Chondrosarcoma in a patient with relapsing polychondritis

Abstract

A case of a chondrosarcoma in a patient with relapsing polychondritis was presented. The association of these two unusual disorders of cartilaginous tissue has never been described before. The clinical presentation and radiologic and histologic features were discussed.

Sweet's syndrome and relapsing polychondritis: Is their appearance in the same patient a coincidental occurrence or a bona fide association of these conditions?

“…Relapsing polychondritis can be associated with connective tissue diseases (ankylosing spondylitis, psoriatic arthritis, Reiter's syndrome, rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus), gastrointestinal diseases (Crohn's disease, primary biliary cirrhosis, and ulcerative colitis), endocrine disorders (diabetes mellitus type I, Graves’ disease, Hashimoto thyroiditis, and hypothyroidism), and systemic vasculitides (such as Behcet's disease/MAGIC [mouth and genital ulcers with inflamed cartilage] syndrome, Churg–Strauss syndrome, microscopic polyarteritis, polyarteritis nodosa, Takayasu arteritis, and temporal arteritis) 27–35 . Paraneoplastic relapsing polychondritis is increasingly being described: Most commonly in patients with myelodysplastic syndrome, 30,31,36–48 but also – less frequently – in individuals with other malignant hematologic disorders 28,30,32,45–47,49–54 or solid tumors 1,30,47,55–60 . Rarely benign neoplasms (thymoma in an individual with myasthenia gravis 61 and enchondroma 62 have been reported in relapsing polychondritis patients.…”

“…A comparison of the epidemiology, clinical and histologic features, treatment, and pathogenesis of Sweet's syndrome and relapsing polychondritis is summarized in Table 1 1,12–73 . In a series of 48 patients with Sweet's syndrome, 6.25% of the individuals (three patients) had relapsing polychondritis 65 .…”

“… † Data for this table has been summarized from several reports: Sweet's syndrome 1–26,36,37,63–73 and relapsing polychondritis 1,27–65 …”

“… ††† Some of the solid tumors reported in patients with relapsing polychondritis include: Adenocarcinoma (unknown primary), 47 bladder, 1 breast, 30 bronchus, 47 chondrosarcoma, 58 colon, 30,57 lung, 30 malignant fibrohistiocytoma, 57 pancreas, 56 prostate, 56,59 rectum, 60 and vocal cords 57 …”

Untitled

“…Similar tumours in the breast, vocal cords, prostate [23], Kaposi sarcoma [24], soft-tissue sarcoma [25], malignant fibrohistiocytoma and urothelial carcinoma [26] have also been recorded in few cases (Table 1). Cutaneous features have been described in 35-50% of RP patients [1].…”