A Case of Duodenal Leiomyoblastoma Accompanied with von Recklinghausen's Disease

Duodenal leiomyoblastoma is an exceedingly rare disease and 14 cases includingours have been reported in Japan. This is the first case of the leiomyoblastoma combined with von Recklinghausen's disease. The patient was a 56-year-old man who had a past history and family history of von Recklinghausen'sdisease. He was admitted with the complaint of a right hypochondrial tumor. Under the diagnosis of a smoothmuscle tumor located in the 1st portion of the duodenum and a gastric ulcer, tumor resection and totalgastrectomy (Roux-Y) was performed. The histological diagnosis was leiomyoblastoma and from its size, the evidence ofinfiltration and pleomorphism it was thought to be malignant though there was no metastasis to regional lymph nodes.Twenty months after the operation the patient is still alive without evidence of recurrence. He should becarefully followed up because the biological behavior of leiomyoblastoma is still controversial.