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

概要

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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.
日本小児外科学会の論文
1992-12-20