Journal
INTERNAL MEDICINE
Volume 55, Issue 24, Pages 3611-3621Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.55.7293
Keywords
adrenal; composite pheochromocytoma; ganglioneuroblastoma; extracellular signal-regulated kinase 5; ankyrin repeat domain 1
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Funding
- Japan Society for the Promotion of Science [15K07963]
- Takeda Science Foundation
- Grants-in-Aid for Scientific Research [15K07963] Funding Source: KAKEN
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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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