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International Journal of Surgical... Apr 2010Pulmonary sarcomatoid carcinomas (PSCs) are currently defined as poorly differentiated non-small-cell carcinomas containing a component with sarcoma or sarcoma-like... (Review)
Review
Pulmonary sarcomatoid carcinomas (PSCs) are currently defined as poorly differentiated non-small-cell carcinomas containing a component with sarcoma or sarcoma-like (spindle and/or giant cell) features. They consist of 5 major histological variants, namely pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The segregation of PSCs into a distinct clinicopathologic entity seems justified on the basis of morphologic, behavioral, and genotypic/phenotypic attributes. As a group, PSCs generally run an aggressive clinical course and may cause major difficulties in the differential diagnosis with other primary and secondary malignancies of the lung. At present, PSCs are believed to represent a family of carcinomas "in transition," in which diverse pathways of clonal evolution account for histological differences of a common ancestor lesion. The sarcomatous or sarcomatoid component of these tumors is thought to derive from carcinoma cells during the progression of carcinogenesis through the activation of an epithelial-mesenchymal transition program leading to sarcomatous transformation or metaplasia (conversion paradigm). Conceivably, targeting the epithelial-mesenchymal transition program could become a valid therapeutic strategy for these life-threatening tumors, whose sensitivity to current medical manipulation is disappointing.
Topics: Biomarkers, Tumor; Carcinoma; Carcinoma, Giant Cell; Carcinosarcoma; Humans; Lung Neoplasms; Neoplasms, Multiple Primary; Pulmonary Blastoma; Sarcoma
PubMed: 19124452
DOI: 10.1177/1066896908330049 -
Pediatrics Apr 2021Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the...
BACKGROUND
Pediatric lung lesions are a group of mostly benign pulmonary anomalies with a broad spectrum of clinical disease and histopathology. Our objective was to evaluate the characteristics of children undergoing resection of a primary lung lesion and to identify preoperative risk factors for malignancy.
METHODS
A retrospective cohort study was conducted by using an operative database of 521 primary lung lesions managed at 11 children's hospitals in the United States. Multivariable logistic regression was used to examine the relationship between preoperative characteristics and risk of malignancy, including pleuropulmonary blastoma (PPB).
RESULTS
None of the 344 prenatally diagnosed lesions had malignant pathology ( < .0001). Among 177 children without a history of prenatal detection, 15 (8.7%) were classified as having a malignant tumor (type 1 PPB, = 11; other PPB, = 3; adenocarcinoma, = 1) at a median age of 20.7 months (interquartile range, 7.9-58.1). Malignancy was associated with the DICER1 mutation in 8 (57%) PPB cases. No malignant lesion had a systemic feeding vessel ( = .0427). The sensitivity of preoperative chest computed tomography (CT) for detecting malignant pathology was 33.3% (95% confidence interval [CI]: 15.2-58.3). Multivariable logistic regression revealed that increased suspicion of malignancy by CT and bilateral disease were significant predictors of malignant pathology (odds ratios of 42.15 [95% CI, 7.43-340.3; < .0001] and 42.03 [95% CI, 3.51-995.6; = .0041], respectively).
CONCLUSIONS
In pediatric lung masses initially diagnosed after birth, the risk of PPB approached 10%. These results strongly caution against routine nonoperative management in this patient population. DICER1 testing may be helpful given the poor sensitivity of CT for identifying malignant pathology.
Topics: Child, Preschool; Cohort Studies; DEAD-box RNA Helicases; Female; Humans; Infant; Infant, Newborn; Length of Stay; Lung Neoplasms; Mutation; Neoplasm Metastasis; Pregnancy; Prenatal Diagnosis; Pulmonary Blastoma; Respiratory Distress Syndrome, Newborn; Retrospective Studies; Ribonuclease III; Tomography, X-Ray Computed
PubMed: 33762310
DOI: 10.1542/peds.2020-028357 -
The New England Journal of Medicine Jun 2018
Topics: DNA Helicases; Female; Germ-Line Mutation; Humans; Infant; Lung Neoplasms; Male; Nuclear Proteins; Pedigree; Pulmonary Blastoma; Teratoma; Transcription Factors
PubMed: 29874541
DOI: 10.1056/NEJMc1803354 -
Neonatology 2017
Topics: Humans; Lung; Pulmonary Blastoma; Respiratory System Abnormalities
PubMed: 27562209
DOI: 10.1159/000447992 -
Journal of Thoracic Oncology : Official... Apr 2007Biphasic pulmonary blastoma is a rare malignancy of the lung composed of proliferating epithelial and mesenchymal cells with a high vessel density at the tumor...
Biphasic pulmonary blastoma is a rare malignancy of the lung composed of proliferating epithelial and mesenchymal cells with a high vessel density at the tumor periphery. We report a rare case of renal metastasis of biphasic pulmonary blastoma that responded to sorafenib, an oral multikinase inhibitor. After 2 months of treatment with sorafenib, the renal tumor size decreased sufficiently to allow a safe laparoscopic radical nephrectomy. We believe that further studies are warranted to confirm the possible effects of sorafenib on pulmonary blastomas.
Topics: Administration, Oral; Adult; Benzenesulfonates; Biopsy, Needle; Chemotherapy, Adjuvant; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Immunohistochemistry; Kidney Neoplasms; Lung Neoplasms; Neoplasm Staging; Nephrectomy; Niacinamide; Phenylurea Compounds; Pneumonectomy; Pulmonary Blastoma; Pyridines; Risk Assessment; Sorafenib; Treatment Outcome
PubMed: 17409808
DOI: 10.1097/01.JTO.0000263719.76944.0a -
European Journal of Cardio-thoracic... Dec 2016
Topics: Child, Preschool; Female; Humans; Lung; Pleura; Pulmonary Blastoma; Radiography; Sternotomy; Tomography, X-Ray Computed
PubMed: 27341849
DOI: 10.1093/ejcts/ezw217 -
Anticancer Research Nov 2015We report a case of a young male who presented with an unresectable, centrally-located classic biphasic pulmonary blastoma (CBPB) involving his bilateral mainstem...
We report a case of a young male who presented with an unresectable, centrally-located classic biphasic pulmonary blastoma (CBPB) involving his bilateral mainstem bronchi and esophagus and a synchronous right testicular seminoma. CBPB is a rare and aggressive tumor that most commonly presents as a solitary mass in the periphery of the lung. Surgical resection is the preferred treatment for CBPB, as chemotherapy and radiation have demonstrated limited effectiveness. In the current case, four cycles of cisplatin, ifosfamide, and etoposide with concurrent radiotherapy resulted in a favorable response at three months. Currently he optimal treatment for unresectable pulmonary blastomas remains undefined.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Cisplatin; Esophageal Neoplasms; Etoposide; Humans; Ifosfamide; Lung Neoplasms; Male; Mediastinal Neoplasms; Neoplasm Staging; Neoplasms, Multiple Primary; Prognosis; Pulmonary Blastoma; Testicular Neoplasms
PubMed: 26504071
DOI: No ID Found -
Pneumonologia I Alergologia Polska 1999During the last decade more diagnoses of pulmonary blastoma were made worldwide than in previous decades. Whether this increased frequency is caused by better diagnostic... (Review)
Review
During the last decade more diagnoses of pulmonary blastoma were made worldwide than in previous decades. Whether this increased frequency is caused by better diagnostic procedures (immunochemistry, electron microscopy) or by the growing number of patients having this neoplasm is difficult to distinguish. We present controversies concerning pulmonary blastoma and related biphasic primary pulmonary neoplasms. We agree with Wick et al. that cases in children (called pleuropulmonary blastoma) should be differentiated from cases in adults, which show many similarities to bronchogenic lung cancer. However, our opinion is that the new classification system proposed by Wick et al. does not take into account cases of PB in young adults with intropulmonary growth of the tumor.
Topics: Adult; Carcinoma; Child; Diagnosis, Differential; Humans; Lung Neoplasms; Prevalence; Pulmonary Blastoma
PubMed: 10570649
DOI: No ID Found -
Indian Journal of Pathology &... Dec 1994
Review
Topics: Adult; Female; Humans; Lung Neoplasms; Pulmonary Blastoma
PubMed: 8613168
DOI: No ID Found -
Respiration; International Review of... 2012A 62-year-old patient diagnosed with pulmonary blastoma with submandibular, scrotum and adrenal metastases was admitted to Sotiria General Hospital in Athens. No other...
A 62-year-old patient diagnosed with pulmonary blastoma with submandibular, scrotum and adrenal metastases was admitted to Sotiria General Hospital in Athens. No other such case has been published to date. The patient started receiving chemotherapy, but the scrotum metastasis grew rapidly and erupted. This led to sepsis despite surgical excision of infected and necrotic tissues and intravenous antibiotics. Treatment strategy in pulmonary blastoma should be defined by a multidisciplinary team, and surgical treatment should be considered as quickly as possible when such a tumor is suspected.
Topics: Adrenal Gland Neoplasms; Biopsy; Bronchoscopy; Diagnosis, Differential; Fatal Outcome; Genital Neoplasms, Male; Humans; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Scrotum; Submandibular Gland Neoplasms; Tomography, X-Ray Computed
PubMed: 21447935
DOI: 10.1159/000324265