosthesis for mechanical failure or prosthetic breakage. Periprosthetic fractures and revisions for polyethylene bushing wear were excluded.

Results: 119 distal femoral, 55 proximal tibial and 47 proximal femoral Kotz endoprostheses (221 in total) have been implanted in our center since 1989. There were 21 revisions (9.5% of total prostheses) for mechanical failure. Of these, 16 were in the distal femur, four in the proximal tibia and one in the proximal femur. Mechanical failures occurred at a mean of 77 months (range 24–170). Of the 21 metal failures, 8 stems broke at the junction of the stem and body, 8 fractured through screw holes in the stem, 3 fractured the derotation lug, one fractured the tibial housing and one lateral side-plate failed. Of these failures only three implants had associated definite loosening and two of these three were cemented. Broken stems initially required extraction whilst preserving as much of the longitudinal and transverse bone stock as possible in order to facilitate osseo-mechanical integration of the revision prosthesis. This was accomplished using trephines to core the ingrown broken stem out of the bone. Over the last 20 years, the 16 broken stems have been revised in 5 patients to larger Kotz uncemented stems, 2 to cemented GMRS stems with an adaptor to the KMFTR system, 3 to Restoration uncemented revision hip stems with a custom adaptor to the KMFTR system, 2 to custom GMRS uncemented stems with an adaptor to the KMFTR system, and 4 to total femurs. All except one patient was alive with no evidence of disease. Post-revision, 14 patients had TESS, MSTS87, MSTS93 scores of 80.5, 25.5 and 70 respectively.

Conclusion: Despite very low aseptic loosening rates, mechanical failure of the Kotz prosthesis continues to be a significant clinical problem even several years after implantation. Fatigue failure often leads to the difficult scenario of removing a well-ingrown uncemented stem. Our data illustrates that these prostheses can often be successfully revised by trephining out the broken stem and inserting new uncemented stems. Functional outcome continues to be good and is comparable to pre-revision levels.


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 8 | Pages 1093 - 1097
1 Aug 2011
Weiss KR Bhumbra R Biau DJ Griffin AM Deheshi B Wunder JS Ferguson PC

Pathological fractures of the humerus are associated with pain, morbidity, loss of function and a diminished quality of life. We report our experience of stabilising these fractures using polymethylmethacrylate and non-locking plates. We undertook a retrospective review over 20 years of patients treated at a tertiary musculoskeletal oncology centre. Those who had undergone surgery for an impending or completed pathological humeral fracture with a diagnosis of metastatic disease or myeloma were identified from our database. There were 63 patients (43 men, 20 women) in the series with a mean age of 63 years (39 to 87).

All had undergone intralesional curettage of the tumour followed by fixation with intramedullary polymethylmethacrylate and plating. Complications occurred in 14 patients (22.2%) and seven (11.1%) required re-operation. At the latest follow-up, 47 patients (74.6%) were deceased and 16 (25.4%) were living with a mean follow-up of 75 months (1 to 184). A total of 54 (86%) patients had no or mild pain and 50 (80%) required no or minimal assistance with activities of daily living. Of the 16 living patients none had pain and all could perform activities of daily living without assistance.

Intralesional resection of the tumour, filling of the cavity with cement, and plate stabilisation of the pathological fracture gives immediate rigidity and allows an early return of function without the need for bony union. The patient’s local disease burden is reduced, which may alleviate tumour-related pain and slow the progression of the disease. The cemented-plate technique provides a reliable option for the treatment of pathological fractures of the humerus.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 266 - 266
1 Jul 2011
Clarkson P Sandford KL LaFrance AE Griffin A Wunder JS Masri BA Goetz TJ
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Purpose: Giant cell tumour (GCT) of the distal radius is associated with high local recurrence rates unless the tumour is aggressively resected, which often leaves a significant skeletal defect. The purpose of this study is to compare the functional outcomes of two commonly used reconstructive techniques, vascularised free fibular transfer (VFF) and non-vascularised structural iliac crest transfer (NIC).

Method: Patients treated for giant cell tumour of the distal radius in either Vancouver or at Mount Sinai Hospital, Toronto were identified in the prospectively collected databases maintained in each centre. Twenty-seven patients were identified, 14 of whom underwent VFF transfer as their primary procedure. The two groups were comparable for age, sex and tumour grade. Functional outcomes were assessed with TESS, MSTS, DASH and the Ankle Osteoarthritis Scale.

Results: Fourteen patients were included in the VFF group, 13 of which were performed as the primary index procedure, one followed prior cementation. Thirteen patients underwent NIC, one followed prior cementation. Two local recurrences occurred in the VFF group and one in NIC group, all treated with local excision. In the VFF group three patients underwent further surgery for cosmesis, hardware removal and tendon release respectively. One is scheduled for future surgery for tendon release. In the NIC group two patients suffered infections requiring debridement, one of which ultimately went on to require free fibular transfer. This patient’s results were included in the NIC group as this was the index procedure. Functional scores showed no differences between the two groups on any of the parameters studied for the upper limb (Mann-Whitney test). The Ankle osteoarthritis scale had a median score of 9% for the six patients on which it was available.

Conclusion: Both VFF and NIC are effective surgical techniques that result in a well-functioning wrist arthrodesis. VFF may be more useful where there is a significant skin defect from previous interventions. We were unable to demonstrate any difference in functional scores between VFF and NIC.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 266 - 267
1 Jul 2011
Jones KB Riad S Griffin A Deheshi B Bell RS Ferguson P Wunder JS
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Purpose: The functional consequences of femoral nerve resection during soft tissue sarcoma management are not well described. Sciatic nerve resection with a sarcoma, once considered an indication for amputation, is now commonly performed during limb salvage. We compared the functional outcomes of femoral and sciatic nerve resections in patients undergoing wide resection of soft-tissue sarcomas.

Method: The prospectively collected database from a tertiary referral center for sarcomas was retrospectively reviewed to identify patients with resection of the femoral or sciatic nerve performed during wide excision of a soft tissue sarcoma. Patient demographics, treatment, complications and functional outcomes were collected.

Results: Ten patients with femoral nerve resections were identified, all women, aged 47 to 78, with large soft tissue sarcomas of varied subtypes. All patients received adjuvant radiotherapy, most pre-operatively. Six patients developed fractures with long-term follow-up, only two of which were in the prior radiation field. Musculoskeletal Tumor Society (MSTS) 1987 scores demonstrated one excellent, 4 good, and 5 fair results. MSTS 1993 scores averaged 71.4 ± 17.2 percent and Toronto Extremity Salvage Scores (TESS) averaged 61.7 ± 21.8. There were no significant differences between the functional scores for patients with femoral or sciatic nerve resections (P=1.0).

Conclusion: Femoral nerve resection appears more morbid than anticipated. The falls to which patients were prone, even years after surgery, subject them to ongoing long-term risks for fractures and other injuries. Nerve-specific functional outcomes should be considered when counseling patients prior possible resection of the femoral nerve for involvement by a soft tissue sarcoma.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_III | Pages 266 - 266
1 Jul 2011
Jones KB Riad S Griffin A Deheshi B Bell RS Ferguson P Wunder JS
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Purpose: Few functional outcomes of total femoral endoprosthetic replacement (TFEPR) using contemporary modular systems are available. We compared functional results between TFEPR patients receiving fixed- and rotating-hinge knee componentry following oncologic resections.

Method: Eighteen TFEPR patients were identified from a prospectively gathered sarcoma database. Six were secondary procedures and 12 primary. Four patients had metastatic carcinoma, 8 osteosarcoma, 4 non-osteogenic spindle cell sarcomas of bone, 1 Ewing’s sarcoma, and 1 femur-invading soft-tissue sarcoma. All reconstructions used modular implants from a single company. Proximally, all were bipolar hip hemiarthoplasties, 12 including abductor reattachment. Distally, 8 had fixed- and 10 had rotating-hinge knee componentry. Toronto Extremity Salvage Score (TESS), and both Musculoskeletal Tumor Society Scores (MSTS) were compared between fixed- and rotating-hinge groups using the Mann-Whitney test.

Results: Complications included 1 hip dislocation, 1 femoral malrotation, and wound problems requiring 3 debridements and 1 amputation. One metastatic carcinoma patient developed local relapse. Follow-up averaged 4 years (range 1 month to 14 years). At latest follow-up, 10 patients had died of disease. Eight remained alive, 6 disease-free, 2 with distant disease. Among patients surviving 6 months, 6 used no assistive devices, 5 used a single cane, and 4 were wheelchair bound, each at least partly due to distant disease progression. TESS averaged 74.5±17.4, MSTS1987 25.2±4.4; and MSTS1993 58.6±22.9 among the 12 patients for whom functional results were available from latest follow-up. No statistically significant differences or even trends were detected between fixed-hinge and rotating-hinge patients (lowest p = 0.755), but both instability problems were in the rotating-hinge group.

Conclusion: While both rotating- and fixed-hinge TFEPR reconstructions may function well, consideration should be given to fixed-hinge knee reconstruction when massive myectomies or poorer conditioning make hip and knee stability a primary concern in the short-term.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 10 | Pages 1475 - 1479
1 Oct 2010
Gortzak Y Kandel R Deheshi B Werier J Turcotte RE Ferguson PC Wunder JS

Various chemicals are commonly used as adjuvant treatment to surgery for giant-cell tumour (GCT) of bone. The comparative effect of these solutions on the cells of GCT is not known. In this study we evaluated the cytotoxic effect of sterile water, 95% ethanol, 5% phenol, 3% hydrogen peroxide (H2O2) and 50% zinc chloride (ZnCI2) on GCT monolayer tumour cultures which were established from six patients. The DNA content, the metabolic activity and the viability of the cultured samples of tumour cells were assessed at various times up to 120 hours after their exposure to these solutions.

Equal cytotoxicity to the GCT monolayer culture was observed for 95% ethanol, 5% phenol, 3% H2O2 and 50% ZnCI2. The treated samples showed significant reductions in DNA content and metabolic activity 24 hours after treatment and this was sustained for up to 120 hours. The samples treated with sterile water showed an initial decline in DNA content and viability 24 hours after treatment, but the surviving cells were viable and had proliferated. No multinucleated cell formation was seen in these cultures.

These results suggest that the use of chemical adjuvants other than water could help improve local control in the treatment of GCT of bone.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 39 - 39
1 Mar 2010
Kulidjian A Deheshi B Ferguson PC Wunder JS Bell RS Griffin A
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Purpose: To review the oncologic outcomes following treatment of soft tissue sarcomas in the foot and ankle, and to determine the results of limb salvage surgery in this anatomically constrained area with often expected close pathologic margins.

Method: One hundred twenty-nine patients with soft tissue sarcomas of the foot were treated at our institution since 1986. Average age at presentations was 55 years old. Females and males were equally represented. Most common diagnosis was MFH in 31 patients, followed by leiomyosarcoma in 16 patients, synovial sarcoma in 13, and clear cell sarcoma in 6, the remainder being other soft tissue sarcomas. Most of sarcomas were intermediate or high grade. Our follow-up averaged 58 months.

Results: Limb salvage surgery was possible in 97 patients. Of those, negative margin excision was achieved in 75 patients, 18 patients had micro-positive margins, and 4 had grossly positive margins. Fifty-three patients in the limb salvage group required free tissue transfer for coverage. Of 97 limb salvage patients, 82 received radiation therapy, 5 patients received chemotherapy. Local recurrence occurred in 24 patients. Two of these were amenable to re-excision, the remaining required amputation. Thirty-two patents developed systemic disease. Average disease-free survival for patients with recurrent disease was 23 months. At last follow-up, 78 patients were alive with no evidence of disease and 24 were alive with disease. Seventeen patients died of disease, and the remainder of other causes.

Conclusion: In the setting of soft tissue sarcoma in foot and ankle, amputation rate is higher than in other anatomic areas. However, limb salvage surgery can be achieved with good oncological outcomes despite often unavoidable close margins. Soft tissue reconstructive procedures aid in achieving good surgical results while radiation therapy aids local control. Given these results, limb salvage in soft tissue tumors of foot and ankle should be the goal.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 38 - 38
1 Mar 2010
Ferguson PC Griffin A Deheshi B Wunder JS
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Purpose: Patients presenting with metastatic soft tissue sarcoma (STS) generally have a poor prognosis. The factors that determine ultimate outcome in this setting are poorly understood. The purpose of this study is to establish factors that affect outcomes in a group of patients with metastatic STS at initial presentation who underwent surgical resection of their primary tumour.

Method: A retrospective review of our institutional database from 1986 to present was carried out. We identified all patients with STS who, at presentation, had metastatic disease. Patients who did not undergo surgical resection of their primary tumour were excluded. Factors affecting overall survival were assessed using the methods of Kaplan and Meier and ANOVA.

Results: We identified 130 patients with metastatic STS (75 male, 55 female). Of these, 81 patients presented with pulmonary metastases, 20 with lymph node metastases, 13 with both lung and lymph node metastases, and in 16 patients initial resection was of a metastatic lesion. Currently only 17 patients (13%) are free of disease. Factors associated with improved survival were lymph node metastases as opposed to pulmonary metastates (p=0.0006), size less than 5 cm (p=0.02), low grade sarcoma (p=0.05) and 3 or fewer pulmonary metastases (p=0.04). Age, gender, histological subtype, anatomic location, use of chemotherapy, depth, bilaterality of pulmonary metastases and pulmonary metastasectomy had no effect on overall survival.

Conclusion: A small percentage of patients presenting with metastatic STS can be cured with aggressive surgical management. Patients with lymph node metastases, small tumours, low grade tumours and 3 or fewer lung metastases at presentation have a better outcome and longer life expectancy.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 64 - 64
1 Mar 2010
Gortzak Y Mahendra A Griffin AM Wunder JS Ferguson PC
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Objectives: A stable shoulder is essential for proper elbow and hand function after oncologic resection of the shoulder girdle. We describe a surgical technique for replacing the shoulder joint capsule using synthetic mesh after resections of the shoulder girdle that resulted in gross intraoperative instability of the shoulder joint.

Methods: 68 patients who underwent shoulder girdle resection between 1989 and 2006 were identified in our prospective database. This report focuses on nine patients whose shoulder joint was reconstructed with synthetic mesh. All patients were followed on a 3 monthly basis. Shoulder joint instability was determined from clinical records, database and radiographs.

Results: Nine patients underwent shoulder joint reconstruction with synthetic mesh. One patient underwent a shoulder disarticulation within 30 days of the index surgery and was excluded from this rapport.

No dislocations were noted during follow-up (range 3–48 months). Radiographs revealed an average vertical displacement of the humeral head compared to its original position of 0.7 cm (range 0–1.7 cm). There were two surgical complications. In one patient the humeral prosthesis migrated proximally and eroded through the skin requiring additional surgery. In another case erosion of the distal clavicle was noted. This was biopsied and foreign body reaction identified.

Conclusions: Joint instability following major resections of the shoulder girdle is a source of morbidity and affects the function of the salvaged limb. Synthetic capsular reconstruction using Marlex mesh is a useful adjunct in patients where insufficient shoulder musculature and joint capsule remains after resection to allow for stable suspension of the upper limb.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 63 - 63
1 Mar 2010
Gortzak Y Mahendra A Griffin AM Lockwood G Wang Y Deheshi B Wunder JS Ferguson PC
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Objectives: To formulate a scoring system enabling decision making for prophylactic stabilization of the femur following surgical resection of a soft tissue sarcoma (STS) of the thigh.

Methods: A logistic regression model was developed using patient variables collected from a prospective database. The test group included 22 patients with radiation-related pathological femur fracture following surgery and radiation for a thigh STS. The control group of 79 patients had similar treatment but without a fracture. No patients received chemotherapy. Mean follow-up was 8.6 years. Variables examined were: Age (< 49, 50–70, > 70 years), gender, tumor size (0–7, 8–14, > 14 cm), radiation dose (low=5000 cGy, high> 6000 cGy), extent of periosteal stripping (< 10, 10–20, > 20 cm) and thigh compartment (posterior, adductor, anterior). A score was assigned to each variable category based on the coefficients obtained in the logistic regression model.

Results: Based on the regression model and an optimal cut-point, the ability to predict radiation associated fracture risk was 91% sensitive and 86% specific. The area under the Receiver Operating Characteristic (ROC) curve was 0.9, which supports this model as a very accurate predictor.

Conclusions: Radiation-related femur fractures following combined surgery and radiation treatment for STS are uncommon, but are difficult to manage and their non-union rate is extremely high. These results suggest that it is possible to predict radiation-associated pathological fracture risk with high sensitivity and specificity. This would allow identification of high risk patients and treatment with prophylactic IM nail stabilization. Presentation of this model as a clinical nomogram will facilitate its clinical use.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 64 - 64
1 Mar 2010
Mahendra A Griffin AM Yu C Gortzak Y Bell Ferguson PC Wunder JS Davis A
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Objectives: To investigate whether components of MSTS-87 (Pain, ROM, Strength, Stability, Deformity, Acceptance and Function) correlate with function as measured by TESS following endoprosthetic replacement (EPR) for patients with bone sarcoma.

Methods: 255 patients with extremity bone sarcoma treated by resection & EPR were identified from a prospective database. From this group we investigated 111 patients with primary bone sarcoma with > 2 years follow up, evaluated by both MSTS-87 & TESS, no local recurrence, metastasis or major complication for at least 2 years prior to the follow-up. Upper extremity patients were excluded due to small numbers. We examined the influence of patient demographics and tumour characteristics on functional outcome scores. Correlation between MSTS-87 & TESS was performed using linear regression analysis.

Results: Age, gender, tumour size, anatomical site, chemotherapy treatment and presence of pathological fracture did not significantly correlate with TESS. Linear regression analysis of MSTS-87 individual criteria and total score revealed that only pain, ROM and function helped explain the TESS score (p < 0.05) while strength, stability, deformity & acceptance had no significant effect on overall functional outcome.

Conclusions: Of the seven MSTS-87 variables, only pain, ROM and function significantly correlate with overall functional outcome as measured by TESS following EPR for bone sarcoma. This suggests that patients with decreased strength, stability, deformity and acceptance as defined by MSTS-87 scores, may still adapt well with good overall functional outcomes.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 64 - 65
1 Mar 2010
Mahendra A Gortzak Y Griffin AM Wunder JS Ferguson PC
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Objectives: Well fixed, ingrown stems in patients with infected, uncemented knee tumor prostheses are difficult to remove. We have shown that it?s not necessary to remove them for infection control. We describe a technique for intraoperative fabrication of antibiotic-impregnated temporary cement prostheses using rush pin endoskeleton during first stage revision of infected, uncemented knee tumor prostheses.

Methods: We used this method in 7 patients with infected uncemented tumor prostheses around the knee. Two patients are awaiting second stage procedures. Surgery involved removing exchangeable components and retaining well-ingrown stemmed intramedullary components. Spacers were made of Rush pins that were bent and placed through hinge of retained component to allow knee movement. Antibiotic impregnated cement was placed around pins.

Results: This method allowed partial weight-bearing, knee movement, stability and pain control between first and second stages. In 4/5 patients infection was eradicated. Second-stage reimplantations were accomplished successfully in 3/5 patients with no obvious metal wear. Mean interval between the 2 stages was 12 weeks. One patient had residual infection requiring amputation and one patient did not agree to further surgery. Overall functional outcome following revision was TESS 82.3/100, MSTS93 73.3/100, MSTS87 23.3/35.

Conclusions: The technique allows maintenance of length, stability and knee joint movement, thus subsequent revision becomes technically simpler. Cement construct has large surface area to maximize antibiotic elution. This method has advantages of low cost, availability of numerous pin dimensions, which allows a custom component. Although there is no obvious wear, this remains a potential concern at articulation of rush rods and retained component.


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_I | Pages 38 - 38
1 Mar 2010
Gortzak Y Lockwood G Mahendra A Wang Y Griffin A Deheshi B Wunder JS Ferguson PC
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Purpose: To formulate a scoring system enabling decision making for prophylactic stabilization of the femur following surgical resection of a soft tissue sarcoma (STS) of the thigh.

Method: A logistic regression model was developed using patient variables collected from a prospective database. The test group included 22 patients with radiation-related pathological femur fracture following surgery and radiation for a thigh STS. The control group of 79 patients had similar treatment but without a fracture. No patients received chemotherapy. Mean follow-up was 8.6 years. Variables examined were: Age (70 years), gender, tumor size (0–7, 8–14, > 14 cm), radiation dose (low=5000 cGy, high> 6000 cGy), extent of periosteal stripping (20 cm) and thigh compartment (posterior, adductor, anterior). A score was assigned to each variable category based on the coefficients obtained in the logistic regression model.

Results: Based on the regression model and an optimal cut-point, the ability to predict radiation associated fracture risk was 91% sensitive and 86% specific. The area under the Receiver Operating Characteristic (ROC) curve was 0.9, which supports this model as a very accurate predictor.

Conclusion: Radiation-related femur fractures following combined surgery and radiation treatment for STS are uncommon, but are difficult to manage and their non-union rate is extremely high. These results suggest that it is possible to predict radiation-associated pathological fracture risk with high sensitivity and specificity. This would allow identification of high risk patients and treatment with prophylactic IM nail stabilization. Presentation of this model as a clinical nomogram will facilitate its clinical use.


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_II | Pages 253 - 253
1 May 2009
Saidi K London O Bell RS Griffin AM Saidi K Wunder JS
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Radiation induced pathologic fractures present a difficult problem for musculoskeletal oncologists. The purpose of this study was to determine the outcomes of management of radiation-induced pathologic fractures in a group of patients who had previously undergone combined management of extremity soft tissue sarcoma.

A review of our retrospective database was undertaken. From 1986 to present, thirty-two patients with soft tissue sarcomas were found to have radiation induced pathologic fractures. The records of these patients were reviewed for patient demographics, tumour size and anatomic site, presence of periosteal stripping at time of surgery, radiation dose, time to fracture, fracture treatment and fracture outcome.

There were twenty-three females and nine males with a mean age of sixty-three (range thirty-six to eighty-nine) years. Fractures occurred at a mean of forty-five months after resection of the sarcoma (range three to one hundred and fifty months). Anatomic distribution of fractures were : proximal femur(twelve), femoral diaphysis (eight), distal femur (two) tibia (five), acetabulum (two), metatarsal (two) and patella (one). Periosteal stripping was performed in half of the patients. Twenty-three patients had received high dose radiation (6600Gy). Seven fractures were managed conservatively while twenty-five were treated surgically. Only eleven of the thirty-two fractures united. Six patients underwent amputation, three for local recurrence and three for non-union of their fracture. Eight patients ultimately underwent arthroplasty, while seven patients have persistent non-unions. In the proximal femur, only three out of twelve fractures healed while six patients eventually underwent arthroplasty and three continue to have non-unions. Of eight femoral diaphyseal fractures, only one united. Patients who eventually underwent prosthetic replacement had good function and pain relief.

Radiation induced pathologic fractures are a difficult clinical problem. In particular patients with fractures in the proximal femur often undergo multiple attempts at fixation before definitive management with resection and endoprosthetic replacement. Fractures of the femoral diaphysis rarely heal despite aggressive surgical management. Primary arthroplasty may be considered in some patients as an alternative to fixation in radiation-induced pathologic fractures of the femur in order to avoid long term morbidity and repeated operations.


The Journal of Bone & Joint Surgery British Volume
Vol. 88-B, Issue 11 | Pages 1480 - 1486
1 Nov 2006
Pradhan A Cheung YC Grimer RJ Abudu A Peake D Ferguson PC Griffin AM Wunder JS O’Sullivan B Hugate R Sim FH

We have investigated the significance of the method of treatment on the oncological and functional outcomes and on the complications in 184 patients with soft-tissue sarcomas of the adductor compartment managed at three international centres. The overall survival at five years was 65% and was related to the grade at diagnosis and the size of the tumour. There was no difference in overall survival between the three centres. There was, however, a significant difference in local control with a rate of 28% in Centre 1 compared with 10% in Centre 2 and 5% in Centre 3.

The overall mean functional score using the Toronto Extremity Salvage Score in 70 patients was 77% but was significantly worse in patients with wound complications or high-grade tumours. The scores were not affected by the timing of radiotherapy or the use of muscle flaps.

This large series of soft-tissue sarcomas of the adductor compartment has shown that factors influencing survival do not vary across the international boundaries studied, but that methods of treatment affect complications, local recurrence and function.


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_II | Pages 111 - 111
1 Feb 2003
Abudu A Driver N Wunder JS Griffin AM Pearce D O’Sullivan B Catton CN Bell RS Davis AM
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812 consecutive patients with soft tissue sarcoma of the extremity were studied to compare the characteristics and outcome of patients who had primary amputations and limb preserving surgery.

Patients with primary amputations were more likely to have metastases at presentation, high-grade tumours, larger tumours and were older.

The most frequent indications for primary amputation were tumour excision which would result in inadequate function and large extracompartmental tumours with composite tissue involvement including major vessels, nerves and bone.

The requirement for primary amputation was a poor prognostic factor independent of tumour grade, tumour size and patients’ age.


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_II | Pages 111 - 111
1 Feb 2003
Gerrand CH Wunder JS Kandel RA O’Sullivan B Catton CN Bell RS Griffin AM Davis AM
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To determine if rates of local recurrence and metastasis differ in upper versus lower extremity sarcomas.

Prospectively collected data relating to patients undergoing limb-sparing surgery for extremity soft tissue sarcoma between January 1986 and April 1997 were analysed. Local recurrence-free and metastasis-free rates were calculated using the method of Kaplan and Meier. Univariate and multivariate analyses of potential predictive factors were evaluated with the log-rank test and the Cox proportional hazards model.

Of 480 eligible patients, 48 (10. 0%) had a local recurrence and 131 (27. 3%) developed metastases. Median follow-up of survivors was 4. 8 years (0. 1 to 12. 9). There were 139 upper and 341 lower extremity tumours. Upper extremity tumours were more often treated by unplanned excision before referral (89 vs 160, p< 0. 001) and were smaller (6. 0cm vs 9. 3cm, p< 0. 000). Lower extremity tumours were more often deep to or involving the investing fascia (280 vs. 97, p< 0. 003). The distribution of histological types differed in each extremity. Fewer upper extremity tumours were treated with adjuvant radiotherapy (98 vs. 289, p< 0. 000).

The 5-year local recurrence-free rate was 82% in the upper and 93% in the lower extremity (p< 0. 002). Local recurrence was predicted by surgical margin status (hazard ratio 3. 16, p< 0. 000) but not extremity (p=0. 127) or unplanned excision before referral (p=0. 868).

The 5-year metastasis-free rate was 82% in the upper and 69% in the lower extremity (p< 0. 013). Metastasis was predicted by high histological grade (hazard ratio 17. 28, p< 0. 000), tumour size in cm (hazard ratio 1. 05, p< 0. 001) and deep location (hazard ratio 1. 93, p< 0. 028) but not by extremity (p=0. 211).

Local recurrence is more frequent after treatment for upper compared with lower extremity sarcomas. Variation in the use of radiotherapy and differences in histological type may be contributory. Metastasis is more frequent after treatment for lower extremity sarcomas because tumours tend to be large and deep.


The Journal of Bone & Joint Surgery British Volume
Vol. 84-B, Issue 6 | Pages 870 - 872
1 Aug 2002
Plasschaert F Craig C Bell R Cole WG Wunder JS Alman BA

Localised Langerhans-cell histiocytosis of bone (eosinophilic granuloma) is a benign tumour-like condition with a variable clinical course. Different forms of treatment have been reported to give satisfactory results. However, previous series all contain patients with a wide age range. Our aim was to investigate the effect of skeletal maturity on the rate of recurrence of isolated eosinophilic granuloma of bone excluding those arising in the spine.

We followed up 32 patients with an isolated eosinophilic granuloma for a mean of five years; 17 were skeletally immature. No recurrences were noted in the skeletally immature group even after biopsy alone. By contrast, four of 13 skeletally mature patients had a recurrence and required further surgery. This suggests that eosinophilic granuloma has a low rate of recurrence in skeletally immature patients.


The Journal of Bone & Joint Surgery British Volume
Vol. 83-B, Issue 8 | Pages 1149 - 1155
1 Nov 2001
Gerrand CH Wunder JS Kandel RA O’Sullivan B Catton CN Bell RS Griffin AM Davis AM

We considered whether a positive margin occurring after resection of a soft-tissue sarcoma of a limb would affect the incidence of local recurrence. Patients with low-grade liposarcomas were expected to be a low-risk group as were those who had positive margins planned before surgery to preserve critical structures. Two groups, however, were expected to be at a higher risk, namely, patients who had undergone unplanned excision elsewhere with a positive margin on re-excision and those with unplanned positive margins occurring during primary resection.

Of 566 patients in a prospective database, 87 with positive margins after limb-sparing surgery and adjuvant radiotherapy were grouped according to the clinical scenario by an observer blinded to the outcome. The rate of local recurrence differed significantly between the two low- (4.2% and 3.6%) and the two high-risk groups (31.6% and 37.5%). This classification therefore provides useful information about the incidence of local recurrence after positive-margin resection.


The Journal of Bone & Joint Surgery British Volume
Vol. 82-B, Issue 1 | Pages 120 - 125
1 Jan 2000
Lan F Wunder JS Griffin AM Davis AM Bell RS White LM Ichise M Cole W

We used dual-energy x-ray absorptiometry (DEXA) to evaluate the extent of periprosthetic bone remodelling around a prosthesis for distal femoral reconstruction, the Kotz modular femoral tibial replacement (KMFTR; Howmedica, Rutherford, New Jersey). A total of 23 patients was entered into the study which had four parts: 1) 17 patients were scanned three times on both the implant and contralateral legs to determine whether the precision of DEXA measurements was adequate to estimate bone loss surrounding the anchorage piece of the KMFTR; 2) in 23 patients the bone mineral density (BMD) in different regions of interest surrounding the diaphyseal anchorage was compared with that of the contralateral femur at the same location to test whether there was consistent evidence of loss of BMD adjacent to the prosthetic stem; 3) in 12 patients sequential studies were performed about one year apart to compare bone loss; and 4) bone loss was compared in ten patients with implants fixed by three screws and in 13 without screws.

The mean coefficients of variation (SD/mean) for the 17 sets of repeated scans ranged from 2.9% to 7.8% at different regions of interest in the KMFTR leg and from 1.4% to 2.5% in the contralateral leg. BMD was decreased in the KMFTR leg relative to the contralateral limb and the percentage of BMD loss in general increased as the region of interest moved distally in the femur. Studies done after one year showed no consistent pattern of progressive bone loss between the two measurements. The ten patients with implants fixed by screws were found to have a mean loss of BMD of 42% in the most distal part of the femur, while the 13 without screw fixation had a mean loss of 11%.

DEXA was shown to have adequate precision to evaluate loss of BMD around the KMFTR. This was evident relative to the contralateral leg in all patients and generally increased in the most distal part of the femur. In general, it stabilised between two measurements taken one year apart and was greater surrounding implants fixed by cross-locking screws.


The Journal of Bone & Joint Surgery British Volume
Vol. 80-B, Issue 1 | Pages 38 - 42
1 Jan 1998
Gibbons CLMH Bell RS Wunder JS Griffin AM O’Sullivan B Catton CN Davis AM

We describe the functional results in 14 patients (7 men, 7 women) after subtotal scapulectomy for primary bone and soft-tissue tumours at a specialist musculoskeletal oncology unit. Eight had chondrosarcomas, two Ewing’s sarcomas, one aggressive fibromatosis and three soft-tissue sarcomas. The mean follow-up was 52 months (6 to 120). Analysis of residual symptoms and of range and strength of movement by physicians used the Musculoskeletal Tumour Society rating scale (MSTS). Physical disability was measured by the patients using the Toronto Extremity Salvage Score (TESS).

All 14 patients are still alive, two with systemic disease. Nine had more than 80% of their scapula resected but the glenohumeral joint was preserved in all cases. Eight had full movement and another two achieved 90° of flexion. The mean functional results were good to excellent in all except three patients (mean MSTS = 71.6 and TESS = 79.9). Two of these three patients had considerable pain as a result of brachial neuropathy.

Scapulectomy gives an excellent functional result if the glenohumeral joint is preserved. The rotator cuff could be removed without a severe functional deficit provided that the deltoid was reattached to the scapular remnant and the trapezius.