Expression of Extracellular Signal-regulated Kinase 5 and Ankyrin Repeat Domain 1 in Composite Pheochromocytoma and Ganglioneuroblastoma Detected Incidentally in the Adult Adrenal Gland

• Published in: Internal Medicine Vol. 55; no. 24; pp. 3611 - 3621
• Main Authors: Suenaga, Shinta, Ichiyanagi, Osamu, Ito, Hiromi, Naito, Sei, Kato, Tomoyuki, Nagaoka, Akira, Kato, Tomoya, Yamakawa, Mitsunori, Obara, Yutaro, Tsuchiya, Norihiko
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2016
• Subjects: adrenal; Adrenal Gland Neoplasms - metabolism; Adrenal Gland Neoplasms - pathology; Adrenal Gland Neoplasms - surgery; Adrenal Medulla - diagnostic imaging; Adrenal Medulla - metabolism; Adrenal Medulla - pathology; Adrenalectomy; ankyrin repeat domain 1; Case Report; composite pheochromocytoma; extracellular signal-regulated kinase 5; Female; Follow-Up Studies; ganglioneuroblastoma; Ganglioneuroblastoma - metabolism; Ganglioneuroblastoma - pathology; Ganglioneuroblastoma - surgery; Humans; Incidental Findings; Middle Aged; Mitogen-Activated Protein Kinase 7 - metabolism; Muscle Proteins - metabolism; Nuclear Proteins - metabolism; Pheochromocytoma - metabolism; Pheochromocytoma - pathology; Pheochromocytoma - surgery; Repressor Proteins - metabolism; Signal Transduction; Succinate Dehydrogenase - genetics; Treatment Outcome
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.55.7293

Composite pheochromocytoma (cPC) is extremely rare, arising in the adrenal medulla as a mixture of PC and other tumors of neural origin. We herein report on a case of adrenal incidentaloma post-operatively diagnosed as cPC with ganglioneuroblastoma (GNBL). The PC component had 7 points on the PASS, a Ki-67 index of 5.1%, a focal absence of sustentacular cells, and no genetic aberrations in succinate dehydrogenase subunit B. The GNBL component exhibited no N-myc amplification. Tumor cells of both components were stained positively for extracellular signal-regulated kinase 5 and ankyrin repeat domain 1. The aberrant activation of growth signaling may play a role in the marginal malignancy of cPC.