Clinical Trial: Review of the Paediatric Pleuropulmonary Blastoma French Series

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational

Official Title: Review of the Paediatric Pleuropulmonary Blastoma French Series

Brief Summary:

Pleuropulmonary blastoma is a rare embryonic malignant tumour that remains the most frequent malignant tumour of the lung in childhood. The International Pleuro pulmonary Blastoma Registry (IPPB) found only 220 cases in 2009 and 350 in 2015. In France, 20 cases were identified in 2009.

Three histologies are described: type 1 purely cystic, type 2 combined and type 3 solid. Median age at diagnostic is 12 months, 35 months and 41 months respectively. Evolution is possible from type 1 to type 2 or 3 in 10% of the cases.

Since 2009, DICER 1 mutations research is proposed systematically to all families.

PPB symptoms are usually non-specific. Diagnostic is evoked when imaging work up shows bubbles or solid lesions, and confirmed by pathological analysis. However the diagnosis can be difficult because of the proximity with congenital cystic adenomatoid malformation.

The French society of paediatric oncology recommends surgery at first instance. PPB type 1 remains a problem because some are still misdiagnosed as CCAM, a benign lesion. Chemotherapy depends on the PPB type and the quality of the resection. There is a real interest to analyse the French series.

The prognosis of type 2 and 3 is low with a 5 years survival rate of 45-60%, whereas type 1 survival rate is 91%. The French experience reports a 100% survival rate in type 1 and 48% in type 2 and 3. Other prognostic factors are initial size of the tumour, extra pulmonary invasion and quality of surgery.

Early local relapses are possible and late ones concern more often type 2 and 3 with more cerebral metastasis.

In 2009, the frenc

Detailed Summary: Multicentre retrospective study
Sponsor: Rennes University Hospital

Current Primary Outcome:

  • Overall survival [ Time Frame: Within 5 years from the diagnosis ]
  • Progression free survival [ Time Frame: Within 5 years from the diagnosis ]
    Characterize the evolution and the global care of PPB in the French series


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • State of PPB surgical care [ Time Frame: Within 5 years from the diagnosis ]
  • Radiology sign [ Time Frame: Within 5 years from the diagnosis ]
    Identification of a type 1 PPB predictive radiological sign


Original Secondary Outcome: Same as current

Information By: Rennes University Hospital

Dates:
Date Received: January 16, 2017
Date Started: January 2017
Date Completion: July 2017
Last Updated: February 3, 2017
Last Verified: February 2017