A Case of Carcinoma of the Ampulla of Vater Complicated by von Recklinghausen's Disease with Hypoplasia of the Pancreatic Body and Tail

Kouji Tobita, Kazuma Yamazaki, Masaaki Kodama, Okamichi Morikawa, Isao Toura, Akinao Matsunaga, Isao Kawamura and Takenori Ochiai*

Department of Surgery, Shimotsuga General Hospital
Department of Academic Surgery, Graduate School of Medicine, Chiba University*

We report a very rare case of carcinoma of the ampulla of Vater complicated by von Recklinghausen's disease with hypoplasia of the pancreatic body and tail and an anomaly of the portal vein running on the ventral side of the common bile duct. Ours is the fourteenth case of carcinoma of the ampulla of Vater complicated by von Recklinghausen's disease reported in Japan. No report has, to our knowledge, been made on cases of von Recklinghausen's disease complicated by hypoplasia of the pancreatic body and tail or an anomaly of anatomical vessels. The 57-year-old woman underwent pylorus-preserving pancreatoduodenectomty with D3 lymph node dissection for carcinoma of the papilla of Vater. The resected specimen showed a 3.0×8.0 cm tumor. The pathological diagnosis of the resected specimen was moderately differentiated adenocarcinoma, stage III. To ensure better postoperative quality of life (QOL), we avoided total pancretectomy. Unexpectedly, part of the pancreas became ischemic, causing intractable leakage of pancreatic juice. The patient died of recurrence two years after surgery. Total pancreatectomy focusing on radical resection of advanced carcinoma of the ampulla of Vater accompanied by hypoplasia of the pancreatic body and tail would have prevented the postoperative leakage of pancreatic juice.

Key words
carcinoma of the ampulla of Vater, von Recklinghausen's disease, hypoplasia of pancreatic body and tail

Reprint requests
Kouji Tobita Department of Academic Surgery, Graduate School of Medicine, Chiba University
1-8-1 Inohana, Chuo-ku, Chiba, 260-8670 JAPAN

Accepted
October 19, 2004

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