Unusual mixed gangliocytoma-pituitary adenoma in sellar region

• Main Authors: Jie-tian JIN, Dan-feng YU, Zhi-yun YANG, Zhi LI
• Format: Article
• Language: English
• Published: Tianjin Huanhu Hospital 2016-10-01
• Series: Chinese Journal of Contemporary Neurology and Neurosurgery
• Subjects: Ganglioneuroma; Growth hormone-secreting pituitary adenoma; Sella turcica; Immunohistochemistry; Pathology
• Online Access: http://www.cjcnn.org/index.php/cjcnn/article/view/1489

<p><strong>Background</strong> The presence of ganglion cells within an endocrine pituitary adenoma in sellar region is rare, and is usually diagnosed as "mixed gangliocytoma-pituitary adenoma". Due to lack of radiological characteristics, it is very difficult to make an accurate diagnosis preoperatively. Herein we describe one case of unusual mixed gangliocytoma - growth hormone (GH) secreting pituitary adenoma in sellar region and review related literatures, so as to summarize the clinicopathological characteristics and improve the diagnosis and differential diagnosis of this tumor. <strong>Methods and Results</strong> A 28 - year - old female presented with headache and blurred vision for 8 months. She also complained of acromegaly and amenorrhea. Head CT and MRI examinations showed a sellar and suprasellar mass with clear boundary compressing the optic chiasm and buttom of the third ventricle. The mass exhibited isointense signal or mild hypointensity on T₁WI and mild hyperintensity on T₂WI with heterogeneous enhancement on the contrast MRI. The tumor was removed totally. The histological sections demonstrated two parts of intermixed areas. One part of areas was marked by a proliferation of scattered gangliocyte - like cells arranged in a fibrillary background. Other areas were marked by a sheet - like or locally papillary 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 cells were diffusely positive for Syn, and almost 30% cells were positive for GH, and negative for other neurohypophysial hormones. A final diagnosis of mixed gangliocytoma-GH secreting pituitary adenoma in sellar region (WHO grade Ⅰ) was made. The patient did not receive postoperatively adjuvant therapy and was followed-up for one year, without any neurological deficit or signs of recurrence. <strong>Conclusions</strong> Mixed gangliocytoma - pituitary adenoma is extremely rare and a definite diagnosis should be made under microscopy examination. Since the histogenesis of this tumor suggests that the uncommitted stem/progenitor cells consist of both adenohypophysial and neuronal characteristics and are capable of giving rise to pituitary adenoma with neuronal component, a diagnostic term of "pituitary adenoma with ganglionic differentiation" is suggested for this independent entity rather than collision tumor combined by two separate tumors.</p><p> </p><p><strong>DOI: </strong>10.3969/j.issn.1672-6731.2016.10.011</p>