Department of Pneumology, CH Saint Brieuc, France.
Department of Radiotherapy, CAC Mougins, France.
Crit Rev Oncol Hematol. 2018 May;125:48-50. doi: 10.1016/j.critrevonc.2018.02.009. Epub 2018 Mar 3.
Pulmonary blastomas represent about 0.5% of primary pulmonary malignancies. The prognosis is poor. Standard treatment consists of surgical excision. There are no published series on which to judge the efficacy of chemotherapy or radiation therapy. We describe an unusual case of classic biphasic pulmonary blastoma (CBPC), with long-term survival despite numerous and varied cancer-related events and review the literature. Our 71-year-old Caucasian woman presented with history of blood in sputum in 2009. Right lower lobectomy yielded a diagnosis of sarcomatoid carcinoma (pneumoblastoma). Unusually, our patient is still alive 7 years after initial surgery, despite metastatic first relapse after 2 years. Metastatic progression was confirmed histologically on three separate occasions during the disease course. The patient received a combination of cisplatin (or carboplatin) and etoposide on three separate occasions. Molecular biology studies of CBPC are needed to identify effective treatments, and a patient registry should be created.
肺胚细胞瘤约占原发性肺部恶性肿瘤的 0.5%。预后较差。标准治疗方法是手术切除。目前尚无关于化疗或放疗疗效的发表系列。我们描述了一例罕见的经典双相性肺胚细胞瘤（CBPC）病例，尽管发生了多种与癌症相关的事件，但患者仍长期生存，并对文献进行了回顾。我们的这位 71 岁白人女性于 2009 年出现咳痰带血的病史。右肺下叶切除术的诊断为梭形细胞癌（肺胚细胞瘤）。不同寻常的是，尽管在最初手术后 2 年就发生了转移性首次复发，但患者在初始手术后 7 年仍存活。在疾病过程中，有三次分别通过组织学确认了转移进展。该患者在三次不同的情况下接受了顺铂（或卡铂）和依托泊苷的联合治疗。需要对 CBPC 进行分子生物学研究以确定有效的治疗方法，并且应该创建一个患者登记处。
