Surgery and radiation therapy for brain metastases from classic biphasic pulmonary blastoma.

Pulmonary blastoma, a rare malignant lung tumour, can metastasise to the brain. However, there is no evidence for any effective treatment. The aim of this report is to discuss the treatment options for pulmonary blastoma and confirm the necessity for a pathological diagnosis. A 75-year-old man was admitted with progressive right-sided hemiplegia and aphasia. MRI showed multiple brain tumours. A left frontal lobe lesion was surgically resected, after which he underwent whole brain radiation (30 Gy/10 fractions). He died of an acute exacerbation of interstitial pneumonia. On performing autopsy, partial responses in the brain metastases that had been irradiated were confirmed pathologically. Thus, we present pathological confirmation that surgery and radiation therapy have therapeutic effects on brain metastases from pulmonary blastoma.

Pediatric dosimetry for intrapleural lung injections of (32)P chromic phosphate.

Intracavitary injections of (32)P chromic phosphate are used in the therapy of pleuropulmonary blastoma and pulmonary sarcomas in children. The lung dose, however, has never been calculated despite the potential risk of lung toxicity from treatment. In this work the dosimetry has been calculated in target tissue and lung for pediatric phantoms. Pleural cavities were modeled in the Monte Carlo code MCNP within the pediatric MIRD phantoms. Both the depth-dose curves in the pleural lining and into the lung as well as 3D dose distributions were calculated for either homogeneous or inhomogeneous (32)P activity distributions. Dose-volume histograms for the lung tissue and isodose graphs were generated. The results for the 2D depth-dose curve to the pleural lining and tumor around the pleural cavity correspond well with the point kernel model-based recommendations. With a 2 mm thick pleural lining, one-third of the lung parenchyma volume gets a dose more than 30 Gy (V(30)) for 340 MBq (32)P in a 10 year old. This is close to lung tolerance. Younger children will receive a larger dose to the lung when the lung density remains equal to the adult value; the V(30) relative lung volume for a 5 year old is 35% at an activity of 256 MBq and for a 1 year old 165 MBq yields a V(30) of 43%. At higher densities of the lung tissue V(30) stays below 32%. All activities yield a therapeutic dose of at least 225 Gy in the pleural lining. With a more normal pleural lining thickness (0.5 mm instead of 2 mm) the injected activities will have to be reduced by a factor 5 to obtain tolerable lung doses in pediatric patients. Previous dosimetry recommendations for the adult apply well down to lung surface areas of 400 cm(2). Monte Carlo dosimetry quantitates the three-dimensional dose distribution, providing a better insight into the maximum tolerable activity for this therapy.

Prognostic factors in pleuro-pulmonary blastoma.

PURPOSE: To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB). PATIENTS AND METHODS: Clinicopathological findings, treatment, and outcome of 22 PPB cases observed in 13 Italian Associations for Pediatric Hematology and Oncology centers are reported. Clinical data, surgical notes, pathologic findings, and summaries of treatment were taken from the charts and correlated with outcome by standard statistical methods. RESULTS: The series included 22 patients (14 males) with a median age of 30.5 months followed up for a median of 22 months (range 2-176 months). In nine patients the PPB developed with lung involvement only. Congenital lung cysts were recorded in five cases. Nine patients had recurrences. Gender, side, tumor size, pre-existing lung cysts, and extent of surgical resection at diagnosis did not significantly affect survival by univariate analysis. Achieving total resection of the tumor at any time of treatment resulted in a significantly better prognosis (P = 0.01), whereas extrapulmonary involvement at diagnosis resulted in a significantly worse prognosis (P = 0.01). Estimated 15-year event-free and overall survival rates were 44 and 49% for all patients, respectively. CONCLUSIONS: PPB is an aggressive neoplasm. Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis.

Unexpected response of a pulmonary blastoma on radiotherapy: a case report and review of the literature.

Pulmonary blastoma (PB) is a rare malignant tumor of the lung. Treatment is primarily surgical, although, combination chemotherapy has been reported to result in objective responses in inoperable tumors or after incomplete resections. To our knowledge, this is the first report of a very radiosensitive PB, which showed major tumor reduction after several fractions of radiotherapy without further tumor regression after additional chemotherapy with cisplatin and etoposide. The literature on the treatment of PB is reviewed.

[A case report of pulmonary blastoma--biphasic pulmonary blastoma].

We report a biphasic pulmonary blastoma in a 77-year-old man. In a routine chest radiographic examination, in the left upper lung field, a massive shadow that had been entirely absent one year before was detected. The preoperative imaging films showed a 10 x 8 cm, well-circumscribed solid tumor in the upper lobe of the left lung. A preoperative clinical diagnosis of primary lung cancer was considered. The intraoperative findings were that the tumor had invaded the visceral pleura with adhesion to the parietal pleura. Left upper lobectomy with lymph node sampling was performed, and since invasion of the pulmonary artery wall was confirmed, complete removal of tumor was not possible. The postoperative diagnosis was biphasic pulmonary blastoma. The patient was treated with radiotherapy up to a limit of 50 Gy, covering the area around of the left hilum. At the latest follow-up, 1 year postoperatively, the patient was clinically and radiologically free of the disease.

Radical resection of a pulmonary blastoma involving the mediastinum.

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.

Tumores malignos pleuropulmonares primitivos de la infancia / Malignant pleuropulmonary primary tumors in childhood

Se presentan 3 casos de tumores primitivos de origen pleuropulmonar:2 blastomas pleuropulmonares y un mesotelioma.Se obtuvieron inicialmente remisiones completas con quimioterapia convencional,cirugía y en uncaso se agregó radioterapia.Un paciente murió a los 14 meses y 2 están en progresión de enfermedad 12 y 14 meses después del diagnóstico.Terapia multimodal,cirugía,poliquimioterapia y radioterapia,juegan un rol importante en el tratamiento de estos tumores,sin embargo es insuficiente y no previenen la recurrencia local y/o metástasis.Se hace hincapié en el mal pronóstico de este tipo de tumores

Tumores malignos pleuropulmonares primitivos de la infancia / Malignant pleuropulmonary primary tumors in childhood

Se presentan 3 casos de tumores primitivos de origen pleuropulmonar:2 blastomas pleuropulmonares y un mesotelioma.Se obtuvieron inicialmente remisiones completas con quimioterapia convencional,cirugía y en uncaso se agregó radioterapia.Un paciente murió a los 14 meses y 2 están en progresión de enfermedad 12 y 14 meses después del diagnóstico.Terapia multimodal,cirugía,poliquimioterapia y radioterapia,juegan un rol importante en el tratamiento de estos tumores,sin embargo es insuficiente y no previenen la recurrencia local y/o metástasis.Se hace hincapié en el mal pronóstico de este tipo de tumores

Pulmonary blastoma: case report of a patient with a 7-year remission and review of chemotherapy experience in the world literature.

BACKGROUND: Pulmonary blastoma is a rare malignant neoplasm for which there currently are no treatment guidelines. METHODS: A patient with locally advanced pulmonary blastoma is described. The treatment modality is discussed and the world literature is reviewed with respect to the use of chemotherapy. RESULTS: A 54-year-old man had a 7-year disease free survival despite subtotal resection. He was treated with adjuvant radiotherapy and combination chemotherapy. Three cycles of cisplatin and etoposide were administered. The world literature was reviewed with regard to the use of adjuvant chemotherapy in the treatment of pulmonary blastoma. CONCLUSIONS: Surgery, adjuvant radiotherapy, and combination chemotherapy with cisplatin and etoposide should be considered in the treatment of patients with this rare pulmonary neoplasm.