Sarathi Vijaya
Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Center, Bengaluru, Karnataka, India.
Indian J Endocrinol Metab. 2017 May-Jun;21(3):470-474. doi: 10.4103/ijem.IJEM_558_16.
The "rule of 10" used to describe pheochromocytoma/paragangliomas (PCC/PGLs) has been challenged. However, recent studies suggested that pediatric PCC/PGLs may follow a pattern. Hence, we reviewed the available literature to verify the same. We searched PubMed, Scopus, ProQuest, and Google Scholar for studies describing the genotype and/or phenotype characteristics of pediatric PCC/PGL cohorts published after 2000 in English language and those with sample size more than 35 were included in this review. Pediatric PCC/PGLs were malignant in 10%, synchronous bilateral in 20%, extra-adrenal in 30%, among which, 30% were extra-abdominal and familial in 40%. PCC/PGL diagnosed during pediatric age recurs in 50% by 30 years of follow-up and 60% cases occur in boys. Seventy percent of children with PCC/PGL are likely to have sustained hypertension. Germline mutations could be identified in 80% of children with PCC/PGL and 90% are secretory. The review concludes that pediatric PCC/PGLs follow a pattern, which we call "10%-90% rule." This new rule will help easily remember the characteristics of pediatric PCC/PGLs.
用于描述嗜铬细胞瘤/副神经节瘤（PCC/PGLs）的“10法则”受到了挑战。然而，最近的研究表明小儿PCC/PGLs可能遵循一种模式。因此，我们回顾了现有文献以进行验证。我们在PubMed、Scopus、ProQuest和谷歌学术上搜索了2000年后发表的、用英文描述小儿PCC/PGL队列基因型和/或表型特征且样本量超过35的研究，符合条件的研究纳入本综述。小儿PCC/PGLs中10%为恶性，20%为双侧同时发生，30%为肾上腺外，其中30%为腹外，40%为家族性。小儿期诊断的PCC/PGLs在随访30年时有50%复发，60%的病例发生在男孩中。70%患有PCC/PGL的儿童可能患有持续性高血压。80%患有PCC/PGL的儿童可检测到胚系突变，90%为分泌性。本综述得出结论，小儿PCC/PGLs遵循一种模式，我们称之为“10%-90%法则”。这一新法则将有助于轻松记住小儿PCC/PGLs的特征。
