Angiosarcoma in a chronically lymphedematous leg: an unusual presentation of Stewart-Treves syndrome.

Abstract: Angiosarcoma arising from chronic lymphedema is referred to as Stewart-Treves syndrome. It typically occurs as a complication of long-lasting lymphedema of the arm after mastectomy and/or radiotherapy for breast cancer. Angiosarcoma associated with idiopathic lymphedema of the lower extremity is extremely rare. We report a case of diffuse angiosarcoma of the leg in a patient with a 25-year history of idiopathic lymphedema. Despite rapid aggressive surgical treatment, the patient died 6 weeks after diagnosis.

Key Words: angiosarcoma, chronic lymphedema

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Key Points

* All patients with chronic lymphedema of the extremity should have close follow-up.

* Angiosarcoma arising from chronic lymphedema has a poor prognosis despite rapid aggressive surgical treatment.

Case Report

A 60-year-old white man was referred to the department of surgical oncology because of a painful lesion of the left leg. The patient had a 25-year history of chronic lymphedema of the lower extremities with recurrent episodes of erysipelas. At age 7 he had surgery for a right inguinal hernia. Medical history was otherwise noncontributory. At physical examination, both legs were grossly lymphedematous, and there was marked lymphedema of the groin region and the anterior abdominal wall. The skin on the lower extremities was indurated and pale, with multiple papillomatous, skin-colored nodules. On the left popliteal fossa was an ulcerated, purplish lesion measuring 15 cm in diameter, with multiple purplish satellite nodules of 1 to 3 cm (Fig. 1). There were no other abnormal physical findings. Computed tomography (CT) showed a 4.0 x 7.0 x 7.5 cm tumor within the soft tissue of the left thigh, posterior to the popliteal artery and extending upward. Infiltration of extensor muscles, subcutaneous tissue, and skin was also noticed on the CT scans, as well as extensive infiltration of subcutaneous tissue within the posterior and medial part of the left thigh. Findings on chest x-ray, abdominal ultrasonography, and x-ray films of the lower left extremity were normal. Microscopic examination of an incisional biopsy specimen showed infiltration of malignant mesenchymal GIII neoplasm, suggesting a differential diagnosis of angiosarcoma. Because of excessive lymphedema, radiotherapy was not indicated, and left above-knee amputation was done. Pathologic studies revealed malignant mesenchymal neoplasm of angiosarcoma type. Infiltration of muscles, adipose tissue, and skin within all parts of the extremity was noted, as well as elephantiasis. Infiltration of neoplastic cells was present within surgical margins. Six days after surgery, multiple purplish nodules were seen within the left inguinal region. Incisional biopsy showed infiltration of malignant mesenchymal cells. On the 20th day after surgery, a chest x-ray showed bilateral hydrothorax and right pneumothorax. Cytologic study of the fluid drained from both pleural cavities showed anaplastic cells. Radiotherapy was not indicated. The patient died 6 weeks after diagnosis.

[FIGURE 1 OMITTED]

Angiosarcoma is a malignant tumor of vascular origin. It accounts for less than 1% of all sarcomas and is usually located in the skin, breast, liver, and deep soft tissue. (1) Angiosarcoma arising in a region of chronic lymphedema is referred toas Stewart-Treves syndrome. It typically occurs in postmastectomy lymphedema of the arm and sporadically in a lymphedematous leg. (2) The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor. (3)

Discussion

Angiosarcoma accounts for less than 1% of all sarcomas. (1) Factors that have been considered in the pathogenesis of this tumor include radiation, lymphedema, ulceration, and chemicals such as vinyl chloride, arsenic, and thorium dioxide. (4) Angiosareoma arising in an area of chronic lymphedema is referred to as Stewart-Treves syndrome. Stewart and Treves (2) first described lymphangiosarcoma occurring in postmastectomy lymphedema of the arm in 1948. Since then, several authors have reported this severe postmastectomy complication. Angiosarcoma arising in a chronically lymphedematous leg is even less common. Chronic lymphedema in the lower extremity can be caused by previous radiotherapy (5) or filiariasis (6) or less frequently can be idiopathic, (7) as in our case. In some cases, a history of recurrent erysipelas in the lymphedematous extremity has been reported. (6) This was also present in our case. In the majority of patients, the angiosarcoma is relatively large (at least 10 cm in diameter) at the time of presentation because of a significant delay in diagnosis. The delay is usually a consequence of a chronic, clinically irrelevant history of lymphedema (up to 40 yr (7)) and the presence of multiple hyper-keratotic nodules and fissures that may occur with lymphedema and thus lead to overlooking the first symptoms of sarcoma. (5) Our patient had had lymphedema for 25 years before being referred to our center because of pain. Pathologic examination revealed sarcoma infiltrating and destroying most of the tissue in both the thigh and crus. This finding suggests that angiosarcoma had been present for a long period and its management could have been more successful if diagnosed earlier.

Treatment of Stewart-Treves syndrome should consist of radical, wide surgical excision (or limb amputation if obtaining wide negative margins by excision is doubtful) and subsequent radiotherapy. (8) Nevertheless, even aggressive therapy has resulted in a mean survival rate of 24 months and a 5-year survival rate of only 10%. (3) In our case, amputation was considered first-line treatment. Radiotherapy was not applied because rapid dissemination occurred shortly after surgical treatment. The patient survived for only 1 month.

The incidence of Stewart-Treves syndrome is low. The prevalence has been estimated at between 0.45 and 0.07% of patients treated for early carcinoma of the breast. (9) Stewart-Treves syndrome in the lower extremity is reported only occasionally. (5-7) Our case illustrates the possibility of angiosarcoma arising in a chronically lymphedematous extremity and thus the need for careful follow-up of all patients with chronic lymphedema.

References

(1.) Enzinger FM, Weiss SW. Malignant vascular tumors, in Enzinger FM, Weiss SW (eds): Soft Tissue Tumors. St. Louis, C.V. Mosby, 1988, ed 2, pp 545 580.

(2.) Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema. Cancer 1948;1:64-81.

(3.) Caldwell JB, Ryan MT, Benson PM, James WD. Cutaneous angiosarcoma arising in the radiation site of congenital hemangioma. J Am Acad Dermatol 1995;33:865-870.

(4.) Nanus DM, Kelsen D, Clark DG. Radiation-induced angiosarcoma. Cancer 1987;60:777-779.

(5.) Azurdia RM. Guerin DM, Verbov JL. Chronic lymphedema and angiosarcoma. Clin Exp Dermatol 1999;24:270-272.

(6.) Hallel-Halevy D, Yerushalmi J, Grunwald MH, Avinoach I, Halevy S. Stewart-Treves syndrome in a patient with elephantiasis. J Am Acad Dermatol 1999;41:349-350.

(7.) Sinclair SA, Sviland L, Natarajan S. Angiosarcoma arising in a chronically lymphedematous leg. Br J Dermatol 1998;138:692-694.

(8.) Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma: A report of 67 patients and a review of the literature. Cancer 1996;77:2400-2406.

(9.) Fitzpatrick PJ. Lymphangiosarcoma in breast cancer. Can J Surg 1969; 12:172.

From the Department of Surgical Oncology, Center of Oncology, Maria Curie-Sklodowska Memorial Institute of Oncology, Krakow, Poland. Reprint requests to Andrzej Komorowski, MD, Department of Surgical Oncology,Center of Oncology, Maria Curie-Sklodowska Memorial Institute of Oncology, ul.Garncarska 11, 31-115 Krakow, Poland. Email: [e-mail]

Accepted June 19, 2002.