一名2岁分泌睾酮的转移性肾上腺皮质癌男童的长期随访及治疗结果
LONG-TERM FOLLOW UP AND TREATMENT OUTCOMES OF A 2 YEAR-OLD-BOY WITH METASTATIC TESTOSTERONE-SECRETING ADRENOCORTICAL CARCINOMA.
作者信息
LoGiurato Danielle, Antal Zoltan, Zhou Ping
出版信息
AACE Clin Case Rep. 2020 Aug 6;6(6):e300-e304. doi: 10.4158/ACCR-2020-0354. eCollection 2020 Nov-Dec.
OBJECTIVE
Treatment of metastatic adrenocortical carcinoma (ACC) is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited.
METHODS
We describe the case of a 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis.
RESULTS
The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height.
CONCLUSION
We report a case of pediatric ACC with metastasis that was successfully treated with surgery, chemotherapy, and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.
目的
转移性肾上腺皮质癌（ACC）的治疗具有挑战性，长期生存率极低。接受米托坦治疗的儿科患者的长期结局数据非常有限。
方法
我们描述了一名2岁患有ACC并伴有肺转移的男孩的病例。他接受了手术、化疗和米托坦治疗，诊断后13年仍无疾病。
结果
从该病例中学到的关键内分泌问题包括：肾上腺源性性类固醇和胰岛素样生长因子-2水平与疾病状态相关；在米托坦治疗期间需要非常高剂量的糖皮质激素和盐皮质激素；需要及时检测和治疗中枢性性早熟以维持最终成人身高。
结论
我们报告了一例伴有转移的儿科ACC病例，该病例通过手术、化疗和米托坦辅助治疗成功治愈。适当的内分泌检测和管理对长期生存和生活质量很重要。
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