Prasad A, Choudhry P, Sarin Y K, Saran R K
Department of Pediatric Surgery and Pediatrics, Maulana Azad Medical College, New Delhi-110002, India.
Indian J Pediatr. 2001 Aug;68(8):793-6. doi: 10.1007/BF02752427.
A three-and-half-year-old boy presented with recurrent chest infections, fever and weight loss of three month duration not responding to antibiotics. The chest X-ray and CT scan revealed a large well-circumscribed mass in right upper thorax with collapse of right upper lobe. A preoperative diagnosis could not be made even after fine needle aspiration cytology. Thoracotomy and right upper lobectomy was done and the biopsy report was an inflammatory pseudotumor. The child remained well for three months after which his symptoms and the mass recurred. The histopathology slides were reviewed and revealed a biphasic malignant tumor suggestive of Pulmonary Blastoma (PB). Patient received four cycles of chemotherapy followed by re-exploration. The recurrent tumour could only be excised partly and the child succumbed to persistent shock postoperatively. The final histopathological diagnosis was confirmed as PB. Primary pulmonary neoplasms in children are rare and of these PB which is even rarer, constitutes less than 15%. The report highlights that the lack of familiarity with this entity still causes error in the diagnosis of PB.
一名三岁半男孩出现反复胸部感染、发热及体重减轻,持续三个月,对抗生素治疗无反应。胸部X光和CT扫描显示右上胸部有一个边界清晰的大肿块,右上叶萎陷。即使进行了细针穿刺细胞学检查,术前仍无法做出诊断。遂行开胸手术及右上叶切除术,活检报告为炎性假瘤。患儿术后三个月情况良好,但之后症状及肿块复发。复查组织病理学切片显示为双相性恶性肿瘤,提示肺母细胞瘤(PB)。患者接受了四个周期的化疗,随后再次进行探查。复发性肿瘤仅部分切除,患儿术后死于持续性休克。最终组织病理学诊断确诊为PB。儿童原发性肺部肿瘤罕见,其中PB更是罕见,占比不到15%。该报告强调,对这种疾病缺乏了解仍然会导致PB诊断错误。