Mixed gangliocytoma-pituitary adenoma containing GH and GHRH co-secreting adenoma cells. (3rd October 2019)

• Record Type: Journal Article
• Authors: Teramoto, Shinichiro; Tange, Yuichi; Ishii, Hisato; Goto, Hiromasa; Ogino, Ikuko; Arai, Hajime
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2019)
• Publication Date: 2019-10-03
• Subjects: Adult -- Female -- Asian - Japanese -- Japan; Pituitary -- Pituitary -- GHRH -- IGF1 -- GH -- Pituitary adenoma -- Gangliocytoma* -- Diabetes mellitus type 2 -- Acromegaly; Diabetes mellitus type 2 -- Macroglossia -- Face - coarse features -- Feet - large -- Hands - large -- GH -- GHRH -- MRI -- IGF1 -- Glucose tolerance (oral) -- Histopathology -- Haematoxylin and eosin staining -- Immunohistochemistry -- Immunostaining -- Microtubule-associated protein 2* -- Transsphenoidal surgery -- Resection of tumour; Insight into disease pathogenesis or mechanism of therapy -- October -- 2019; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-19-0099
• Languages: English
• ISSNs: 2052-0573

Abstract

Abstract : Summary: A 67-year-old woman with a past history of type 2 diabetes mellitus presented with worsening glycemic control. She had some acromegaly symptoms and magnetic resonance imaging demonstrated a pituitary tumor. Endocrinological examination found the resting growth hormone (GH) level within the normal range, but elevated insulin-like growth factor 1 level. A 75 g oral glucose tolerance test showed inadequate suppression of nadir GH levels. Acromegaly due to GH-secreting pituitary tumor was diagnosed. The patient underwent endoscopic transsphenoidal surgery resulting in gross total removal of the tumor and recovered well postoperatively. Histological examination of the tumor showed coexistence of relatively large gangliocytoma cells and pituitary adenoma cells, suggesting mixed gangliocytoma-pituitary adenoma. In addition, colocalization of GH and GH-releasing hormone (GHRH) in pituitary adenoma cells was revealed, so the adenomatous components were more likely to produce GHRH in our mixed gangliocytoma-pituitary adenoma case. Mixed gangliocytoma-pituitary adenoma is very rare, and the present unique case demonstrated only the adenomatous components associated with GHRH production. Learning points: Sellar gangliocytoma coexisting with pituitary adenoma is recognized as a mixed gangliocytoma-pituitary adenoma and is very rare. A proposed developmental mechanism of growth hormone (GH)-secreting mixed gangliocytoma-pituitary adenoma involves GH-releasing hormone (GHRH) produced by the gangliocytic components promoting the growth of tumor including GH-secreting adenomatous components. Since our present case indicated that the adenomatous components of mixed gangliocytoma-pituitary adenoma could secrete both GH and GHRH simultaneously, progression of GH-secreting mixed gangliocytoma and pituitary adenoma may involve exposure to spontaneously produced GHRH due to the adenomatous components.