Giant Adrenal Cavernous Hemangioma in a Patient with Familial Adenomatous Polyposis

Abstract

Adrenal hemangioma is an uncommon benign vascular tumor that is often discovered incidentally. It has never been reported in association with familial adenomatous polyposis. We report a case of a 60-year old man with a history of familial adenomatous polyposis, in whom a huge retroperitoneal cyst of 18x17 cm was discovered during routine radiologic evaluation. Because of the impossibility of ruling out the presence of malignancy, surgical cystectomy was performed, associated to a scheduled total colectomy. Pathological examination revealed that the cyst corresponded to an adrenal cavernous hemangioma. Colonic adenomas did not show signs of degeneration. Screening for adenomatous polyposis coli (APC) gene mutation was not carried out.As familial adenomatous polyposis is known to involve a variety of extracolonic manifestations, this finding raises the suspicion of a possible variant of this syndrome including adrenal hemangioma. An extensive study based on a larger patient series with genetic exploration is necessary.

Adrenal cavernous hemangioma: A rare cause of chronic lumbar pain - a case report

“…AH can be associated with malignant lung neoplasms (small-cell lung cancer), gynecologic or colic tumors (familial adenomatous polyposis). AH can also be associated with neonatal syndromes such as Klippel-Trenaunay or Sturge-Weber syndromes [ 14 , 15 ].…”

Adrenal Cavernous Hemangioma: A Rarely Perceived Pathology—Case Illustration and Review of Literature

“…Tumors originating from vessels could be associated with syndromes, but these are rather neonatal tumors, and not acquired tumors such as our present case. Nevertheless, there has been a previous single report of an adrenal cavernous hemangioma associated with familial adenomatous polyposis [57].…”

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“…AH may be associated with malignant extra-adrenal lesions such as small-cell carcinoma of the lung, gynecologic cancers and recently with familial adenomatous polyposis [5] . AH are mostly cavernous, unilateral and appear in the sixth or seventh decade of life with a 2:1 female to male ratio.…”