Pinés Corrales P J, González-Albarrán O, Peralta M, Roa C, Antón T
Department of Endocrinology, Hospital Ramón y Cajal, Madrid, Spain.
Horm Res. 2006;66(5):207-10. doi: 10.1159/000094742. Epub 2006 Jul 25.
Familial adenomatous polyposis (FAP) is an autosomal dominant condition characterized by multiple colorectal adenomas that can progress to carcinoma. FAP can be associated with diverse extracolonic manifestation, including desmoid tumors and adrenal masses. We report our experience with a patient diagnosed of FAP, who developed a desmoid tumor and an adrenal mass in the follow-up. To our knowledge, this is the first case in the literature in which a hypersecretion of aldosterone and cortisol in the adrenal mass of a patient diagnosed of FAP has been demonstrated.
家族性腺瘤性息肉病(FAP)是一种常染色体显性遗传病,其特征为多个结肠直肠腺瘤,可发展为癌。FAP可伴有多种结肠外表现,包括硬纤维瘤和肾上腺肿块。我们报告了一例被诊断为FAP的患者的情况,该患者在随访中出现了硬纤维瘤和肾上腺肿块。据我们所知,这是文献中首例被诊断为FAP的患者肾上腺肿块出现醛固酮和皮质醇分泌过多的病例。