Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Ewa Bien¹, Jelena Roganovic², Malgorzata A Krawczyk¹, Jan Godzinski^{3

4}, Daniel Orbach⁵, Giovanni Cecchetto⁶, Winfred Barthlen⁷, Anne-Sophie Defachelles⁸, Andrea Ferrari⁹, Christopher B Weldon¹⁰, Ines B Brecht¹¹, Dominik T Schneider¹², Gianni Bisogno¹³, Alexandra Kolenova¹⁴, Tal Ben-Ami¹⁵, Kata Martinova¹⁶, Calogero Virgone⁶, Teresa Stachowicz-Stencel¹, Denis Kachanov¹⁷, Yves Reguerre¹⁸

Affiliations

¹ Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.
² Department of Pediatrics, Clinical Hospital Center Rijeka, University of Rijeka, Rijeka, Croatia.
³ Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland.
⁴ Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, Wroclaw, Poland.
⁵ SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France.
⁶ Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy.
⁷ Pediatric Surgery, Universitaetsmedizin Greifswald, Greifswald, Germany.
⁸ Centre Oscar Lambret, Lille, France.
⁹ Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
¹⁰ Departments of Surgery, Oncology and Anesthesiology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts, USA.
¹¹ Pediatric Hematology and Oncology, Children's Hospital, Eberhard-Karls-Universitaet Tübingen, Tübingen, Germany.
¹² Clinic of Pediatrics, Dortmund Municipal Hospital, Dortmund, Germany.
¹³ Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padua, Italy.
¹⁴ University Children Hospital, Bratislava, Slovakia.
¹⁵ Pediatric Hematology Unit, Kaplan Medical Center, Rehovot, Israel.
¹⁶ Department of Hematology and Oncology, University Clinic for Children`s Diseases, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of North Macedonia.
¹⁷ Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
¹⁸ Department of Pediatric Hematology and Oncology, Félix Guyon University Hospital, St Denis, Réunion Island, France.

PMID: 34174157
DOI: 10.1002/pbc.29112

Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Ewa Bien et al. Pediatr Blood Cancer. 2021 Jun.

. 2021 Jun:68 Suppl 4:e29112.

doi: 10.1002/pbc.29112.

Authors

Affiliations

¹ Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland.
² Department of Pediatrics, Clinical Hospital Center Rijeka, University of Rijeka, Rijeka, Croatia.
³ Department of Pediatric Surgery, Marciniak Hospital, Wroclaw, Poland.
⁴ Department of Pediatric Traumatology and Emergency Medicine, Wroclaw Medical University, Wroclaw, Poland.
⁵ SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France.
⁶ Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy.
⁷ Pediatric Surgery, Universitaetsmedizin Greifswald, Greifswald, Germany.
⁸ Centre Oscar Lambret, Lille, France.
⁹ Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
¹⁰ Departments of Surgery, Oncology and Anesthesiology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard University, Boston, Massachusetts, USA.
¹¹ Pediatric Hematology and Oncology, Children's Hospital, Eberhard-Karls-Universitaet Tübingen, Tübingen, Germany.
¹² Clinic of Pediatrics, Dortmund Municipal Hospital, Dortmund, Germany.
¹³ Hematology-Oncology Division, Department of Pediatrics, Padova University Hospital, Padua, Italy.
¹⁴ University Children Hospital, Bratislava, Slovakia.
¹⁵ Pediatric Hematology Unit, Kaplan Medical Center, Rehovot, Israel.
¹⁶ Department of Hematology and Oncology, University Clinic for Children`s Diseases, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of North Macedonia.
¹⁷ Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
¹⁸ Department of Pediatric Hematology and Oncology, Félix Guyon University Hospital, St Denis, Réunion Island, France.

PMID: 34174157
DOI: 10.1002/pbc.29112

Abstract

Pancreatoblastoma (PBL) is a rare malignant epithelial neoplasm that affects typically young children. Signs related to advanced upper-abdominal tumor accompanied by elevated serum α-fetoprotein levels in a young child suggest PBL, however histopathological confirmation is mandatory. The mainstay of the treatment is a complete surgical resection. Unresectable and/or metastatic PBL may become amenable to complete delayed surgery after neoadjuvant chemotherapy. This manuscript presents the international consensus recommendations for the diagnosis and treatment of children with PBL, established by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the EU-funded PARTNER (Paediatric Rare Tumors Network - European Registry) project.

Keywords: EXPeRT; PARTNER; children; diagnosis; pancreatoblastoma; recommendations.

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References

REFERENCES

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Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Affiliations

Pancreatoblastoma in children: EXPeRT/PARTNER diagnostic and therapeutic recommendations

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Supplementary concepts

LinkOut - more resources

Full Text Sources

Medical