Zhang May, Bhat Trisha, Gutmann David H, Johnson Kimberly J
Brown School Master of Public Health Program, Washington University in St. Louis, St. Louis, MO.
Dermatol Online J. 2019 Nov 15;25(11):13030/qt5ck3f722.
Neurofibromatosis type 1 (NF1) is a cancer syndrome associated with many different cancer types. There are limited studies examining melanoma risk in this population.
To identify melanoma cases in NF1 patients and compare melanoma incidence rates relative to a general population sample.
A retrospective single institution case review of 857 NF1 patients (seen from 7/1997 to 7/2017) was conducted. We calculated age- and calendar period-adjusted standardized incidence ratios (SIRs) for white patients >20 years old overall (N=282) and for females (N=156) at their last visit date. We obtained general population melanoma reference rates from the Surveillance, Epidemiology, and End Results (SEER) 9 database.
Among 857 patients, 52.2% were female, 54% were <20 (mean±sd=10.9±4.6) years old, and 46% were >20 (40.4±14.9) years old at their last visit date. One white female patient had a malignant melanoma diagnosed at 47 years old. The adjusted SIR was 0.97 (95% CI 0.05-4.78) overall (N=282) and 1.62 (95% CI 0.08-7.98) for females (N=156).
We did not find statistical evidence for an increased melanoma risk in adults with NF1. However, additional large studies are warranted to clarify whether melanoma risk is increased in NF1 patients.
1型神经纤维瘤病（NF1）是一种与多种不同癌症类型相关的癌症综合征。针对该人群黑色素瘤风险的研究有限。
识别NF1患者中的黑色素瘤病例，并将黑色素瘤发病率与普通人群样本进行比较。
对857例NF1患者（于1997年7月至2017年7月就诊）进行了单机构回顾性病例分析。我们计算了20岁以上白人患者（N = 282）以及女性患者（N = 156）在其最后就诊日期时按年龄和日历期调整的标准化发病率（SIR）。我们从监测、流行病学和最终结果（SEER）9数据库中获取了普通人群黑色素瘤参考率。
在857例患者中，52.2%为女性，54%在最后就诊时年龄小于20岁（平均±标准差=10.9±4.6），46%年龄大于20岁（40.4±14.9）。一名47岁的白人女性患者被诊断为恶性黑色素瘤。总体调整后的SIR为0.97（95%可信区间0.05 - 4.78）（N = 282），女性为1.62（95%可信区间0.08 - 7.98）（N = 156）。
我们未发现成年NF1患者黑色素瘤风险增加的统计学证据。然而，需要更多大型研究来明确NF1患者的黑色素瘤风险是否增加。
