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胃原始神经外胚层肿瘤:一例报告

Primitive Neuroectodermal Tumor of the Stomach: A Case Report.

作者信息

Song Min Jeong, An Soyeon, Lee Seung Soo, Kim Beom Su, Kim Jihun

机构信息

University of Ulsan Collage of Medicine, Asan Medical Center, Seoul, Republic of Korea.

University of Ulsan Collage of Medicine, Asan Medical Center, Seoul, Republic of Korea

出版信息

Int J Surg Pathol. 2016 Sep;24(6):543-7. doi: 10.1177/1066896916639371. Epub 2016 Mar 21.

Abstract

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a highly aggressive small round cell tumor that mainly occurs in the bone or soft tissue of children or young adults but is extremely rare in the stomach. A 55-year-old man presented with melena and anemia. On endoscopy, an ulcerofungating mass was observed in the high body and total gastrectomy was performed. Histologically, the mass consisted of small round cells with scanty cytoplasm and inconspicuous nucleoli. They often formed perivascular pseudorosettes and multinucleated giant cells were frequently observed. The tumor cells strongly expressed CD99, FLI1, and chromogranin and weakly expressed synaptophysin and CD56. EWS-FLI1 fusion transcript was confirmed by reverse transcription-polymerase chain reaction. ES/PNET is frequently misdiagnosed because of its similarity with small cell carcinoma. Although gastric ES/PNET is very rare, it should be included in differential diagnoses of small round cell tumor in the stomach.

摘要

尤因肉瘤/原始神经外胚层肿瘤(ES/PNET)是一种侵袭性很强的小圆细胞肿瘤,主要发生于儿童或青年的骨骼或软组织,但在胃中极为罕见。一名55岁男性因黑便和贫血就诊。内镜检查时,在胃体上部发现一个溃疡性蕈伞状肿物,遂行全胃切除术。组织学上,肿物由胞质稀少、核仁不明显的小圆细胞组成。它们常形成血管周围假菊形团,且常可见多核巨细胞。肿瘤细胞强烈表达CD99、FLI1和嗜铬粒蛋白,弱表达突触素和CD56。通过逆转录-聚合酶链反应证实存在EWS-FLI1融合转录本。ES/PNET常因与小细胞癌相似而被误诊。尽管胃ES/PNET非常罕见,但在胃小圆细胞肿瘤的鉴别诊断中应考虑到它。

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