Synovial chondrosarcoma arising in synovial chondromatosis.

Scott Evans,Samena Chaudhry,Michele Boffano,Lee Jeys,Robert Grimer

doi:10.1155/2014/647939

Abstract

Primary synovial chondromatosis (SC) is a rare proliferative disorder that causes pain, swelling, and restriction of movement to the joints it affects. The disease frequently runs a protracted course, often requiring multiple surgical procedures to obtain some control. Few reports exist detailing the natural history of SC, although malignant transformation to synovial chondrosarcoma (CHS) is recognized to be a rare event. The aim of our study was to review a large orthopaedic oncology database in order to evaluate the incidence of CHS arising from SC. We identified 78 patients who have presented to our centre with primary synovial chondromatosis (SC). Of those patients, 5 went on to develop malignant change. This represents a 6.4% incidence of developing synovial chondrosarcoma (CHS) within preexisting primary synovial chondromatosis. The patients had a mean age of 28 years at first diagnosis with synovial chondromatosis with the median time from original diagnosis to malignant transformation being 20 years (range 2.7–39 yrs).

Highlights

Synovial chondromatosis is a rare proliferative, metaplastic disorder of the synovium [1]
We conducted a retrospective search of a prospective tumour database to identify all patients treated at our unit with the diagnosis of primary synovial chondromatosis (SC)
A search of our database, which holds prospectively gathered data on over 30,000 patients including over 3800 primary bone sarcomas, identified 78 patients with SC

Summary

Introduction

Synovial chondromatosis is a rare proliferative, metaplastic disorder of the synovium [1]. The disease was first described by Leannac in 1813 [2]; its current description was not applied until 1958 by Jaffe [3]. The exact prevalence of primary synovial chondromatosis (SC) is unknown but it usually affects the third to fifth decades of life [4], with men being affected two to four times more frequently than women [5]. The knee is the most commonly affected large joint followed by hip, shoulder, elbow, ankle, and wrist [6]. Patient’s clinical symptoms typically include pain, swelling, and restricted joint movement [7]. The symptoms are often insidious at disease onset and are gradually progressive, rare spontaneous regression has been reported [8]. Patients often have a protracted length of symptoms prior to diagnosis, with an average of 5 years [9, 10]

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