Alahwal Mahmoud S, Maniyar Iqbal H, Saleem Faiza, Alshiekh Mariam
Medical Oncology, Faculty of Medicine, King Abdulaziz University, Jeddah 22254, Saudi Arabia.
Case Rep Med. 2012;2012:471613. doi: 10.1155/2012/471613. Epub 2012 Sep 19.
Pulmonary blastoma, a rare primary lung malignancy, is considered to be distinct from other lung tumors based on pathological features, clinical course, and prognosis. More than one hundred cases have been reported in literature highlighting an interesting fact about their distinctive biologic manner from histopathological features. Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. Surgery is the mainstay of treatment. The prognosis of this rare malignancy is poor and the overall 5-year survival is around 15%. Our patient presented with respiratory symptoms and was found to have right-sided chest wall mass. The patient underwent complete surgical excision followed by 6 cycles of platinum-based chemotherapy. The patient showed good subjective and objective response with no evidence of disease recurrence. We report this rare malignancy with a review of literature, and the potential to use adjuvant chemotherapy in the management of this condition.
肺母细胞瘤是一种罕见的原发性肺恶性肿瘤，基于病理特征、临床病程和预后，被认为与其他肺部肿瘤不同。文献中已报道了一百多例病例，凸显了其在组织病理学特征方面独特生物学行为的一个有趣事实。典型的肺母细胞瘤由类似于胎儿肺组织的未成熟上皮和间充质组织混合组成。手术是主要的治疗方法。这种罕见恶性肿瘤的预后很差，总体5年生存率约为15%。我们的患者出现呼吸道症状，被发现右侧胸壁有肿块。患者接受了完整的手术切除，随后进行了6个周期的铂类化疗。患者主观和客观反应良好，无疾病复发迹象。我们报告了这种罕见的恶性肿瘤，并对文献进行了综述，以及在这种疾病管理中使用辅助化疗的可能性。
