Gardner's Syndrome With Adrenal Carcinoma

Abstract

SUMMARYA young woman with familial polyposis of the colon, multiple sebaceous cysts and osteomata (Gardner's syndrome) presented with Cushing's disease associated with carcinoma of the adrenal gland. This appears to be the first recorded case of Gardner's syndrome with adrenal carcinoma, and the first case wherein a metastasizing tumour other than carcinoma of the colon has been fully documented.

Hyperaldosteronism in an adolescent with Gardner's syndrome

“…They developed weight gain, hypertension, and headaches but did not have electrolyte abnormalities. Two of the patients had adrenal cortical carcinomas [6,8], and 2 had adrenal adenomas [2,3]. In all 4 cases, the symptoms were consistent with cortisol hypersecretion or adrenal Cushing's syndrome.…”

“…Desmoid tumors, mesenteric fibromatosis, dental abnormalities, gastric polyps, duodenal polyps, lymphoid hyperplasia, retinal pigment hypertrophy, hepatocellular carcinoma, benign intracranial tumors, and endocrine neoplasms are now considered part of the syndrome [2][3][4][5][6][7][8][9].…”

“…Because most endocrine-associated tumors in patients with GS occur without symptoms, most cases are discovered incidentally or at autopsy. A review of the literature resulted in the identification of 17 cases of adrenal neoplasms (13 adenomas, 4 carcinomas) in patients with GS [2,3,6,8,9]. Only 4 patients, however, were symptomatic.…”

Clinically Inapparent Adrenal Mass in a Patient with Familial Adenomatous Polyposis

“…Since then, other adrenal masses have been reported in association with FAP, the majority of them were discovered at autopsies [1,8,9,10,11,12,13,14,15,16]. Marchesa et al [2] studied 738 patients with FAP, 162 of those had been studied with abdominal TC scan.…”

“…FAP can be associated with diverse extracolonic manifestation, including desmoid tumors and adrenal masses [6]. To date, not many cases of desmoid tumor and adrenal mass in association with FAP have been reported in the literature, and no one of them secreting cortisol and aldosterone jointly [1, 7,8,9,10,11,12,13,14,15,16]. We report our experience with a patient diagnosed of FAP, who developed a desmoid tumor and an adrenal mass that secreted cortisol and aldosterone in the follow-up.…”