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Peutz-Jeghers syndrome and metastasising colonic adenocarcinoma.

A case of metastasising colonic carcinoma associated with Peutz-Jeghers syndrome in a 39 year old man is described. The caecal adenocarcinoma had metastasised widely to regional lymph nodes and was associated with several other colonic Peutz-Jeghers polyps, showing no evidence of dysplasia or malignancy. It was not possible to determine whether the carcinoma had arisen from a Peutz-Jeghers polyp. The patient also had gastric and small intestinal polyps. The serum carcinoembryonic antigen (CEA) was normal at presentation in this patient and in one other reported case. The use of this determination as a screening test for so-called high risk groups is, therefore, not supported by this report. The risk of malignancy is not known. It will only be determined by careful follow up of a defined Peutz-Jeghers syndrome population and comparison of carcinoma incidence with a matched sample of the general population. A national registry of Peutz-Jeghers syndrome patients in the United Kingdom would help to resolve this question.

Adenocarcinoma

[Cancer and polyps of the large intestine].

Incidence and mortality of the carcinoma of the large intestine increase also in the GDR. Carcinomas mostly develop in adenomas of the large intestine in the course of several years. Nowadays the adenoma-carcinoma-sequence is regarded as ascertained. The environmental influences are of decisive importance for the genesis of the colon carcinoma. Apart from this also genetic factors play a part. Villous adenomas more frequently show malignant structures than tubular adenomas. In large adenomas with a diameter of more than 20 mm more frequently invasive carcinomas (20-40%) are found than in small adenomas with a diameter of less than 10 mm (1%). From this is to be derived the demand of the removal of all adenomas of the large intestine with a diameter larger than 5 mm. The method of choice of the treatment is the polypectomy and the technique with the diathermy loop.

Adenoma

Hamartoma of the bladder in a 4-year-old girl with hamartomatous polyps of the gastrointestinal tract.

We report on a girl with hamartomatous intestinal polyps and a large mass that involved most of the posterior wall of the bladder, which on microscopic examination had the characteristics of a hamartoma. Hamartomas are among the rarest of bladder tumors. Our case is only the third reported under this designation, although 3 other cases may fall into this category. Of the 6 patients 4 have been children. Although most polypoid bladder tumors in children are rhabdomyosarcomas of the botryoid type, our case illustrates that rarely other lesions are similar grossly. Recognition of these lesions has clinical implications with regard to therapy and prognosis.

Child, Preschool

The distribution of polyps in the large intestine.

The distribution of polyps of the large intestine was investigated. Comparisons were made between results of autopsy studies and a clinical study consisting of 114 consecutively resected polyps. The shift from distal to proximal predominance of adenomas with increasing age from the 50 to the 80 year age group in autopsy studies, was in accordance with the high frequency of adenomas in the distal part of the large intestine in the prospective clinical study, mean 60 years of age. The present clinical study showed a high frequency of adenomas among men, whereas a tendency towards larger polyps and more polyps larger than 10 mm was seen for women. The present examination confirms the reported discrepancy between a high prevalence of adenomas compared to a much lower prevalence of cancer. Thus, the present results emphasize the need for further studies to gain information on risk factors in polyps or individuals predisposed for malignancy of the large intestine.

Aged

[Malignant colonic polyps: is polypectomy adequate treatment?].

The management of patients with endoscopically removed malignant intestinal polyps is controversial. The risk of residual disease should be assessed against the risk of a surgical operation. The authors report 35 cases of malignant polyps (5.5% of 641 colonoscopically removed adenomas). Sixteen patients had carcinoma in situ and received no further treatment and 19 had invasive carcinoma (sessile in 6, pedunculated in 13). Of these 19, 7 did not undergo surgery--because of old age in 2, minimal invasion in 3, a low rectal location in 1 and refusal in 1. Twelve patients (3 with sessile, 9 with pedunculated polyps) underwent a surgical resection, and residual disease was present in 3 (25%), 1 with positive nodes. Reported criteria of increased risk of residual disease--cancer in lymphatics or veins, incomplete excision, tumour at resection margin, sessile and villous tumours--were present in nine. All three patients with residual disease had microscopically involved margins of resection. The authors believe that the increased risk of recurrence justifies the risk associated with subsequent surgical resection unless the patient is otherwise a poor operative risk.

Adenoma