11/99. Pleuropulmonary blastoma: A case report documenting transition from type I (cystic) to type III (solid). BACKGROUND: Pleuropulmonary blastoma is a rare, aggressive neoplasm that typically occurs in young children. It has been classified as type I, II, or III on the basis of the cystic versus solid nature of the lesion as well as the histologic appearance. Although it has been speculated that type I lesions may have a tendency to progress into type III lesions, no such case has been reported to date. methods: A case of type I pleuropulmonary blastoma in a girl age 2 years 9 months was found in our departmental archive. This case, originally diagnosed as a hamartoma over 20 years ago, predated the description of this pathologic entity. Over a 3-year period, the patient underwent excisions of a primary tumor and 3 subsequent recurrences, thus allowing us to follow the natural history of this neoplasm. RESULTS: The primary tumor was a large, multicystic mass (roughly 90% cystic by volume) with benign histologic appearance except for occasional foci with bland, embryonal rhabdomyosarcomatous features. In subsequent recurrences, the resected specimens became increasingly solid and had an anaplastic, multiphasic mesenchymal pattern. CONCLUSIONS: The course of the patient described here represents the first case in which transition from type I (cystic) to type III (solid) was documented. ( info) |
12/99. Well-differentiated fetal adenocarcinoma of the lung. We describe the case of a 43-year-old woman with a tumor shadow in the upper lobe of the left lung. The tumor was initially suspected to be a carcinoid tumor, following percutaneous needle biopsy. Subsequently, a left upper lobectomy was performed, and a well-differentiated fetal adenocarcinoma was diagnosed histologically. Unlike the biphasic epithelial and stromal features of pulmonary blastoma, it was composed solely of malignant glands of embryonal appearance. ( info) |
| 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. ( info) |
14/99. Pleuropulmonary blastoma: four cases. Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm affecting children. A retrospective review was carried out of patients diagnosed as having PPB at one institute over a period of 16 years. The presentation, diagnosis, treatment, and family history were studied. PPB usually presents with symptoms and signs of respiratory tract infection, and the diagnosis may be delayed. There are no distinguishing clinical features or imaging studies. The diagnosis is made on histologic examination of tumour material and is sometimes difficult to differentiate from benign cystic lung lesions. The treatment is primarily complete excision of the tumour, followed in some cases by intense chemotherapy. PPB is a strong predictor of the presence of tumours in close relatives. Four patients treated at our institute are discussed along with a review of the literature. ( info) |
15/99. Pancreatic metastasis of a pleuropulmonary blastoma in an adult. Pleuropulmonary blastoma (PPB) is a rare dysontogenetic tumor that usually develops in the first decade of life and has been recognized as a distinct clinico-pathological entity different from the ordinary pulmonary blastoma of adulthood. Since the tumor grows aggressively and tends to metastasize early, physicians have to be aware of late onset of symptoms and uncommon manifestations. We report a case of PPB in a young adult and its recurrence in the pancreas after primary surgical treatment and adjuvant chemotherapy. Keeping in mind the moderate prognosis of PPB in children, accurate assessment and treatment of PPB require a team approach of oncology, radiology and surgery to establish new therapeutic guidelines in the future. ( info) |
16/99. Staged resection of bilateral pleuropulmonary blastoma in a two-month old girl. Pleuropulmonary blastoma is a rare unilateral intrathoracic tumor of childhood. We report an unusual case of bilateral pleuropulmonary blastoma in a two-month old girl who underwent staged thoracotomies for complete wedge resection of both neoplasm. She remains well and tumor free two years after the operation. ( info) |
17/99. A 49-year-old woman with well-differentiated fetal adenocarcinoma. A 49-year-old woman underwent a pneumonectomy because of a mass in the middle lobe. Histological examination of the tumour showed a well-differentiated fetal adenocarcinoma (WDFA). This rare tumour appears to be associated with an excellent prognosis in the absence of metastases following surgical resection. ( info) |
| Pulmonary blastomas are a group of rare malignant neoplasms subdivided into three categories: classic biphasic pulmonary blastoma (CBPB), well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB). We report herein the cases of two men with CBPB. Both were heavy smokers and presented with a history of hemoptysis. physical examination revealed slightly significant findings, chest radiographs showed a large pulmonary mass, confirmed by computed tomography, and bronchoscopic biopsies were not diagnostic. A left and right inferior lobectomy was performed and a diagnosis of CPBP was confirmed by histological examination. In the first patient, local recurrence with multiple bilateral lung metastases was found 6 months later and despite chemotherapy, he died of respiratory failure 1 year after his operation. In the second patient, a subcutaneous metastasis was found in the right subscapular region 2 months later, and a cerebral metastasis in the right posterior parietal lobe 4 months later. Partial remission was achieved by cerebral irradiation, but 6 months later the patient died of cardiac failure while in a coma. We conclude that more aggressive and multidisciplinary treatment should be adopted for CBPB, and because of its low incidence, it is important to unify individual experiences in a central registry to gather as much information as possible regarding the biological and clinical features of this unusual disease. ( info) |
19/99. Extension of a pulmonary blastoma into the left atrium. Pulmonary tumors of embryonic origin are rare, and pulmonary blastomas are probably the most uncommon. A thorough literature search disclosed no previous reports of extension of this type of tumor into the heart. We describe a patient whose initial clinical presentation suggested an obstructive left atrial mass; however, clinical and histologic findings indicated the mass was a tumor that originated from a pulmonary blastoma that extended into the left atrium through a pulmonary vein. The unique aspect of this case is that the patient's symptoms were related to the obstructive effects of the atrial mass, not to the primary pulmonary tumor. ( info) |
20/99. Pleuropulmonary blastoma: an aggressive intrathoracic neoplasm of childhood. A case of a 4-year-old girl with pleuropulmonary blastoma is reported. Surgical resection of the tumor was performed and histologic examination revealed pleuropulmonary blastoma with rhabdomyosarcomatous differentiation. Postoperative chemotherapy was administered and 3 weeks after initiation of treatment protocol a second site of lesion in the retroperitoneum was revealed with extension to the mediastinum, which shared similar mesenchymal neoplastic characteristics to the previously diagnosed primary lesion. The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment. ( info) |