Blastoma pleuropulmonar: reporte de un caso / Pleuropulmonary blastoma: case report

El blastoma pleuropulmonar corresponde a una neoplasia primaria de pulmón, exclusiva de la edad pediátrica, poco frecuente y de características agresivas. La edad de diagnóstico varía entre 1 mes y 12 años. No se identifica una distinción de sexos. Se localiza en la pleura o el pulmón. Suele presentarse como dificultad respiratoria, con o sin neumotórax, dolor torácico y fiebre, por lo que es mal diagnosticada como neumonía. Radiológicamente, suele hallarse una masa de gran tamaño cercana a la pleura en la base pulmonar derecha sin broncograma aéreo. Cuenta con tres tipos histológicos: tipo I (quístico), tipo II (mixto), tipo III (sólido). Su tratamiento incluye resección y quimioterapia multimodal. El tipo I tiene un pronóstico favorable, pero los tipos II y III tienen mal pronóstico. Se presenta un caso de blastoma pleuropulmonar en una niña de 2 años, con énfasis en su diagnóstico clínico-radiológico.

Pleuropulmonary Blastoma corresponds to a malignant primary lung disorder, exclusive of pediatric age, infrequent and of aggressive characteristics. Age on diagnosis is 1 month-12 years. Rates per sex are equal. It can be found inside pleura or lungs. Respiratory distress associated or not with pneumothorax, chest pain and fever are classical clinical signs. These symptoms could be misdiagnosed as pneumonia. Radiologically, a large mass near the pleura at the base of the right lung without air bronchogram is its most common form. It has three histological types: type I (cystic), type II (mixed) and type III (solid). Its treatment requires tumor excision and multimodal chemotherapy. Pleuropulmonary Blastoma type I has good prognosis; type II and III variants have lower survival. We report a 2-year-old girl, pointing to the clinical-radiological diagnosis.

Pulmonary blastoma in a young adult.

Pulmonary blastoma is a rare but aggressive malignancy of the lung comprising epithelial and mesenchymal elements that resemble fetal lung tissue. This report described a case of an 18-year-old male who presented with cough and weight loss for a month. Computed tomography (CT) of the thorax revealed a large mass with mixed solid and cystic lesions on the right side of chest along with pleural effusion and mediastinal lymphadenopathy. Massive debulking was performed followed by chemotherapy. A biphasic pulmonary blastoma was diagnosed on histopathology.

Pleuropulmonary blastoma in an infant.

Pleuropulmonary blastomas (PPB) are rare and highly aggressive tumors. Herein, we report an infantile case of type III PPB. A 9-mth-old boy presented to our unit with a history of cough and tachypnea for 2 days. Chest computed tomography revealed a mass in the left upper lobe, emphysema in the left upper lung, and mediastinum and heart shifted towards the right. The mass was removed completely by left upper lobectomy and histology confirmed diagnosis of type III PPB. The immature blastematous tissue was positive for vimentin while benign epithelium was positive for epithelial membrane antigen and cytokeratin. No lymph nodule metastasis was found in the 7 lymph nodules obtained from the hilum of the lung near the tumor. Currently, the patient is under close follow-up and is doing well.