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Vol.48, No.3, 2014
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Archive (2009~
Archive-the Korean Journal of Pathology(1967~2008)
Archive-the Korean Journal of Cytopathology(1990~2008)
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  Vol.47, No.1:77-81, February 2013
Case studies
Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study

Joon Mee Kim· Young Chae Chu· Chang Hwan Choi · Lucia Kim· Suk Jin Choi · In Suh Park· Jee Young Han · Kyung Rae Kim1· Yoon-La Choi2 · Taeeun Kim3

Departments of Pathology and 1Surgery, Inha University Hospital, Inha University School of Medicine, Incheon; 2Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul; 3Department of Pathology, Gachon University of Medicine and Science, Incheon, Korea

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 11.0×6.0 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.
Key Words : Neuroectodermal tumor, primitive, peripheral; Intestine, small; Osteogenesis; Metaplasia; EWSR1

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