ArticlePediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
Pleuropulmonary Blastoma: Evolution of an Entity as an Entry into a Familial Tumor Predisposition Syndrome.
Article in Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 77 citations in OpenAlex.
- Surgical Management of Congenital Lung Malformations: Indications, Timing, and Outcomes.Journal of chest surgery · 2026Review
- [General anatomic and histopathological diagnostic aspects of hereditary tumor syndromes : The role of pathology].Pathologie (Heidelberg, Germany) · 2026Review
- Structural insights into disease-associated mutations in the microRNA processing machinery.Experimental & molecular medicine · 2026Review
- Expanding the Molecular Data ofHuman mutation · 2026Article
- Pleuropulmonary Blastoma in a Pediatric Patient: A Case Report and Review of Current Diagnostic and Treatment Strategies.Clinical case reports · 2025Article
- Type III pleuropulmonary blastoma: A case report.Oncology letters · 2025Article
- Imaging findings of primary lung tumors in childrenDiagnostic and interventional radiology (Ankara, Turkey) · 2024Article
- Pediatric pleuropulmonary blastoma: analysis of four cases.BMC cancer · 2024Article
- Congenital lung malformations.Nature reviews. Disease primers · 2023Review
- KRAS Hijacks the miRNA Regulatory Pathway in Cancer.Cancer research · 2023Article
- Cancer-associated Mutations in Congenital Pulmonary Malformations: A Prospective Cohort.American journal of respiratory and critical care medicine · 2023Article
- Defining the spatial landscape of KRAS mutated congenital pulmonary airway malformations: a distinct entity with a spectrum of histopathologic features.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
- Fetal imaging of congenital lung lesions with postnatal correlation.Pediatric radiology · 2022Review
- International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines.Ecancermedicalscience · 2022Article
- Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature.Radiology case reports · 2021Article
- Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations.Pediatric blood & cancer · 2021Article
- Previously unreported somatic variants in two patients with pleuropulmonary blastoma with metastatic brain recurrence.Pediatric blood & cancer · 2021Article
- Deletion ofDisease models & mechanisms · 2020Article
- Imaging of DICER1 syndrome.Pediatric radiology · 2019Review
- NCAM1/FGF module serves as a putative pleuropulmonary blastoma therapeutic target.Oncogenesis · 2019Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
Abstract
Pleuropulmonary blastoma (PPB) is the most common primary malignant neoplasm of the lung in children. Like other solid dysontogenic neoplasms, this tumor typically presents before 7 years of age. The earliest manifestation is the presence of a lung cyst(s), which is usually recognized in the first year of life and is difficult to differentiate on the basis of imaging studies from non-neoplastic cysts of early childhood. From a multilocular cyst, PPB has the potential to progress to a high-grade multipatterned primitive sarcoma. More than 65% of all affected children have a heterozygous germline mutation in DICER1. The DICER1 PPB familial tumor predisposition syndrome is initially recognized in most cases on the basis of PPB alone but also by several other unique and characteristic extrapulmonary tumors, including pediatric cystic nephroma, nasal chondromesenchymal hamartoma, nodular lesions of the thyroid, embryonal rhabdomyosarcoma of the cervix, and ciliary body medulloepithelioma.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.