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Vol. 40. Issue 4.
Pages 188-190 (April 2004)
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Vol. 40. Issue 4.
Pages 188-190 (April 2004)
Case Reports
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Persistent Hemoptysis Secondary to Extensive Epithelioid Angiosarcoma
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4035
L. López, M. Iriberri
Corresponding author
secretaria.neumo@hcru.osakidetza.net

Correspondence: Dra. M. Iriberri. Unidad de Patología Respiratoria. Hospital de Cruces. Pza. de Cruces, s/n. 48903 Baracaldo. Vizcaya. España
, L. Cancelo, A. Gómez, F. Uresandi, V. Atxotegui
Unidad de Patología Respiratoria, Hospital de Cruces, Baracaldo, Vizcaya, Spain
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Abstract
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Pulmonary angiosarcoma is an unusual cause of diffuse pulmonary hemorrhage. Angiosarcomas are rare malignant vascular tumors accounting for 1% to 2% of all sarcomas. Angiosarcomas have been detected in nearly all organs, but lung involvement is unusual, accounting for less than 7%. The literature describes approximately 10 isolated cases of primary pulmonary angiosarcoma as opposed to the more common metastatic type. Given that primary and metastatic types are clinicopathologically similar, the presence of a distant primary sarcoma must be ruled out before a diagnosis of primary pulmonary angiosarcoma can be made. A pathological diagnosis requires a finding of polygonal or oval cells with atypical irregular nuclei and vascular spaces lined with such cells surrounded by hemorrhagic phenomena. Immunohistochemical analysis is positive for specific endothelial cell markers such as CD31 and factor VIII, and coexpression of keratin is a frequent finding.

Key words:
Angioendotheliomatosis
Epithelioid angiosarcoma
Lung sarcoma
Hemoptysis

Una causa excepcional de hemorragia pulmonar difusa es la presencia de un angiosarcoma pulmonar. Éste es un tumor vascular maligno que representa del 1-2% del total de sarcomas. Los angiosarcomas se han descrito en casi todos los órga-nos, siendo la afección pulmonar rara, de alrededor del 7%. En la bibliografía se describen aproximadamente 10 casos ais-lados de tumores primarios y es más frecuente la afección me-tastásica pulmonar. Desde el punto de vista clinicopatológico son superponibles las formas primarias y las metastásicas, por lo cual, ante la presencia de un angiosarcoma pulmonar pri-mario, hay que descartar la existencia de un posible tumor primario a distancia. El diagnóstico anatomopatológico se ca-racteriza por células poligonales u ovoides de núcleos atípicos irregulares, y espacios vasculares tapizados por dichas células tumorales con fenómenos hemorrágicos alrededor. Las técni-cas de inmunohistoquímica que demuestran la naturaleza en-do telial del tumor son la positividad del CD-31 y factor anti-proteína VIII, así como la positividad de la queratina, coexpresión frecuente del angiosarcoma epitelioide.

Palabras clave:
Hemangioendotelioma
Sarcomas pulmonares
Hemoptisis
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Copyright © 2004. Sociedad Española de Neumología y Cirugía Torácica (SEPAR)
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