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一例胰腺混合性神经内分泌-非神经内分泌肿瘤(MiNEN)(胰腺神经内分泌肿瘤及伴有破骨细胞样巨细胞的胰腺未分化癌),其神经内分泌成分转移至肝脏。

A pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) (NET and undifferentiated carcinoma of the pancreas with osteoclast-like giant cells) with metastatic neuroendocrine component to the liver.

作者信息

Gao Guofeng, Qorbani Amir, Zhou Chihong Heidi

机构信息

University of California, Davis Medical Center, Department of Pathology & Laboratory Medicine, Sacramento, CA, USA.

University of California, San Francisco Medical Center, Department of Pathology & Laboratory Medicine, San Francisco, CA, USA.

出版信息

Autops Case Rep. 2020 Dec 8;11:e2020201. doi: 10.4322/acr.2020.201. eCollection 2021.

DOI:10.4322/acr.2020.201
PMID:34277482
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8101651/
Abstract

Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UCOGCs) is an extremely rare morphologically and clinically distinct variant of pancreatic ductal adenocarcinoma (PDAC), exhibiting a characteristic component of reactive osteoclast-like giant cells admixed with neoplastic mononuclear cells. Sommers and Meissner first described it in 1954 as an "unusual carcinoma of the pancreas". Later it acquired many different names. In 2010, the WHO classified these tumors as a variant of PDAC under the heading of "undifferentiated carcinoma with osteoclast-like giant cells". Here we describe the first case of pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) composed of UCOGC and pancreatic neuroendocrine tumor (NET), which occurred in a 78-year-old man with biliary colic and pancreatitis. The mass did not respond to the chemotherapy, and he soon developed liver metastasis from the NET component, and unfortunately, the patient passed away 10 months later. Since UCOGC is extremely rare, and its association with NET has not been reported yet, our case expands the knowledge regarding its unusual presentation and poor prognosis.

摘要

伴有破骨细胞样巨细胞的胰腺未分化癌(UCOGC)是胰腺导管腺癌(PDAC)一种极为罕见的形态学和临床特征均独特的变异型,其特征性成分是反应性破骨细胞样巨细胞与肿瘤性单核细胞混合存在。1954年,索默斯和迈斯纳首次将其描述为“胰腺罕见癌”。后来它有了许多不同的名称。2010年,世界卫生组织将这些肿瘤归类为PDAC的一种变异型,归在“伴有破骨细胞样巨细胞的未分化癌”名下。在此,我们报告首例由UCOGC和胰腺神经内分泌肿瘤(NET)组成的胰腺混合性神经内分泌-非神经内分泌肿瘤(MiNEN),该病例发生在一名患有胆绞痛和胰腺炎的78岁男性身上。肿块对化疗无反应,患者很快出现了NET成分的肝转移,不幸的是,患者在10个月后去世。由于UCOGC极为罕见,且其与NET的关联尚未见报道,我们的病例拓展了对其异常表现和不良预后的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/87c0eb7457ee/autopsy-11-e2020201-gf05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/d76588cdd24f/autopsy-11-e2020201-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/91312126ce8c/autopsy-11-e2020201-gf02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/4b8725963d29/autopsy-11-e2020201-gf03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/52c3f2d8379f/autopsy-11-e2020201-gf04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/87c0eb7457ee/autopsy-11-e2020201-gf05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/d76588cdd24f/autopsy-11-e2020201-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/91312126ce8c/autopsy-11-e2020201-gf02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/4b8725963d29/autopsy-11-e2020201-gf03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/52c3f2d8379f/autopsy-11-e2020201-gf04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0047/8101651/87c0eb7457ee/autopsy-11-e2020201-gf05.jpg

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