WDHA 症候群を呈した VIP 産生神経節芽腫の1例 : VIP産生神経原性腫瘍46例の検討

• 遠藤 豪一

  福島県立医科大学第1外科

• 金沢 幸夫

  福島県立医科大学第1外科

• 佐藤 志以樹

  福島県立医科大学第1外科

• 井上 仁

  福島県立医科大学第1外科

• 元木 良一

  福島県立医科大学第1外科

• 二宮 規郎

  寿泉堂病院小児科

書誌事項

タイトル別名

• A Case of WDHA Syndrome Associated With Ganglioneuroblastic VIPoma

抄録

We report a case of 26-month-old boy with WDHA syndrome in association with ganglioneuroblastic VIPoma. A patient was admitted for dehydration from protoracted watery diarrhea. Serum analysis revealed hypokalemia (2.4mEq/L) and high VIP levels (220pg/dl). Abdominal roentogenogram and computed tomography demonstrated a calcified tumor in the left paravertevral region. VIP secreting neurogenic tumor was highly suspected. In operation a well encapsulated tumor measuring 45×32×30mm was removed. The histological diagnosis was ganglioneuroblastoma. VIP level of the tumor was high (5500-95000ng/gwet). Soon after resection of the tumor, the diarrhea improved and serum VIP level returned within normal level. Forty-three months after the surgery, the patient remained healthy without evidence of tumor recurrence. In the literatures 45 cases of WDHA syndrome associated with neurogenic VIPoma have been reported since 1973, and we discussed them further.

収録刊行物

• Journal of the Japanese Society of Pediatric Surgeons

  Journal of the Japanese Society of Pediatric Surgeons 28 (7), 1364-1371, 1992

  特定非営利活動法人 日本小児外科学会

キーワード

• WDHA syndrome
• neurogenic tumor

詳細情報

• CRID: 1390282679787433856
• NII論文ID: 110002103762
• DOI: 10.11164/jjsps.28.7_1364
• ISSN: 21874247
• ISSN: 0288609X
• Web Site: https://search.jamas.or.jp/link/ui/1993205710
• 本文言語コード: ja
• データソース種別:
  • JaLC
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可