A Case of Peutz-Jeghers Syndrome Associated with an Advanced Carcinoma and Adenomas Arising in Hamartomatous Polyps of the Colon

Hideki Ohdan, Hisashi Oshiro, Yasuji Yamamoto, Issei Tanaka, Kazuo Inagaki, Kazuo sumimoto, Takashi Maeda, Takao Hinoi

First Department of Surgery, Hiroshima Prefectural Hiroshima Hospital

A 47-year-old male was referred to our hospital for evaluation of occult bleeding in the stool. Pigment spots were known to have been present on the lips since early childhood. At the age of 25, ilectomy was performed for intussusception due to intestinal polyps, and a diagnosis of Peutz-Jeghers (P-J) syndrome was made. Investigation of the family revealed similar mucocutaneous pigmentation and intestinal polyps in his mother, two brothers and a nephew. Fiberoptic colonoscopy revealed many polyps in the colon and advanced carcinoma of the sigmoid colon. The patient was treated by sigmoidectomy and R₃ lymphoidectomy. During the same operation, several colonic and rectal polyps were removed by colotomy and polypectomy. Histologically, the tumor in the sigmoid colon was mucinous carcinoma (n₁, ss, stage III), and most resected polyps were hamartoma. Adenomatous epithelium, however, was identified in three hamartomatous polyps: in the ascending colon, transverse colon and rectum. This paper reports the occurrence, in a patient with P-J syndrome, of advanced carcinoma and of adenomas arising in hamartomatous polyps. The literature on the occurrence of neoplastic changes in hamartomatous polyps of the P-J syndrome is reviewed.

Key words
Peutz-Jeghers syndrome, adenomas, carcinomas

Reprint requests
Hideki Ohdan First Department of Surgery, Hiroshima Prefectual Hiroshima Hospital
1-5-54 Ujinakanda, Hiroshima, 734 JAPAN

Accepted
September 4, 1991

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