A Case of von Recklinghausen's Disease with Multiple Gastrointestinal Stromal Tumors of the Small Intestine and Leiomyoma of the Appendix

We present a case of von Recklinghausen's disease with multiple gastrointestinal stromal tumors of the small intestine and leiomyoma of the appendix. A 63-year-old man with von Recklinghausen's disease seen for lower abdominal pain was found in abdominal US and CT examinations to have a tumor of about 11cm in diameter with an indented center. On the day following hospitalization, he reported increased abdominal pain and peri-toneal irritation, necessitating surgery. Upon laparotomy, bloody ascites was found in Douglas's pouch. A 17×13×6.5cm tumor mainly occupied in the mesenterium of the jejunum and contacted the jejunum. A 19 mm tu-mor was present in the jejunum and a 21 mm tumor present in the appendix. The 3 lesions were excised. The large jejunal tumor cross-section showed a yellowish-white mass with an indentation intersecting with the je-junal lumen. Microscopic findings for the 3 tumors showed fascicular proliferation of spindle shaped cells. Two jejunal tumors were diagnosed as GIST and appendiceal tumor was diagnosed as leiomyoma according to im-munohistochemical expression of marker proteins. The patient died of recurrece 2 years and 3 months after surgery.