Stewart-Treves Syndrome: A Case Report and Review of the Literature

The Stewart-Treves syndrome is a rare and deadly entity, which is defined as angiosarcoma arising in the setting of chronic lymphedema. It typically presents in women who develop lymphedema in the upper extremity secondary to axillary lymph node dissection for breast cancer surgery. It is extremely...

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Main Authors: Roberto Berebichez-Fridman, Yehuda E. Deutsch, Thomas M. Joyal, Pablo Montero Olvera, Pasquale W. Benedetto, Andrew E. Rosenberg, Daniel H. Kett
Format: Article
Language:English
Published: Karger Publishers 2016-04-01
Series:Case Reports in Oncology
Subjects:
Online Access:http://www.karger.com/Article/FullText/445427
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author Roberto Berebichez-Fridman
Yehuda E. Deutsch
Thomas M. Joyal
Pablo Montero Olvera
Pasquale W. Benedetto
Andrew E. Rosenberg
Daniel H. Kett
author_facet Roberto Berebichez-Fridman
Yehuda E. Deutsch
Thomas M. Joyal
Pablo Montero Olvera
Pasquale W. Benedetto
Andrew E. Rosenberg
Daniel H. Kett
author_sort Roberto Berebichez-Fridman
collection DOAJ
description The Stewart-Treves syndrome is a rare and deadly entity, which is defined as angiosarcoma arising in the setting of chronic lymphedema. It typically presents in women who develop lymphedema in the upper extremity secondary to axillary lymph node dissection for breast cancer surgery. It is extremely uncommon in the lower extremities as a result of idiopathic chronic lymphedema. Here, we present the case of a 63-year-old female patient with idiopathic chronic lymphedema of the lower extremities having morbid obesity (BMI 82.6) and multiple comorbidities. She developed multiple confluent, hemorrhagic and necrotic elevated purple-black papules in the lower extremities, for which the initial diagnosis was cellulitis. Because there was no improvement with antibiotics, a lower extremity ultrasound and biopsy was performed which showed multiple masses in the left inner upper calf with solid and cystic components. The pathology results of the punch biopsies were consistent with angiosarcoma. Immunohistochemical studies revealed positivity for CD31, FLI-1, and a high Ki-67 proliferation rate. Because of the patient's weight and medical comorbidities, no further extensive diagnostic tests were performed to detect metastatic disease, and because of contraindications, no further medical treatment was provided. The patient subsequently died 1 month after diagnosis.
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spelling doaj.art-f88a7520e6fa4952a850051a447cad452022-12-21T21:59:08ZengKarger PublishersCase Reports in Oncology1662-65752016-04-019120521110.1159/000445427445427Stewart-Treves Syndrome: A Case Report and Review of the LiteratureRoberto Berebichez-FridmanYehuda E. DeutschThomas M. JoyalPablo Montero OlveraPasquale W. BenedettoAndrew E. RosenbergDaniel H. KettThe Stewart-Treves syndrome is a rare and deadly entity, which is defined as angiosarcoma arising in the setting of chronic lymphedema. It typically presents in women who develop lymphedema in the upper extremity secondary to axillary lymph node dissection for breast cancer surgery. It is extremely uncommon in the lower extremities as a result of idiopathic chronic lymphedema. Here, we present the case of a 63-year-old female patient with idiopathic chronic lymphedema of the lower extremities having morbid obesity (BMI 82.6) and multiple comorbidities. She developed multiple confluent, hemorrhagic and necrotic elevated purple-black papules in the lower extremities, for which the initial diagnosis was cellulitis. Because there was no improvement with antibiotics, a lower extremity ultrasound and biopsy was performed which showed multiple masses in the left inner upper calf with solid and cystic components. The pathology results of the punch biopsies were consistent with angiosarcoma. Immunohistochemical studies revealed positivity for CD31, FLI-1, and a high Ki-67 proliferation rate. Because of the patient's weight and medical comorbidities, no further extensive diagnostic tests were performed to detect metastatic disease, and because of contraindications, no further medical treatment was provided. The patient subsequently died 1 month after diagnosis.http://www.karger.com/Article/FullText/445427DiagnosisStewart-Treves syndromeAngiosarcomaLymphedemaMorbid obesityCancerTreatment
spellingShingle Roberto Berebichez-Fridman
Yehuda E. Deutsch
Thomas M. Joyal
Pablo Montero Olvera
Pasquale W. Benedetto
Andrew E. Rosenberg
Daniel H. Kett
Stewart-Treves Syndrome: A Case Report and Review of the Literature
Case Reports in Oncology
Diagnosis
Stewart-Treves syndrome
Angiosarcoma
Lymphedema
Morbid obesity
Cancer
Treatment
title Stewart-Treves Syndrome: A Case Report and Review of the Literature
title_full Stewart-Treves Syndrome: A Case Report and Review of the Literature
title_fullStr Stewart-Treves Syndrome: A Case Report and Review of the Literature
title_full_unstemmed Stewart-Treves Syndrome: A Case Report and Review of the Literature
title_short Stewart-Treves Syndrome: A Case Report and Review of the Literature
title_sort stewart treves syndrome a case report and review of the literature
topic Diagnosis
Stewart-Treves syndrome
Angiosarcoma
Lymphedema
Morbid obesity
Cancer
Treatment
url http://www.karger.com/Article/FullText/445427
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