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KMID : 0357920130470010077
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Korean Journal of Pathology
2013 Volume.47 No. 1 p.77 ~ p.81
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Peripheral Primitive Neuroectodermal Tumor with Osseous Component of the Small Bowel Mesentery: A Case Study
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Kim Joon-Mee
Chu Young-Chae
Choi Chang-Hwan
Kim Lucia
Choi Suk-Jin
Park In-Suh
Han Jee-Young
Kim Kyung-Rae
Choi Yoon-La
Kim Tae-Eun
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Abstract
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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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KEYWORD
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Neuroectodermal tumor, primitive, peripheral, Intestine, small, Osteogenesis, Metaplasia, EWSR1
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