title: Neoadjuvant Chemoradiotherapy Treatment for a Classic Biphasic Pulmonary Blastoma with High PD-L1 Expression.
abstract: Pulmonary blastomas are rare malignant tumors, comprising only 0.25-0.5% of all malignant lung neoplasms. The prognosis of pulmonary blastoma is very poor, with an overall five-year survival of 16%. No standard treatment has been defined for unresectable disease. We present the case of a 25-year-old woman with unresectable locally advanced classic biphasic pulmonary blastoma (CBPB) successfully treated with neodjuvant chemoradiotherapy based on two chemotherapy induction cycles of cisplatin plus etoposide, followed by concurrent weekly cisplatin to 50.4 Gy radiotherapy treatment. The patient had a significant reduction in tumor size, allowing for complete resection by pneumonectomy. Molecular study for epidermal growth factor receptor (EGFR) mutations, anaplastic lymphoma kinase (ALK), proto-oncogene receptor tyrosine kinase (ROS1) and rearranged during transfection (RET) rearrangements, and programmed death ligand 1 (PD-L1) expression was performed in the pre-treatment tumor sample. Our patient presented a high expression (>90% of tumor cells) of PD-L1. To our knowledge, this is the first report of PD-L1 expression in CBPB. This could lead to new treatment options based on new immunotherapy agents blocking the PD-1/PD-L1 pathway for this rare disease with poor prognosis.
authors.affiliation: Department of Medical Oncology, Catalan Institute of Oncology, Doctor Josep Trueta University Hospital, Girona, Spain Girona Biomedical Research Institute (IDIBGi), Girona, Spain Department of Medical Sciences, Medical School, University of Girona, Girona, Spain jbosch@iconcologia.net rrosell@iconcologia.net.
authors.forename: Joaquim
authors.initials: J
authors.lastname: Bosch-Barrera
authors.affiliation: Department of Medical Oncology, Catalan Institute of Oncology, Doctor Josep Trueta University Hospital, Girona, Spain.
authors.forename: Francia
authors.initials: F
authors.lastname: Holguin
authors.affiliation: Department of Thoracic Surgery, Doctor Josep Trueta University Hospital, Girona, Spain.
authors.forename: Xavier
authors.initials: X
authors.lastname: Baldó
authors.affiliation: Department of Thoracic Surgery, Doctor Josep Trueta University Hospital, Girona, Spain.
authors.forename: Matilde
authors.initials: M
authors.lastname: Rubio
authors.affiliation: Department of Medical Oncology, Catalan Institute of Oncology, Doctor Josep Trueta University Hospital, Girona, Spain Girona Biomedical Research Institute (IDIBGi), Girona, Spain Department of Medical Sciences, Medical School, University of Girona, Girona, Spain.
authors.forename: Rut
authors.initials: R
authors.lastname: Porta
authors.affiliation: Department of Radiotherapy. Catalan Institute of Oncology, Doctor Josep Trueta University Hospital, Girona, Spain.
authors.forename: Rafael
authors.initials: R
authors.lastname: Fuentes
authors.affiliation: Pangaea Biotech, Quirón Dexeus University Institute, Barcelona, Spain.
authors.forename: Cristina
authors.initials: C
authors.lastname: Teixidó
authors.affiliation: Catalan Institute of Oncology, Germans Trias i Pujol University Hospital, Badalona, Spain.
authors.forename: José Luis
authors.initials: JL
authors.lastname: Ramirez
authors.affiliation: Nuclear Medicine Unit, Department of Radiology, Diagnostic Imaging Institute, Girona, Spain.
authors.forename: Nuria
authors.initials: N
authors.lastname: Ferran
authors.affiliation: Department of Thoracic Surgery, Doctor Josep Trueta University Hospital, Girona, Spain.
authors.forename: Fernando
authors.initials: F
authors.lastname: Sebastián
authors.affiliation: Cancer Biology and Precision Medicine Program, Germans Trias i Pujol University Hospital, Badalona, Spain Dr Rosell Oncology Institute, Quirón Dexeus University Hospital, Barcelona, Spain Molecular Oncology Research Foundation (MORe), Barcelona, Spain jbosch@iconcologia.net rrosell@iconcologia.net.
authors.forename: Rafael
authors.initials: R
authors.lastname: Rosell
mesh_terms: D000328:Adult; D060890:B7-H1 Antigen / Q000378:metabolism*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000628:therapy*; D020360:Neoadjuvant Therapy; D049268:Positron-Emission Tomography; D000090063:Proto-Oncogene Mas; D018202:Pulmonary Blastoma / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000628:therapy*; D014057:Tomography, X-Ray Computed
chemical_list: D060890:B7-H1 Antigen; C423236:CD274 protein, human; C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas
keywords: ALK-EML4 translocation; EGFR mutation; PD-L1 expression; Pulmonary blastoma; chemoradiotherapy; neoadjuvant treatment; pembrolizumab