We report on a 9-year-old boy with von Recklinghausen's disease who developed a huge retroperitoneal tumor continuous with spinal tumors. He had a history of cafe-au-lait spots and multiple subcutaneous tumors on his entire body from birth. He was admitted to our hospital with complaints of anal prolapse and anal discomfort. A large lower abdominal tumor was detected by palpation. The tumor was diagnosed as a neurofibroma based on transrectal needle biopsy. CT scan and MRI demonstrated a large retroperitoneal tumor occupying the pelvic cavity. The tumor communicated with spinal tumors through the sacral canal, and extended to the dorsal space of the pancreas. First we excised the bilateral caudal spinal tumors. Laparotomy was performed secondarily. The tumor invaded to the urinary bladder and the colon along the nervous system and vessels. We resected the tumor together with the sigmoidal colon, but preserved the lower rectum and the urinary bladder at his parents' request. The notworthy feature of this case is the rarity of a huge retroperitoneal tumor in a child with von Recklinghausens disease. Except for two cases, similar cases have not been reported in the literature.
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