Fuller C E, Williams G T
Department of Pathology, University of Wales College of Medicine, Cardiff, UK.
Histopathology. 1991 Jul;19(1):1-11. doi: 10.1111/j.1365-2559.1991.tb00888.x.
Gastrointestinal involvement in von Recklinghausen's disease occurs in three principal forms: hyperplasia of the submucosal and myenteric nerve plexuses and mucosal ganglioneuromatosis which leads to disordered gut motility; gastrointestinal stromal tumours showing varying degrees of neural or smooth muscle differentiation; and a distinctive glandular, somatostatin-rich carcinoid of the periampullary region of the duodenum that contains psammoma bodies and which may be associated with phaeochromocytoma. This review describes the histopathological features of these lesions and discusses potential pitfalls in their differential diagnosis. Their accurate identification has significant implications for clinical management and may even provide the first pointer to the diagnosis of neurofibromatosis.
黏膜下和肌间神经丛增生以及黏膜神经节瘤病，可导致肠道运动紊乱；胃肠道间质瘤，表现出不同程度的神经或平滑肌分化；十二指肠壶腹周围区域一种独特的富含生长抑素的腺性类癌，含有砂粒体，可能与嗜铬细胞瘤有关。本文综述了这些病变的组织病理学特征，并讨论了鉴别诊断中的潜在陷阱。准确识别它们对临床管理具有重要意义，甚至可能为神经纤维瘤病的诊断提供首个线索。
