Mixed gangliocytoma–pituitary adenoma in MEN1 syndrome: A case report and literature review. Issue 2 (8th February 2022)

• Record Type: Journal Article
• Authors: Martin, Elaine C.; Bitner, Benjamin F.; Perez‐Rosendahl, Mari; Kuan, Edward C.; Linskey, Mark E.
• Is Part Of: Neuropathology. Volume 42:Issue 2(2022)
• Page Start: 155
• Page End: 159
• Publication Date: 2022-02-08
• Subjects: endoscopic pituitary surgery -- gangliocytoma -- MEN1 -- pituitary adenoma -- pituitary neoplasms; Nervous system -- Diseases -- Periodicals; Nervous system -- Pathophysiology -- Periodicals; 616.8047
• DOI: 10.1111/neup.12788
• Languages: English
• ISSNs: 0919-6544

Abstract

Abstract : Pituitary adenoma is one of the three most common neoplasms described in multiple endocrine neoplasia type 1 (MEN1), and patients with pituitary adenoma occupies 30‐50% of those with MEN1‐related tumor. Mixed gangliocytoma–pituitary adenoma (MGPA) is a rare clinical entity in which gangliomatous cells are intermixed with adenomatous cells. This tumor has been estimated to account for 0.52–1.26% of all pituitary tumors. We report a rare case of MGPA in a patient with MEN1. A retrospective chart review was conducted on a patient with MEN1 diagnosed with MGPA in 2019 at a single tertiary academic medical center. A review of the literature was performed on MGPA and pituitary adenoma in MEN1. MGPA is rare, with only 174 cases previously reported in the literature and only three prior case reported in a patient with MEN1. There are multiple hypotheses regarding their pathogenesis, and it is unclear whether the MEN1 gene ( menin ) plays a role in the pathogenesis of MGPA. This tumor in MEN1 is a rare clinical entity of unknown etiology. Further studies are required with difficulty due to its low incidence.