title: Pulmonary artery sarcoma with angiosarcoma phenotype mimicking pleomorphic malignant fibrous histiocytoma: a case report.
abstract: Primary sarcomas of the major blood vessels can be classified based on location in relationship to the wall or by histologic type. Angiosarcomas are malignant neoplasms that arise from the endothelial lining of the blood vessels; those arising in the intimal compartment of pulmonary artery are rare. We report a case of pulmonary artery angiosarcoma in a 36-year old female with pulmonary masses. The patient had no other primary malignant neoplasm, thus excluding a metastatic lesion. Gross examination revealed a thickened right pulmonary artery and a necrotic and hemorrhagic tumor, filling and occluding the vascular lumen. The mass extended distally, within the pulmonary vasculature of the right lung. Microscopically, an intravascular undifferentiated tumor was identified. The tumor cells showed expression for vascular markers VEGFR, VEGFR3, PDGFRa, FGF, Ulex europaeus, FVIII, FLI-1, CD31 and CD34; p53 was overexpressed and Ki67 proliferative rate was increased. Intravascular angiosarcomas are aggressive neoplasms, often associated with poor outcome.
authors.affiliation: Department of Pathology, Christus Mugerza UPAEP University Hospital, Puebla, 72000, Mexico. olga.bohn@gmail.com
authors.forename: Olga L
authors.initials: OL
authors.lastname: Bohn
authors.forename: Eric Acosta-Ponce
authors.initials: EA
authors.lastname: de León
authors.forename: Oscar
authors.initials: O
authors.lastname: Lezama
authors.forename: Nina P
authors.initials: NP
authors.lastname: Rios-Luna
authors.forename: Sergio
authors.initials: S
authors.lastname: Sánchez-Sosa
authors.forename: Antonio
authors.initials: A
authors.lastname: Llombart-Bosch
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D049109:Cell Proliferation; D017024:Chemotherapy, Adjuvant; D003951:Diagnostic Errors; D005260:Female; D006394:Hemangiosarcoma / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D051677:Histiocytoma, Malignant Fibrous / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D010641:Phenotype; D011013:Pneumonectomy; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor