鞍区混合性神经节细胞瘤-垂体腺瘤3例临床病理分析

Mixed gangliocytoma-adenoma in sellar region: a clinicopathological analysis of 3 cases

目的探讨鞍区混合性神经节细胞瘤-垂体腺瘤的临床病理特征及发病机制。方法分析3例混合性神经节细胞瘤-垂体腺瘤的临床资料、病理组织学特点、免疫表型及预后,并进行该疾病相关文献复习。结果 1例为男性,2例为女性,年龄38~59岁。1例伴有生长激素水平增高及肢端肥大症,1例伴催乳素水平升高但无其他临床症状,另1例无激素水平改变但伴有反复低钠。3例病理组织学均为混合性生长激素细胞腺瘤与神经节细胞瘤,其中2例为稀疏颗粒型。垂体腺瘤垂体特异性转录因子(Pit-1)及GH(+),神经节细胞区域除了Neu N和Calretinin(CR)(+),部分还Pit-1及CK18(+)。3例随访7~90个月,均无复发。结论神经节细胞瘤常与稀疏颗粒型生长激素腺瘤并发,常有肢端肥大症或无临床症状。更多实验结果支持生长激素腺瘤发生神经元转化是其发生机制,有待进一步研究;其预后与单纯垂体腺瘤相同。

Objective To investigate the clinicopathological features and pathogenesis of mixed gangliocytoma-adenoma in sellar region. Methods Clinical data,histological features,immunohistochemical findings,and follow-up data were analyzed in three cases of mixed gangliocytoma-adenoma. The cases were collected from 2010 to 2019 at Liaocheng People’s Hospital. Literature review was performed. Results One case was male and two were female. The age of the patients ranged from 38 to 59 years. One patient had increased growth hormone levels and acromegaly,one had elevated prolactin levels but no clinical symptoms,and the other had no hormone changes but recurrent low sodium. The histopathology of 3 cases were mixed growth of somatotroph adenoma and ganglioma,2 of them were of sparsely granulated type. The expression of pituitary specific transcription factor-1(Pit-1) and GH was positive in pituitary adenomas,while Neu N and calretinin(CR) were positive in ganglion cells,and Pit-1 and CK18(cyclokeratin18) were also positive in some areas. All the three cases were followed up for 7-90 months without recurrence. Conclusion Gangliocytoma is often complicated with sparsely granulated somatotroph adenoma,often with acromegaly or no clinical symptoms. More experimental results support that the mechanism is neuronal transformation of growth hormone adenoma,which needs further study. The prognosis is the same as that of simple pituitary adenoma.