Unusual mixed gangliocytoma-pituitary adenoma in sellar region

Chinese Journal of Contemporary Neurology and Neurosurgery

Jie-tian JIN¹  Dan-feng YU¹  Zhi LI¹  Zhi-yun YANG² 

[1] Department of Pathology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong, China;Department of Radiology, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Guangdong, China;

关键词: Ganglioneuroma;    Growth hormone-secreting pituitary adenoma;    Sella turcica;    Immunohistochemistry;    Pathology;   

DOI  :  

来源: DOAJ

【 摘 要 】

Background The presence of ganglion cells within anendocrine pituitary adenoma in sellar region is rare, and is usually diagnosedas "mixed gangliocytoma-pituitary adenoma". Due to lack of radiologicalcharacteristics, it is very difficult to make an accurate diagnosispreoperatively. Herein we describe one case of unusual mixed gangliocytoma -growth hormone (GH) secreting pituitary adenoma in sellar region and reviewrelated literatures, so as to summarize the clinicopathological characteristicsand improve the diagnosis and differential diagnosis of this tumor.Methods and Results A 28 - year - old female presented withheadache and blurred vision for 8 months. She also complained of acromegaly andamenorrhea. Head CT and MRI examinations showed a sellar and suprasellar masswith clear boundary compressing the optic chiasm and buttom of the thirdventricle. The mass exhibited isointense signal or mild hypointensity onT₁WI and mild hyperintensity on T₂WI with heterogeneousenhancement on the contrast MRI. The tumor was removed totally. The histologicalsections demonstrated two parts of intermixed areas. One part of areas wasmarked by a proliferation of scattered gangliocyte - like cells arranged in afibrillary background. Other areas were marked by a sheet - like or locallypapillary proliferation of round and oval cells. Immunohistochemically,cytoplasm of gangliocyte-cells were diffusely positive for synaptophysin (Syn),and negative for adenohypophysial hormones; cytoplasm of round and oval cellswere diffusely positive for Syn, and almost 30% cells were positive for GH, andnegative for other neurohypophysial hormones. A final diagnosis of mixedgangliocytoma-GH secreting pituitary adenoma in sellar region (WHO grade Ⅰ) wasmade. The patient did not receive postoperatively adjuvant therapy and wasfollowed-up for one year, without any neurological deficit or signs ofrecurrence. Conclusions Mixed gangliocytoma - pituitary adenomais extremely rare and a definite diagnosis should be made under microscopyexamination. Since the histogenesis of this tumor suggests that the uncommittedstem/progenitor cells consist of both adenohypophysial and neuronalcharacteristics and are capable of giving rise to pituitary adenoma withneuronal component, a diagnostic term of "pituitary adenoma with ganglionicdifferentiation" is suggested for this independent entity rather than collisiontumor combined by two separate tumors.

 

DOI: 10.3969/j.issn.1672-6731.2016.10.011

【 授权许可】

Unknown