Lymphangiosarcoma arising in congenital lymphedema

An 85-year-old woman with congenital lymphedema of the right upper extremity developed a small purplish papule on the forearm, which was the first clinical evidence of lymphangiosarcoma. The lesion grew rapidly and became necrotic and ulcerated. The patient experienced severe pain. A mid-arm amputation was performed. Microscopical examination of the amputated limb showed widespread histological involvement of skin, muscle, and subcutaneous tissue by lymphangiosarcoma. The need for continuous monitoring of patients with both primary and secondary lymphedema is emphasized, since early diagnosis of the developing lymphangiosarcoma and rapid surgical intervention provide the best prognosis for survival.