Lewis Jennifer A, Petty William J, Urbanic James, Bernstein Eric D, Ahmed Tamjeed
Internal Medicine, Vanderbilt University, Nashville, USA.
Internal Medicine, Wake Forest School of Medicine, Winston-Salem, USA.
Cureus. 2018 Nov 13;10(11):e3586. doi: 10.7759/cureus.3586.
Pulmonary blastoma is a rare lung cancer classified into three subtypes: classic biphasic pulmonary blastoma (CBPB), well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB) of childhood. Compared to the other subtypes, CPPB is an aggressive tumor with an overall five-year survival of 16% across all stages. We present two cases of biopsy-proven metastatic CBPB, who have been disease-free for over 10 years since treatment completion. Both patients were treated with surgery to the primary tumor followed by an adjuvant cisplatin-based chemotherapy for four cycles and thoracic radiation. One patient relapsed shortly after the completion of thoracic radiation with brain metastases and underwent craniotomy, gamma knife radiosurgery (GKRS), and whole brain radiation therapy. The other patient presented with synchronous pelvic metastases and underwent metastasectomy after the completion of chemotherapy but before the initiation of thoracic radiation. We review the literature regarding surgical, chemotherapeutic, and radiation treatment for patients with metastatic pulmonary blastoma. Based on our experience and review of the existing case reports, aggressive tri-modality treatment including surgery, chemotherapy with a cisplatin backbone, and a definitive treatment of oligometastatic lesions amenable to local therapy including resection or radiosurgery is reasonable to consider for medically fit patients with CBPB.
肺母细胞瘤是一种罕见的肺癌,分为三种亚型:经典双相肺母细胞瘤(CBPB)、高分化胎儿腺癌(WDFA)和儿童胸膜肺母细胞瘤(PPB)。与其他亚型相比,CBPB是一种侵袭性肿瘤,所有阶段的总体五年生存率为16%。我们报告两例经活检证实的转移性CBPB病例,自治疗结束以来已无病生存超过10年。两名患者均接受了原发性肿瘤手术,随后进行了四个周期的以顺铂为基础的辅助化疗和胸部放疗。一名患者在胸部放疗结束后不久复发并出现脑转移,接受了开颅手术、伽玛刀放射外科治疗(GKRS)和全脑放射治疗。另一名患者出现同步盆腔转移,在化疗结束后但在开始胸部放疗前接受了转移灶切除术。我们回顾了关于转移性肺母细胞瘤患者手术、化疗和放射治疗的文献。根据我们的经验和对现有病例报告的回顾,对于身体状况适合的CBPB患者,考虑积极的三联疗法,包括手术、以顺铂为主的化疗以及对适合局部治疗(包括切除或放射外科治疗)的寡转移病灶进行确定性治疗是合理的。