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Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study

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±èÁع̠( Kim Joon-Mee ) 
Inha University Hospital Department of Pathology

ÁÖ¿µÃ¤ ( Chu Young-Chae ) 
Inha University Hospital Department of Pathology
ÃÖâȯ ( Choi Chang-Hwan ) 
Inha University Hospital Department of Pathology
±è·ç½Ã¾Æ ( Kim Lucia ) 
Inha University Hospital Department of Pathology
ÃÖ¼®Áø ( Choi Suk-Jin ) 
Inha University Hospital Department of Pathology
¹ÚÀμ­ ( Park In-Suh ) 
Inha University Hospital Department of Pathology
ÇÑÁö¿µ ( Han Jee-Young ) 
Inha University Hospital Department of Pathology
±è°æ·¡ ( Kim Kyung-Rae ) 
Inha University Hospital Department of Surgery
ÃÖÀ±¶ó ( Choi Yoon-La ) 
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
±èÅÂÀº ( Kim Tae-Eun ) 
Gachon University of Medicine and Science Department of Pathology

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 11.0¡¿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.

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Neuroectodermal tumor; primitive; peripheral; Intestine; small; Osteogenesis; Metaplasia; EWSR1

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