Stewart-Treves Syndrome: Lymphangiosarcoma Following Mastectomy

Heitmann, Christoph; Ingianni, G.

doi:10.1097/00000637-200044010-00012

Cited by 18 publications

(9 citation statements)

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“…11 They account for less than 2% of all visceral and soft tissue sarcomas, and various clinical forms have been described, including primary scalp, radiation-induced, primary breast, angiosarcoma associated with lymphedema (Stewart-Treves Syndrome) 12 , and vinyl-chloryde induced liver sarcomas. 13 Most are spontaneous, however, several well described risk factors exist, including radiation, chronic lymphoedema, exogenous toxins and familial syndromes.…”

Section: Discussionmentioning

confidence: 99%

Systemic therapy in primary angiosarcoma of the spleen

et al. 2012

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Primary splenic angiosarcoma is a very rare neoplasm with a high propensity for metastatic disease and poor prognosis. There is a paucity of literature concerning this specific sarcoma subtype and the role of systemic therapy is not well defined. A retrospective review of the prospectively maintained University of Washington/Seattle Cancer Care Alliance Sarcoma Unit database was performed to identify patients with splenic angiosarcoma treated between 2007 and 2012. In total there were 19 patients with angiosarcoma treated at the Seattle Cancer Care Alliance from 2007 to 2012. The number of patients with splenic angiosarcoma was 2 (11%). The first patient was a woman aged 57 years who was referred with metastatic splenic angiosarcoma to the liver, post-splenectomy. She was treated with 4 cycles of weekly paclitaxel prior to metastatic resection and 4 cycles of the same drug in an adjuvant scenario, achieving a pathological complete response to treatment. She is alive and on third-line systemic therapy. The second patient was a male patient aged 30 years who presented with metastatic high-grade splenic angiosarcoma and was treated with 3 lines of systemic therapy, including doxorubicin, paclitaxel and gemcitabine+docetaxel, but developed a gastrointestinal metastasis with subsequent gastrointestinal bleeding. Splenic angiosarcoma is a very rare neoplasm. Surgery remains the mainstay of management for localized disease. Paclitaxel administered weekly proved to be well-tolerated and resulted in a good radiological response in one of our patients, enabling resection of metastatic disease. Durable clinical benefit can be achieved in metastatic splenic angiosarcoma with multi modality management.

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Section: Discussionmentioning

confidence: 99%

Systemic therapy in primary angiosarcoma of the spleen

et al. 2012

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“…It is a rare condition, and c‐STS is even more infrequent. The diagnosis is easier in the presence of infiltrative single or multiple nodular hardened areas associated with tumor, plaque, or macula . There is no usual dermatological presentation, as shown in this review.…”

Section: Discussionmentioning

confidence: 70%

“…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.…”

Section: Resultsmentioning

confidence: 99%

“…The diagnosis is easier in the presence of infiltrative single or multiple nodular hardened areas associated with tumor, plaque, or macula. 1,2,[7][8][9][10][11][12][13][15][16][17][19][20][21][23][24][25][27][28][29][30][31][32] There is no usual dermatological presentation, as shown in this review. Papules are associated with bullous erysipelas, but the presence of multicolored lesions should raise suspicion.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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Background Stewart Treves–Syndrome (STS) was first characterized as angiosarcoma in the homolateral limb of a patient with breast cancer and lymphedema. Now, other conditions represent STS. It's a rare condition. The diagnosis is easier in the presence of single or multiple purple nodules. Even though other dermatological aspects have been reported, no study has grouped its characteristics. Aim Evaluate the dermatological characteristics of classical STS (c‐STS). Methods and Results We report a patient with chronic lymphedema with a history of recurrent erysipelas that rapidly developed multiple papules in the superior limb. It was initially diagnosed as bullous erysipelas, but unsatisfactory evolution led to biopsy, which demonstrated an unsuspected epithelioid angiosarcoma. We have also performed a review of dermatologic aspects of c‐STS using PubMed and Lilacs databases. PICTOS methodology and PRISMA flow chart were considered. The main dermatological aspects associated with c‐CTS were summarized. Using a systematic evaluation from 109 articles, 29 were selected and 44 patients were described to whom we added one case. The mean time with lymphedema was 10 years. Of the patients analyzed, 97.2% were female; 95.6% were submitted to radical mastectomy; 81.2% presented with multiple lesions, 67.4% of the lesions were reported as nodules or tumors, 53.4% were purple, 33.4% were associated with an ecchymotic halo, and 33.4% were ulcerated lesions. Conclusion When evaluating patients with chronic lymphedema with new dermatological abnormalities, clinical suspicion, or unfavorable evolution, the knowledge of clinical signs is important for diagnosis, and a biopsy must be considered. Papules associated with erythematous‐wine color and bluish hematoma aspect must raise clinical suspicion.

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“…One of these malignancies is lymphangiosarcoma (Stewart-Treves syndrome) [25]. The incidence rate of this syndrome in patients with lymphedema 5 years after breast cancer surgery is 0.07–0.45%, according to previous reports [26]. Other malignant tumors that appear with increased frequency in lymphedematous limbs include Kaposi's sarcoma, squamous cell carcinoma, malignant lymphoma, and melanoma.…”

Section: Pathophysiology Of Lymphedemamentioning

confidence: 99%

Lymphedema and Therapeutic Lymphangiogenesis

Saito

Nakagami

Kaneda

et al. 2013

BioMed Research International

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Lymphedema is a disorder of the lymphatic vascular system characterized by impaired lymphatic return and swelling of the extremities. Lymphedema is divided into primary and secondary forms based on the underlying etiology. Despite substantial advances in both surgical and conservative techniques, therapeutic options for the management of lymphedema are limited. Although rarely lethal, lymphedema is a disfiguring and disabling condition with an associated decrease in the quality of life. The recent impressive expansion of knowledge on the molecular mechanisms governing lymphangiogenesis provides new possibilities for the treatment of lymphedema. This review highlights the lymphatic biology, the pathophysiology of lymphedema, and the therapeutic lymphangiogenesis using hepatocyte growth factor.

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Stewart-Treves Syndrome: Lymphangiosarcoma Following Mastectomy

Cited by 18 publications

References 0 publications

Systemic therapy in primary angiosarcoma of the spleen

Systemic therapy in primary angiosarcoma of the spleen

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

Lymphedema and Therapeutic Lymphangiogenesis

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