多发性内分泌瘤(MEN)Ⅱb综合征的完整临床表现包括嗜铬细胞瘤、甲状腺髓癌、粘膜神经瘤和顽固性便秘,类似巨结肠(Hirshsprung病)的临床和X线表现并不少见。但与该病的无神经节不同,肠肌神经丛大片增生,故被称为神经节细胞瘤病,发生机理还不清楚。本文报告的病变超微结构有不典型“侵入”型神经增殖,同时有相对多的充满小颗粒的神经末梢,表明有一个神 The complete clinical manifestations of multiple endocrine tumor (MEN) IIb syndrome include pheochromocytoma, thyroid medullary cancer, muscarebral neuroma, and refractory constipation. The clinical and X-ray findings of Hirshsprung’s disease are not uncommon. However, unlike the disease’s absence of ganglion, the intestinal muscle plexus is hyperplastic, so it is called gangliocytoma and its mechanism is unclear. The ultrastructure of the lesion reported in this article has an atypical “invasive” type of neural proliferation, while there are relatively many nerve endings filled with small particles, suggesting that there is a god