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THE VARIABILITY IN THE PRESENTATION OF SYNOVIAL SARCOMA IN CHILDREN – A PLEA FOR GREATER AWARENESS.



Abstract

Introduction: The aim of this study was to identify the pattern of symptoms in patients presenting with synovial sarcoma, examine how these corresponded to the symptoms outlined by the NICE guidelines on the rapid referral of patients with a suspected sarcoma and spot factors that led to long delays in diagnosis.

Methods: Early symptoms and the results of clinical and radiological investigation were reviewed along with the presumed diagnoses that had been made for 35 children. The total duration of symptoms was separated into patient delay and doctor delay.

Results: Using the four clinical findings suggestive of sarcoma according to the NICE guidance, only half of the patients had one or more of them at the time of initial symptoms. The most common presentation was a painless mass (n=16), and in 10 children there was no mass identified. Seven patients had an inexplicable joint contracture, many having been extensively investigated unsuccessfully. The mean duration of symptoms before the diagnosis was made was 98 weeks (range 2 to 364). The mean number of doctors seen prior to referral was 3 (range 1–6) and for 15 patients the diagnosis was obtained after inadvertent excision. The factors associated with long duration of symptoms before diagnosis were knee and elbow location (p=0.0047) or periarticular location (p=0.01), absence of lump (p=0.016) or painful mass as early symptom (p=0.04), the presence of calcifications on x-rays (p=0.01) and a fixed joint contracture (p=0.0003).

We could not show that delay in diagnosis led to a worse prognosis.

Discussion: This paper highlights the sometimes bizarre symptoms associated with synovial sarcoma and hopefully this will increase awareness of the condition among relevant sections of the medical profession and help to reduce the delay in diagnosing these cases.

Correspondence should be addressed to BOOS c/o British Orthopaedic Association, 35-43 Lincoln’s Inn Fields, London WC2A 3PE, England