Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma

Grigoletto, Veronica; Tagarelli, Arianna; Sparber‐Sauer, Monika; Kościelniak, Ewa; Orbach, Daniel; Duplan, Marcel; Stachowicz‐Stencel, Teresa; Bień, Ewa; Almaraz, Ricardo López; Ben-Ami, Tal; Pourtsidis, Apostolos; Österlundh, Gustaf; Mazánek, Pavel; Zsíros, József; Farinha, Nuno; Ferrari, Andrea; Bisogno, Gianni

doi:10.1007/s00431-019-03566-7

Cited by 9 publications

(6 citation statements)

References 19 publications

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“…Estimates focused on cancers primarily affecting adults given the quality of available incidence and treatment data. Primarily pediatric cancers (e.g., blastomas) were not included in global estimates given paucity of global incidence and treatment data and their relatively low incidence [20] , [91] . It is unlikely that including pediatric cancers would substantially change estimates given their low global incidence.…”

Section: Methodsmentioning

confidence: 99%

Global burden of ototoxic hearing loss associated with platinum-based cancer treatment: A systematic review and meta-analysis

Dillard

Lopez-Perez

Martinez

et al. 2022

Cancer Epidemiology

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Section: Methodsmentioning

confidence: 99%

Global burden of ototoxic hearing loss associated with platinum-based cancer treatment: A systematic review and meta-analysis

Dillard

Lopez-Perez

Martinez

et al. 2022

Cancer Epidemiology

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“…As no prospective or comparative prospective studies have been published in PPB so far, all recommendations presented here are therefore based on a level IV or V grade of evidence. 5,9,[12][13][14][15][16][17][18][19] 3 RECOMMENDATIONS…”

Section: Methodsmentioning

confidence: 99%

“…Due to the rarity of PPB and the necessary multidisciplinary treatment, a tumor board discussion is highly recommended at diagnosis and during therapy (Level V, Grade A). The enrolment of patients in a prospective trial if available and data collection in national or international databases should be proposed to patients and families 14 (http://www.PPBregistry.org) (Level IV, Grade A). Even if many aspects of PPB treatment could not be fully supported by evidence‐based medicine, EXPeRT group proposes a possible overall strategy in order to help physicians to treat patients.…”

Section: Recommendationsmentioning

confidence: 99%

Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Bisogno

Sarnacki

Stachowicz‐Stencel

et al. 2021

Pediatric Blood & Cancer

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Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico‐pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo‐ or adjuvant treatments can be challenging taking into account potential long‐term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union‐funded project PARTNER (Paediatric Rare Tumours Network ‐ European Registry).

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“…Epidemiological data and source of information have previously been described 6 . Patients were retrospectively and some also prospectively collected within eight national registries from Italy, France, Greece, Poland, Spain, Israel, Slovakia and Germany (CWS).…”

Section: Methodsmentioning

confidence: 99%

Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis

Sparber‐Sauer

Tagarelli

Seitz

et al. 2021

Pediatric Blood & Cancer

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Background: Children with progressive (PD) or relapsed disease (RD) of pleuropulmonary blastoma (PPB) type II/III are known to have a very poor outcome. Methods: A retrospective review of children registered in national and European databases and trials (2000-2018) with diagnosis of PPB type II/III and PD or RD was performed. Results: A total of 35 patients with PPB were analysed: patients with PD (n = 9) and RD (n = 26). Patients experienced PD at the median age of 3.9 years [range, 0.5-17.8] despite surgery, chemotherapy (CHT, n = 9) and radiotherapy (RT, n = 1) with a median time to progression of 0.58 years [range, 0.02-1.27] from diagnosis. All of them died. Patients suffered from RD at the median age of 4.3 years [1.7-15.1], median delay to relapse 1.03 years [range, 0.03-2.95]. RD occurred locally (n = 12), combined (n = 1) and in metastatic sites (n = 13): central nervous system (n = 11) and unspecified site (n = 2). Patients were treated with salvage CHT (n = 20), surgery (n = 10) ± RT (n = 10).

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Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma

Cited by 9 publications

References 19 publications

Global burden of ototoxic hearing loss associated with platinum-based cancer treatment: A systematic review and meta-analysis

Global burden of ototoxic hearing loss associated with platinum-based cancer treatment: A systematic review and meta-analysis

Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations

Children with progressive and relapsed pleuropulmonary blastoma: A European collaborative analysis

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