Solcia E, Fiocca R, Rindi G, Villani L, Cornaggia M, Capella C
University of Pavia, Italy.
Endocrinol Metab Clin North Am. 1993 Dec;22(4):795-821.
Among endocrine tumors occurring in the gastrointestinal tract, midgut argentaffin EC cell carcinoids, gastric argyrophil ECL cell carcinoids, duodenal gastrin cell tumors, and rectal trabecular L cell carcinoids (in order of decreasing frequency) are those occurring more frequently. Together, they account for more than 80% of such tumors. Duodenal somatostatin cell tumors, gangliocytic paragangliomas, and differentiated neuroendocrine carcinomas are also well-defined tumor entities. The carcinoid syndrome, either classical, with intermittent flushing, hypotension, and diarrhea, or atypical, with persistent histamine-type red flushing, bronchospasm, and no diarrhea, and Zollinger-Ellison syndrome, with severe peptide ulcer disease, are the only hyperfunctional syndromes consistently found in association with these tumors. The carcinoid syndrome occurs in about 10% of gastrointestinal carcinoids, usually in their advanced, metastatic stage. The Zollinger-Ellison syndrome occurs in association with about 40% of intestinal gastrin cell tumors, including small intramural growths. Tumor prognosis depends on the mode and site of presentation, histology, cell type(s), size, level of invasion, metastases (especially distant metastases), and associated clinical syndrome or background disease. Hormones, trophic factors, inherited genetic traits, somatic mutations, and some chronic inflammatory processes are pathogenetically important in a large proportion of cases.
在发生于胃肠道的内分泌肿瘤中，中肠嗜银EC细胞类癌、胃嗜银ECL细胞类癌、十二指肠胃泌素细胞瘤和直肠小梁L细胞类癌（按发生频率递减顺序排列）较为常见。它们合计占此类肿瘤的80%以上。十二指肠生长抑素细胞瘤、神经节细胞副神经节瘤和分化型神经内分泌癌也是明确的肿瘤实体。类癌综合征，无论是典型的，表现为间歇性潮红、低血压和腹泻，还是非典型的，表现为持续性组胺型红潮、支气管痉挛且无腹泻，以及佐林格-埃利森综合征，伴有严重的肽性溃疡病，是与这些肿瘤始终相关的仅有的高功能综合征。类癌综合征见于约10%的胃肠道类癌，通常处于晚期转移阶段。佐林格-埃利森综合征见于约40%的肠道胃泌素细胞瘤，包括壁内小肿瘤。肿瘤预后取决于呈现方式和部位、组织学、细胞类型、大小、浸润程度、转移情况（尤其是远处转移）以及相关的临床综合征或背景疾病。在很大一部分病例中，激素、营养因子、遗传性状、体细胞突变和一些慢性炎症过程在发病机制上具有重要意义。
