title: Pleuropulmonary angiosarcoma involving the liver, the jejunum and the spine, developed from chronic tuberculosis pyothorax: Multidisciplinary approach and review of literature.
abstract: Pleuropulmonary angiosarcomas are very rare, with less than fifty cases reported in the literature. In most cases, the etiology is unknown but the presence of a chronic tuberculous pyothorax has been reported in several Asian case reports as a possible risk factor. We report the case of a Caucasian 68-year old man who presented with a pleuropulmonary angiosarcoma that arose from a chronic tuberculous pyothorax and which involved the ribs and the vertebrae, the psoas muscle, and the jejunum. The patient received adapted anti-tuberculosis treatment, embolization of the mass in the small bowel, palliative external beam radiotherapy on the spine and systemic chemotherapy with liposomal non-pegylated doxorubicin and ifosfamide. With this multidisciplinary approach the patient's symptoms were well controlled and he achieved a complete metabolic response after six cycles of chemotherapy. Unfortunately, the patient died after eight months from the beginning of chemotherapy due to an acute lung injury secondary to extensive bilateral interstitial infiltrates. Opportunistic pathogens or drug-induced lung toxicity were the most probable causes. Treatment with liposomal non-pegylated doxorubicin and ifosfamide could be a reasonable option in pleuropulmonary angiosarcoma but it should be validated in clinical trials. Chronic pyothorax seems to be a predisposing factor for the development of pleural angiosarcoma but further investigations are required to assess a causal association.
authors.affiliation: Medical Oncology Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Gema
authors.initials: G
authors.lastname: Bruixola
authors.affiliation: Medical Oncology Department, University Hospital La Fe, Valencia, Spain. Electronic address: diaz_rob@gva.es.
authors.forename: Robert
authors.initials: R
authors.lastname: Díaz-Beveridge
authors.affiliation: Pathology Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Enrique
authors.initials: E
authors.lastname: Jiménez
authors.affiliation: Medical Oncology Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Caballero
authors.affiliation: Infectious Diseases Unit, Internal Medicine Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Miguel
authors.initials: M
authors.lastname: Salavert
authors.affiliation: Medical Oncology Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Corina
authors.initials: C
authors.lastname: Escoin
authors.affiliation: Medical Oncology Department, University Hospital La Fe, Valencia, Spain.
authors.forename: Jorge
authors.initials: J
authors.lastname: Aparicio
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D004654:Empyema, Tuberculous / Q000150:complications*; D017809:Fatal Outcome; D006394:Hemangiosarcoma / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D006801:Humans; D007583:Jejunum / Q000473:pathology; D008099:Liver / Q000473:pathology; D008175:Lung Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D008297:Male; D010997:Pleural Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D049268:Positron-Emission Tomography; D013131:Spine / Q000473:pathology; D014057:Tomography, X-Ray Computed
keywords: Angiosarcoma; Chemotherapy; Pleura; Pyothorax; Sarcoma; Tuberculosis