El-Rifai Nahida, Shahine Samar, Sidani Hassan, Sabeh Aion Ali, Deschildre Antoine, Copin Marie-Christine
Department of Pediatrics, Makassed General Hospital, Beirut, Lebanon.
Department of Pathology, Makassed General Hospital, Beirut, Lebanon.
Case Rep Pediatr. 2016;2016:6714351. doi: 10.1155/2016/6714351. Epub 2016 Nov 22.
Lung tumors are extremely rare in the pediatric population, comprising only 0.2% of all malignancies in children. Among them, mucoepidermoid carcinoma (MEC) is even rarer with a reported frequency of 0.1% to 0.2%. MEC is defined by the World Health Organization as a tumor characterized by a combination of mucus-secreting, squamous, and intermediate cell types. We describe the case of a 4-year-old girl who presented with a history of intermittent fever and nonproductive cough of 1-month duration after foreign body aspiration. The chest X-ray showed complete collapse of the left lung. After removal of the foreign body, the lung expanded well after. However, the control chest X-ray done after 5 days showed again complete collapse of the left lung. The biopsy specimen taken during bronchoscopy confirmed the diagnosis of low-grade MEC. Fluorescence in situ hybridization (FISH) confirmed the presence of MAML2 rearrangement. Complete surgical resection with preservation of lung parenchyma was performed. No adjuvant therapy was needed. Repeat bronchoscopy was performed 2 months after surgery and showed no recurrence of the tumor. In conclusion, a remote chest X-ray after removal of a foreign body is necessary to avoid missing a rare serious underlying disease such as MEC. According to the size and the location of the tumor, complete surgical removal is sufficient without additional treatment in case of low-grade tumor. The presence of MAML2 rearrangement confers a favorable outcome and may have long-term implications for the clinical management.
肺肿瘤在儿童群体中极为罕见,仅占儿童所有恶性肿瘤的0.2%。其中,黏液表皮样癌(MEC)更为罕见,报告的发生率为0.1%至0.2%。世界卫生组织将MEC定义为一种以黏液分泌细胞、鳞状细胞和中间细胞类型组合为特征的肿瘤。我们描述了一名4岁女孩的病例,她在误吸异物后出现了持续1个月的间歇性发热和干咳病史。胸部X线显示左肺完全萎陷。取出异物后,肺随后扩张良好。然而,5天后进行的对照胸部X线检查再次显示左肺完全萎陷。支气管镜检查期间获取的活检标本确诊为低级别MEC。荧光原位杂交(FISH)证实存在MAML2重排。进行了保留肺实质的完整手术切除。无需辅助治疗。术后2个月进行了重复支气管镜检查,未显示肿瘤复发。总之,异物取出后进行远期胸部X线检查对于避免漏诊罕见的严重潜在疾病如MEC很有必要。根据肿瘤的大小和位置,对于低级别肿瘤,完整的手术切除就足够了,无需额外治疗。MAML2重排的存在预示着良好的预后,可能对临床管理具有长期意义。