Abstract
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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 163-167 |
| Number of pages | 5 |
| Journal | Pediatric and Developmental Pathology |
| Volume | 20 |
| Issue number | 2 |
| DOIs | |
| State | Published - 2017 |
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Keywords
- Cytoreductive surgery
- Hyperthermic intraperitoneal chemotherapy
- Immunohistochemistry
- Metastatic angiosarcoma
- Ovarian tumor
- Pediatric sarcoma
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Pathology and Forensic Medicine
Cite this
Angiosarcoma of the Pelvis in a 13-year-old girl : Case report and review of the literature. / El-Sharkawy, Farah; Isabel Delgado, Patricia; Podda, Antonello; Neville, Holly; Rojas, Claudia Patricia.
In: Pediatric and Developmental Pathology, Vol. 20, No. 2, 2017, p. 163-167.Research output: Contribution to journal › Article
}
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
AU - Rojas, Claudia Patricia
PY - 2017
Y1 - 2017
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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UR - http://www.scopus.com/inward/citedby.url?scp=85027159631&partnerID=8YFLogxK
U2 - 10.1177/1093526616686007
DO - 10.1177/1093526616686007
M3 - Article
VL - 20
SP - 163
EP - 167
JO - Pediatric and Developmental Pathology
JF - Pediatric and Developmental Pathology
SN - 1093-5266
IS - 2
ER -