title: Primary cardiac malignant fibrous histiocytoma in the right ventricular infundibulum treated with a cavo-pulmonary shunt and coronary embolization.
abstract: A 51-year-old woman presented with progressive right ventricular infundibular wall thickening and outflow obstruction. She had had an aorto-coronary bypass for left main coronary artery disease 1 year after radiation therapy for left mammary cancer. Enhanced computed tomography showed a mass in the right ventricular free wall with no connection to the mediastinum; the tumor extended into the main pulmonary artery, but there was no other evidence of a primary or metastatic tumor. A biopsy specimen was obtained and based on the microscopic and immuno-histochemical findings (vimentin and Kp-1 positive) the diagnosis was primary cardiac malignant fibrous histiocytoma, which is very rare. A cavo-pulmonary artery connection lessened her symptoms, but embolization of the coronary artery to try and to reduce the mass had minimal effect. Four months after the tumor was diagnosed she died of extended pulmonary artery obstruction.
authors.affiliation: Third Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan. omafumi@hsp.md.shinshu-u.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Owa
authors.forename: T
authors.initials: T
authors.lastname: Higashikata
authors.forename: H
authors.initials: H
authors.lastname: Shimada
authors.forename: H
authors.initials: H
authors.lastname: Kitahara
authors.forename: M
authors.initials: M
authors.lastname: Asano
authors.forename: J
authors.initials: J
authors.lastname: Nakayama
authors.forename: H
authors.initials: H
authors.lastname: Hikita
authors.forename: J
authors.initials: J
authors.lastname: Koyama
authors.forename: S
authors.initials: S
authors.lastname: Sakurai
authors.forename: S
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D001157:Arterial Occlusive Diseases; D001943:Breast Neoplasms / Q000532:radiotherapy; D004621:Embolization, Therapeutic; D017809:Fatal Outcome; D005260:Female; D018610:Heart Bypass, Right; D006338:Heart Neoplasms / Q000473:pathology* / Q000601:surgery; D018219:Histiocytoma, Benign Fibrous / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000473:pathology / Q000628:therapy; D018497:Ventricular Dysfunction, Right / Q000473:pathology* / Q000628:therapy