Pulmonary blastoma: case report in an adult.

During January 1997, a 48 year old man was admitted to our clinic for exertional dyspnoea which had developed 2 months before, wheezing and retrosternal chest pain spreading to the right axillary region. Low-grade fever (37.4°C) began 4 days before admission. The patient had worked as a welder. He had a tobacco smoking history of 5 pack/years. His physical examination was unremarkable. A chest X-ray showed the presence of a large (16 cm) round mass in the upper right lobe, compressing the trachea (figure 1). Routine laboratory tests demonstrated a mild normochromic normocytic anemia (hemoglobin 12.3 g/dL; mean corpuscolar volume 81 fL; mean corpuscolar hemoglobin 27 pg), increased erythrocyte sedimentation rate (81 mm/h), and hyperfibrinogenemia (908 mg/dL). Pulmonary function tests showed a small decrease in the vital capacity, (74% of the predicted value), forced expiratory volume in one second (FEV1) (69% of the predicted value) and total lung capacity (72% of the predicted value), with a normal FEV1/VC ratio (93% of the predicted value). Arterial blood gases were within the normal limits. Cytological examination of the sputum was repeatedly negative for the presence of neoplastic cells. A fibreoptic bronchoscopy revealed a left-convex deviation of the trachea and occlusion, ab estrinseco, of the apical right segmental bronchus. Pathological examination of the bronchoalveolar lavage and of a blind bronchial biopsy did not demonstrate the presence of neoplastic cells. A computed tomographic (CT) scan of the chest (figure 1) showed the presence in the right upper lobe of a large (12 cm) round mass, well-demarcated, centrally hypodense and with a peripheral ring of contrast enhancement. Epiaortic, paratracheal, and subcarenal lymph nodes were enlarged. Cranial and abdominal CT were negative. A radionuclide bone scan showed an area of hypercaptation inside the eleventh rib on the left side. Pathological examination of the material collected through a transthoracic needle aspiration of the mass was consistent with a diagnosis of biphasic pulmonary blastoma. The patient underwent right upper lobectomy. By gross examination, a 16 cm partly necrotic and haemorrhagic mass occupies much of the resected lobe. Histopathological examination (figure 2) confirmed the diagnosis of biphasic pulmonary blastoma infiltrating the visceral pleura without the presence of metastasis in the lymph nodes. The immunohistochemistry staining for the anti-oncogene protein p53 was negative. The patient was treated with 4 cycles of postoperative chemotherapy (epirubicin 75 mg/m2 and ifosfamide 1500 mg/m2) and follow-up visits showed a regular course until 18 months after the surgery when the