Torrisi A, Carillio G, Libra M, Lanzafame S
Dipartimento di Anatomia, Patologia Diagnostica, Medicina Legale e Sanità Pubblica G.F. Ingrassia, Sezione di Anatomia e Istologia Patologica, Dipartimento di Scienze Biomediche, Università di Catania.
Pathologica. 2003 Aug;95(4):192-5.
Ganglioneuromas may occur in the small and large intestine as either solitary lesions or, more commonly, as multiple lesions (ganglioneuromatosis). The former are very rare, whereas ganglioneuromatosis may be associated with von Recklinghausen's disease and multiple endocrine neoplasia (MEN) type II B. We described the clinicopathologic features of a case of solitary polypoid ganglioneuroma of the ileocecal valve. The lesion was endoscopically diagnosed in a 27 year old man, with a history of abdominal pain. No association with von Reckling-hausen's disease or MEN was identified. Mutational analysis for RET was negative. Microscopically, the tumor consisted of a proliferation of well differentiated Schwann cells and ganglion cells in the lamina propria. The solitary polipoid ganglioneuroma is invariably benign. It shows no evidence of recurrence after total excision.
神经节瘤可发生于小肠和大肠，表现为孤立性病变，或更常见的是多发性病变（神经节瘤病）。前者非常罕见，而神经节瘤病可能与冯·雷克林豪森病和II B型多发性内分泌肿瘤（MEN）有关。我们描述了一例回盲瓣孤立性息肉样神经节瘤的临床病理特征。该病变在一名有腹痛病史的27岁男性中经内镜诊断。未发现与冯·雷克林豪森病或MEN有关。RET基因的突变分析为阴性。显微镜下，肿瘤由固有层中分化良好的施万细胞和神经节细胞增生组成。孤立性息肉样神经节瘤总是良性的。全切除后无复发迹象。
