Aggressive pituitary tumors in the young and elderly.

Abstract

Aggressive pituitary tumors (APTs) represent rare pituitary adenomas (PAs) with local invasion of surrounding tissues, increased risk for multiple recurrence, rapid tumor growth, or resistance to standard therapies. The most common APTs in children and adolescents are giant prolactinomas and somatotropinomas. Few cases of Crooke's cell adenomas, silent corticotroph adenomas and pituitary carcinomas havealso been reported in the literature. Pediatric patients with APTs have higher risk of harboring germline genetic defects, most commonly in the MEN1 and AIP genes. Since certain genetic defects confer a more aggressive behaviorto PAs, genetic testing should be considered in tumors with young onset and positive family history. The management of pediatric APTs involves usually acombination of standard therapies (surgical, medical, radiation). Newer agents, such as temozolomide, have been used in few cases of pediatric pituitary tumors with promising results. In the elderly, PAs are more commonly non-functioning. Their management often poses dilemmas given the coexistence of age-related comorbidities. However, standard surgical treatment and temozolomide seem to be safe and well tolerated in elderly patients.