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 30 free, full-text research articles on human participants. First 3 results:

Insulin-like Growth Factor 2 Gene Expression Molecularly Differentiates Pleuropulmonary Blastoma and Embryonal Rhabdomyosarcoma.
 

Author(s): Rajkumar Venkatramani, Timothy J Triche, Larry Wang, Hiroyuki Shimada, Leo Mascarenhas

Journal: J. Pediatr. Hematol. Oncol.. 2015 Aug;37(6):e356-60.

 

The sarcomatous element in pleuropulmonary blastoma (PPB) is often histologically indistinguishable from embryonal rhabdomyosarcoma (ERMS). A diagnosis of PPB is often made after definitive surgical resection based on pathologic features, most notably the presence of hamartomatous ...

Last Updated: 18 Jul 2015

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Fibroblast Growth Factor 9 Regulation by MicroRNAs Controls Lung Development and Links DICER1 Loss to the Pathogenesis of Pleuropulmonary Blastoma.
 

Author(s): Yongjun Yin, Angela M Castro, Marrit Hoekstra, Thomas J Yan, Ajay C Kanakamedala, Louis P Dehner, D Ashley Hill, David M Ornitz

Journal:

 

Pleuropulmonary Blastoma (PPB) is the primary neoplastic manifestation of a pediatric cancer predisposition syndrome that is associated with several diseases including cystic nephroma, Wilms tumor, neuroblastoma, rhabdomyosarcoma, medulloblastoma, and ovarian Sertoli-Leydig cell tumor. ...

Last Updated: 18 May 2015

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Pleuropulmonary blastoma: a case report.
 

Author(s): Sümeyye Ekmekci, Anıl Aysal, Nur Olgun, Mustafa Olguner, Handan Çakmakci, Erdener Özer

Journal: Turk Patoloji Derg. 2015 ;31(1):68-71.

 

Pleuropulmonary blastoma is rare embryonal tumor of infancy and early childhood and it often arises from lung and more rarely from the parietal pleura. We present this entity which has no systematic data associated with its incidence in order to discuss clinical, histopathological, ...

Last Updated: 6 Jan 2015

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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 1 free, full-text review articles on human participants. First 3 results:

Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood.
 

Author(s): J C Manivel, J R Priest, J Watterson, M Steiner, W G Woods, M R Wick, L P Dehner

Journal: Cancer. 1988 Oct;62(8):1516-26.

 

The authors studied 11 pediatric intrathoracic neoplasms that share clinicopathologic features and constitute a specific tumor in children. These neoplasms were intrapulmonary, mediastinal, or pleural-based masses. A common histologic feature was the presence of small, primitive cells ...

Last Updated: 8 Nov 1988

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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: 1 Feb 2016

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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: 4 Jun 2016

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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; Goiter; Sarcoma; Pineoblastoma; Pituitary Tumors; Wilms Tumor

 

Last Updated: 28 Jul 2016

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