Clinically Inapparent Adrenal Mass in a Patient with Familial Adenomatous Polyposis

Corrales, Pedro José Pinés; González-Albarrán, O.; Peralta, M.Garrido; Roa, C.; Anton, Todd

doi:10.1159/000094742

Cited by 6 publications

(2 citation statements)

References 40 publications

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“… 69 , 74 Most adrenal adenomas do not produce hormones, although production of cortisol, 66 , 69 , 76 aldosterone 65 and their combination have been reported. 73 Smith et al. described a patient with a pheochromocytoma.…”

Section: Fap and Adrenal Tumorsmentioning

confidence: 99%

Extra-Intestinal Manifestations of Familial Adenomatous Polyposis

et al. 2008

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Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited disorder, which results from a germ line mutation in the APC (adenomatous polyposis coli) gene. FAP is characterized by the formation of hundreds to thousands of colorectal adenomatous polyps. Although the development of colorectal cancer stands out as the most prevalent complication, FAP is a multisystem disorder of growth. This means, it is comparable to other diseases such as the MEN syndromes, Von Hippel-Lindau disease and neurofibromatosis. However, the incidence of many of its clinical features is much lower. Therefore, a specialized multidisciplinary approach to optimize health care-common for other disorders-is not usually taken for FAP patients. Thus, clinicians that care for and counsel members of high-risk families should have familiarity with all the extra-intestinal manifestations of this syndrome. FAPrelated complications, for which medical attention is essential, are not rare and their estimated lifetime risk presumably exceeds 30%. Affected individuals can develop thyroid and pancreatic cancer, hepatoblastomas, CNS tumors (especially medulloblastomas), and various benign tumors such as adrenal adenomas, osteomas, desmoid tumors and dental abnormalities. Due to improved longevity, as a result of better prevention of colorectal cancer, the risk of these clinical problems will further increase.We present a clinical overview of extra-intestinal manifestations, including management and treatment options for the FAP syndrome. Furthermore, we provide recommendations for surveillance of FAP complications based on available literature.

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Section: Fap and Adrenal Tumorsmentioning

confidence: 99%

Extra-Intestinal Manifestations of Familial Adenomatous Polyposis

et al. 2008

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“…FAP-associated adrenal tumors can produce mineralocorticoids, 10 corticosteroids, 5 or both. 11 In addition, there are reports of FAP patients developing pheochromocytoma 2 and adrenocortical carcinoma. 5 In a mortality study that investigated the causes of death in 132 FAP patients, one patient was found to have died of adrenal cancer.…”

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confidence: 99%

Adrenal Incidentaloma in Familial Adenomatous Polyposis: A Long-Term Follow-Up Study and Schema for Management

Will¹,

Hansmann²,

Phillips

et al. 2009

Diseases of the Colon &Amp; Rectum

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Familial adenomatous polyposis-associated adrenal incidentaloma may warrant long-term follow-up. Although the natural history is similar to lesions occurring sporadically, these patients have concomitant familial adenomatous polyposis-associated manifestations under radiologic surveillance. In this rare condition, development of a robust protocol will require evidence from worldwide patient cohorts. However, a tailored schema is suggested as a consistent basis for future modification.

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