Malignant Transformation of Synovial Chondromatosis: A Systematic Review

Vincent Y Ng, Philip Louie, Stephanie Punt, Ernest U Conrad, Vincent Y Ng, Philip Louie, Stephanie Punt, Ernest U Conrad

Abstract

Background: Synovial chondromatosis (SCh) can undergo malignant transformation. Pathologic diagnosis of secondary synovial chondrosarcoma (SChS) is challenging and misdiagnosis may result in over- or undertreatment.

Method: A systematic review revealed 48 cases of SChS published in 27 reports since 1957. Data was collected to identify findings indicative of SChS and outcomes of treatment.

Results: At median follow-up of 18 months, patients were reported as alive (10%), alive without disease (22%), alive with disease (15%), dead of disease (19%), dead of pulmonary embolism (4%), and unknown (29%). Initial diagnosis of SChS (grade: low/unknown 48%, intermediate/high 52%) was after biopsy in 58%, local resection in 29%, and amputation in 13%. Seventy-four percent of patients underwent 1.8 (mean) resections. Patients treated prior to 1992 were managed with amputation in 79% of cases compared to 48% after 1992. Symptoms were present for 72 mos prior to diagnosis of SChS. Synovial chondrosarcoma demonstrated symptom progression over several months (82%), rapid recurrence after complete resection (30%), and medullary canal invasion (43%). The SChS tumor dimensions were seldom quantified.

Conclusion: Malignant degeneration of synovial chondromatosis is rare but can necessitate morbid surgery or result in death. Pathognomonic signs for SChS including intramedullary infiltration are present in the minority of cases. Progression of symptoms, quick local recurrence, and muscle infiltration are more suggestive of SChS. Periarticular cortical erosion, extra-capsular extension, and metaplastic chondroid features are non-specific. Although poorly documented for SChS, tumor size is a strong indicator of malignancy. Biopsy and partial resection are prone to diagnostic error. Surgical decisions are frequently based on size and clinical appearance and may be in conflict with pathologic diagnosis.

Keywords: Chondrosarcoma; Malignant transformation; Osteochondromatosis; Size; Synovial chondromatosis.

Figures

Fig. (1)
Fig. (1)
Search Flow Diagram.
Fig. (2)
Fig. (2)
Synovial chondrosarcoma in the setting of synovial chondromatosis 47 year-old male with two prior ankle injuries and pain over the past 3 years now presenting with recent progression of pain and swelling. Initial biopsy was consistent with grade II/III chondrosarcoma, confirming the radiographic appearance (MRI, A; Plain XR, B) and clinical presentation. He underwent below-knee amputation.
Fig. (3)
Fig. (3)
Synovial chondrosarcoma in the setting of synovial chondromatosis 43 year-old female presenting with a massive localized chondroid lesion suspected to have malignant degeneration of synovial chondromatosis based on imaging (MRI, A) and symptomatology. Histologic evidence of chondrosarcoma, however, was only detected after resection of essentially the entire lesion.

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