TY - JOUR
T1 - Angiosarcoma of the Pelvis in a 13-year-old girl
T2 - Case report and review of the literature
AU - El-Sharkawy, Farah
AU - Isabel Delgado, Patricia
AU - Podda, Antonello
AU - Neville, Holly Leigh
AU - Rojas, Claudia Patricia
N1 - Publisher Copyright:
© Society for Pediatric Pathology, 2017 All rights reserved.
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2017/4
Y1 - 2017/4
N2 - Angiosarcomas are highly aggressive malignancies of vascular origin and are very rarely found in children. We report a case of a 13-year-old girl with a history of abdominal pain and increased abdominal girth. Radiologic imaging showed significant ascites and large pelvic masses involving bilateral adnexa with abdominal spread. Microscopic examination of a biopsy revealed pleomorphic epithelioid and spindle cells with brisk mitotic activity, intracytoplasmic vacuoles, vascular channels, and large areas of hemorrhage and necrosis. Immunohistochemistry analysis showed strong and diffuse positivity for CD31, D2-40, ERG, FLI-1, and focally for CD34, vWF, and EMA. The diagnosis of metastatic angiosarcoma was rendered. The patient was treated aggressively with systemic chemotherapy, immunotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy, with a favorable response after 1-year follow-up. Angiosarcoma should be considered when encountering a vascular tumor with pleomorphism, brisk mitotic activity, and necrosis. Immunohistochemistry studies are necessary for proper diagnosis.
AB - Angiosarcomas are highly aggressive malignancies of vascular origin and are very rarely found in children. We report a case of a 13-year-old girl with a history of abdominal pain and increased abdominal girth. Radiologic imaging showed significant ascites and large pelvic masses involving bilateral adnexa with abdominal spread. Microscopic examination of a biopsy revealed pleomorphic epithelioid and spindle cells with brisk mitotic activity, intracytoplasmic vacuoles, vascular channels, and large areas of hemorrhage and necrosis. Immunohistochemistry analysis showed strong and diffuse positivity for CD31, D2-40, ERG, FLI-1, and focally for CD34, vWF, and EMA. The diagnosis of metastatic angiosarcoma was rendered. The patient was treated aggressively with systemic chemotherapy, immunotherapy, cytoreductive surgery, and hyperthermic intraperitoneal chemotherapy, with a favorable response after 1-year follow-up. Angiosarcoma should be considered when encountering a vascular tumor with pleomorphism, brisk mitotic activity, and necrosis. Immunohistochemistry studies are necessary for proper diagnosis.
KW - Cytoreductive surgery
KW - Hyperthermic intraperitoneal chemotherapy
KW - Immunohistochemistry
KW - Metastatic angiosarcoma
KW - Ovarian tumor
KW - Pediatric sarcoma
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U2 - 10.1177/1093526616686007
DO - 10.1177/1093526616686007
M3 - Article
C2 - 28326959
AN - SCOPUS:85027159631
VL - 20
SP - 163
EP - 167
JO - Pediatric and Developmental Pathology
JF - Pediatric and Developmental Pathology
SN - 1093-5266
IS - 2
ER -