SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma mimicking solid pseudopapillary neoplasm: A case report and review of the literature.
SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma mimicking solid pseudopapillary neoplasm: A case report and review of the literature.
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DOI：
10.3748/wjg.v26.i36.5520
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发表时间：
2020-09-28
影响因子：
4.3
通讯作者：
Rampisela D
中科院分区：
文献类型：
--
作者：
Hua Y;Soni P;Larsen D;Zreik R;Leng B;Rampisela D
SMARCB1/INI1缺陷的胰腺未分化横纹肌样癌是一种侵袭性很强的肿瘤，文献报道很少。肿瘤以横纹肌样细胞为主，不同的生长方式已有报道。患者为59岁女性，表现为弥漫性腹痛，程度加重，并伴有体重减轻、恶心和呕吐。影像显示胰头肿块。细针抽吸可见非典型上皮样细胞，其假乳头状生长模式提示为实性假乳头状肿瘤。切除的肿瘤表现为单调的上皮样和局灶性梭形细胞，具有假乳头状结构、横纹肌样结构和SMARCB1蛋白表达缺失的野生型KRAS，与SMARCB1/INI1缺陷的未分化横纹肌样癌一致。术后病情迅速恶化，术后3个月死亡。在本文中，我们报告了第一例SMARCB1/INI1缺陷的未分化胰腺横纹样癌，类似于实性假乳头状肿瘤。
SMARCB1/INI1-deficient pancreatic undifferentiated rhabdoid carcinoma is a very aggressive tumor that is rarely reported in the literature. The tumor has a predominant rhabdoid cell component and different patterns of growth have been reported. A 59-year-old woman presented with diffuse abdominal pain, increasing in severity and accompanied by weight loss, nausea, and vomiting. Imaging showed a pancreatic head mass. Fine needle aspiration demonstrated atypical epithelioid cells with a pseudopapillary growth pattern suggestive of solid pseudopapillary neoplasm. The excised neoplasm showed monotonous epithelioid and focally spindle cells with pseudopapillary structures, rhabdoid features, and loss of SMARCB1 protein expression with wild-type KRAS, consistent with a SMARCB1/INI1-deficient undifferentiated rhabdoid carcinoma. The patient’s condition deteriorated rapidly following surgery and she expired 3 mo post operation. In this article, we report the first case of SMARCB1/INI1-deficient undifferentiated pancreatic rhabdoid carcinoma mimicking solid pseudopapillary neoplasm.
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影响因子：
7.5
作者：
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通讯作者：
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影响因子：
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DOI：
10.1097/pas.0000000000000689
发表时间：
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期刊：
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影响因子：
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作者：
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3.5
作者：
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