Lymphangiosarcoma is known to be associated with chronic lymphedema of an extremity, especially when it is secondary to radical mastectomy. Only rarely has this malignant neoplasm been secondary to chronic lymphedema without the previous occurrence of a malignant neoplasm. Our patient had lymphangiosarcoma secondary to congenital lymphedema, which is a very rare occurrence. Clinical, microscopic, histochemical, and ultrastructural studies were performed. Multiple early biopsy specimens of this patient's lesion showed no definite neoplasm. If an area of ulceration occurs within a chronically lymphedematous extremity, multiple deep biopsies should be performed; one or two normal biopsy findings do not rule out the presence of malignant disease. The prognosis in this malignant condition is poor, but the best chance of long-term survival is prompt diagnosis and radical surgery.
Dubin HV, Creehan EP, Headington JT. Lymphangiosarcoma and Congenital Lymphedema of the Extremity. Arch Dermatol. 1974;110(4):608-614. doi:10.1001/archderm.1974.01630100064017