Advances in pediatric surgery

Korean Association of Pediatric Surgeons (대한소아외과학회)
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Ganglioneuroblastoma with WDHA Syndrome

WDHA증후군을 동반한 신경절모세포종

  • Kim, Dae-Yeon (Department of Surgery, Seoul National University College of Medicine) ;
  • Kim, Ki-Hong (Department of Surgery, Seoul National University College of Medicine) ;
  • Kim, Sang-Beom (Department of Surgery, Seoul National University College of Medicine) ;
  • Jung, Sung-Eun (Department of Surgery, Seoul National University College of Medicine) ;
  • Lee, Seong-Cheol (Department of Surgery, Seoul National University College of Medicine) ;
  • Park, Kwi-Won (Department of Surgery, Seoul National University College of Medicine) ;
  • Kim, Woo-Ki (Department of Surgery, Seoul National University College of Medicine)
  • 김대연 (서울대학교 의과대학 외과학교실) ;
  • 김기홍 (서울대학교 의과대학 외과학교실) ;
  • 김상범 (서울대학교 의과대학 외과학교실) ;
  • 정성은 (서울대학교 의과대학 외과학교실) ;
  • 이성철 (서울대학교 의과대학 외과학교실) ;
  • 박귀원 (서울대학교 의과대학 외과학교실) ;
  • 김우기 (서울대학교 의과대학 외과학교실)
  • Published : 2000.06.30
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Abstract

The WDHA syndrome characterized by watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) is rare, and with neurogenic tumors. A 20-month-old girl presenting with symptoms of WDHA syndrome was transferred to our hospital. She had a ganglioneuroblastoma of the adrenal gland. The serum level of VIP was elevated. After complete excision of the tumor, all symptoms related to the WDHA syndrome were relieved and serum VIP level dropped to normal. The postoperative course was uneventful. The patient was treated with postoperative chemotherapy and radiation therapy. There was no evidence of disease 33 months after operation.

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