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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 -
Archivos Argentinos de Pediatria Jun 2018Pleuropulmonary Blastoma corresponds to a malignant primary lung disorder, exclusive of pediatric age, infrequent and of aggressive characteristics. Age on diagnosis is...
Pleuropulmonary Blastoma corresponds to a malignant primary lung disorder, exclusive of pediatric age, infrequent and of aggressive characteristics. Age on diagnosis is 1 month-12 years. Rates per sex are equal. It can be found inside pleura or lungs. Respiratory distress associated or not with pneumothorax, chest pain and fever are classical clinical signs. These symptoms could be misdiagnosed as pneumonia. Radiologically, a large mass near the pleura at the base of the right lung without air bronchogram is its most common form. It has three histological types: type I (cystic), type II (mixed) and type III (solid). Its treatment requires tumor excision and multimodal chemotherapy. Pleuropulmonary Blastoma type I has good prognosis; type II and III variants have lower survival. We report a 2-year-old girl, pointing to the clinicalradiological diagnosis.
Topics: Child, Preschool; Cough; Female; Hemoptysis; Humans; Prognosis; Pulmonary Blastoma; Survival
PubMed: 29756723
DOI: 10.5546/aap.2018.e455 -
Clinics in Perinatology Jun 2012Confusion, controversy, and uncertainty are all terms applicable to the diagnosis and management of congenital lung lesions both prenatally and postnatally. This review... (Review)
Review
Confusion, controversy, and uncertainty are all terms applicable to the diagnosis and management of congenital lung lesions both prenatally and postnatally. This review examines the current status of fetal diagnosis and treatment of these lesions; reviews the various classifications, including congenital cystic adenomatoid malformation/congenital pulmonary airway malformation, sequestrations, variants and hybrid lesions; discusses the risk of malignant transformation or misdiagnosis with pleuropulmonary blastoma; presents the arguments in favor and against resection of asymptomatic lesions, the timing of such resection, and the long-term pulmonary function after resection; and reviews the experience with thoracoscopic resection of congenital lung lesions.
Topics: Betamethasone; Bronchopulmonary Sequestration; Cystic Adenomatoid Malformation of Lung, Congenital; Diagnosis, Differential; Female; Humans; Hydrops Fetalis; Lung Neoplasms; Pregnancy; Prenatal Diagnosis; Pulmonary Atresia; Pulmonary Blastoma; Ultrasonography
PubMed: 22682383
DOI: 10.1016/j.clp.2012.04.009 -
Advances in Anatomic Pathology May 2011Malignant biphasic tumors of the lungs are rare primary tumors of the bronchopulmonary system. These tumors are composed of malignant epithelial and mesenchymal elements... (Review)
Review
Malignant biphasic tumors of the lungs are rare primary tumors of the bronchopulmonary system. These tumors are composed of malignant epithelial and mesenchymal elements and together comprise <2% of all primary pulmonary neoplasms. The tumors belonging to this group include pulmonary blastoma, pleuropulmonary blastoma, and carcinosarcoma. In this study, the clinicopathologic features, immunohistochemical phenotype, molecular biological characteristics, and the differential diagnosis of these uncommon neoplasms are discussed.
Topics: Adult; Carcinosarcoma; Child; Child, Preschool; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Lung; Lung Neoplasms; Male; Prognosis; Pulmonary Blastoma
PubMed: 21490435
DOI: 10.1097/PAP.0b013e318216980c -
Tumori 2002There is an increased risk of cancer after organ transplantation mainly due to the immunosuppressive therapy required in these patients. We report a case of biphasic...
There is an increased risk of cancer after organ transplantation mainly due to the immunosuppressive therapy required in these patients. We report a case of biphasic pulmonary blastoma in an adult male who underwent liver transplant for hepatocellular carcinoma in March 1999, followed by immunosuppressive treatment and adjuvant chemotherapy with epirubicin. Disease-free survival lasted 18 months, then a diagnosis of biphasic pulmonary blastoma was made and the patient underwent a lung lobectomy. Five months after surgical resection a recurrence of this rare tumor was recorded and two cycles of cisplatin + etoposide and ifosfamide + etoposide and one cycle of second-line chemotherapy with vinorelbine were administered. The tolerability and the efficacy of this treatment were poor. The patient died less than one year after diagnosis. To our knowledge this is the first reported case of pulmonary blastoma in a transplant patient. Our findings confirm that organ transplant recipients deserve long-term medical surveillance also in the absence of graft complications, and that pulmonary blastoma is an aggressive tumor with a poor prognosis.
Topics: Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Hepatocellular; Chemotherapy, Adjuvant; Cisplatin; Disease-Free Survival; Etoposide; Fatal Outcome; Humans; Ifosfamide; Immunosuppressive Agents; Liver Neoplasms; Liver Transplantation; Lung Neoplasms; Male; Middle Aged; Prognosis; Pulmonary Blastoma; Risk Factors; Vinblastine; Vinorelbine
PubMed: 12088262
DOI: 10.1177/030089160208800219 -
Pediatric Surgery International Jul 1999Pulmonary 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...
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.
Topics: Abdominal Pain; Adult; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Causality; Child, Preschool; Cough; Diagnosis, Differential; Dyspnea; Female; Fever; Humans; Lung Neoplasms; Pulmonary Blastoma; Radiotherapy, Adjuvant; Tomography, X-Ray Computed
PubMed: 10415291
DOI: 10.1007/s003830050604 -
European Journal of Pediatric Surgery :... Oct 2019The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and...
Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution.
PURPOSE
The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB).
MATERIALS AND METHODS
An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed.
RESULTS
A total of 51 patients with a pathologic diagnosis of CPAM ( = 45; 88.2%) or PPB ( = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann-Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; = 0.003) for symptomatic versus asymptomatic patients, respectively.
CONCLUSION
CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult.
Topics: Asymptomatic Diseases; Child; Child, Preschool; Cystic Adenomatoid Malformation of Lung, Congenital; Databases, Factual; Disease Progression; Female; Humans; Infant; Infant, Newborn; Length of Stay; Male; Postoperative Period; Pulmonary Blastoma; Retrospective Studies; Thoracotomy; Time Factors
PubMed: 29920635
DOI: 10.1055/s-0038-1661333 -
Surgery Today 1999A 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...
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.
Topics: Adult; Female; Genes, p53; Humans; Immunohistochemistry; Lung Neoplasms; Polymorphism, Single-Stranded Conformational; Pulmonary Blastoma
PubMed: 10483763
DOI: 10.1007/BF02482333 -
The Journal of Pediatrics Jul 2011
Topics: Chest Tubes; Child, Preschool; Cough; Humans; Lung Neoplasms; Male; Pneumonectomy; Pneumothorax; Pulmonary Blastoma; Radiography
PubMed: 21349540
DOI: 10.1016/j.jpeds.2010.12.056 -
The European Respiratory Journal Jun 2016
Topics: Adult; DEAD-box RNA Helicases; Female; Humans; Infant; Lung Neoplasms; Mutation; Neoplasm Staging; Pulmonary Blastoma; Ribonuclease III
PubMed: 27126690
DOI: 10.1183/13993003.00172-2016