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

Naoki Futamura, Masasumi Matsutomo, Tsutomu Marui, Mikio Yasumura and Ken-ichiro Tateyama

Department of Surgery, Hashima City Hospital

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 11 cm in diameter with an indented center. On the day following hospitalization, he reported increased abdominal pain and peritoneal irritation, necessitating surgery. Upon laparotomy, bloody ascites was found in Douglas's pouch. A 17×13×6.5 cm tumor mainly occupied in the mesenterium of the jejunum and contacted the jejunum. A 19 mm tumor 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 jejunal 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 immunohistochemical expression of marker proteins. The patient died of recurrece 2 years and 3 months after surgery.

Key words
von Recklinghausen's disease, gastrointestinal stromal tumor, gastrointestinal mesenchymal tumor

Reprint requests
Naoki Futamura Department of Surgery, Gujo City Hospital
1264 Shimatani, Hachiman-cho, Gujo, 501-4222 JAPAN

Accepted
February 25, 2004

To read the PDF file you will need Abobe Reader installed on your computer.