Cases reported "Pulmonary Blastoma"

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1/6. Biphasic pulmonary blastoma in a child.

    pulmonary blastoma (PB) is a rare malignant pulmonary tumor composed of immature mesenchyme and/or epithelium that resembles an embryonic lung at 10-16 weeks gestation. PBs constitute only 0.25 to 0.5 percent of all primary malignant lung tumors. Approximately 20 percent of the reported cases have occurred in pediatric patients. A seven-year-old girl presented with fever, cough, respiratory distress and chest pain on the left side. An x-ray, ultrasonography and a computed tomographic scan of the chest showed a large mass consisting of solid and cystic components almost completely occupying the left hemithorax associated with pleural effusion. The diagnosis of biphasic PB was established by histological examination of thoracotomy material. The patient was considered inoperable due to tumor involvement of the mediastinum, and she died two days after the initiation of chemotherapy. We report this case of PB to raise attention to the clinical, radiological and pathological features of PB in childhood because of its rarity.
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keywords = effusion
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2/6. Pleuropulmonary blastoma in a child presenting with spontaneous pneumothorax.

    The authors described a 27-month-old boy with the diagnosis of pleuropulmonary blastoma who presented with spontaneous pneumothorax. The child was admitted to our hospital with the chief complaint of respiratory distress for 8 months. Initial chest X-ray revealed tension pneumothorax on the right side. After chest tube insertion to the right side, a repeated chest X-ray showed minimal pleural effusion and a mass-like lesion at the right lower lung field. Computed tomography (CT) of the chest showed a cavity with intramural mass confined in the right lower lung accompanied with hydropneumothorax. The surgery revealed a cystic and solid mass occupying the right pleural space medially displacing the right lower lung. Total removal of the mass was performed, the histopathologic findings revealed a mixed cystic and solid type of pleuropulmonary blastoma which was composed of primitive blastema with multidirectional differentiation. Combination chemotherapy consisting of vincristine, cyclophosphamide, doxorubicin and dactinomycin was administered two weeks after surgery. The child has been well for almost 6 months since the surgery, without any signs of metastasis or recurrence.
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keywords = effusion
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3/6. Case report: pulmonary blastoma: presentation of two atypical cases and review of the literature.

    pulmonary blastoma is a relatively rare aggressive adult malignancy. The clinical and radiographic features of two cases are described and the literature reviewed. pulmonary blastoma usually presents radiologically as a well-defined mass lesion on chest radiography, which may be large enough to completely opacify the hemithorax and cause mediastinal shift. On CT, pulmonary blastoma is seen as a mixed solid and cystic lesion with variable contrast enhancement and a necrotic centre. pleural effusion may be present but is not the predominant abnormality. Our cases are unusual as recurrent pleural effusions in the absence of a significant lung mass were the presenting findings.
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keywords = effusion
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4/6. pulmonary blastoma in an adult presenting as a chronic loculated effusion: a diagnostic problem.

    A 33 year old woman posed a diagnostic problem for two years with recurrent left sided chest pain and radiographic features suggestive of a loculated effusion. diagnosis was finally made at thoracotomy which revealed an encapsulated cystic mass. Histological examination confirmed a rare monomorphic pulmonary blastoma.
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keywords = effusion
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5/6. Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts.

    pulmonary blastoma is a rare and aggressive malignant tumor that affects children and adults. Recently a 3-year-old boy with a 2-year history of bilateral unilocular pulmonary cysts was transferred for evaluation of a cough and high spiking fever. A chest radiogram showed left pulmonary consolidation with pleural effusion, but thoracentesis was unsuccessful. Computerized tomography (CT) was suggestive of a pulmonary abscess, but CT-guided drainage did not yield any purulent fluid. Percutaneous biopsies were performed, and the cytology showed malignant cells. During thoracotomy, a large tumor involving the left lower lobe and pleural space was found, and a biopsy was performed. A frozen section showed blastemal and mesenchymal components devoid of neoplastic epithelium, consistent with the pleural variant of pulmonary blastoma. A left lower lobectomy, with tumor decortication of the pleural space, achieved total gross tumor removal. The child received aggressive multiagent chemotherapy, and midway through it he underwent elective excision of the opposite lung cyst. It has been 17 months since the lobectomy; he is off chemotherapy and has no evidence of disease. A review of the literature showed that a large number of pediatric pulmonary blastomas are associated with cystic lung disease. Because total tumor removal offers the only chance of a good long-term outcome, surgical excision or close follow-up of pulmonary cysts in children is strongly recommended.
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keywords = effusion
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6/6. A rare tumor masquerading as an empyema: pleuropulmonary blastoma.

    Pleuropulmonary blastoma, although rare, should be considered in the differential diagnosis of children who present with a pleural effusion and symptoms of respiratory tract infection when the empyema fails to respond to medical management. A case of a 2-year-old boy with this rapidly growing tumor is reported. The treatment and outcome of pleuropulmonary blastoma in children is reviewed.
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ranking = 1
keywords = effusion
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