Xu D, Chen Z M, Gu W Z, Wang Y S, Huang M X, Tang L F, Zhang Y Y, Jiang Y
Department of Pulmonology, the Children's Hospital of Zhejiang University School of Medicine, Hangzhou 310052, China.
Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):298-303. doi: 10.3760/cma.j.issn.0578-1310.2017.04.013.
To understand the clinical manifestation, imaging characteristics and outcomes of pulmonary malignant tumors in children. We retrospectively collected information about seven cases of pulmonary malignant tumors in children in our hospital from Jan 2010 to Dec 2016. The information included clinical manifestation, imaging characteristics, pathologic results, and treatment. (1) All the seven patients firstly visited pediatric internal medicine departments. Symptoms included cough (=6), dyspnea (=4), fever (=2), anorexia (=2), chest tightness (=1), chest pain (=1), lameness (=1), abdominal distension and constipation (=1). We did not find hemoptysis, wheezing or weight loss in those patients. Physical examinations revealed unilateral reduced breath sounds (=5), moist rales and wheezes (=1), and normal (=1). Extrapulmonary signs included abdominal distension (=2), left hip tenderness (=1), and mass in left scrotum. We did not identify clubbing finger, anemic appearance, lymph node enlargement, or hepatosplenomegaly etc. (2) Laboratory examination results: complete blood count showed white blood cells in normal range except one case (17.44×10(9)/L). Neutrophil percentage ranged from 0.348 to 0.767. C reactive protein ranged between<1 and 162 mg/L. Hemoglobin was normal. Three out of four cases had abnormal blood tumor markers.(3) Imaging results showed multiple nodes (=3), multiple cystic lesions in lungs (=2) (both with pleuropulmonary blastoma), endobronchial soft tissue mass (=1), pulmonary round-shaped mass (=1), and mediastinal mass (=1). Imaging results also found atelectasis (=3), pneumonia (=2), pneumothorax (=2), longitudinal diaphragmatic hernia (=2), pleural effusion (=1), subcutaneous emphysema (=1). (4) All the patients underwent tumor puncture biopsy or tumor resection. Pathology revealed the final diagnosis of pleuropulmonary blastoma (=3), endodermal sinus tumors (=2), squamous cell carcinoma of lung (=1), and thyroid papillary carcinoma (=1). All of them were malignant tumors. We followed up them. Two patients died (both with pleuropulmonary blastoma) after their parents refused any medical help. Two cases were lost to follow-up. Three patients survived (followed up for 19 months, 11 months and tow months, respectively). One case with thyroid papillary carcinoma pulmonary metastasis underwent right thyroid cancer radical plus left thyroid lobe resection plus modified selective central lymph node dissection, then iodine (131) treatment. One case with endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies, resection of left retroperitoneal tumor and left testicular tumor, and six additional chemotherapies. Another one case of endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies and was discharged. Pulmonary malignant tumors are rare diseases in children. Clinical signs are often non-specific. For those with chest CT showing multiple cystic lesions, endobronchial soft tissue mass or multiple nodes, but no significant infection manifestation or no response to anti-infection therapy, pulmonary malignant tumors should be considered. Biopsy may be needed to confirm the final diagnosis.
为了解儿童肺恶性肿瘤的临床表现、影像学特征及预后。我们回顾性收集了我院2010年1月至2016年12月期间7例儿童肺恶性肿瘤的相关信息。这些信息包括临床表现、影像学特征、病理结果及治疗情况。(1) 7例患者均首诊于儿科内科。症状包括咳嗽(6例)、呼吸困难(4例)、发热(2例)、厌食(2例)、胸闷(1例)、胸痛(1例)、跛行(1例)、腹胀及便秘(1例)。这些患者中未发现咯血、喘息或体重减轻。体格检查发现单侧呼吸音减弱(5例)、湿啰音及哮鸣音(1例)、正常(1例)。肺外体征包括腹胀(2例)、左髋部压痛(1例)、左侧阴囊肿物。未发现杵状指、贫血貌、淋巴结肿大或肝脾肿大等。(2) 实验室检查结果:血常规显示除1例(白细胞17.44×10⁹/L)外,其余患者白细胞均在正常范围。中性粒细胞百分比在0.348至0.767之间。C反应蛋白在<1至162mg/L之间。血红蛋白正常。4例中有3例血液肿瘤标志物异常。(3) 影像学结果显示多发结节(3例)、肺部多发囊性病变(2例)(均为胸膜肺母细胞瘤)、支气管内软组织肿块(1例)、肺圆形肿块(1例)及纵隔肿块(1例)。影像学结果还发现肺不张(3例)、肺炎(2例)、气胸(2例)、纵膈疝(2例)、胸腔积液(1例)、皮下气肿(1例)。(4) 所有患者均接受了肿瘤穿刺活检或肿瘤切除术。病理最终诊断为胸膜肺母细胞瘤(3例)、内胚窦瘤(2例)、肺鳞状细胞癌(1例)及甲状腺乳头状癌肺转移(1例)。均为恶性肿瘤。我们对其进行了随访。2例患者(均为胸膜肺母细胞瘤)在其父母拒绝任何医疗救助后死亡。2例失访。3例患者存活(分别随访19个月、11个月和2个月)。1例甲状腺乳头状癌肺转移患者接受了右甲状腺癌根治术加左甲状腺叶切除术加改良选择性中央淋巴结清扫术,然后进行碘(131)治疗。1例内胚窦瘤肺转移患者接受了3次化疗,切除左腹膜后肿瘤及左睾丸肿瘤,并追加6次化疗。另1例内胚窦瘤肺转移患者接受了3次化疗后出院。儿童肺恶性肿瘤是罕见疾病。临床症状常不具有特异性。对于胸部CT显示多发囊性病变、支气管内软组织肿块或多发结节,但无明显感染表现或抗感染治疗无效者,应考虑肺恶性肿瘤。可能需要活检以确诊。