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

被引：13

作者：

Bisogno, Gianni ^{[1
]}

Sarnacki, Sabine ^{[2
]}

Stachowicz-Stencel, Teresa ^{[3
]}

Minard Colin, V. ^{[4
]}

Ferrari, Andrea ^{[5
]}

Godzinski, Jan ^{[6
]}

Gauthier Villars, Marion ^{[7
]}

Bien, Ewa ^{[3
]}

Hameury, Frederic ^{[8
]}

Helfre, Sylvie ^{[9
]}

Schneider, Dominik T. ^{[10
]}

Reguerre, Yves ^{[11
]}

Lopez Almaraz, Ricardo ^{[12
]}

Janic, Dragana ^{[13
]}

Cesen, Maja ^{[14
]}

Kolenova, Alexandra ^{[15
,16
]}

Rascon, Jelena ^{[17
]}

Martinova, Kata ^{[18
]}

Cosnarovici, Rodica ^{[19
]}

Pourtsidis, Apostolos ^{[20
]}

Ben Ami, Tal ^{[21
]}

Roganovic, Jelena ^{[22
]}

Koscielniak, Ewa ^{[23
]}

Schultz, Kris Ann P. ^{[24
]}

Brecht, Ines B. ^{[25
]}

Orbach, Daniel ^{[26
]}

机构：

[1] Padova Univ Hosp, Div Hematol Oncol, Dept Womens & Childrens Hlth, Padua, Italy

[2] Univ Paris, Dept Pediat Surg, Hop Necker Enfants Malad, Paris, France

[3] Med Univ Gdansk, Dept Pediat Hematol & Oncol, Gdansk, Poland

[4] Univ Paris Saclay, Dept Paediat & Adolescent Oncol, INSERM U1015, Gustave Roussy, Villejuif, France

[5] Fdn IRCCS Ist Nazl Tumori Milano, Pediat Oncol Unit, Milan, Italy

[6] Marciniak Hosp, Dept Pediat Surg, Wroclaw, Poland

[7] Paris Sci Lettres Res Univ, Inst Curie, Dept Genet, Paris, France

[8] Hop Femme Mere Enfant, Dept Pediat Surg, Lyon, France

[9] Inst Curie, Dept Radiotherapy, Paris, France

[10] Dortmund Municipal Hosp, Pediat Clin, Dortmund, Germany

[11] Ctr Hosp Univ, Pediat Hematol Oncol Dept, St Denis, France

[12] Univ Hosp Cruces, Pediat Oncol Hematol Unit, Barakaldo Bizkaia, Spain

[13] Univ Childrens Hosp Univ Belgrade, Dept Hematol & Oncol, Belgrade, Serbia

[14] Univ Med Ctr, Ljubljana, Slovenia

[15] Natl Inst Childrens Dis, Dept Pediat Hematol & Oncol, Bratislava, Slovakia

[16] Comenius Univ, Bratislava, Slovakia

[17] Vilnius Univ, Inst Clin Med, Pediat Clin, Fac Med, Vilnius, Lithuania

[18] Ss Cyril & Methodius Univ Skopje, Univ Clin Childrens Dis Med Fac, Dept Hematol & Oncol, Skopje, North Macedonia

[19] Oncol Inst Prof Dr Ion Chiricuta, Oncopediat Dept, Cluj Napoca, Romania

[20] PA Kyriakou Childrens Hosp, Dept Oncol, Athens, Greece

[21] Kaplan Med Ctr, Pediat Hematol Unit, Rehovot, Israel

[22] Univ Rijeka, Dept Pediat, Clin Hosp Ctr Rijeka, Rijeka, Croatia

[23] Klinikum Stuttgart Olgahosp, Pediat Oncol Hematol Immunol 5, Stuttgart Canc Ctr, Stuttgart, Germany

[24] Childrens Minnesota, Int PPB DICER1 Registry, Canc & Blood Disorders, Minneapolis, MN USA

[25] Eberhard Karls Univ Tuebingen, Childrens Hosp, Pediat Hematol & Oncol, Tubingen, Germany

[26] PSL Univ, Inst Curie, SIREDO Oncol Ctr Care Innovat & Res Children Adol, Paris, France

来源：

PEDIATRIC BLOOD & CANCER | 2021年 / 68卷

关键词：

children; PARTNER; pleuropulmonary blastoma; therapeutic recommendations; very rare tumors; PROGNOSTIC-FACTORS; DICER1; MUTATIONS; PREDISPOSITION; MALFORMATION;

D O I：

10.1002/pbc.29045

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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).

引用

页数：7

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