Department of Molecular Medicine, University of Pavia and Pathology Unit, IRCCS San Matteo Hospital, Pavia, Italy.
Service of Clinical Pathology, Institute of Pathology, Lausanne University Hospital, Lausanne, Switzerland.
Neuroendocrinology. 2018;107(2):114-126. doi: 10.1159/000489902. Epub 2018 Jun 12.
Gastric neuroendocrine neoplasms (NENs) are very heterogeneous, ranging from mostly indolent, atrophic gastritis-associated, type I neuroendocrine tumors (NETs), through highly malignant, poorly differentiated neuroendocrine carcinomas (pdNECs), to sporadic type III NETs with intermediate prognosis, and various rare tumor types. Histologic differentiation, proliferative grade, size, level of gastric wall invasion, and local or distant metastases are used as prognostic markers. However, their value remains to be tailored to specific gastric NENs.
Series of type I NETs (n = 123 cases), type III NETs (n = 34 cases), and pdNECs (n = 43 cases) were retrospectively collected from four pathology centers specializing in endocrine pathology. All cases were characterized clinically and histopathologically. During follow-up (median 93 months) data were recorded to assess disease-specific patient survival.
Type I NETs, type III NETs, and pdNECs differed markedly in terms of tumor size, grade, invasive and metastatic power, as well as patient outcome. Size was used to stratify type I NETs into subgroups with significantly different invasive and metastatic behavior. All 70 type I NETs < 0.5 cm (micro-NETs) were uneventful. Ki67-based grading proved efficient for the prognostic stratification of type III NETs; however, grade 2 (G2) was not associated with tumor behavior in type I NETs. Although G3 NETs (2 type I and 9 type III) had a very poor prognosis, it was found that patient survival was longer with type III G3 NETs compared to pdNECs.
Given the marked, tumor type-related behavior differences, evaluation of gastric NEN prognostic parameters should be tailored to the type of neoplastic disease.
胃神经内分泌肿瘤（NENs）具有高度异质性，范围从主要为惰性、萎缩性胃炎相关的 I 型神经内分泌肿瘤（NETs），到高度恶性、低分化的神经内分泌癌（pdNECs），再到具有中间预后的散发性 III 型 NETs，以及各种罕见的肿瘤类型。组织学分化、增殖分级、大小、胃壁侵犯程度、局部或远处转移均被用作预后标志物。然而，它们的价值仍需针对特定的胃 NENs 进行调整。
从四个专门从事内分泌病理学的病理中心回顾性收集了一系列 I 型 NETs（n=123 例）、III 型 NETs（n=34 例）和 pdNECs（n=43 例）。所有病例均进行了临床和组织病理学特征描述。在随访期间（中位时间为 93 个月）记录数据以评估疾病特异性患者生存率。
I 型 NETs、III 型 NETs 和 pdNECs 在肿瘤大小、分级、侵袭和转移能力以及患者预后方面存在显著差异。大小用于将 I 型 NETs 分为具有显著不同侵袭和转移行为的亚组。所有 70 例大小<0.5cm（微 NETs）的 I 型 NETs 均无进展。基于 Ki67 的分级对于 III 型 NETs 的预后分层非常有效；然而，I 型 NETs 中 G2 级与肿瘤行为无关。尽管 G3 NETs（2 例 I 型和 9 例 III 型）预后极差，但发现与 pdNECs 相比，III 型 G3 NETs 的患者生存时间更长。
鉴于明显的、与肿瘤类型相关的行为差异，评估胃 NEN 预后参数应针对肿瘤疾病的类型进行调整。
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