A Case of Stewart-Treves Syndrome on Lower Extremity Related to Hysterectomy and Total Hip Replacement Surgery / 대한피부과학회지

Stewart-Treves syndrome (STS) is a rare cutaneous angiosarcoma that develops in chronic lymphedema. The majority of STS is described in the upper extremity after aggressive locoregional therapy for breast cancer and is rarely reported in lower extremities. A 68-year-old woman presented with a 3-month history of multiple purpuric tumorous plaques and nodules on the right posterior thigh. She had a history of radical hysterectomy with lymph node dissection and postoperative radiotherapy due to uterine cervical cancer 16 years ago. She received right total hip replacement surgery due to hip joint avascular necrosis 14 years ago. She had suffered from chronic leg edema, especially on the right side. Skin biopsy on the right posterior thigh showed irregular vascular channels lined by atypical endothelial cells. Special stains showed positivity for CD31, CD34, factor VIII, and D2~40, which are pan-vascular or lymphatic markers. She showed a pelvic mass and pelvic bone metastasis on radiologic staging work-up. She refused all treatment, including surgery, radiotherapy, and chemotherapy, except for pain control. She died 2 months after diagnosis of this highly malignant tumor. The lymphedema on both lower extremities after uterine cervical cancer treatment was aggravated especially on the right lower extremity after right total hip replacement surgery. Increased weight of the right lower extremity resulted in 4 episodes of recurrent hip dislocation. We contend that these multiple factors (uterine cervical cancer treatment, total hip replacement surgery on the right side, and recurrent hip dislocations) attributed to development of Stewart-Treves syndrome. We herein report a case of Stewart-Treves syndrome of the lower extremity following chronic leg lymphedema after uterine cervical cancer treatment and hip surgery.

Síndrome de Stewart-Treves / Stewart-Treves Syndrome

El síndrome de Stewart-Treves es un angiosarcoma cutáneo, poco frecuente, que se produce en pacientes con linfedema crónico. Se describe clásicamente luego de realizar mastectomía radical con vaciamiento ganglionar y/o radioterapia. Su mortalidad es elevada. Comunicamos el caso de una paciente de 76 años de edad, sometida a mastectomía radical con vaciamiento ganglionar axilar, seguido de quimioterapia y radioterapia. Consulta 13 años después por dolor intenso y lesión tumoral exofitica, ulcerada, angiomatosa, sangrante y maloliente en cara anterior del brazo izquierdo. Presentaba edema del miembro de varios años de evolución. Luego de realizar biopsia por punch de la lesión, los resultados histopatológicos resultaron compatibles con linfangiosarcoma. La familia junto con el servicio de Cirugía decide realizar la extirpación del tumor, con conservación del miembro. Falleció luego de once meses de realizado el diagnóstico de síndrome de Stewart-Treves, en nuestro Servicio.

The Stewart-Treves Syndrome is a rare cutaneous angiosarcoma, that occurs in patients with chronic lymphedema. It is classically described after performing radical mastectomy with lymph node dissection and/or radiation therapy. Its mortality is high. We have reported the case of a 76 years old patient, who underwent radical mastectomy with axillary lymph node dissection, followed by chemotherapy and radiotherapy. She came 13 years later with an intense pain and exophytic, ulcerated, angiomatous, bleeding and smelly tumor injury in front side of the left arm. She had an edema of the left upper limb with several years of evolution. After performing punch biopsy of the lesion the histopathological results were compatible with lymphangiosarcoma. The family, jointly with the surgery service decided to remove the tumor with limb preservation. She died 11 months after the diagnosis of Stewart-Treves syndrome, performed in our department.

Stewart-Treves syndrome: a rare entity.

Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma that develops in long - standing chronic lymphedema. Most commonly, this tumor is a result of lymphedema induced by radical mastectomy to treat breast cancer. Stewart-Treves syndrome occurs in 0.5% of patients, who survive mastectomy for more than 5 years. The mean age at appearance of the angiosarcoma is 62 years, and the mean interval between mastectomy and the appearance of the tumor is 10.5 years. The exact pathogenesis is not known so far. On histopathology, vascular channels infiltrate the normal structures in a disorganized fashion, as if trying to line every available tissue space with a layer of endothelial cells. The prognosis is poor as it is very aggressive. Only early diagnosis and prompt radical excision with or without adjuvant radiotherapy, can promote survival.

Linfangiosarcoma en un varón con linfedema crónico / Lymphangiosarcoma in a male with chronic lymphedema

Comunicamos un caso de un paciente de sexo masculino de 54 años de edad, con un linfangiosarcoma símil síndrome de Stewart-Treves post-linfedema crónico, secundario a un traumatismo. El enfermo rechazó la amputación por lo que fue irradiado y luego recibió quimioterapia, interferón y talidomida; no obstante ello, fallece debido a metástasis pulmonares a los cuatro años de efectuado el diagnóstico. Se efectúa una revisión de los angiosarcomas en general, del linfangiosarcoma, como asimismo una nota conceptual sobre los llamados hemangioendoteliomas.

A 54 years-old man with a lymphangiosarcoma, associated to a post traumatic chronic lymphedema Stewart-Treves like, was reported. The patient refused the amputation of the superior left arm so radiation therapy was done. Despite the adjuvant chemotherapy and subcutaneous interferon, together with thalidomide the patient death four years after the diagnosis due to disseminated lung metastasis. A brief review of the cutaneous angiosarcoma and a conceptual nosological position of the so called hemangioendotheliomas, were made.