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Clinical Imaging 2004To determine the radiologic findings of pulmonary blastoma on chest radiograph and CT.
PURPOSE
To determine the radiologic findings of pulmonary blastoma on chest radiograph and CT.
MATERIALS AND METHODS
Chest radiographs and CT scans of five patients with pathologically proven pulmonary blastoma were retrospectively evaluated and radiologic findings were correlated with histopathologic findings.
RESULTS
On chest radiograph, the pulmonary blastoma manifested as a solitary parenchymal mass (n = 2), a solitary parenchymal nodule (n = 1), hilar bulging with fingerlike parenchymal opacities (n = 1), or opacification of hemithorax (n = 1). On CT, the pulmonary blastoma manifested as a solitary parenchymal mass (n = 2), a solitary parenchymal nodule (n = 1), an endobronchial mass with postobstructive pneumonitis (n = 1), or a parenchymal mass with multiple subpleural masses and pleural effusion (n = 1).
CONCLUSION
Pulmonary blastomas most commonly manifested as a solitary parenchymal mass on chest radiograph and CT. These radiologic features are nonspecific and many primary or metastatic tumors of the lung could be included in differential diagnosis.
Topics: Adult; Female; Humans; Lung Neoplasms; Male; Pulmonary Blastoma; Radiography, Thoracic; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 15050223
DOI: 10.1016/S0899-7071(03)00240-7 -
Seminars in Ultrasound, CT, and MR Feb 2022Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary... (Review)
Review
Pleuropulmonary blastomas are rare, potentially aggressive embryonal cancers of the lung parenchyma and pleural surfaces that account for 0.25%-0.5% of primary pulmonary malignancies in children. Pleuropulmonary blastomas are classified as cystic (type I), mixed cystic and solid (type II), and solid (type III). Pleuropulmonary blastoma occurs in the same age group (0-6 years) as other more common solid tumors such as neuroblastoma and Wilms tumor. Differential diagnosis includes metastasis from Wilms tumor and macrocystic congenital pulmonary airway malformation (CPAM). A key pathologic and genetic discriminator is the DICER1 germline mutation found in patients with pleuropulmonary blastoma. Imaging, histopathologic, and clinical data are important to use in conjunction in order to determine the diagnosis and risk stratification of pleuropulmonary blastomas. Survival varies from poor to good, depending on type. However, the spectrum of pleuropulmonary blastoma is insufficiently understood due to the variable presentation of this rare disease. We present a current review of the literature regarding pleuropulmonary blastomas in this article.
Topics: Child; Child, Preschool; Cystic Adenomatoid Malformation of Lung, Congenital; DEAD-box RNA Helicases; Diagnosis, Differential; Humans; Infant; Infant, Newborn; Lung Neoplasms; Multimodal Imaging; Pulmonary Blastoma; Ribonuclease III
PubMed: 35164911
DOI: 10.1053/j.sult.2021.05.007 -
Virchows Archiv : An International... Sep 2020Congenital pulmonary airway malformation (CPAM) occurs most commonly in infants. It is divided into 5 types. The most common types 1 and 2 are cystic, type 0 presents as...
Congenital pulmonary airway malformation (CPAM) occurs most commonly in infants. It is divided into 5 types. The most common types 1 and 2 are cystic, type 0 presents as bronchial buds without alveolar tissue, most likely corresponding to alveolar dysgenesis, while type 3 is composed of branching bronchioles and appears as a solid lesion. A defect in the epithelial-mesenchymal crosstalk might be the underlying mechanism for all. Type 4 is a peripheral cystic lesion with a thin cyst wall covered by pneumocytes. CPAM 4 has been mixed up with pleuropulmonary blastoma (PPB) type I and some authors question its existence. We investigated five cases of CPAM type 4 for the presence or absence of rhabdomyoblasts, and for markers associated with CPAM development. In addition, all cases were evaluated for mutations within the Dicer gene and for mutations of the RAS family of oncogenes. All five cases showed smooth muscle actin and desmin-positive cells; however, only one case showed a few cells positive for MyoD. The same case showed a mutation of Dicer 1. All cases were negative for mutations of the RAS family of genes. Fibroblast growth factor 10 was similarly expressed in all cases, and thus cannot be used to differentiate CPAM4 from PPB-I. Low expression of the proliferation marker Ki67 was seen in our CPAM 4 cases and the probable PPB-I case. YingYang-1 protein seems to play an active role in the development of PPB-I. CPAM 4 can be separated from PPB-I based on the presence of rhabdomyoblasts and mutations in Dicer 1 gene. These cells might not be numerous; therefore, all available tissue has to be evaluated. As CPAM 4 morphologically looks very similar to PPB-I, it might be speculated, that there exists a potential for progression from CPAM 4 to PPB-I, by acquiring somatic mutations in Dicer 1.
Topics: Adolescent; Biomarkers, Tumor; Cystic Adenomatoid Malformation of Lung, Congenital; DEAD-box RNA Helicases; Diagnosis, Differential; Disease Progression; Female; Genes, ras; Humans; Infant; Lung Neoplasms; Male; Middle Aged; Mutation; Pulmonary Blastoma; Ribonuclease III; Young Adult
PubMed: 32193603
DOI: 10.1007/s00428-020-02789-6 -
Kyobu Geka. the Japanese Journal of... Jan 2022A 62-year-old man was referred to our hospital for a lung tumor. Computed tomography (CT) of the chest showed a 62×55×68 mm well-circumscribed tumor in the upper lobe...
A 62-year-old man was referred to our hospital for a lung tumor. Computed tomography (CT) of the chest showed a 62×55×68 mm well-circumscribed tumor in the upper lobe of the right lung. A transbronchial lung biopsy was performed, but a diagnosis was not achieved. Positron emission tomography-CT demonstrated intense F-fluorodeoxyglucose uptake in the mediastinal side of the tumor. Surgery was performed under the suspicion of primary lung cancer. The intraoperative pathological examination indicated a non-small-cell carcinoma. Thus, right upper lobectomy and wedge resection of the right lower lobe were performed. Microscopically, the tumor was composed of epithelial components, mimicking fetal lung tissue and embryogenic stroma. Therefore, the tumor was diagnosed as a biphasic pulmonary blastoma (p-StageⅡA). After the post-operative adjuvant chemotherapy, he has remained healthy without recurrence six years after the surgery.
Topics: Fluorodeoxyglucose F18; Humans; Lung; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 35249079
DOI: No ID Found -
Indian Journal of Pathology &... 2019Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are...
Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such cases presenting clinically and radiologically as congenital pulmonary airway malformation. On histology, a final diagnosis of type I pleuropulmonay blastoma was made. Thereafter, chemotherapy was administered following complete surgical excision.
Topics: Child, Preschool; Cystic Adenomatoid Malformation of Lung, Congenital; Diagnosis, Differential; Drug Therapy; Female; Histological Techniques; Humans; Infant; Lung; Lung Neoplasms; Pleural Neoplasms; Pulmonary Blastoma
PubMed: 31611448
DOI: 10.4103/IJPM.IJPM_713_18 -
Journal of Cytology Apr 2009Pulmonary blastomas are rare lung neoplasms constituting 0.5% of all lung tumors. This tumor has an aggressive course and needs to be recognized on cytology. A...
Pulmonary blastomas are rare lung neoplasms constituting 0.5% of all lung tumors. This tumor has an aggressive course and needs to be recognized on cytology. A preoperative diagnosis of pulmonary blastoma is difficult to obtain by cytopathologic methods. A diagnosis of biphasic pulmonary blastoma should be considered when there is a dimorphic population of cells on cytology. A 30-year-old male presented with gradually progressing breathlessness and left-sided chest pain for the past one month. Chest radiograph and computed tomography of thorax revealed an anterior mediastinal mass that was subjected to ultrasound-guided fine-needle aspiration cytology. Aspiration cytology showed a highly cellular lesion with a dimorphic population of tumor cells in a necrotic background. The possibility of a non-small cell carcinoma was suggested. Subsequent histopathology revealed the tumor to be a pulmonary blastoma. The importance of recognizing the dimorphic population of cells in cytology is discussed.
PubMed: 21938157
DOI: 10.4103/0970-9371.55226 -
Journal of Radiology Case Reports Sep 2017Pulmonary blastomas are rare malignancies, representing 0.25% to 0.5% of all primary lung neoplasms with often aggressive progression and poor prognosis. Clinical...
Pulmonary blastomas are rare malignancies, representing 0.25% to 0.5% of all primary lung neoplasms with often aggressive progression and poor prognosis. Clinical management of pulmonary blastomas depends on histologic subtype, staging, and presentation, and may consist of surgery, chemotherapy, and radiation. Biphasic pulmonary blastoma is a subtype of pulmonary blastoma that exhibits biphasic histology, with both epithelial and mesenchymal malignant elements. We report a case of biphasic pulmonary blastoma in a 33-year-old female with 1 pack per day history of smoking for approximately 16 years, who presented with left-sided pleuritic chest pain on deep inspiration without otherwise significant pat medical history. Imaging evaluation using chest radiography, computed tomography, and magnetic resonance imaging identified a heterogenous, well-circumscribed, left lower lobe mass with extensive necrosis and hemorrhage. No lymphadenopathy or distant metastasis was detected through imaging evaluation. Surgical resection of the tumor followed by histopathological analysis confirmed a biphasic pulmonary blastoma.
Topics: Adult; Female; Humans; Lung; Lung Neoplasms; Magnetic Resonance Imaging; Perfusion Imaging; Pulmonary Blastoma; Smoking; Tomography, X-Ray Computed
PubMed: 29299105
DOI: 10.3941/jrcr.v11i9.3153 -
Annals of Thoracic and Cardiovascular... 2012We report a rare case of classic pulmonary blastema (CPB) without recurrence for 3 years after the operation. A 70-year-old man presented with cough and sputum for a...
We report a rare case of classic pulmonary blastema (CPB) without recurrence for 3 years after the operation. A 70-year-old man presented with cough and sputum for a month. Chest computed tomography (CT) showed a 5cm-sized mass in the right middle lobe. Bronchoscopic examination was performed, and the mass was suspected as adenocarcinoma of the lung. Right middle lobectomy and lymph node dissection were performed. The pathologic histology diagnosis was classic pulmonary blastoma, a subtype of biphasic pulmonary blastoma.
Topics: Adenocarcinoma; Adenocarcinoma of Lung; Aged; Biomarkers, Tumor; Biopsy; Bronchoscopy; Cough; Diagnostic Errors; Humans; Immunohistochemistry; Lung Neoplasms; Lymph Node Excision; Male; Pneumonectomy; Predictive Value of Tests; Pulmonary Blastoma; Sputum; Time Factors; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 22001215
DOI: 10.5761/atcs.cr.11.01693 -
Revue Des Maladies Respiratoires Nov 2010The authors report the case of a 24-year-old woman in complete remission 4 years after treatment for a biphasic pulmonary blastoma. After a left lower lobectomy, the...
The authors report the case of a 24-year-old woman in complete remission 4 years after treatment for a biphasic pulmonary blastoma. After a left lower lobectomy, the patient developed a local recurrence that was treated by chemotherapy. In the light of this case, the authors review the clinical, radiological and therapeutic features of this very rare malignant lung tumour.
Topics: Female; Humans; Lung Neoplasms; Pulmonary Blastoma; Young Adult
PubMed: 21111284
DOI: 10.1016/j.rmr.2010.04.008 -
Onkologie Dec 2006Classic biphasic pulmonary blastoma (CBPB) is a rare and aggressive primary malignancy, brain metastases of this type of tumor are even rarer. (Review)
Review
BACKGROUND
Classic biphasic pulmonary blastoma (CBPB) is a rare and aggressive primary malignancy, brain metastases of this type of tumor are even rarer.
CASE REPORT
A 51-year-old male patient with a solitary cerebral metastasis, diagnosed ten months after left pneumonectomy for a CBPB, was treated by surgery and accelerated hypofractionated radiotherapy.
RESULTS
The patient died 15 months after partial removal of the brain metastasis. Literature review revealed only 4 cases of solitary brain metastases from this type of malignancy. The present case is the second one reported with a combined treatment of surgery and radiotherapy resulting in the longest survival.
CONCLUSION
The best treatment for CBPB is difficult to determine because of the small number of cases, however, the combination of surgery with radiotherapy seems to be effective. The effectiveness of chemotherapy has not been ascertained.
Topics: Brain Neoplasms; Humans; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Rare Diseases
PubMed: 17202827
DOI: 10.1159/000096708