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An analysis of 130 neuroendocrine tumors G3 regarding prevalence, origin, metastasis, and diagnostic features
Virchows Archiv ( IF 3.5 ) Pub Date : 2021-09-09 , DOI: 10.1007/s00428-021-03202-6
Atsuko Kasajima 1, 2 , Björn Konukiewitz 1, 3 , Anna Melissa Schlitter 1, 2 , Wilko Weichert 1, 2 , Günter Klöppel 1
Affiliation  

Limited data exist on high-grade neuroendocrine tumors (NETs G3) which represent a new category among neuroendocrine neoplasms (NEN). We analyzed NETs G3 in a consultation series regarding prevalence, origin, metastasis, and diagnostic problems. Based on the WHO classification of digestive system tumors, 130 NETs G3 (9%) were identified in 1513 NENs. NET G3 samples were more often obtained from metastatic sites (69%) than NET G1/G2 samples (24%). NET G3 metastases presented most frequently in the liver (74%) and originated from the pancreas (38/90, 42%), followed by the lung (9%), ileum (7%), stomach (3%), rectum (1%), and rare sites (2%) such as the prostate and breast. The primaries remained unknown in 15%. NETs G3 had a median Ki67 of 30% that distinguished them from NECs (60%), though with great overlap. The expression of site-specific markers, p53, Rb1, and SST2 was similar in NETs G3 and NETs G1/G2, except for p53 and Rb1 which were abnormally expressed in 8% and 7% of liver metastases from NET G3 but not from NET G1/G2. NETs G3 were frequently referred as NECs (39%) but could be well distinguished from NECs by normal p53 (92% versus 21%) and Rb1 expression (93% versus 41%) expression. In conclusion, NETs G3 are frequently discovered as liver metastases from pancreatic or pulmonary primaries and are often misinterpreted as NEC. p53 and Rb1 are powerful markers in the distinction of NET G3 from NEC. Rarely, carcinomas from non-digestive, non-pulmonary organs with neuroendocrine features may present as NET G3.



中文翻译:

130 种神经内分泌肿瘤 G3 的患病率、起源、转移和诊断特征分析

高级别神经内分泌肿瘤 (NETs G3) 的数据有限,它代表了神经内分泌肿瘤 (NEN) 中的一个新类别。我们在关于患病率、起源、转移和诊断问题的咨询系列中分析了 NETs G3。根据消化系统肿瘤的 WHO 分类,在 1513 个 NEN 中确定了 130 个 NETs G3 (9%)。NET G3 样本(69%)比 NET G1/G2 样本(24%)更常从转移部位获得。NET G3 转移最常见于肝脏 (74%) 并起源于胰腺 (38/90, 42%),其次是肺 (9%)、回肠 (7%)、胃 (3%)、直肠 ( 1%) 和罕见部位 (2%),如前列腺和乳房。15% 的初选仍然未知。NETs G3 的 Ki67 中值为 30%,这将它们与 NECs (60%) 区分开来,尽管有很大的重叠。位点特异性标记的表达,p53、Rb1 和 SST2 在 NETs G3 和 NETs G1/G2 中相似,除了 p53 和 Rb1 在 NET G3 的 8% 和 7% 的肝转移中异常表达,但在 NET G1/G2 中没有。NETs G3 经常被称为 NECs (39%),但可以通过正常的 p53(92% 对 21%)和 Rb1 表达(93% 对 41%)表达与 NECs 很好地区分。总之,NETs G3 经常被发现为胰腺或肺原发灶的肝转移,并且经常被误解为 NEC。p53 和 Rb1 是区分 NET G3 和 NEC 的有力标记。极少数情况下,来自具有神经内分泌特征的非消化性、非肺器官的癌可能表现为 NET G3。除了 p53 和 Rb1 在 8% 和 7% 的 NET G3 肝转移灶中异常表达,但在 NET G1/G2 肝转移灶中没有异常表达。NETs G3 经常被称为 NECs (39%),但可以通过正常的 p53(92% 对 21%)和 Rb1 表达(93% 对 41%)表达与 NECs 很好地区分。总之,NETs G3 经常被发现为胰腺或肺原发灶的肝转移,并且经常被误解为 NEC。p53 和 Rb1 是区分 NET G3 和 NEC 的有力标记。极少数情况下,来自具有神经内分泌特征的非消化性、非肺器官的癌可能表现为 NET G3。除了 p53 和 Rb1 在 8% 和 7% 的 NET G3 肝转移灶中异常表达,但在 NET G1/G2 肝转移灶中没有异常表达。NETs G3 经常被称为 NECs (39%),但可以通过正常的 p53(92% 对 21%)和 Rb1 表达(93% 对 41%)表达与 NECs 很好地区分。总之,NETs G3 经常被发现为胰腺或肺原发灶的肝转移,并且经常被误解为 NEC。p53 和 Rb1 是区分 NET G3 和 NEC 的有力标记。极少数情况下,来自具有神经内分泌特征的非消化性、非肺器官的癌可能表现为 NET G3。NETs G3 经常被发现为胰腺或肺原发灶的肝转移,并且经常被误解为 NEC。p53 和 Rb1 是区分 NET G3 和 NEC 的有力标记。极少数情况下,来自具有神经内分泌特征的非消化性、非肺器官的癌可能表现为 NET G3。NETs G3 经常被发现为胰腺或肺原发灶的肝转移,并且经常被误解为 NEC。p53 和 Rb1 是区分 NET G3 和 NEC 的有力标记。极少数情况下,来自具有神经内分泌特征的非消化性、非肺器官的癌可能表现为 NET G3。

更新日期:2021-09-10
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