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Review of the Paediatric Pleuropulmonary Blastoma French Series (PPB)

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ClinicalTrials.gov Identifier: NCT03044834
Recruitment Status : Recruiting
First Posted : February 7, 2017
Last Update Posted : April 9, 2018
Sponsor:
Information provided by (Responsible Party):
Rennes University Hospital

Tracking Information
First Submitted Date January 16, 2017
First Posted Date February 7, 2017
Last Update Posted Date April 9, 2018
Actual Study Start Date January 9, 2017
Estimated Primary Completion Date July 9, 2018   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures
 (submitted: February 3, 2017)
  • 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 Measures Same as current
Change History Complete list of historical versions of study NCT03044834 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures
 (submitted: February 3, 2017)
  • 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 Measures Same as current
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title Review of the Paediatric Pleuropulmonary Blastoma French Series
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 french cases were collected, but no update has been performed since. The aim of this retrospective review of the cases since 2000, is to audit the care of PPB patients in France and update the French rare tumour database.

Evoking PPB diagnosis is difficult when imaging shows a neonatal cystic lesion. There are no radiologic criteria in the literature that differentiate congenital pulmonary cystic lesion and PPB type 1. Radiological presentation is however overlapping. Another aim of this study will be to look for a predictive sign of type 1 PPB.

Detailed Description Multicentre retrospective study
Study Type Observational
Study Design Observational Model: Cohort
Time Perspective: Retrospective
Target Follow-Up Duration Not Provided
Biospecimen Not Provided
Sampling Method Non-Probability Sample
Study Population Patients born between 01/01/2000 and 01/01/2016, followed up for PPB, treated in a French department of paediatric oncology or paediatric surgery.
Condition Pleuropulmonary Blastoma
Intervention Other: PPB
Global current care
Study Groups/Cohorts Pleuropulmonary Blastoma
  • Patients born between 01/01/2000 and 01/01/2016 ;
  • Followed up for PPB
  • Treated in a French department of paediatric oncology or paediatric surgery
  • Study agreement
Intervention: Other: PPB
Publications * Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status Recruiting
Estimated Enrollment
 (submitted: February 3, 2017)
40
Original Estimated Enrollment Same as current
Estimated Study Completion Date July 9, 2018
Estimated Primary Completion Date July 9, 2018   (Final data collection date for primary outcome measure)
Eligibility Criteria

Inclusion Criteria:

  • Patients born between 01/01/2000 and 01/01/2016 ;
  • Followed up for PPB
  • Treated in a French department of paediatric oncology or paediatric surgery
  • Study agreement

Exclusion Criteria:

  • Part of the care out of France
  • Study disagreement
Sex/Gender
Sexes Eligible for Study: All
Ages 1 Year to 17 Years   (Child)
Accepts Healthy Volunteers No
Contacts
Contact: Alexis ARNAUD, MD alexis.arnaud@chu-rennes.fr
Listed Location Countries France
Removed Location Countries  
 
Administrative Information
NCT Number NCT03044834
Other Study ID Numbers 35RC15_3009_PPB
15.18 ( Other Identifier: Comité d'Ethique Régional de Rennes )
15.468 ( Other Identifier: CCTIRS )
915465 ( Other Identifier: CNIL )
Has Data Monitoring Committee No
U.S. FDA-regulated Product Not Provided
IPD Sharing Statement Not Provided
Responsible Party Rennes University Hospital
Study Sponsor Rennes University Hospital
Collaborators Not Provided
Investigators
Principal Investigator: Alexis ARNAUD, MD Rennes University Hospital
PRS Account Rennes University Hospital
Verification Date April 2018