Clinical and roentgen features of the intestinal polyposis syndromes

Dodds, Wylie J.

doi:10.1007/bf02256355

Cited by 26 publications

(6 citation statements)

References 54 publications

(41 reference statements)

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“…The extraintestinal manifestations and risk of malignancy of the familial adenomatous polyposis syndromes and of Peutz-Jeghers polyposis have been well documented in the radiology literature [1]. However, reports of hereditary juvenile polyposis are scant.…”

mentioning

confidence: 98%

Hereditary generalized juvenile polyposis: Association with arteriovenous malformations and risk of malignancy

Radin

1994

Abdom Imaging

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A young man with hereditary juvenile polyposis, a duodenal adenoma with carcinoma in situ, and multiple arteriovenous malformations of the lungs and liver is reported. Because of the risk of malignancy, it is suggested that patients with juvenile polyposis have endoscopic or radiographic surveillance of the upper gastrointestinal tract in addition to either prophylactic colectomy or regular colonoscopic surveillance with polypectomy. In view of the serious potential complications of associated pulmonary, cerebral, and hepatic arteriovenous malformations, screening of patients with juvenile polyposis with chest radiography, magnetic resonance imaging of the brain, and sonography of the liver may be warranted.

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mentioning

confidence: 98%

Hereditary generalized juvenile polyposis: Association with arteriovenous malformations and risk of malignancy

Radin

1994

Abdom Imaging

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“…As discussed by Wade et al [5], this entity differs from familial polyposis coli by the lack of a family history of polyps, and, in 2 patients, by the somewhat older age of presentation and fewer number of polyps [1,2]. It is unlikely that these cases represent a coincidence of multiple polyps and neurocrest tumors.…”

Section: Discussionmentioning

confidence: 97%

“…The multiple polyposis syndromes, and the multiple endocrine neoplasia (MEN) syndromes, have been well-described in the radiologic literature [1][2][3][4]. Recently, Wade et al [5] described a new syndrome of colonic neoplastic polyps associated with malignant neurocrest tumors which they observed in 3 cases.…”

Section: Casementioning

confidence: 99%

Radiologic manifestations of the syndrome of neurocrest and colonic tumors

Shapir

Frank

1985

Gastrointest Radiol

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This report describes the radiologic findings in the recently described syndrome of multiple colonic polyps and malignant tumors of neurocrest origin. One patient had multiple endocrine neoplasia syndrome type 11B with malignant medullary thyroid carcinoma, another had malignant pheochromocytoma, and the third had malignant carcinoid tumor. All patients had multiple colonic tubular or tubulovillous adenomas. In 2 patients, there were 8 sessile or pedunculated polyps distributed throughout the colon. In the patient with multiple endocrine neoplasia syndrome type 11B, the colon was carpeted with small polyps, 2-6 mm in size.

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“…Although uncommon, jejunal polyps are identified as enhancing sessile nodules at CT enterography or MR enterography (37). At doublecontrast barium enema study, multiple polyps measuring from pinhead size up to 1.5 cm and giving a serrated contour to the colonic wall are commonly seen (38).…”

Section: Familial Adenomatous Polyposismentioning

confidence: 99%

Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management

Katabathina¹,

Menias

Khanna³

et al. 2019

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Hereditary gastrointestinal (GI) cancer syndromes due to specific germline mutations are characterized by an increased risk of GI tract malignancies, extra-GI tract cancers, and benign abnormalities. These syndromes include Lynch syndrome, familial adenomatous polyposis, juvenile polyposis syndrome, Peutz-Jeghers syndrome, Cowden syndrome, hereditary diffuse gastric cancer, and hereditary pancreatic cancer. Timely identification of the responsible genes will help predict future cancer risks in these patients and their family members. Early detection of cancers is possible with appropriate screening methods; risk-reducing measures will help in cancer prevention. Select malignancies and benign conditions associated with these syndromes have distinctive imaging features that can aid in classifying the syndromes. Imaging also plays a pivotal role in screening and surveillance of patients as well as their at-risk relatives and is invaluable for follow-up of treated malignancies. The American College of Gastroenterology has established specific guidelines for diagnosis and management of hereditary GI cancer syndromes. Knowledge of the imaging features of various pathologic conditions and screening strategies will guide appropriate management of patients and at-risk family members. ©

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Clinical and roentgen features of the intestinal polyposis syndromes

Cited by 26 publications

References 54 publications

Hereditary generalized juvenile polyposis: Association with arteriovenous malformations and risk of malignancy

Hereditary generalized juvenile polyposis: Association with arteriovenous malformations and risk of malignancy

Radiologic manifestations of the syndrome of neurocrest and colonic tumors

Hereditary Gastrointestinal Cancer Syndromes: Role of Imaging in Screening, Diagnosis, and Management

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