Aggressive pituitary tumors in the young and elderly

被引:26
|
作者
Tatsi, Christina [1 ]
Stratakis, Constantine A. [1 ]
机构
[1] Eunice Kennedy Shriver Natl Inst Child Hlth & Hum, Sect Genet & Endocrinol, NIH, Clin Res Ctr, 10 Ctr Dr,Bldg 10,Room 1-3330,MSC1103, Bethesda, MD 20892 USA
来源
基金
美国国家卫生研究院;
关键词
Pituitary; Children; Adolescent; Aggressive; Adenoma; LINKED ACROGIGANTISM SYNDROME; GIANT PROLACTINOMAS; PHASE-II; CLINICAL CHARACTERISTICS; ADRENOCORTICAL-CELLS; GERMLINE MUTATIONS; EUROPEAN-SOCIETY; CUSHINGS-DISEASE; GENE-MUTATIONS; ADENOMAS;
D O I
10.1007/s11154-019-09534-8
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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 have also been reported in the literature. Pediatric patients with APTs have higher risk of harboring germline genetic defects, most commonly in theMEN1andAIPgenes. Since certain genetic defects confer a more aggressive behavior to PAs, genetic testing should be considered in tumors with young onset and positive family history. The management of pediatric APTs involves usually a combination 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.
引用
收藏
页码:213 / 223
页数:11
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