Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study.
- Author:
Joon Mee KIM
1
;
Young Chae CHU
;
Chang Hwan CHOI
;
Lucia KIM
;
Suk Jin CHOI
;
In Suh PARK
;
Jee Young HAN
;
Kyung Rae KIM
;
Yoon La CHOI
;
Taeeun KIM
Author Information
1. Department of Pathology, Inha University Hospital, Inha University School of Medicine, Incheon, Korea. ycchu@inha.ac.kr
- Publication Type:Case Report
- Keywords:
Neuroectodermal tumor, primitive, peripheral;
Intestine, small;
Osteogenesis;
Metaplasia;
EWSR1
- MeSH:
Abdominal Pain;
Fluorescence;
Gene Rearrangement;
In Situ Hybridization;
Intestine, Small;
Mesentery;
Metaplasia;
Neuroectodermal Tumors, Primitive;
Neuroectodermal Tumors, Primitive, Peripheral;
Osteogenesis;
Phosphopyruvate Hydratase;
Sarcoma, Ewing;
Vimentin
- From:Korean Journal of Pathology
2013;47(1):77-81
- CountryRepublic of Korea
- Language:English
-
Abstract:
A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with osseous component is reported. A 23-year-old man was admitted to our hospital because of acute severe abdominal pain. Abdominal computed tomography revealed a large solid and cystic, oval shaped mass, measuring 11.0x6.0 cm in the pelvic cavity. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming Homer-Wright rosettes and perivascular pseudorosettes, and showed areas of osteoid and bone formation. Immunohistochemical studies revealed that tumor cells expressed positivity against CD99 (MIC2), CD57, neuron-specific enolase, and vimentin. Fluorescence in situ hybridization study revealed Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement on chromosome 22q12. To the authors' knowledge this is the first documentation of a peripheral neuroectodermal tumor with osteoid and bone formation of the small bowel mesentery.
