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胰腺母细胞瘤:一种罕见的成人胰腺肿瘤,具有多种形态。

Pancreatoblastoma: a rare, adult pancreatic tumor with many faces.

机构信息

Department of Pathology, University of Michigan, Ann Arbor, 48109, USA.

出版信息

Arch Pathol Lab Med. 2013 Sep;137(9):1224-6. doi: 10.5858/arpa.2013-0272-CR.

DOI:10.5858/arpa.2013-0272-CR
PMID:23991735
Abstract

Pancreatoblastomas are malignant epithelial neoplasms of the pancreas that are heterogeneous and have variable cellular differentiation, complicating the diagnosis. We report a case of pancreatoblastoma occurring in an adult patient, presenting as a pancreatic head mass with liver metastasis and jaundice. The initial liver biopsy diagnosis was metastatic neuroendocrine carcinoma based on morphology and synaptophysin positivity. At pancreatic resection, the diagnostic features of pancreatoblastoma were recognized. We review the radiologic and pathologic differential diagnosis, histologic heterogeneity, clinical presentation, and associated genetic syndromes for this unusual tumor that can mimic other types of pancreatic neoplasia.

摘要

胰腺母细胞瘤是胰腺的恶性上皮性肿瘤,具有异质性和可变的细胞分化,这使得诊断变得复杂。我们报告了一例发生在成年患者的胰腺母细胞瘤病例,表现为胰头部肿块伴肝转移和黄疸。初始的肝活检诊断基于形态学和突触素阳性表现为转移性神经内分泌癌。在胰腺切除术中,认识到了胰腺母细胞瘤的诊断特征。我们回顾了这种罕见肿瘤的影像学和病理学鉴别诊断、组织学异质性、临床表现和相关的遗传综合征,因为它可以模拟其他类型的胰腺肿瘤。

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