Background: Chronic lymphoedema is classically complicated by recurring episodes of cellulitis. Degeneration to the angiosarcoma form (Stewart-Treves syndrome) is much less common. It occurs mainly in the upper limbs following surgery or radiotherapy for mammary neoplasia. Herein we report a rare case of Stewart-Treves syndrome (STS) of the lower limb as a complication of congenital lymphoedema.
Patients and methods: A 69-year-old woman treated for bilateral lower-limb oedema present for 30years developed painful necrotic lesions in her left lower limb. A diagnosis of angiosarcoma was made based on biopsy of a nodular lesion. Since surgical excision was precluded by the extent of the lesions, chemotherapy was initiated with paclitaxel 175mg/m2 every 21days. The outcome was rapidly unfavourable and the patient died at her home during the third course of treatment.
Discussion: Angiosarcoma is an extremely rare complication of primary lymphoedema; treatment is poorly codified and the prognosis is unfavourable.
Keywords: Angiosarcoma; Angiosarcome; Endothelial tumor; Lymphœdème primaire; Primary lymphedema; Stewart-Treves; Tumeur endothéliale.
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