Stewart Treves Syndrome

Abstract

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

Unsuspected Stewart–Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart–Treves syndrome

“…For initial evaluation, 35 articles were selected. When patients were selected for analysis, 29 articles met the criteria of inclusion, within which 44 patients were identified. Three articles presented multiple cases, and their cases were assessed separately.…”

“…Limb infection may be considered in the differential diagnosis, depending on the color of the lesions, or associated lymphedema. The presentation of papules is low (7.0%). Multiple colors were described: purplish to brownish, erythematous, erythematous‐blue, erythematous‐violet, blue‐red, blue‐black, purple‐red, purple, hemorragic or ecchymotic .…”

“…Of the lesions, 79.1% had an associated nodule, 67.4% were reported as nodule or tumor, 33.3% evolved with ulceration and bleeding, and the description/observation of papules was low (7.0%). Different colors were reported, the main color was purple/purplish in 53.4%, blue-red in 22.2%, and 54.5% described just one color. The ecchymotic halo ( Figure 2B) was described in 15.6% of the cases reviewed, but, considering the characteristics observed in the images presented in the articles, 17.8% were added, resulting in a total of 33.4% of the cases.…”

Brazilian consensus on the diagnosis and treatment of extremities soft tissue sarcomas

“…Immunosuppressive agents, such as HIV, and the use of immunosuppressive medications, such as posttransplant and chemotherapy medications, are related to the development of Kaposi sarcoma 6 . The presence of chronic lymphedema after mastectomy correlates with the occurrence of angiosarcomas 7 …”

“…6 The presence of chronic lymphedema after mastectomy correlates with the occurrence of angiosarcomas. 7 The incidence of STS ranges from 12% to 21% in patients with Li-Fraumeni syndrome-that is, those with mutations in TP53. 8 In Brazil, the pR337H is the most prevalent mutation in Li-Fraumeni patients, especially in the southeast and south, and this variant is associated with several peculiarities, such as high incidence of adrenocortical carcinoma in childhood; earlier breast cancer; and a higher prevalence of papillary thyroid tumor, kidney cancer, and pulmonary adenocarcinoma in relation to the classical form of Li-Fraumeni.…”