混合性节细胞瘤/垂体腺瘤4例临床病理观察

Mixed gangliocytoma/pituitary adenoma:a clinicopathologic study of four cases and review of literature

目的探讨混合性节细胞瘤/垂体腺瘤发生机制、临床病理特征、诊断及鉴别诊断。方法复习4例混合性节细胞瘤/垂体腺瘤患者的临床资料,并对其进行组织学观察和免疫组化(EnVisionplus法)标记。结果4例混合性节细胞瘤/垂体腺瘤患者均为女性,年龄10～35岁,平均26.5岁。临床上3例表现内分泌症状,1例有癫痫症状。组织学上肿瘤由两种不同成分组成,一种结构主要由较多神经节细胞组成,节细胞体积大,可见圆形的尼氏小体,核大,核仁突出,免疫组化标记瘤细胞表达CgA、Syn、S100蛋白,不表达GFAP;另一部分腺瘤细胞大小形态较一致,圆形或卵圆形,胞质丰富,嗜酸性或空淡,间质血窦丰富,瘤细胞表达GH和PRL。结论混合性节细胞瘤/垂体腺瘤是蝶鞍区极少见的肿瘤,好发于青年女性,常合并肢端肥大症。该瘤的诊断主要依靠组织病理学,并辅以免疫组化标记。治疗主要采用单纯手术切除,预后较好。

Purpose To investigate the pathogenesis, clinicopathological characteristics, diagnosis and differential diagnosis of the mixed gangliocytoma/pituitary adenoma （MGPA）. Methods Four cases of MGPA were studied with HE and immunohistochemical EnVision-plus staining. Results All patients were female, with age ranged from 10 to 35 years （ mean 26.5 years）. Clinically, three cases were endocrinologically symptomatic and one case was falling sickness. Histopathologically, two distinct neoplastic cell populations were observed in the same tumor mass. The first population was composed of many ganglionic cells showing large nuclei, prominent nucleoli, and Nissl bodies, which were positive for CgA, Syn, S-100 protein and negative for GFAP. The second population was similar cells in sizes and shapes, with round-oval nuclei, abundant and acidophilic or pale cytoplasm, which were immunoreactive for GH and PRL. Conclusions MGPA is a very rare mixed pituitary tumor with good prognosis. It arises mostly on young woman presenting with acromegaly. The diagnosis deoends mainly on HE as well as immunohistochemical staining.