Balaji Raghunandan, Kumar Prasanna, Garg Isha, Das Kanishka
Department of Paediatric Surgery, St. John's National Academy of Health Sciences, St. John's Medical College Hospital, Bengaluru, Karnataka, India.
Department of Pathology, St. John's National Academy of Health Sciences, St. John's Medical College Hospital, Bengaluru, Karnataka, India.
J Indian Assoc Pediatr Surg. 2017 Jan-Mar;22(1):57-59. doi: 10.4103/0971-9261.194629.
A 9-year-old female presented with fever, cough, and hemoptysis for a week. The chest skiagram and contrast-enhanced computerized tomography delineated a well-defined solid lesion localized to the superior segment of the right lower lobe with features of a congenital pulmonary airway malformation. The lesion was surgically managed with a segmentectomy and histopathology confirmed a contained pulmonary rhabdomyosarcoma (RMS). No other primary site of origin was evident, and a final diagnosis of "primary" pulmonary RMS was made. She received adjuvant chemotherapy and was disease free after 6 years of surveillance. The unique clinicoradiological features of the case are discussed and the sparse literature is reviewed.
一名9岁女性因发热、咳嗽和咯血1周就诊。胸部X光片和增强计算机断层扫描显示右下叶上段有一个边界清晰的实性病变，具有先天性肺气道畸形的特征。该病变通过肺段切除术进行手术治疗，组织病理学证实为局限性肺横纹肌肉瘤（RMS）。未发现其他原发部位，最终诊断为“原发性”肺RMS。她接受了辅助化疗，经过6年的随访无疾病复发。本文讨论了该病例独特的临床放射学特征，并对相关的稀少文献进行了综述。
