Pseudocystic pheochromocytoma: An incidental finding and a rare entity

Abstract

Cystic adrenal neoplasms are uncommon and may be incidentally discovered during radiologic procedure not directly performed for the evaluation of adrenal disease. Cystic pheochromocytomas are uncommon neoplasms that originate from the adrenal medulla, clinically differing from the more frequent solid pheochromocytomas. Here, we report a case of 72 year-old woman affected by asymptomatic pseudocystic pheochromocytoma (70x50mm), incidentally discovered, with a false negative 123I-monoiodobenzylguanidine (123IMIBG) scintigraphy. Histopathological examination led to diagnosis of pseudocystic pheochromocytoma with typical architecture. The screening for germline mutation of succinate dehydrogenase complex subunits (SDHB, SDHC), Von Hipple-Lindau syndrome (VHL) and multiple endocrine neoplasia type 2 (RET) genes were negative, finding only some polymorphisms for RET and SDHB. We emphasize the relevance of an accurate pre-operative diagnosis, especially in rare diseases, and the requirement of experience and multidisciplinary management in these rare pathologies.