Purpose of the study: Extra-abdominal desmoids tumours are benign tumours generally managed by more or less wide surgical resection. This surgery can be mutilating and carries a risk of recurrence to the order of 50% according to several authors.

Material and methods: We followed a series of 26 patients from 1989 to 2009 with non-operated extra-abdominal desmoids tumours. Our objective was to study the clinical, radiographic and pathological course of these tumours and identify prognostic factors. For 11 patients, no medical or surgical treatment was delivered beyond the diagnostic biopsy; for 15, the tumour recurred after surgery and no other adjuvant treatment was given.

Results: Twenty-four tumours stabilised at 13.2 months on average for primary tumours and 20.9 for recurrent tumours. Thus en general, the tumour progression was less than 3 years. In all cases, once the tumour stopped progressing, it did not progress again. One primary tumour regressed spontaneously and one recurrence continued to progress at last follow-up of 23 months. Surgery was not required for any of the patients, excepting biopsy procedures to confirm diagnosis. MRI was the exploration of choice for follow-up.

Discussion: This series, which is the largest devoted to followed extra-abdominal desmoid fibromas, confirms recent data in the literature. A wait-and-see attitude should always be discussed with this type of tumour.

Purpose of the study: Diagnosis and treatment of primary malignant tumors of the pelvis raise difficult problems. The purpose of this retrospective study was to analyze the functional and cancerological results observed after surgical treatment in a single center.

Material and methods: Between 1973 and 2002, 24 patients (16 men and 8 women) underwent surgery in our unit for histological proven malignant tumors. A posterior approach was used for curettages and sacrectomies of the apex. A combined anterior and posterior approach was used for total sacrectomy and hemisacrectomy. Oncological results were assessed in terms of local recurrence, presence of metastasis and patient status at last follow-up. Overall survival and disease-free survival were calculated with the Kaplan-Meier method.

Results: Mean age was 53.38 years. Mean follow-up in our series was 54 months. Mean time to diagnosis was 16 months. Pain was the predominant symptom. Sixteen patients presented neurological manifestations and the digital rectal examination was positive in all. Chondroma was the most frequent histological type (18/24). None of the patients had metastatic disease at diagnosis. A posterior approach was used for 15 patients and a combined approach for the others. There was a clear correlation between type of resection and volume of blood loss (p=0.0002). Wide dissection was wide in five patients, marginal in five and oncologically insufficient in 14. Mean operative time was 1.34 hours for posterior approaches and 9 hours for combined approaches. The postoperative period was uneventful for ten patients. Infection was the most frequent complication. Adjuvant radiotherapy, delivered in 16 patients, effectively retarded the occurrence of local recurrence. Functional disorders were correlated with the level of the neurological sacrifice. At least one S3 root had to be preserved to limit the urological and digestive incapacity. At last follow-up, local recurrence was present in 12 patients. Mean time to first recurrence was 32 months. There was a strong correlation between quality of the resection and time to local recurrence. There was a significant difference between patients with a wide resection and those with an oncologically insufficient resection (p=0.0312). Five patients had metastases. Five-year actuarial survival was 73±12%. At ten years it was 32±14%. Local recurrence-free survival was 55±11% at five years and zero at 10 years.

Discussion and conclusion: In light of these results, factors of poor prognosis were: late diagnosis, soft tissue invasion, proximal extension, marginal or insufficient resection.

Purpose: We report three cases of extensive resection of the ulna without reconstruction.

Material and methods: For the first two patients, resection was performed for ulnar tumour, on low-grade osteosarcoma and one adamantinoma. In the third patient with a voluminous giant-cell tumour, the distal part of the ulna was resected and used as a graft for arthrodesis after resection of the distal third of the homolateral radius. Resection involved the distal half, three-quarters, and one-third of the ulna in these three patients.

Results: At follow-up of 4, 23 and 1 year respectively, wrist motion is normal in the two patients and elbow motion is normal in two out of three. None of the patients experienced wrist pain or ulnar stump pain. Grip force decreased in the two patients who underwent isolated ulnar resection.

Discussion: Our observations corroborate reports in the literature leading to the conclusion that reconstruction of the ulna is not justified when one-quarter of the bone can be preserved. The ulna offers an exceptional graft material for reconstruction of the homolateral radius.

Purpose: Hinged knee prostheses are mainly used for reconstruction after major tumour resection. Aseptic loosening is the main problem with these implants. One of the solutions proposed to reduce the rate of loosening is to add a hydroxyapatite collar on the shaft stems. This work was conducted to study bone ingrowth with a new hinged implant with a hydroxyapatite collar at the junction between the zone of resection and the shaft.

Material and methods: Twenty-nine massive prostheses with a hydroxyapatite collar were implanted between 1998 and 2001. Nine patients were excluded from the analysis because follow-up was less than two years. This retrospective analysis thus compared twenty massive prostheses with twenty matched hinged GUEPAR prostheses without a collar. Bony ingrowth was measured on plain x-rays (two orthogonal views) at 6, 12, 24, and 36 months. Filling of the gap between the bone and the implant was also assessed. Signs of loosening were noted.

Results: Mean bony ingrowth in implants with a hydroxyapatite collar was 6.58 mm at 6 months 9.84 mm at 12 months, 12.3 mm at 24 months and 13.25 mm at 36 months. Mean bony ingrowth in the implants without a hydroxyapatite collar was 1.65 mm at 6 months, 3.31 mm at 12 months, 4.8 mm at 24 months and 4.35 mm at 36 months. In the implants with a collar, gap filling was partial in five cases and total in 15. In implants without a collar, there was no gap filling in eight cases, partial filling in two cases and total filling in fifteen cases.

Discussion: Prostheses with a hydroxyapatite collar enable better radiological bony ingrowth than observed in implants without a hydroxyapatite collar. Gap filling is better for prostheses with a collar. There was no case of loosening at last follow-up for implants with a hydroxyapatite collar.

Conclusion: In light of these results, shaft anchorage appears to be better with implants with a hydrosyapatite collar. Confirmation of improvement in clinical outcome and lower rate of aseptic loosening will require longer follow-up.

Purpose: We report a retrospecitve series of 88 benign osteolytic tumours of the knee treated by curettage-filling between 1973 and 2000. The purpose of this analysis was to evaluate the role of curettage-filling in the treatment of this type of tumour.

Material and methods: Mean patient age was 31 years. The sex ratio was 1. Pain was the main sign and 9% of patients had a pathologic fracture. An equivalent number of tumours were found in the lower extremity of the femur and the upper extremity of the tibia. We analysed clinical features, imaging findings, treatments and complications, recurrence, and treatment of recurrence.

Results: Giant-cell tumours predominated (n=63), followed by aneurysmal cysts (n=7) and chondroblastomas (n=6). Tumours were treated by curettage associated with filling (n=83) and osteosynthesis (n=51). There were six cases of mechanical complications, but only two required total knee arthroplasty. No re-operations for arthrolysis were required. The recurrence rate after curettage was 23%; a second curettage-filling was performed after 90% of the recurrences.

Discussion: This study confirms that curettage-filling is the standard surgical treatment for benign osteolytic tumours of the knee, independently of histological type. This simple procedure with a low complication rate enables preservation of the joint in young subjects. We prefer this approach to resection-arthroplasty. We were unable to identify any factor predictive of local recurrence (histologic type of osteolytic tumour). Repeated curettage-filling is an appropriate treatment for recurrence.

Purpose: The purpose of this work was to analyse and compare survival in patients with osteosarcoma (OS) or Ewing sarcoma (EW) of the pelvis as a function of treatment.

Material and methods: This retrospective series included 31 patients with OS (n=15) or EW (n=16) of the pelvis who were given a homogeneous therapeutic sequence associating chemotherapy, surgery and/or radiotherapy. Mean follow-up was 37 months (2–144). Mean age was 20 years for EW and 28 years for OS. Localisations in the pelvis were: zone I (n=12), zone I and II (n=4), zone II (n=1), zone II and III (n=7), zone III (n=1), and zone I, II and III (n=6). All patients were given chemotherapy, 15 underwent surgery, and 16 were given radiotherapy alone. Five patients were given complementary radiotherapy after surgery. Actuarial survival curves were compared with the logrank test. Comparison factors were presence of surgical resection, presence of initial or secondary metastasis, tumour response (radiographic measure), and pathology (good or poor responder) after chemotherapy.

Results: Five-year survival rate for patients with EW was 53%, 31% for OS. There was no significant difference in survival rates between tumour type. The only factor significantly correlated with lower survival rate was presence of initial metastasis.

Discussion and conclusion : The pelvic localisation of osteosarcoma and Ewing sarcoma is a factor of poor prognosis. Unlike data reported in the literature, surgery did not appear to influence outcome, not being found to be a factor of better prognosis. Surgery does however appear to improve short-term survival. In the pelvic localisation, osteosarcoma appears to have a poorer prognosis in terms of survival than Ewing tumour.

Aims: The authors report oncological and functional results after 15 acetabular reconstructions, followed tumor resection, with an original technique using homo-lateral proximal femoral autograft and total hip prosthesis. Material and method: The modiþed Musculoskeletal Tumor Society Score (MSTS) and the Toronto Extremity Salvage Score (TESS) were used for functional analysis. Functional results were available for only 10 patients who presented with a minimal of 2 years follow-up. Results: Fifteen consecutive patients, 9 men and 6 women with a median age of 50 years, were managed in our department for acetabular bone malignant tumor. The tumors involved the zones II in 5 patients, the zones II and III in 5 patients. The tumors included 10 chon-drosarcomas, 1 malignant þbrous histiocytoma, 1 radio-induced sarcoma, 1 myeloma and 2 metastasis. The mean follow-up was 31 months (Range, 12 to 50 months). Local recurrence occurred in 1 case and metastases in 3 cases. Three patients died of tumoral disease and one of intercurrent disease. Eight complications were observed: aseptic failure (n=1), obturator nerve damage (n=1), deep infection (n=4), skin necrosis (n=2), pros-thesis dislocations (n=1) and deep venous thrombosis (n=1). Five revision were performed. The mean MTS was 72% (range, 40 to 96%) and the mean TESS was 82.5% (range, 56 to 86%). Fusion occurred in all reconstructions and all patients recovered an active hip abduction and ßexion. Discussion: This original technique, using an autograft and a standard total hip prosthesis, is available for zone II and III acetabular reconstructions.

Aims: The authors reported an original technique for proximal humerus reconstruction followed tumor resection using a delta composite prosthesis. Seven patients undergoing this technique Technique: Proximal humeral resection was conducted usually. The host tendons of rotator cuff were resected; the deltoid muscle must be preserved. The glenosphere was inserted with three screws. A long humeral stem was used to provide a distal anchorage of 10 cm; this stem was cemented into the allograft and into the humerus host. The patient was immobilized in 90¡ of abduction during 6 weeks. Results: 7 patients aged 38 to 56 years, who presented a chondro-sarcoma or an osteosarcoma of the proximal humerus were enrolled in this study. The mean follow-up was 20 months (6 to 24). None patient presented with pain and the mean of active abduction was 120¡. No local or general recurrence was detected. Discussion: The Delta prosthesis of Gramon is usually used for shoulder arthritis with rotator cuff rupture. In our experience, reconstruction of the proximal humerus with composite prosthesis provides good functional results but after 3 years, a graft resorption was observed and the functional results decrease. The delta prosthesis could provide good functional results without reconstruction of the rotator cuff. In our technique, we sutured the rotator cuff to avoid dislocation. However, the glenoid þxation is incertain for a long term because its a constrained prosthesis. This technique could be used when the deltoid muscle can be preserved.

Purpose: The purpose of this retrospective study was to assess the carcinological and functional results of resection-arthrodesis procedures for giant-cell tumours of the lower extreminty of the radius.

Material and methods: This series included 16 patients, nine men and seven women, mean ate 39 years (19–63). The initial symptom was pain in all patients. Nine of the 16 patients were referred for recurrence after primary curettage-filling: the seven others presented voluminous tumours encompasing nearly all of the epiphyseome-taphyseal region rendering curettage-filling impossible. En bloc resection of the tumour with reconstruction using two tibial splints applied proximally on the radius and distally on the first ray (eight cases) or the second ray (eight cases) of the carpus was performed in all cases. A plaster cast or external fixation protected the construct. All tumours were benign. Nine patients were reviewed for function (pain, motion, force) and radiographic assessment (lateral view of wrist in maximal flexion and extension). We collected data recorded at the last visit for the other patients.

Results: Mean follow-up was 70 months (12–205). The functional outcome was good with 15 patients totally pain free. For the eight patients whose mediocarpus could be preserved, dorsal flexion was 30° and palmar flexion was 15°. Pronosupination varied from 10° to 170°. Bone fusion was obtained in 15 patients. One developed nonunion which was revised with a bone graft and plate fixation and finally healed. Two graft fractures secondary to trauma consolidated normally after plate-screw fixation associated with a new graft. Three of the patients developed local recurrence in the form of subcutaneous nodules which were resected. One of these three patients had a bony recurrence at the graft-radius junction which was treated by a new bone resection and achieved cure.

Discussion: Resection-arthrodeis is indicated for recurrence after curettage-filling and for voluminous giant-cell tumours with extraosseous extension and failure of curetae-filling. Curettage is rarely possible in this location due to invasion of soft tissues and destruction of the joint surface which occurs early. It appears preferable to perform an arthrodesis between the radius and the first ray of the carpal bones to preserve partial motion of the wrist and good function.

Purpose: Fibrous dysplasia is a rare condition accounting for about 1% of benign bone tumours. Both mono- and polyostotic dysplasia is described. Treatment of small tumours is not particularly difficult, but extensive or complicated tumours are another problem. The purpose of this work was to present the results of surgical treatment of a consecutive series of 64 patients. Particular focus was placed on complications.

Material and methods: This retrospective series included 64 patients with fibrous dysplasia. Mean age was 32 years. There were 37 women and 27 men. Monostotic dysplasia was noted in 58 cases. The femur was the most frequent localisation (n=19). Seven patients had a fracture. Histological proof was obtained in 61 patients. In three patients, fibrous dysplasia was associated with another tumour (aneurysmal cyst or adamantinoma). Several therapeutic strategies were proposed: abstention for three patients, biopsy alone for 13, resection for six, curettage for 34. The curettage was filled in 23 cases with osteosynthesis in 11. Eight patients were given other surgical treatments (prosthesis, osteosynthesis, amputation).

Results: We had five postoperative complications. At mean follow-up of 45 months, 14% of the patients still suffered from generally mild to moderate pain. Radiologically, there were four recurrences, 12 unchanged, 18 regressions, and 19 remissions (generally after resection or curettage with filling). Long-term follow-up revealed malignant transformation in two patients.

Discussion: Lesions treated by biopsy alone cured or regressed in 30% of the cases while curettage with filling led to cure or regression in 70% of the cases. We did not include patients treated by bisphosphonates in this series because of insufficient follow-up. Nevertheless, this treatment appears to have a promising effect on pain.

Conclusion: Results of treatment for fibrous dysplasia are not as good as might be suspected for this benign disease. The role of surgery appears to be on the decline, to be replaced by bisphosphonates whose long-term efficacy must be evaluated.

Purpose: The prognosis is dramatic in patients with malignant Schwannoma. These tumours appear to progress invariably irrespective of the complementary treatment given. In order to determine whether this clinical impression is founded, we reviewed retrospectively patients cared for conjointly by the Cochin Hospital and the Curie Institute since 1956.

Material and methods: This series included 32 patients (17 men and 15 women) including seven who had a Recklinghausen neurofibromatosis (three men and four women). The logrank test was used to compare the actuarial survival curves and determine prognostic factors and potential benefit of chemotherapy and radiotherapy protocols.

Results: Mean age at diagnosis was 42.1 years (11.8–78.9). Ten percent of the schwannomas were grade I, 25% grade II and 65% grade III. All patients except two underwent surgery. The resection was considered wide in three cases, marginal in 17, contaminated in seven and non-classifiable in three. There were 14 cases of local recurrence within a mean delay of 12 months (1–66 months). Twelve patients developed secondary local relapse within a mean delay of 36 months (0–169 months). Different neoadjuvant or adjuvant treatments were administered, including chemotherapy and radiotherapy. Twenty-four patients died with a median survival of 25 months. The survival curves showed that complementary treatments did not significantly improve survival. The only factor with prognostic value was the histological grade and the quality of the resection.

Discussion: Our findings are in general agreement with data in the literature demonstrating the lack of efficacy of complementary treatments in patients with malignant schwannomas. Inversely Recklinghausen disease did not appear to modify prognosis in our patients.

Purpose: This retrospective analysis was based on observations in 15 patients, seven men and eight women, mean age 48 years (19–72) treated between 1988 and 2000 at the Curie Institute. The tumour was located in limbs in eight patients (one humerus, two femurs, four tibias, one fibula), in the axial skeleton in five (four pelvi, one sacrum), and in the rib cage and the scapula in one each. Histology examination of the dedifferentiated component displayed fibrosacroma in six cases, HFM in two, rhabdomyosarcoma in two and leiomyosarcoma and osteosarcoma in one each. Six patients were given neoadjuvant and adjuvant chemotherapy of the osteosarcoma type and underwent conservative surgery of the affected limb in three out of four cases. Total histological necrosis was observed in three out of six cases. Six patients were given adjuvant treatment alone using an osteosarcoma protocol. Three unoperable patients were given palliative chemotherapy and radiotherapy.

Results: Nine patients died from their disease. The most frequent metastatic site was the lung; mean survival was 20 months. Six patients survived including five with no progression (1+, 5+, 6+, 7+, 12+ years). Three out of five had had preoperative chemotherapy with a complete histological response and two out of five had had osteosarcoma protocol adjuvant chemotherapy.

Conclusion: Dedifferentiated chondrosarcoma is generally considered to have very poor prognosis and should lead to an adapted therapeutic strategy. In our series, the osteosarcoma protocol provided complete histological response in three out of six patients. Five patients had prolonged survival, all had been given an osteosarcoma type chemotherapy protocol.

Purpose: We report oncological and functional outcome after ten pelvis reconstructions using the Puget technique.

Material and methods: Ten patients (six men and four women), mean age 50 years (37–71) with malignant bone tumours, generally a chondrosarcoma, were included in this series. The tumour involved zone II in five patients, zones II and III in the other five. Resection was followed by reconstruction using the superior portion of the homolateral femur and a cemented total hip arthroplasty. Mean resection was 13 cm (7–23). Mean operative time was 386 min and mean blood loss was 5490 ml. The resection was wide in seven patients, marginal in two and resection margins were contaminated in one. The patients were reviewed at three, six and twelve months then each year. The function score (Enneking) was recorded for all patients. Living patients also filled out a TESS quality of life questionnaire.

Results: At mean follow-up of 22 months (7–42), four patients were living and disease free and two patients were living with recurrent disease. Three patients had died from their disease and one from pulmonary embolism. Postoperative complications were: one dislocation, one phlebitis, one reflex dystrophy, one injury to the internal genital nerve, two infections and one necrosis of the scar tissue. Bone healing was obtained at a mean five months. The mean Enneking function score was 68% and the mean TESS score was 75%.

Discussion: The high proportion of deaths is related to the severe prognosis of these pelvic tumours. This reconstruction technique described by Puget, provides an interesting alternative to other reconstruction methods for the acetabular region. The autogenous graft combined with a standard total hip arthroplasty makes this a rather easy to perform and low-cost procedure.

Conclusion: Functional outcome has been, in our experience, better than with arthrodesis, acetabular prosthesis, or saddle prosthesis and massive allograft of the pelvis.

Purpose: We report cancerological and functional outcome in 41 patients who underwent interilioabdominal disarticulation for malignant tumours.

Material and methods: This retrospective series included 27 men and 14 women, mean age 49 years, most of whom underwent surgery for chondrosarcoma. In ten patients, the disarticulation followed a resection-reconstruction procedure. In five patients, it followed curettage or contaminated margin resection. For seven patients it was performed after radiotherapy alone. None of the patients had metastatic dissemination prior to surgery. The resection margins were in healthy tissue in 24 cases and contaminated in 17. Mean follow-up was 62 months.

Results: Twenty-eight patients died from their disease and one died from pulmonary embolism. At last follow-up, among the 13 living patients, five had local or general relapse. For the 17 patients who had contaminated resection margins, ten developed a recurrent tumour compared with five recurrent tumours among the 25 patients with resection margins in healthy tissue. Mean five-and ten-year survival rates were 30% and 25% respectively. Initial treatment, tumour size and tumour histology did not have any significant effect on prognosis. The only factor with a significant effect on survival was the quality of the resection margins. All patients were able to walk with two crutches.

Discussion: Interilioabdominal disarticulation is a very mutilating procedure. Since the development of conservative surgery of the pelvis, indications for interilioabdominal disarticulation are generally limited to very voluminous endopelvic tumours with vessel and nerve invasion. For local recurrence after surgical resection of the pelvis or proximal femur, especially in patients with infection or radiated tissue, interilioabdominal disarticulation may be the only solution providing satisfactory cancerological resection. Careful exploration of the locoregional and general extension is necessary before proposing this mutilating procedure, with its inherent psychological and functional impact, in order to properly select patients free of metastasis who could benefit from the cancerological resection provided by inter-ilioabdominal disarticulation.

Purpose: Primary bone tumours located in the upper limb are mainly found in the proximal portion of the humerus. Tumour resection raises difficult situations due to the sacrifice of the cuff tendons. We propose a decision making scheme for determining the best reconstruction strategy.

Material and methods: This retrospective analysis was based on 35 cases (19 massive prostheses, ten composite prostheses, three inverted prostheses and three composite arthrodeses). All the clinical and radiographic data were reviewed to examine function, active motion, pain, and use of the upper limb in everyday activities.

Results: Massive humeral prostheses provided a technically simple solution but produced mediocre functional results: painless shoulder without active motion, but preservation of elbow and forearm function. Composite humeral prostheses (prosthesis + allograft) did not, in our experience provide any gain in function compared with massive prostheses. Allografts were resorbed after four to five years. Composite arthrodesis with allograft and vascularised fibular graft provided a mobile shoulder with a useful amplitude and a pain free joint. Results persisted. The inverted Delta prostheses (Grammont) covered with allografts and with preservation of the rotator cuff tendons offered an interesting alternative with functional results superior to arthrodesis and similar to those with shoulder prostheses implanted for degenerative disease. The persistence of the glenoid anchorage remains uncertain.

Conclusions: We use the following scheme for reconstruction of the proximal humerus. Resection of the proximal humerus with preservation of the deltoid in patients in good general health: inverted composite prosthesis. Resection of the proximal humerus without preservation of the deltoid in patients in poor general health: composite arthrodesis. Resection of the proximal humerus in patients in very poor general health when complex surgery with long post-operative care is not possible: massive prosthesis.

Purpose of the study: Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinical, radiographical and histological findings have been well established since the first description by Ribbert in 1894. Sacral chordomas are however difficult to manage and remain a challenge for surgeons and radiotherapists alike. The purpose of this study was to evaluate the long-term results of surgical treatment and patterns of failure in patients treated for chordoma of the sacrum in our department.

Materials and methods: This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in all cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection in two.

Results: Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences were observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of recurrences was partial surgical removal with radiotherapy (40 to 70 Grays). Three patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall survival was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Average disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years.

Discussion: Chordoma is a slow-growing tumor allowing survival for several years despite recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death after intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete resection. Because radiation therapy seems to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection.