Leser-Trélat徴候を示した小腸bizarre leiomyoblastomaの1例

Bizarre Leiomyoblastoma of the Ileum Associated with Sign of Leser-Trélat

　著者らは最近，Leser-Trélat徴候に一致した皮膚症状を伴う回腸悪性腫瘍例で，その組織像がStoutの提唱したbizarre leiomyoblastomaであった稀有なる1例を経験したので，文献的考察を加えて報告する．

　A 62-year-old man visited our hospital with a complaint of intermittent melena. X-ray examination of the small intestine by double contrast method and compression study after duodenal intubation demonstrated two small niches and a diverticulum-like shadow at the ileum (Fig. 3).

　At surgical exploration, a large mass measuring 7.5×7.5×4.5cm in size was found arising from the small bowel about 60 cm from the ileocecal valve and was adherent to the urinary bladder and the posterior parietal peritoneum. Metastasis and spread to the peritoneum was apparent. Resection of tumor with adjacent intestine was done and about three-fourth of the tumor was removed. There was a large cavity within the mass which was communicating with the lumen of the small intestine (Fig. 4). The tissue was reported as bizarre leiomyoblastoma (Fig. 5, 6).

　Leiomyoblastoma of the small intestine is a rare disease and yet eight cases have already been reported in Japan, of which five cases have diagnosed as malignant tumor including our case (Table2). Microscopically, the mucosa extended from the inside of the ileum to a distance of several cm in the body of the tumor, which suggested Meckel's diverticulum. But a “Meckel-like” diverticulum can result from the presence of cavitation in a myomatous tumor of the ileum (Fig. 7). The origin of the tumor in this patient is unknown. In addition, this patient had rapidly developed multiple, seborrheic keratoses which was considered as sign of Leser-Trélat (Fig. 1, 2). These three points were discussed with some reference to the literature.