| We herein describe a 27-year-old male presenting with a pulmonary blastoma. The patient was admitted to our hospital with the chief complaints of fever and left back pain. Chest roentgenograms showed a tumor measuring 10 cm in diameter in a lower lobe of the left lung. Computed tomography and magnetic resonance imaging revealed a well demarcated and heterogeneously enhanced tumor. Although a histological diagnosis could not be obtained by a transbronchial biopsy, image analyses led us to suspect it to be malignant. The patient underwent a left lower lobectomy with lymph node dissection. A histopathological examination revealed the tumor to be a biphasic type of pulmonary blastoma. Because of the rapid progress of the tumor and the difficulty in making a preoperative diagnosis in such cases, an immediate surgical resection is therefore recommended in cases with even the slightest suspicion of malignancy. ( info) |
2/99. Pleuropulmonary blastoma in an adult: an initial case report. BACKGROUND: Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood. Its primitive, sarcomatous features are analogous to those of other dysembryonic or dysontogenetic tumors, such as wilms tumor, hepatoblastoma, neuroblastoma, and embryonal rhabdomyosarcoma. PPB typically presents in young children, most younger than 5 years, as a pulmonary and/or pleural-based tumor with cystic, solid, or combined cystic and solid features. These neoplasms are characterized histologically by primitive mesenchymal or a mixture of primitive and sarcomatous components and generally have an unfavorable clinical outcome: death occurs within 1-2 years after diagnosis. methods: Clinicopathologic and radiographic findings of a man age 36 years with a cystic and solid mass in the left hemithorax were reviewed and compared with previously studied cases of PPB. RESULTS: Pathologic examination of the mass revealed a cystic and solid neoplasm composed of malignant mesenchymal cells that were immunoreactive for vimentin and muscle specific actin and focally for desmin. The architectural and cytologic appearances as well as the immunohistochemical profile were those of type II PPB. CONCLUSIONS: To the authors' knowledge, all previously reported cases of PPB occurred in children age 12 years or younger. They believe that this case represents the first occurrence of PPB in an adult and documents the finding that, although it is uncommon, adults can develop primitive neoplasms that are usually associated with the pediatric population. In addition, the clinicopathologic features observed in the authors' adult patient were consistent with their experience with this tumor type in children. The patient died less than 1 year after diagnosis. ( info) |
3/99. pulmonary blastoma: diagnostic and therapeutic aspects. pulmonary blastoma (PB) is an extremely uncommon intrathoracic neoplasm in children. It accounts for 0.5% of all primary malignant tumors of the lung, and 20%-25% of the cases present in childhood. A new case of PB in a 3-year-old girl is discussed with special emphasis on diagnosis and treatment. The authors point out that the possibility of a primary lung tumor should be considered for any childhood cases with intractable signs and symptoms of a space-occupying lesion. A primary lung malignancy should not be excluded only on the basis of the patient's age, and childhood lung disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment given to adults. ( info) |
4/99. Pleuropulmonary blastoma: a rare pathology with an even rarer presentation. BACKGROUND: Pleuropulmonary blastoma is among the rarest tumors of childhood. Three types have been described: cystic, solid, and mixed. To date, bilateral disease has not been documented. methods AND RESULTS: A 5-week-old girl presented with a history of fever. Chest x-ray showed bilateral diffuse cystic lesions. Bowel obstruction developed that required laparotomy. Multiple small bowel polyps were resected. The patient was readmitted 4 months later with deteriorating respiratory status. She underwent sequential thoracotomies for resection of multiple bullae under high-frequency oscillatory ventilation. Small bowel polypectomies were again required because of obstruction. lung lesions were compatible with pulmonary blastoma but could not be correlated with intestinal polyposis. Bilateral cystic renal lesions were seen on ultrasound scan. Her disease progressed, despite chemotherapy, with the appearance of metastatic iris lesions. She again underwent laparotomies for multiple recurrent generalized small bowel polyps that were causing obstruction. Expanding renal cysts affected kidney function, and she died at 14 months of age. CONCLUSIONS: The rare association between pleuropulmonary blastoma and Wilms' tumor or nephroblastomatosis is known but rarely reported. Lacking pathological evidence, we can only speculate that this was the case. We have been unable to demonstrate any histological association between the renopulmonary and digestive lesions. Despite many unanswered questions, we are likely dealing with a "syndrome" of sorts with a dire outcome, despite aggressive treatments. ( info) |
| pulmonary blastoma is a rare primary lung tumour associated with poor prognosis. Despite modern diagnostic imaging and biopsy techniques, the diagnosis is usually not considered initially. A case of a 22-year-old man with a large biphasic left-sided pulmonary blastoma, extending to the mediastinum, is reported. It was treated by pneumonectomy and pericardiectomy with radical tumour resection from the anterior mediastinum, under cardiopulmonary bypass. Adjuvant radiotherapy was used. 3 years postoperatively there is no sign of recurring disease. ( info) |
| A 30-year-old woman was admitted to our hospital for investigation of an abnormal shadow in the right pulmonary hilus on a chest x-ray film. A percutaneous needle biopsy was performed, which revealed pulmonary blastoma. A right upper lobectomy was performed and the pathological stage was confirmed to be IIIa (T3N0M0). An analysis of preoperative cytological specimens showed that epithelial tumor cells with thin cytoplasm were either tubular or papillary, while some mesenchymal tumor cells with elliptic and spindle-shaped nuclei were also found in the necrotic background. Thus, pulmonary blastoma should be considered when a two-cell pattern consisting of both epithelial and mesenchymal components is observed. dna analysis was performed on previously identified areas of the epithelial or sarcomatous components, using a microdissection method. An analysis of the p53 gene by the single-strand conformation polymorphysm method showed an abnormal band with shifted mobility of exon 8 in only the sarcomatous component. ( info) |
7/99. Pleuropulmonary blastoma: long-term survival and literature review. BACKGROUND AND PROCEDURE: Pleuropulmonary blastoma (PPB) identifies different types of pulmonary tumors in the pediatric age. It is extremely uncommon and is known to have a very poor prognosis, with only few cases surviving after 10 years follow-up. Our experience with such a patient and long-term follow-up (12 years) is therefore of interest. She was a 2-year-old girl admitted with a history of respiratory distress and underwent a left thoracotomy. The tumor was removed and after 1 month a polychemotherapy course was started using dactinomycin and vincristine for 6 months. The patient remains disease-free 12 years after the operation. CONCLUSIONS: The biologic behavior of the tumor is considered unpredictable, and for this reason much effort has been expended in trying to identify prognostic factors. The preoperative size of the mass (size <5 cm) in our patient, its complete excision, and the histologic aspects might be helpful in identifying favorable prognostic factors. ( info) |
8/99. Pleuropulmonary blastoma and ovarian teratoma. We report a patient with pleuropulmonary blastoma who had a benign teratoma as a second primary tumor. The radiology, clinical course, and the biological importance of this rare neoplasm are discussed. ( info) |
9/99. The cytomorphology of pleuropulmonary blastoma. Pleuropulmonary blastoma is a rare, primitive primary neoplasm of the thorax in young children. The tumor, which is often but not always associated with cystic lung lesions, may arise in pulmonary parenchyma, the mediastinum, and pleura. Histologically, it is characterized by a biphasic neoplastic population of undifferentiated-appearing small round cells and larger spindle-shaped cells. A proportion of these cancers may also manifest more specific mesenchymal differentiation. In contrast to the pulmonary blastoma of adults, a malignant epithelial component does not occur. We present herein the third known case of a fine needle aspiration biopsy of a pleuropulmonary blastoma in a 5-year-old girl. The smears were moderately cellular and included an admixture of the characteristic small ovoid blastemal elements and scattered spindled mesenchymal tumor cells. ( info) |
10/99. Bilateral cystic pleuropulmonary blastoma in early infancy. We report 2 cases of multifocal cystic (type 1) pleuropulmonary blastoma, diagnosed during the first 6 months of life. This rare entity must be recognized before evolution into the prognostically unfavorable type 2 or type 3 pleuropulmonary blastoma. ( info) |