Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, Baltimore, Maryland;
Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin.
Pediatrics. 2021 Apr;147(4). doi: 10.1542/peds.2020-028357. Epub 2021 Mar 24.
Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy.
A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB).
None of the 344 prenatally diagnosed lesions had malignant pathology ( < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, = 11; other PPB, = 3; adenocarcinoma, = 1) at a median age of 20.7 months (interquartile range, 7.9-58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel ( = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2-58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43-340.3; < .0001] and 42.03 [95% CI, 3.51-995.6; = .0041], respectively).
In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology.
小儿肺部病变是一组主要为良性的肺部异常，其临床表现和组织病理学表现广泛。我们的目的是评估接受原发性肺部病变切除术的儿童的特征，并确定术前恶性肿瘤的危险因素。
我们对美国 11 家儿童医院的手术数据库进行了一项回顾性队列研究。采用多变量逻辑回归分析，研究了术前特征与恶性肿瘤风险之间的关系，包括肺囊性腺瘤样畸形（PPB）。
344 例产前诊断的病变中无一例为恶性（ <.0001）。在 177 例无产前检测病史的患儿中，15 例（8.7%）被归类为恶性肿瘤（I 型 PPB， = 11；其他 PPB， = 3；腺癌， = 1），中位年龄为 20.7 个月（四分位距，7.9-58.1）。8 例（57%）PPB 病例存在 DICER1 突变。无恶性病变存在系统供血血管（ =.0427）。术前胸部 CT（computed tomography）对恶性病理的敏感性为 33.3%（95%置信区间[CI]：15.2-58.3）。多变量逻辑回归显示，CT 对恶性肿瘤的怀疑程度增加和双侧病变是恶性病理的显著预测因素（优势比分别为 42.15[95%CI：7.43-340.3； <.0001]和 42.03[95%CI：3.51-995.6； =.0041]）。
在出生后最初诊断为小儿肺部肿块的患者中，PPB 的风险接近 10%。这些结果强烈警告不要对该患者人群进行常规的非手术治疗。鉴于 CT 对识别恶性病变的敏感性差，DICER1 检测可能会有所帮助。
