颅外异位垂体腺瘤3例临床病理观察

Extracranial ectopic pituitary adenoma: clinicopathologic analyses of three cases

目的探讨颅外异位垂体腺瘤(EPA)的临床病理特点。方法搜集并分析3例EPA的临床病理、免疫组化及电镜特点。结果 3例均为男性,平均年龄50岁;2例位于鼻腔,1例位于鼻咽顶正中。镜下见形态单一的肿瘤细胞排列呈实性或器官样、梁索状,瘤细胞巢间分布纤细血管,核多形性、核分裂象及坏死罕见。3例肿瘤细胞Syn、Cg A和CD56均强(+),2例分别为ACTH、TSH(+)。结论 EPA是一种罕见的预后良好的肿瘤,结合临床(特异内分泌症状)、影像学(蝶鞍外中线部位界清肿块)及病理组织学(神经内分泌及垂体激素标记物阳性)可确诊,手术完全切除可治愈,不能全切者术后辅以放疗或药物治疗。

Objective To investigate the clinicopathological characteristics of extracranial ectopic pituitary adenoma.Methods Three cases of extracranial ectopic pituitary adenoma were analyzed by light microscopy and immunohistochemical staining,electronic microscopy. Results Three cases were all male,with average age of 50 years. Two cases were found in nasal cavity,one case in the midline roof of nasopharynx. Microscopically,tumor cells arranged in packets,organoid,ribbons,with prominent delicate vascularized stroma. Nuclear pleomorphism,mitoses,and necrosis were uncommon. Immunohistochemical staining showed that 3 cases were strongly positive expression of Syn,Cg A and CD56; 2 cases expressed ACTH and TSH respectively. Conclusions Extracranial ectopic pituitary adenoma is a rare benign tumor with good prognosis. The final diagnosis depends on combined application of clinical symptoms（ specific endocrinological disorder）,imaging examinations（ a normal sella turcica with distinct midline tumour）,histology and immunohistochemical markers（ neuroendocrine markers and pituitary hormones）. Radically surgical resection is curative,adjuvant radiotherapy and chemotherapy can be helpful if the tumor is large or incompletely excised.