Malignant Transformation of Synovial Chondromatosis: A Systematic Review

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.

Synovial chondrosarcoma: a single‐institution experience with molecular investigations and review of the literature

“…In fact, imaging findings suggestive of synovial chondrosarcoma (including extensive involvement of joints by an articular mass, cortical erosion, and involvement of both soft tissue and underlying bone) may also be observed in synovial chondromatosis. [5][6][7][8][9][10][11][12][13][14] Clinically, malignant progression of synovial chondromatosis may be suspected in the presence of sudden worsening of pain or an early local recurrence. 1,5 The histological criteria for differentiating between synovial chondromatosis and synovial chondrosarcoma proposed by Bertoni et al 1 (Table 3) have been widely adopted, 8,12,20,21,35 and have proved beneficial in enabling a correct diagnosis.…”

“…[1][2][3][4] Previous reports have described three distinct clinical settings: (i) synovial chondrosarcoma presenting in patients with a history of pre-existing synovial chondromatosis but without areas of chondromatosis at the onset of chondrosarcoma; (ii) synovial chondrosarcoma presenting in patients without a pre-existing history of synovial chondromatosis but with histological evidence of chondromatosis coexisting with synovial chondrosarcoma; and (iii) synovial chondrosarcoma presenting in patients without a prior history or histological evidence of synovial chondromatosis. [1][2][3][4][5][6][7][8][9] The clinical and radiological features of synovial chondrosarcoma overlap with those of aggressive synovial chondromatosis, thereby hindering the reliable separation of these two entities, [5][6][7][8][9] although malignant evolution should be suspected if there is either a rapid recurrence after complete resection of synovial chondromatosis or pain worsens rapidly. [5][6][7][9][10][11][12][13][14] In 1991, Bertoni et al 1 proposed a set of histological criteria whose presence would favour a diagnosis of synovial chondrosarcoma over one of synovial chondromatosis.…”

“…[1][2][3][4][5][6][7][8][9] The clinical and radiological features of synovial chondrosarcoma overlap with those of aggressive synovial chondromatosis, thereby hindering the reliable separation of these two entities, [5][6][7][8][9] although malignant evolution should be suspected if there is either a rapid recurrence after complete resection of synovial chondromatosis or pain worsens rapidly. [5][6][7][9][10][11][12][13][14] In 1991, Bertoni et al 1 proposed a set of histological criteria whose presence would favour a diagnosis of synovial chondrosarcoma over one of synovial chondromatosis. Such criteria include loss of clustering of chondrocytes, the presence of myxoid change of the stroma, peripheral hypercellularity, peripheral spindling of chondrocytes, necrosis, and permeation of trabecular bone in a 'filling-up' pattern.…”

Osteocondromatosis sinovial de rodilla: una causa infrecuente de gonalgia en edad pediátrica

“…O t r a p o t e n c i a l c o m p l i c a c i ó n d e l a osteocondromatosis sinovial es la malignización a condrosarcoma. Pese a que existen solamente 48 casos publicados desde 1957, 13 hay artículos que hablan de una tasa de hasta el 5 %, 14 dada la difícil distinción entre la recidiva y el condrosarcoma de bajo grado. La malignización debe sospecharse ante la exacerbación súbita de los síntomas, la infiltración intramedular en las pruebas de imagen o histológicas, la recurrencia en menos de 12 meses o la infiltración muscular adyacente.…”

Potential utility of anti‐TNF drugs in synovial chondromatosis associated with ankylosing spondylitis

“…Although mostly benign, some patients with SC undergo a malignant transformation especially in patients with a recurrent disease …”