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- Shinta Suenaga, Osamu Ichiyanagi, Hiromi Ito, Sei Naito, Tomoyuki Kato, Akira Nagaoka, Tomoya Kato, Mitsunori Yamakawa, Yutaro Obara, and Norihiko Tsuchiya.
- Department of Urology, Yamagata University Faculty of Medicine, Japan.
- Intern. Med. 2016 Jan 1; 55 (24): 361136213611-3621.
AbstractComposite 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.
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