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.

Keywords: Hemangioma; adrenal gland; cavernous; familial adenomatous polyposis.

Figure 1.

Macroscopic and histologic features of adrenal cavernous hemangioma: A) huge cyst with smooth surface; B) laminated adrenal tissue adhering to the external surface of the cyst wall (arrow). Note blood clots in the internal side; C) anfractuous vascular cavities separated by fibrous septa (HEx200); D) positive immunostaining of endothelial cells with CD34 (IHCx200).

Figure 2.

Macroscopic and histologic features of colonic adenomatous polyposis: A) numerous colonic polyps of various sizes; B) tubular adenoma with low-grade dysplasia (HEx200).