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Pediatric Blood & Cancer Mar 2007To evaluate the prognostic factors in a series of children affected by pleuropulmonary blastoma (PPB).
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.
Topics: Antineoplastic Combined Chemotherapy Protocols; Carboplatin; Chemotherapy, Adjuvant; Child; Child, Preschool; Combined Modality Therapy; Dactinomycin; Disease Progression; Disease-Free Survival; Doxorubicin; Epirubicin; Etoposide; Female; Follow-Up Studies; Humans; Ifosfamide; Infant; Kaplan-Meier Estimate; Lung Neoplasms; Male; Neoplasm Metastasis; Neoplasm Staging; Pleural Neoplasms; Pneumonectomy; Prognosis; Pulmonary Blastoma; Radiotherapy, Adjuvant; Retrospective Studies; Risk Factors; Second-Look Surgery; Treatment Outcome; Vincristine
PubMed: 16619221
DOI: 10.1002/pbc.20842 -
Archivos Argentinos de Pediatria Feb 2016Pleuropulmonary blastoma is a rare lung tumor of childhood that can occur with cystic or solid lesions, as a radiological finding with or without respiratory symptoms....
Pleuropulmonary blastoma is a rare lung tumor of childhood that can occur with cystic or solid lesions, as a radiological finding with or without respiratory symptoms. We report the case of a 2 year old toddler in his first pulmonary obstructive episode with suspected toracic malformation of the left upper lobe in his chest x-ray and tomography. Surgery was performed showing cystic malformation of the left upper lobe. We received the pathology report with diagnosis of type I pleuropulmonary blastoma. He began follow-up with Oncology initiating treatment with cyclophosphamide and vincristine, well tolerated. Currently, there is controversy about the management of congenital lung cysts, tilting the balance towards the surgical procedure because of serious difficulties in differentiating benign pulmonary cysts from pleuropulmonary blastoma without histopathologic review.
Topics: Child, Preschool; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 26914086
DOI: 10.5546/aap.2016.e25 -
European Journal of Pediatric Surgery :... Aug 2001We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass...
We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.
Topics: Antineoplastic Agents; Chemotherapy, Adjuvant; Child, Preschool; Dactinomycin; Disease-Free Survival; Humans; Ifosfamide; Lung Neoplasms; Male; Pleural Neoplasms; Pneumonectomy; Pulmonary Blastoma; Vincristine
PubMed: 11558020
DOI: 10.1055/s-2001-17149 -
Indian Pediatrics Dec 1993
Topics: Combined Modality Therapy; Diagnosis, Differential; Drug Therapy; Empyema, Pleural; Fatal Outcome; Humans; Lung; Lung Neoplasms; Male; Pulmonary Blastoma
PubMed: 8077035
DOI: No ID Found -
Kyobu Geka. the Japanese Journal of... Sep 2020A 63-year-old man was pointed out a mass lesion in his chest X-ray at a medical checkup and referred to our hospital for further examination. Chest computed tomography...
A 63-year-old man was pointed out a mass lesion in his chest X-ray at a medical checkup and referred to our hospital for further examination. Chest computed tomography showed a 4 cm-diameter tumor in the left upper lobe. He was diagnosed with adenocarcinoma by bronchoscopic biopsy. Therefore, we performed left upper lobectomy. The tumor was diagnosed as pulmonary blastoma with the differentiation of adenocarcinoma and large cell neuroendocrine carcinoma.
Topics: Adenocarcinoma; Adult; Carcinoma, Neuroendocrine; Cell Differentiation; Female; Humans; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma
PubMed: 32879279
DOI: No ID Found -
Australasian Radiology Aug 2001Serial plain radiographic, ultrasound and CT findings of an unusual case of pulmonary blastoma are described with a review of the literature. (Review)
Review
Serial plain radiographic, ultrasound and CT findings of an unusual case of pulmonary blastoma are described with a review of the literature.
Topics: Child; Humans; Lung Neoplasms; Male; Pneumothorax; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 11531773
DOI: 10.1046/j.1440-1673.2001.00881.x -
European Journal of Cardio-thoracic... Nov 2002Identification of clinical features suggestive of pulmonary blastoma (PB) through a retrospective comparison with cases of non-small cell lung cancer (NSCLC) operated...
OBJECTIVE
Identification of clinical features suggestive of pulmonary blastoma (PB) through a retrospective comparison with cases of non-small cell lung cancer (NSCLC) operated during the same period.
METHODS
Between 1977 and 1999, five patients were operated for PB at Geneva University Hospital (four women and one man, aged 32-46 years--mean 36.8) versus 1913 consecutive patients (1558 men and 355 women, mean age 61.2) for primary NSCLC. In the PB subgroup (0.3%), the pulmonary tumor was single, located in an upper lobe in all but one instance, and measured between 5 and 13 cm (mean 9.6), whereas in the total NSCLC group, 27% of patients had tumors <3 cm (T1), evenly distributed in both lungs. All but one PB patients were symptomatic, compared to 45% in the NSCLC group.
RESULTS
The five patients with PB underwent curative pulmonary excisions (lobectomy in three and pneumonectomy in two) with mediastinal lymph node sampling. Pathological examination revealed extensive tumor necrosis in four, and N2 lymph node metastases in four (in the total NSCLC group, N2 disease was diagnosed in 21%). Postoperatively, three PB patients received radio- and/or chemotherapy. Four patients died between six and 30 months after the operation (mean 15), whereas 5-year survival in the NSCLC group was 32%, with a median survival of 3.7 years; the fifth patient is alive 28 months later, without any sign of recurrence.
CONCLUSIONS
Compared to operated NSCLC, PB are rare, large, and symptomatic tumors; they affect younger patients and carry a worse prognosis.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma, Non-Small-Cell Lung; Combined Modality Therapy; Diagnosis, Differential; Female; Humans; Lung Neoplasms; Male; Middle Aged; Prognosis; Pulmonary Blastoma; Radiography; Retrospective Studies; Survival Rate
PubMed: 12414034
DOI: 10.1016/s1010-7940(02)00529-8 -
Turk Patoloji Dergisi 2024Pulmonary blastoma (PB) is an exceedingly rare and aggressive malignant lung neoplasm that has distinct biphasic morphology. In this report, we document rare...
Pulmonary blastoma (PB) is an exceedingly rare and aggressive malignant lung neoplasm that has distinct biphasic morphology. In this report, we document rare manifestations and molecular alterations in PB. A 59-year-old non-smoker female, presented with cough and hemoptysis for 4 months. The high-resolution computed tomography chest scan showed a 3.5x2.7 cm mass in the basal segment of the left lung. Positron emission tomography and computed tomography revealed a fluorodeoxyglucose avid lobulated mass in the superior segment of the lower lobe of the left lung. On core biopsy, the diagnosis of pleomorphic carcinoma in a background of adenocarcinoma was made. A definite diagnosis of pulmonary blastoma was established on the left lung lobectomy specimen based on morphological and immunohistochemical findings. Post-surgical biopsy from the scalp swelling showed metastatic deposits. On Next Generation Sequencing (NGS), in addition to conventional CTNNB1 gene mutation, new pathogenic MYCN and ATM gene mutations were detected. Post-chemotherapy, the patient was doing well after 10 months of close follow-up. PB exhibited rare associations in the form of non-smoker status, scalp metastasis, and MYCN and ATM gene mutations on NGS in addition to conventional CTNNB1 gene mutation. Large cohort studies are required to discover the incidence, significance and therapeutic implications of these co-existing pathogenic molecular alterations in PB.
Topics: Female; Humans; Middle Aged; Hemoptysis; Lung; Lung Neoplasms; N-Myc Proto-Oncogene Protein; Pulmonary Blastoma
PubMed: 38235566
DOI: 10.5146/tjpath.2023.01597 -
The Annals of Thoracic Surgery May 2002Pulmonary blastomas are rare lung tumors that morphologically resemble fetal pulmonary structure and can exist in two forms, biphasic and monophasic. We reviewed our...
BACKGROUND
Pulmonary blastomas are rare lung tumors that morphologically resemble fetal pulmonary structure and can exist in two forms, biphasic and monophasic. We reviewed our experience over a 12-year period with emphasis on the clinical features, management, and outcome.
METHODS
Patients with a diagnosis of pulmonary blastoma from January 1988 to July 1999 were identified from the database of the Department of Histopathology, Llandough Hospital, Cardiff. Specimens had been obtained from bronchoscopy, fine-needle aspiration, trucut biopsy, and thoracotomy.
RESULTS
Six patients were identified from 2,720 histologically proven lung cancers (0.2%). Median age was 35.5 years and sex ratio was equal. Overall, 4 patients underwent resection and are all alive (median, 43.5 months). Three of these had advanced tumors at presentation (stage IIIb or IV), two of which were successfully downstaged with neoadjuvant chemotherapy, and the third treated with postoperative radiotherapy. Nonresected cases succumbed at a median of 5.5 months.
CONCLUSIONS
Although pulmonary blastomas are rare, those affected represent a group of patients with advanced tumors for whom a coordinated approach from both oncologists and surgeons can achieve excellent medium-term results.
Topics: Adult; Aged; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Lung Neoplasms; Male; Middle Aged; Neoadjuvant Therapy; Neoplasm Staging; Pulmonary Blastoma; Survival Rate; Treatment Outcome
PubMed: 12022552
DOI: 10.1016/s0003-4975(02)03494-x -
General Thoracic and Cardiovascular... Feb 2009We report a rare case of biphasic pulmonary blastoma (BPB) with rapid progression in a 75-year-old man. Computed tomography (CT) of the chest revealed a well-defined...
We report a rare case of biphasic pulmonary blastoma (BPB) with rapid progression in a 75-year-old man. Computed tomography (CT) of the chest revealed a well-defined tumor shadow measuring 8 x 6 cm in the right upper lobe. CT-guided lung biopsy revealed malignant cells with sarcomatous elements. Right upper lobectomy and systemic lymph node dissection with chest wall resection were performed. Histopathologically, the tumor was composed of immature embryoniclike mesenchymal and epithelial components that resembled embryonic lung tissue. The final diagnosis was BPB classified as pathological stage IIB (T3N0M0). Two months later, the tumor recurred in the right supraclavicular lymph nodes. The patient died of respiratory failure due to tumor progression 7 months after surgery.
Topics: Aged; Biopsy; Diagnosis, Differential; Disease Progression; Fatal Outcome; Follow-Up Studies; Humans; Lung Neoplasms; Male; Pneumonectomy; Pulmonary Blastoma; Time Factors; Tomography, X-Ray Computed
PubMed: 19214452
DOI: 10.1007/s11748-008-0327-7