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Vol. 46. Issue 1.
Pages 44-46 (January 2010)
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Vol. 46. Issue 1.
Pages 44-46 (January 2010)
Case Report
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Pulmonary Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Case Report and a Review of the Literature
Sarcoma de Ewing pulmonar/tumor neuroectodérmico primitivo (PNET): aportación de un caso y revisión de la bibliografía
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Juan Suárez Antelo
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jsuaant@telefonica.net

Corresponding author.
, Carlota Rodríguez García, Carmen Montero Martínez, Héctor Verea Hernando
Servicio de Neumología, Complejo Hospitalario Universitario, A Coruña, Spain
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Abstract

Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.

We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.

Keywords:
Ewing sarcoma/primitive neuroectodermal tumor
Primary lung tumor
Diagnosis
Resumen

Los sarcomas primarios de tórax son muy poco frecuentes. Sarcoma sinovial, angiosarcomas, leiomiosarcomas, rabdomiosarcomas y mesoteliomas sarcomatoides son las variantes intratorácicas más comunes. Aunque el sarcoma de Ewing/tumor neuroectodérmico primitivo (PNET) torácico se desarrolla habitualmente en la pared torácica, se ha descrito en la literatura médica algún caso de localización pulmonar primaria.

Presentamos el caso de una mujer de 22 años diagnosticada de sarcoma de Ewing/PNET pulmonar mediante muestra broncoscópica por sus características histológicas, inmunohistoquímicas y técnicas de hibridación in situ. Se excluyó el origen metastásico mediante radiografía, gammagrafía y biopsia de médula ósea. Se inició quimioterapia según el esquema VACD-IE (vincristina, actinomicina D, ciclofosfamida, doxorrubicina, ifosfamida y etopósido), con buena respuesta. En la actualidad acude de forma regular a consultas ambulatorias.

Palabras clave:
Sarcoma de Ewing/PNET
Tumor pulmonar primario
Diagnóstico
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Copyright © 2010. Sociedad Española de Neumología y Cirugía Torácica
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