A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema

Abstract Background stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. Objectives This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. Methods Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. Results The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. Study limitations Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. Conclusions STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

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.

The MRI findings of Stewart-Treves syndrome / 实用放射学杂志

Objective To evaluate the value of MRI diagnosis in Stewart-Treves syndrome(STS).Methods The 48 STS nodules proved by pathology were retrospectively studied,which compared the MRI findings and pathological results.Results All nodules were solid and more than 4 mm in diameter.According to the expression of D2-40 and CD31,all nodules were divided into two types.The 47 nodules were divided into STS type I(hemangiosarcoma).The MRI findings showed slightly low signal on T1 WI and slightly high signal on T2 WI,while were higher than the muscular signal.Additionally,all nodules of STS type I were remarkably enhanced.The 1 mass-like-shape cases wsa divided into STS type Ⅱb (mixed lymphangiosarcoma).The MRI findings showed heterogeneous signal on both T1 WI and T2 WI,in which the high signal was leading.And the enhancement showed the low-level inhomogeneous signal.Conclusion MRI could be a valuable tool for the diagnosis in STS because of the characteristic signal on MRI.And if combined with the history of chronic upper limb lymphedema,the diagnostic accuracy could be improved before 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.

Stewart-Treves Syndrome Arising in a Chronic Lymphedematous Leg / 대한피부과학회지

Angiosarcoma is a rare malignancy of a vasculo-eptihelial origin. It is divided into three categories. The first category is primary anigosarcoma, which occurs in the face and scalp of elderly patients without any predisposing factors. The second is Stewart-Treves Syndrome, which is a subset of chronic lymphedema. The third is a post irradiation angiosarcoma that occurs at the site of prior irradiation. Generally in Stewart-Treves Syndrome, there is a pre-existing chronic lymphedema on the upper extremities due to the wide excision of breast cancer with or without radiation therapy. We here report on a case of a 60-year-old female with chronic lymphedema on the leg and who had a history of hysterectomy due to cervical cancer. The patient had purple-colored tender nodule and ulcer with exudate on the chronic lymphedematous leg and was diagnosed as angiosarcoma by a skin biopsy.

A Case of Stewart-Treves Syndrome Associated with Disseminated Intravascular Coagulation / 대한피부과학회지

Stewart-Treves syndrome is an angiosarcoma that develops in chronic lymphedematous skin. Kasabach-Merritt syndrome is characterized by the occurrence of disseminated intravascular coagulation (DIC) in combination with benign angiomatous tumors in most cases, but angiosarcoma has also been reported to be associated with DIC in rare cases. Here we report on a case of a 74-year-old woman who had angiosarcoma on a large area of the right trunk and arm around a previous mastectomy site. She developed DIC after laminoplasty, and she finally expired from multiorgan failure 3 months after the initial diagnosis of DIC. Her preoperative laboratory findings were normal, and she did not have any other underlying diseases that were possibly related with the development of DIC, except the large area of angiosarcoma. In conclusion, we report here on a rare case of Stewart-Treves syndrome that co-occurred with DIC and we believe that accurate evaluation of a skin lesion is important for Stewart-Treves syndrome patients who display systemic symptoms.

Angiosarcoma in a Chronically Lymphedematous Leg: A Case of Stewart-Treves Syndrome / 대한피부과학회지

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 lymphedema of the lower extremity is rare. We report a case of angiosarcoma of the leg in a patient with a 15-year history of lymphedema due to cervical cancer operation and radiotherapy.

A case of Stewart-Treves syndrome

Several months after left radical mastectomy without irradiation therapy for breast cancer, a 74-year-old woman developed severe edema on the homolateral arm extending to the axilla. Ten years later, purplish to brownish blotch and nodules accompanied with heating sensation and pain appeared and increased in size gradually on the left forearm. The patient was treated by irradiation therapy under the clinical and histopathologic diagnosis of Stewart-Treves syndrome and almost all of the skin lesions and symptoms disappeared after irradiation of 6450 rads.