Pleuropulmonary blastoma

Common Name(s)

Pleuropulmonary blastoma

Pleuropulmonary blastoma (PPB) is a rare pediatric tumor of the lung that arises during fetal lung development and is often part of an inherited cancer syndrome ({5:Hill et al., 2009}). PPBs contain both epithelial and mesenchymal cells. Early in tumorigenesis, cysts form in lung airspaces, and these cysts are lined with benign-appearing epithelium. Mesenchymal cells susceptible to malignant transformation reside within the cyst walls and form a dense 'cambium' layer beneath the epithelial lining. In a subset of patients, overgrowth of the mesenchymal cells produces a sarcoma, a transition that is associated with a poorer prognosis ({6:Priest et al., 1996}). In approximately 35% of families in which a child has PPB, the patient or a family member manifests 1 or more additional conditions from an unusual array of dysontogenetic-dysplastic and malignant conditions, known as the 'PPB family tumor and dysplasia syndrome' (PPBFTDS). Cystic nephroma, which are benign lesions of the kidney, are found in 9 to 10% of family members affected by PPB (summary by {1:Bahubeshi et al., 2010}). Larger studies have shown that DICER1 mutations are associated with a variety of tumor types, indicating that this disorder can be considered a tumor predisposition syndrome (summary by {7:Slade et al., 2011}).
 

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Condition Specific Organizations

Following organizations serve the condition "Pleuropulmonary blastoma" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Pleuropulmonary blastoma" returned 16 free, full-text research articles on human participants. First 3 results:

FGF10 Signaling differences between type I pleuropulmonary blastoma and congenital cystic adenomatoid malformation.
 

Author(s): Guillaume Lezmi, Virginie Verkarre, Naziha Khen-Dunlop, Shamila Vibhushan, Alice Hadchouel, Caroline Rambaud, Marie-Christine Copin, Jean-Luc Rittie, Alexandra Benachi, Jean-Christophe Fournet, Christophe Delacourt

Journal:

 

Type I pleuropulmonary blastoma (PPB) and congenital cystic adenomatoid malformation of the lung (CCAM) are cystic lung diseases of childhood. Their clinical and radiological presentations are often similar, and pathologic discrimination remains difficult in many cases. As a consequence, ...

Last Updated: 15 Nov 2013

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Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry.
 

Author(s): Kris Ann P Schultz, M Cristina Pacheco, Jiandong Yang, Gretchen M Williams, Yoav Messinger, D Ashley Hill, Louis P Dehner, John R Priest

Journal: Gynecol. Oncol.. 2011 Aug;122(2):246-50.

 

Pleuropulmonary blastoma (PPB) is a childhood cancer arising from pleuropulmonary mesenchyme. This neoplasm is a sentinel disease in a familial tumor syndrome recently found to be associated with germline mutations in DICER1. Observations of ovarian sex cord-stromal tumors (OSCST) ...

Last Updated: 18 Jul 2011

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Bilateral cystic nephroma with pleuropulmonary blastoma.
 

Author(s): Anand Kumar Bhardwaj, Prem Dass Sharma, Amit Mittal, Akshay Sharma

Journal:

 

Cystic nephroma is a rare benign renal neoplasm that is purely cystic and is lined by an epithelium. Bilateral cystic nephromas are even rarer with only a handful cases reported in the literature. A case of a 2-year-old male child who presented with bilateral renal cystic masses later ...

Last Updated: 12 Jun 2012

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Pleuropulmonary blastoma" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

International Pleuropulmonary Blastoma (PPB) Treatment and Biology Registry
 

Status: Recruiting

Condition Summary: Pleuropulmonary Blastoma

 

Last Updated: 16 Aug 2013

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DICER1-related Pleuropulmonary Blastoma Cancer Predisposition Syndrome: A Natural History Study
 

Status: Recruiting

Condition Summary: Pleuropulmonary Blastoma; Cystic Nephroma; Ovarian Sertoli-Leydig Cell Tumors; Ocular Medulloepithelioma; Nasal Chondromesenchymal Hamartoma

 

Last Updated: 11 Nov 2014

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Elucidating the Genetic Basis of the Pleuropulmonary Blastoma (PPB) Familial Cancer Syndrome
 

Status: Recruiting

Condition Summary: Pleuropulmonary Blastoma; Cystic Nephroma; Sertoli-Leydig Cell Tumor of Ovary; Medulloepithelioma; Embryonal Rhabdomyosarcoma of Cervix

 

Last Updated: 9 Sep 2014

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