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Cancer Feb 2023Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion that has a microscopic population of...
BACKGROUND
Pleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion that has a microscopic population of primitive small cells with or without rhabdomyoblastic features and may progress to type II or III PPB, whereas type Ir lacks primitive small cells.
METHODS
Children with suspected PPB were enrolled in the International PPB/DICER1 Registry. Pathology was centrally reviewed, and follow-up was ascertained annually.
RESULTS
Between 2006 and 2022, 205 children had centrally reviewed type I or Ir PPB; 39% of children with type I and 5% of children with type Ir PPB received chemotherapy. Outcomes were favorable, although 11 children (nine with type I and two with type Ir PPB) experienced progression to type II/III (n = 8) or regrowth of type I PPB at the surgical site (n = 3), none of whom received chemotherapy before progression. Age and cyst size in combination were more suitable than either factor alone in predicting whether a particular lesion was type I or Ir PPB.
CONCLUSIONS
For young children with type I PPB, outcomes are favorable, but complete resection is indicated because of the risk for progression. Chemotherapy may be useful in a subset of children at increased risk for recurrence/progression. Efforts to risk stratify children with type I PPB to optimize outcomes while reducing treatment-related side effects are underway.
Topics: Child; Humans; Child, Preschool; Pulmonary Blastoma; Lung Neoplasms; Registries; Ribonuclease III; Drug-Related Side Effects and Adverse Reactions; DEAD-box RNA Helicases
PubMed: 36541021
DOI: 10.1002/cncr.34593 -
BMC Cancer Aug 2020Pulmonary blastoma (PB) is a rare lung primary malignancy with poorly understood risk factors and prognosis. We sought to investigate the epidemiologic features and...
BACKGROUND
Pulmonary blastoma (PB) is a rare lung primary malignancy with poorly understood risk factors and prognosis. We sought to investigate the epidemiologic features and long-term outcomes of PB.
METHODS
A population-based cohort study was conducted to quantify the death risk of PB patients. All subjects diagnosed with malignant PB from 1988 to 2016 were screened from the Surveillance, Epidemiology and End Results database. Cox regression model of all-cause death and competing risk analysis of cause-specific death were performed.
RESULTS
We identified 177 PB patients with a median survival of 108 months. The 5 and 10-year survival rate in all PB patients were 58.2 and 48.5%, as well as the 5 and 10-year disease-specific mortality were 33.5 and 38.6%. No sex or race disparities in incidence and prognosis was observed. The death risk of PB was significantly associated with age at diagnosis, clinical stage, histologic subtype and surgery treatment (p<0.01). On multivariable regression analyses, older age, regional stage and no surgery predicted higher risk of both all-cause and disease-specific death in PB patients.
CONCLUSION
We described the epidemiological characteristics of PB and identified its prognostic factors that were independently associated with worse clinical outcome.
Topics: Adolescent; Adult; Age Factors; Aged; Female; Follow-Up Studies; Humans; Incidence; Kaplan-Meier Estimate; Lung; Lung Neoplasms; Male; Middle Aged; Neoplasm Staging; Pneumonectomy; Pulmonary Blastoma; Retrospective Studies; Risk Factors; SEER Program; Survival Rate; Treatment Outcome; United States; Young Adult
PubMed: 32847556
DOI: 10.1186/s12885-020-07323-0 -
Pediatric Radiology Mar 2010Pulmonary blastoma is a rare malignant primary lung neoplasm typically seen in adults that accounts for 0.3-1.3% of lung malignancies. It has been categorized as a...
Pulmonary blastoma is a rare malignant primary lung neoplasm typically seen in adults that accounts for 0.3-1.3% of lung malignancies. It has been categorized as a subtype of sarcomatoid carcinoma according to the current World Health Organization Classification of Lung Tumours and is distinct from the more common pediatric lung tumor pleuropulmonary blastoma. We report a case of neonatal pulmonary blastoma, illustrating the imaging characteristics of this rare tumor. The subject of pediatric lung masses is confusing because the terms pleuropulmonary blastoma and pulmonary blastoma have been used interchangeably in previously reported cases. We recommend use of the current WHO classification to differentiate these lesions as additional cases are described. Additionally, we discuss distinctive cytogenetic features of this case.
Topics: Diagnosis, Differential; Humans; Infant, Newborn; Lung Neoplasms; Male; Pleural Neoplasms; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 19902198
DOI: 10.1007/s00247-009-1456-6 -
Surgery Today 2001Pulmonary blastomas are a group of rare malignant neoplasms subdivided into three categories: classic biphasic pulmonary blastoma (CBPB), well-differentiated fetal...
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.
Topics: Aged; Fatal Outcome; Hemoptysis; Humans; Lung Neoplasms; Male; Middle Aged; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Pulmonary Blastoma; Smoking
PubMed: 11381509
DOI: 10.1007/s005950170136 -
Journal of Cardiothoracic Surgery Jun 2013Pulmonary blastoma is a rare primary lung neoplasm, in that monophasic variety is far too rare. There are no specific clinical features seen for pulmonary blastoma;...
Pulmonary blastoma is a rare primary lung neoplasm, in that monophasic variety is far too rare. There are no specific clinical features seen for pulmonary blastoma; computed tomography and histopathology are diagnostic. Surgical excision is the treatment of choice; however, adjuvant chemotherapy and radiotherapy may be required in large and aggressive tumors.
Topics: Adolescent; Biopsy; Chemotherapy, Adjuvant; Diagnosis, Differential; Diagnostic Imaging; Female; Humans; Lung Neoplasms; Pulmonary Blastoma; Thoracotomy
PubMed: 23758909
DOI: 10.1186/1749-8090-8-144 -
Der Chirurg; Zeitschrift Fur Alle... Jan 2001Pulmonary blastoma is a rare non-small-cell lung cancer. In a statistically significant proportion of cases it is combined with cystic lung disease in children. The...
Pulmonary blastoma is a rare non-small-cell lung cancer. In a statistically significant proportion of cases it is combined with cystic lung disease in children. The biphasic pulmonary blastoma and the well-differentiated fetal adenocarcinoma are the most frequent manifestations of pulmonary blastoma. If metastatic disease is ruled out, surgical therapy should be guided by the principles for radical therapy of NSCLC. There are no sufficient recommendations yet for therapy of stage IIIb and IV (UICC 1997) disease.
Topics: Aged; Diagnosis, Differential; Humans; Lung; Lung Neoplasms; Male; Neoplasm Staging; Pneumonectomy; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 11225463
DOI: 10.1007/s001040051273 -
The Indian Journal of Chest Diseases &... 2012Pulmonary blastoma is a rare but aggressive malignancy of the lung comprising epithelial and mesenchymal elements that resemble fetal lung tissue. This report described...
Pulmonary blastoma is a rare but aggressive malignancy of the lung comprising epithelial and mesenchymal elements that resemble fetal lung tissue. This report described a case of an 18-year-old male who presented with cough and weight loss for a month. Computed tomography (CT) of the thorax revealed a large mass with mixed solid and cystic lesions on the right side of chest along with pleural effusion and mediastinal lymphadenopathy. Massive debulking was performed followed by chemotherapy. A biphasic pulmonary blastoma was diagnosed on histopathology.
Topics: Adolescent; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 23008928
DOI: No ID Found -
Zhonghua Zhong Liu Za Zhi [Chinese... Jan 2016
Topics: Humans; Lung Neoplasms; Pulmonary Blastoma
PubMed: 26796805
DOI: 10.3760/cma.j.issn.0253-3766.2016.01.008 -
Cancer Treatment and Research 1995
Review
Topics: Humans; Lung Neoplasms; Prognosis; Pulmonary Blastoma
PubMed: 7702991
DOI: 10.1007/978-1-4615-2630-8_16 -
Thoracic Cancer Oct 2020Biphasic pulmonary blastoma is a rare but lethal type of lung malignancy with characteristic histology of both epithelial and mesenchymal components. Previously reported...
Biphasic pulmonary blastoma is a rare but lethal type of lung malignancy with characteristic histology of both epithelial and mesenchymal components. Previously reported cases have been limited to presentation at advanced stages, suggesting that the clinical course of the disease is usually aggressive. Here, we report a case of incidental diagnosis of biphasic pulmonary blastoma by imaging surveillance in a patient previously treated for adenocarcinoma of the lung. The patient was diagnosed with stage 1 disease and underwent successful resection. Next-generation sequencing (NGS) revealed a high mutation burden, a finding not previously reported in a patient with biphasic pulmonary blastoma.
Topics: Adenocarcinoma of Lung; Female; High-Throughput Nucleotide Sequencing; Humans; Lung Neoplasms; Middle Aged; Neoplasm Staging; Pulmonary Blastoma
PubMed: 32833349
DOI: 10.1111/1759-7714.13629