Intestinal polyposis and brain tumor in a family

Sayed, Atef K.; Jafri, Syed; Shenoy, Sadashiv S.

doi:10.1007/bf02586938

Cited by 18 publications

(3 citation statements)

References 14 publications

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“…Medulloblastoma and high‐grade gliomas are most commonly seen and may develop before polyposis 9,10. We found only two cases of pituitary adenoma reported in association with FAP; neither case was evaluated for APC germline mutation 11,12. Here we describe a third patient with FAP and a pituitary adenoma, who also had features consistent with a clinical diagnosis of Gardner's syndrome.…”

Section: Introductionmentioning

confidence: 76%

“…Fifty‐eight percent of the brain tumors associated with BTP syndrome type 2 were medulloblastoma; the remainder were predominantly gliomas. Only 2 patients with pituitary adenoma were identified 11,12. Both had FAP detected during adolescence, but neither was reported to have desmoid tumors or other extracolonic manifestations of Gardner's syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Prolactinoma as the first manifestation of Gardner's syndrome

Goodin

McCarville

Thibodeau³

et al. 2006

Pediatric Blood & Cancer

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Familial adenomatous polyposis (FAP) is an inherited condition causing numerous adenomatous colorectal polyps and a markedly elevated risk of colon cancer. FAP may be associated with various extracolonic manifestations such as desmoid fibromatosis and osteomas (termed Gardner's syndrome) and brain tumors, usually medulloblastoma or glioma [termed Brain Tumor Polyposis (BTP) syndrome type 2]. We describe a pediatric patient who initially presented with prolactinoma and later was found to have Gardner's syndrome. A germline mutation of the APC (adenomatous polyposis coli) gene was identified. Our case illustrates the association between prolactinoma and FAP, which may represent a rare subtype of Gardner's and BTP syndromes.

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Section: Introductionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Prolactinoma as the first manifestation of Gardner's syndrome

Goodin

McCarville

Thibodeau³

et al. 2006

Pediatric Blood & Cancer

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“…Only five families with at least two sibs affected with fully expressed TS have been reported [ 1,4,. Some more cases of TS were reported with familial recurrence in more than one generation [9,13] or as sporadic 13, [14][15][16][17][18][19][20][21][22][23].…”

Section: Introductionmentioning

confidence: 99%

Turcot's syndrome with intestinal lymphoma in a child: An unusual case of triple tumor

Parigi

Locatelli

Bragheri

et al. 1990

Med. Pediatr. Oncol.

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Turcot's syndrome, the association of brain tumor (usually glioblastoma, medullo-blastoma, or astrocytoma) and colonic polyps, is a very rare condition of which about 20 cases have been reported. It has been described only once previously with cancer in a third organ system. In this paper, we report a child affected with colonic polyposis and astrocytoma (i.e., Turcot's syndrome) associated with intestinal non-Hodgkin's lymphoma.

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Association of endocrine neoplasia with multiple polyposis of the colon

Schneider

Cubilla

Chaganti

1983

Cancer

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Endocrine neoplasms have occasionally been noted in patients with Gardner's syndrome and other polyposis coli (PC) syndromes; 15 such cases were found in a survey of the English literature. This article reports four additional patients with PC in whom occult endrocrine neoplasms were found at autopsy. Four sets of 100 consecutive autopsy reports, each bracketing but excluding one of the above cases, were reviewed. The incidence of endrocrine neoplasia in patients with non‐PC‐colorectal cancer (2/18) was similar to that in the entire sample (32/400), while four of five PC patients had such tumors. These data suggest that endrocrine neoplasia is significantly more frequent in PC than in other cancer patients, and a more frequent part of the PC syndromes than generally recognized. The relevance of these data to the issue of the genetics of the PC syndromes is discussed.

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Intestinal polyposis and brain tumor in a family

Cited by 18 publications

References 14 publications

Prolactinoma as the first manifestation of Gardner's syndrome

Prolactinoma as the first manifestation of Gardner's syndrome

Turcot's syndrome with intestinal lymphoma in a child: An unusual case of triple tumor

Association of endocrine neoplasia with multiple polyposis of the colon

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