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 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.
The William J. Harrington Medical Training Programs for Latin America and the Caribbean, University of Miami, Miller School of Medicine, Miami, Fla., USA; School of Medicine, Faculty of Health Sciences, Anáhuac University, Mexico City, Mexico.
Roberto
R
Berebichez-Fridman
Departments of Hematology/Oncology, University of Miami, Miller School of Medicine, Miami, Fla., USA.
Yehuda E
YE
Deutsch
Departments of Pathology and Laboratory Medicine, University of Miami, Miller School of Medicine, Miami, Fla., USA.
Thomas M
TM
Joyal
School of Medicine, Faculty of Health Sciences, Anáhuac University, Mexico City, Mexico.
Pablo Montero
PM
Olvera
Departments of Sylvester Comprehensive Cancer Center, University of Miami, Miller School of Medicine, Miami, Fla., USA; Department of Hematology/Oncology, Jackson Memorial Hospital, Miami, Fla., USA.
Pasquale W
PW
Benedetto
Departments of Pathology and Laboratory Medicine, University of Miami, Miller School of Medicine, Miami, Fla., USA.
Andrew E
AE
Rosenberg
Department of Critical Care Medicine, University of Miami, Miller School of Medicine, Miami, Fla., USA.
Daniel H
DH
Kett
Angiosarcoma; Cancer; Diagnosis; Lymphedema; Morbid obesity; Stewart-Treves syndrome; Treatment