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Pheochromocytoma Developed in a Boy with Multiple Endocrine Neoplasia Type 2A Confirmed by the RET Proto-oncogene Mutation

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Author(s)
So Yun ParkMin Ji JinEun Mi ChoiSeok Jin KangJin Hyeok ChoiYe Jee ShimHeung Sik KimEun Young JungHee Jung LeeMi Sun ChoiHye Won Kim
Keimyung Author(s)
Choi, Jin HyeokKim, Heung SikShim, Ye JeeJung, Eun YoungLee, Hee JungChoe, Mi SunKim, Hae Won
Department
Dept. of Pediatrics (소아청소년학)
Dept. of Surgery (외과학)
Dept. of Radiology (영상의학)
Dept. of Pathology (병리학)
Dept. of Nuclear Medicine (핵의학)
Journal Title
Clinical Pediatric Hematology-Oncology
Issued Date
2017
Volume
24
Keyword
ChildMultiple endocrine neoplasiaRET proto-oncogenePheochromocytoma
Abstract
A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.
Alternative Title
RET 유전자변이로 확진된 제2A형 다발성내분비샘종 남자 환자에서 발병한 크롬모세포종
Keimyung Author(s)(Kor)
최진혁
심예지
김흥식
정은영
이희정
최미선
김해원
Publisher
School of Medicine
Citation
So Yun Park et al. (2017). Pheochromocytoma Developed in a Boy with Multiple Endocrine Neoplasia Type 2A Confirmed by the RET Proto-oncogene Mutation. Clinical Pediatric Hematology-Oncology, 24, 75–79. doi: 10.15264/cpho.2017.24.1.75
Type
Article
ISSN
2233-5250
DOI
10.15264/cpho.2017.24.1.75
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/32389
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Nuclear Medicine (핵의학)
1. School of Medicine (의과대학) > Dept. of Pathology (병리학)
1. School of Medicine (의과대학) > Dept. of Pediatrics (소아청소년학)
1. School of Medicine (의과대학) > Dept. of Radiology (영상의학)
1. School of Medicine (의과대학) > Dept. of Surgery (외과학)
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