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

Shinta Suenaga; Osamu Ichiyanagi; Hiromi Ito; Sei Naito; Tomoyuki Kato; Akira Nagaoka; Tomoya Kato; Mitsunori Yamakawa; Yutaro Obara; Norihiko Tsuchiya

doi:10.2169/internalmedicine.55.7293

CASE REPORTS

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

Shinta Suenaga, Osamu Ichiyanagi, Hiromi Ito, Sei Naito, Tomoyuki Kato, Akira Nagaoka, Tomoya Kato, Mitsunori Yamakawa, Yutaro Obara, Norihiko Tsuchiya

Author information

Shinta Suenaga
Department of Urology, Yamagata University Faculty of Medicine, Japan
Osamu Ichiyanagi
Department of Urology, Yamagata University Faculty of Medicine, Japan
Hiromi Ito
Department of Urology, Yamagata University Faculty of Medicine, Japan
Sei Naito
Department of Urology, Yamagata University Faculty of Medicine, Japan
Tomoyuki Kato
Department of Urology, Yamagata University Faculty of Medicine, Japan
Akira Nagaoka
Department of Urology, Yamagata University Faculty of Medicine, Japan
Tomoya Kato
Department of Pathological Diagnostics, Yamagata University Faculty of Medicine, Japan
Mitsunori Yamakawa
Department of Pathological Diagnostics, Yamagata University Faculty of Medicine, Japan
Yutaro Obara
Department of Pharmacology, Yamagata University Faculty of Medicine, Japan
Norihiko Tsuchiya
Department of Urology, Yamagata University Faculty of Medicine, Japan

Keywords: adrenal, composite pheochromocytoma, ganglioneuroblastoma, extracellular signal-regulated kinase 5, ankyrin repeat domain 1

JOURNAL OPEN ACCESS
Supplementary material

2016 Volume 55 Issue 24 Pages 3611-3621

DOI https://doi.org/10.2169/internalmedicine.55.7293

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Abstract

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.

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