Rindi G, Ubiali A, Villanacci V
Department of Pathology and Laboratory Medicine, University of Parma, Via Gramsci 14, I-43100 Parma, Italy.
Dig Liver Dis. 2004 Feb;36 Suppl 1:S26-30. doi: 10.1016/j.dld.2003.11.010.
Endocrine tumours of gut and pancreas tract are rare entities originating from cells of the diffuse endocrine system. The endocrine phenotype is assessed by the expression of general and specific endocrine markers. General endocrine markers associate to organelles like large dense core vesicles (e.g. chromogranin A) and small synaptic-like vesicles (e.g. synaptophysin), or to the cytosol, like neuron specific enolase and protein gene product 9.5 (PGP9.5). The specific markers correspond to the hormones produced by tumour cells. Two major categories of endocrine tumours are identified as (i) well-differentiated and (ii) poorly differentiated neoplasms. Well-differentiated tumours/carcinomas (also known as carcinoids) express all general markers of endocrine differentiation and various hormones. Poorly differentiated endocrine carcinomas lack large dense core vesicles markers (chromogranin A), while widely express synaptophysin and cytosol endocrine markers. The clinical behaviour of endocrine tumours spans from benign to low-grade malignant for well-differentiated tumours/carcinomas to high grade malignant for poorly differentiated carcinomas. The Multiple Endocrine Neoplasia type 1 syndrome (MEN1) gene is involved in the genesis of a proportion of both well- and poorly differentiated sporadic tumours. p53 gene abnormality appears as restricted to poorly differentiated endocrine carcinomas.
胃肠道和胰腺的内分泌肿瘤是起源于弥漫性内分泌系统细胞的罕见实体。内分泌表型通过一般和特异性内分泌标志物的表达来评估。一般内分泌标志物与细胞器相关，如大的致密核心囊泡（如嗜铬粒蛋白A）和小的突触样囊泡（如突触素），或与胞质溶胶相关，如神经元特异性烯醇化酶和蛋白基因产物9.5（PGP9.5）。特异性标志物对应于肿瘤细胞产生的激素。内分泌肿瘤主要分为两类：（i）高分化肿瘤和（ii）低分化肿瘤。高分化肿瘤/癌（也称为类癌）表达内分泌分化的所有一般标志物和各种激素。低分化内分泌癌缺乏大的致密核心囊泡标志物（嗜铬粒蛋白A），而广泛表达突触素和胞质溶胶内分泌标志物。内分泌肿瘤的临床行为范围从高分化肿瘤/癌的良性到低级别恶性，再到低分化癌的高级别恶性。多发性内分泌腺瘤1型综合征（MEN1）基因参与了一部分高分化和低分化散发性肿瘤的发生。p53基因异常似乎仅限于低分化内分泌癌。
