Norio Kohrogi

Neoplastic transformation arising in Peutz-Jeghers polyposis

PURPOSE AND METHODS: To clarify the potential for malignancy of Peutz-Jeghers polyposis, we investigated 75 gastrointestinal polyps resected surgically or endoscopically from seven patients with this syndrome. RESULTS: There were 19 polyps in the stomach, 18 in the duodenum, 22 in the small intestine, and 16 in the large intestine, and these were histologically composed of 1 pyogenic granuloma, 1 cancer in adenoma, 2 adenomas, and 71 Peutz-Jeghers polyps. Nine of these Peutz-Jeghers polyps were accompanied by an adenomatous component, and, in addition, two of these showed a cancerous transformation with stalk invasion. A total of 12 neoplastic polyps (16 percent) were found in three relatively young patients (aged 20, 25, and 43 years), all of which were pedunculated and located either in the duodenum or in the jejunum. There was no statistical significance in size between the neoplastic polyps (mean ±SD, 20.1±10.8 mm) and the completely hamartomatous polyps (mean ±SD, 15.8±9.0 mm). Moreover, the configuration of these types of polyps seemed similar. CONCLUSION: Neoplastic transformation is not a rare event, and our results may indicate evidence of a hamartoma-adenoma-carcinoma sequence in Peutz-Jeghers polyposis.

Natural history of fundic gland polyposis in patients with familial adenomatosis coli/Gardner's syndrome

In order to study the natural history of fundic gland polyposis, 23 patients with familial adenomatosis coli/Gardners syndrome were examined over a follow-up period ranging from 17 mo to 13 yr (average 6 yr). Examinations included gastric radiography and endoscopy with biopsy. Fundic gland polyps were found in 10 individuals. The size and number of polyps varied considerably. During the follow-up period, there was an increase in number or size of polyps, or both, in 5 patients (aged 8-27 yr), a decrease or disappearance in 2 patients (aged 36 and 41 yr), an initial decrease or disappearance followed by a late-occurring increase in 2 patients (aged 28 and 35 yr), In addition, malignant or adenomatous changes of fundic gland polyps were not observed in any patient. Therefore, fundic gland polyposis in patients with familial adenomatosis coli/Gardners syndrome may appear as early as 8 yr of age. In some patients there is a gradual increase in number and size of polyps, whereas in others, polyp proliferation ceases and polyps may even decrease in number and size. Our findings indicate that the fundic gland polyposis does not require prophylactic surgery and that careful periodic follow-up should suffice.

Gastrointestinal Manifestations of Cowdenʼs Disease: Report of Four Cases

Four patients with an established diagnosis of Cowdens disease underwent barium meal study, upper gastrointestinal endoscopy, barium enema examination, and colonoscopy. In three, the esophagus was affected by small protrusions, which were diagnosed as glycogenic acanthosis. Numerous hyperplastic polyps were found in the stomach in three patients, and in one an inflammatory fibroid polyp was also detected. Either lymphangiectasia or lymphoid polyps were found in the duodenum in two patients. In all patients, the colon showed polyps that varied in histology and included adenoma, hamartomatous polyp, and ganglioneurofibroma. In addition, jejunal lymphangiomas were found in one of the three patients in whom the small intestine could be precisely evaluated. These findings suggest that the gastrointestinal involvement in Cowdens disease is characterized by various benign lesions, especially esophageal glycogenic acanthosis, numerous gastric hyperplastic polyps, and multiple hamartomatous polyps in the rectosigmoid colon. Detection of these gastrointestinal manifestations may lead to early diagnosis of this potentially malignant disease.

Villous adenoma of the duodenum in a patient with familial adenomatosis coli

A case of familial adenomatosis coli with villous adenoma of the third portion of the duodenum, which falls in the category of a Gardners syndrome, is described. The patient, who had complained of an abdominal mass which had been diagnosed as a desmoid tumor after surgical resection, had numerous adenomatous polyps throughout the colon confirmed by colonoscopy with biopsy. Endoscopic examination of the upper gastrointestinal tract revealed fundic gland polyposis in the stomach and numerous small adenomas in the duodenum. In addition, there was a pedunculated polyp in the third portion of his duodenum, measuring 30 mm in diameter, the surface of which had a cauliflowerlike appearance. The polyp was removed with the electrocautery snare and was histologically diagnosed as villous adenoma. Our case report supports the concept that villous adenoma, which possesses a high malignant potential, may occur in the upper gastrointestinal tract in patients with familial adenomatosis coli, and careful examination of the upper gastrointestinal tract including the distal duodenum seems to be necessary in the follow-up of patients with this disease.

Esophageal ulcer complicated by Reiter's syndrome. A case report.

A 40-year-old woman with Reiters syndrome had low-grade fever, a psoriasislike eruption on feet and hands, and multiple esophageal ulcers. She had keratoderma blennorrhagica, aseptic vaginitis, and ileosacral arthritis, but no ocular lesions. The patient was HLA-B27 negative. Radiographic and endoscopic examinations of the upper gastrointestinal (GI) tract showed multiple round or irregularly shaped small ulcers in the middle and distal portion of the esophagus. One month later, all esophageal ulcers disappeared spontaneously. A case like this of Reiters syndrome with esophageal involvement has not been reported before.