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Results for “Intestinal Polyposis”

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[Genetic counseling in cancerology].

The authors have studied the different situations that prompt a request for genetic counseling if different members of the same family suffer from cancer. Six possibilities are considered: the cancer concerned is a genetic disease per se (e.g. retinoblastoma, thyroid cancer with amyloid stroma); the genetic disease is often complicated with cancer (e.g. intestinal polyposis); the genetic disease is occasionally complicated with cancer (e.g. neurofibromatosis); cancer is part and parcel of the genetic disease (e.g. chromosomal abnormalities); in addition, there are two special situations: "cancer-prone families" and families who request genetic counseling after one single case (e.g. cancer of leukaemia in a child).

Genetic Counseling↗

[Fibrofolliculoma. Treatment with copper vapor laser].

There are many clinical presentations to fibrofolliculoma, described by Birt, Hogg and Dubé: the solitary and multiple forms, with or without other skin tumors, could also be markers of intestinal polyposis. Little is known of its pathogenesis. A case of multiple fibrofolliculoma of the face and neck is described. A new therapeutic approach by copper vapour laser is proposed.

Adult↗

[A recurrent desmoid tumor of the popliteal fossa with vascular compromise].

We report a case of a 18-year-old male patient with his fourth relapse of a desmoid tumour of the left popliteal hollow after a traumatism in this area. Arterial displacement and extrinsic compression were showed by arteriography. Tumoration was extirpated including popliteal artery, in this way a terminal by-pass from distal femoral to peroneo-tibial trunks with contralateral great saphena was made. Diagnosis of intestinal polyposis and of the little osseous malformations, was discarded these pathologies frequently are related with these tumours. The research for 8 years and 4 months doesn't show any relapse, nor local neither multicentral. Furthermore, some considerations about this theme are reported.

Adolescent↗

Clinicopathologic findings in the Bannayan-Riley-Ruvalcaba syndrome.

BACKGROUND: The term Bannayan-Riley-Ruvalcaba syndrome has been proposed to reflect the clinical overlap of 3 conditions previously described as separate entities, each inherited in an autosomal dominant fashion. They are the Riley-Smith, Bannayan-Zonana, and Ruvalcaba-Myhre-Smith syndromes. OBSERVATIONS: We studied 2 kindreds with the Bannayan-Riley-Ruvalcaba syndrome. Characteristic cutaneous findings included multiple subcutaneous lipomas and vascular malformations, lentigines of the penis and vulva, verrucae, and acanthosis nigricans. Macrocephaly with normal ventricular size, mental retardation, central nervous system vascular malformations, intestinal polyposis, skeletal abnormalities, and thyroid tumors were the most common systemic featues. A striking clinical finding in 1 patient was widespread verrucous changes of both lips that histologically showed epidermal hyperplasia with papillomatosis and hyperkeratosis. Biopsy specimens of facial papules demonstrated the histological features of both syringomas and trichilemmomas. Lentiginous hyperplasia of the epidermis with increased pigment in the basal layer and a slight increase in the number of melanocytes were seen in biopsy specimens of the penile lentigines. CONCLUSIONS: The histologic findings of both the facial lesions and the pigmented macules of the penis in the Bannayan-Riley-Ruvalcaba syndrome have not, to our knowledge, been reported previously. The similarities between the Bannayan-Riley-Ruvalcaba syndrome and Cowden disease raise the possibility of a common genetic pathogenesis for these 2 diseases.

Abnormalities, Multiple↗

[Hypertrophy of retinal pigment epithelium--epidemiology, clinical characteristics and differential diagnosis].

The aim of the study is to show the clinical features of hypertrophy of the retinal pigment epithelium. The authors have studied five own cases (all women, mean age 42 years). All patients had a complete ophthalmological examination including fluorescein angiography (the follow-up time was 6 to 12 months). They also had general medical examination to find the possible association with intestinal polyposis with probable malignant transformation (Gardner's syndrome). Although hypertrophy of the retinal pigment epithelium is so far a benign lesion, the patients should be checked up every year. All hyperpigmented lesions of the ocular fundus are serious for clinicians because of the suspicion of their malignancies.

Adult↗

Inhibition of polyposis in the small intestine of BALB/c mice by intestinal bacteria.

The incidence of polyposis and the number of polyps per mouse were significantly lower in conventionalized (CVz) mice than in germ-free (GF) mice. There was no significant difference in the average number of polyps between GF and gnotobiotic (GB) mice monoassociated with the various strains of intestinal bacteria. However, the incidence of polyposis and the number of polyps per mouse were significantly lower for mice associated with either chloroform-resistant bacteria (CRB) or fusiform bacteria (FB) than for GF mice. This study demonstrated that polyposis was suppressed by FB and CRB in the small intestine of BALB/c mice.

Animals↗

Polyposis of the small intestine in a young cat. A comparison with polyposis in man and dogs.

A one year old female Foreign White cat is described with a history of six days of unthriftiness, anorexia, anaemia and depressed temperature. The necropsy revealed multiple sessile polyps in the jejunum. Histologically a hyperplasia of the crypt epithelium with an inflammation of the mucosa and submucosa was found. A comparison of polyposis in man, dogs and cats is made.

Animals↗

High-grade non-Hodgkin's lymphoma complicating polypoid nodular lymphoid hyperplasia and multiple lymphomatous polyposis of the intestine.

Three patients with multiple lymphoid polyps of the small intestine--two with nodular lymphoid hyperplasia and one with multiple lymphomatous polyposis--developed high-grade B-cell lymphomas. A literature search has revealed only 10 previous cases of nodular lymphoid hyperplasia complicated by lymphoma and none of an association between multiple lymphomatous polyposis and high-grade lymphoma.

Adult↗

Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas.

Upper gastrointestinal polyps and extraintestinal tumors are well recognized in association with familial adenomatous polyposis (FAP). Although carcinoid tumors have been reported in association with sporadic colonic neoplasms and ulcerative colitis, to date, carcinoids have not been reported in association with FAP. We report a patient with FAP who has recurrent carcinoid tumors located at the bases of duodenal adenomas. The genetic basis of carcinoid neoplasms is still uncertain. This report may represent the clinical effect of the APC gene mutation on the enterochromaffin cell line manifesting as recurrent carcinoid tumors in physical association with intestinal adenomas. Future genetic analysis and epidemiological studies may be of value in determining whether a true association exists.

Adenoma↗

[Diffuse polyposis of the large intestine complicated by amyloidosis of the internal organs].

Polyposis of the large intestine is a common affliction of the intestine which usually occurs as a result of inflammatory diseases or of dysplasia of embryonic anlages. Malignization of diffuse polyposis is observed in 70-100% of cases. A case of diffuse polyposis of the large intestine with initial phenomena of malignization in a male patient aged 44 years is described. The case in question was characterized by the presence of accompanying diffuse amyloidosis of a number of internal organs (kidney, spleen, pancreas). The onset of amyloidosis in this case was associated apparently with the character of absorption by a pathologically changed surface of the large intestine mucosa and with the enlargement of the surface of absorption of products of the protein metabolism.

Adult↗

[Adenomas and carcinomas of the small intestine in familial polyposis and Gardner syndrome--analysis of a literature survey].

An analysis of 70 case records from the literature of patients with familial polyposis or Gardner syndrome and adenomas or carcinomas of the small bowel demonstrated that there is no difference between the neoplasms of the small bowel in familial polyposis or in Gardner syndrome. Between the adenomas and the carcinomas of the small bowel it was possible to show, that there exists a similar close relation as in the large intestine. In some carcinomas of the small bowel rests of adenomas can be observed. The peak incidence of small bowel adenomas is more than a decade earlier than that of carcinomas. The distribution of adenomas in the small bowel is quite similar to that of carcinomas. Therefore it can be supposed, that the significance of familial polyposis coli and Gardner syndrome for the importance of an adenoma-carcinoma-sequence in the large bowel is also existing in the small bowel.

Adenomatous Polyposis Coli↗