A Case of Cowden's Disease Accompanied by Hamartoma of the Liver and Rectal Cancer

Satoshi Yano¹⁾, Tomoyoshi Atsuta¹⁾, Fujio Watanabe¹⁾, Kazuhiko Naoe¹⁾, Takeshi Kawamura¹⁾, Niwako Saito²⁾, Hiroo Ueno³⁾, Keiji Furuuchi⁴⁾, Hiroyuki Kato⁵⁾

Department of Surgery¹⁾ and Medicine²⁾, Otaru Kyoukai Hospital
Department of Pathology, Otaru City Hospital³⁾
Section of Genetice, Institute of Cancer Research, Hokkaido University, School of Medicine⁴⁾
The Second Department of Surgery, Hokkaido University, School of Medicine⁵⁾

A case of Cowden's disease is reported. A 59-year-old man sought medical treatment for diarrhea and anal bleeding. A series of endoscopic examinations showed multiple Yamada type 2 esophageal, gastric and colonic polyps, as well as type 3 rectal cancer. A careful orocutaneous examination with biopsy revealed acral and postauricular keratotic papules, cutaneous hemangioma on the lower right side of the abdomen and a papilloma of the right tonsilar area. These findings were sufficient to diagnose as Cowden's disease. On abdominal CT scan, we also found a hepatic tumor in the lateral segment. Miles' operation and partial resection of liver were performed. Histological findings showed that all the polyps were of hyperplastic types, and the liver tumor was a cavernous hemangioma (hamartoma), and the rectal cancer was a moderately differentiated adenocarcinoma. In addition, human papillomavirus was detected at both of the sites of rectal cancer and liver hamartoma. There are many problems needed to be solved in the pathogenesis of Cowden's disease. That may be helped by the accumulation of more detailed genetic studies.

Key words
Cowden's disease, hamartoma, human papillomavirus

Reprint requests
Satoshi Yano Department of Surgery, Otaru Kyoukai Hospital
1-6-15 Suminoe, Otaru, 047 JAPAN

Accepted
November 13, 1996

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