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Pancreatic Tumors

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Rare Tumors in Children and Adolescents

Part of the book series: Pediatric Oncology ((PEDIATRICO))

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Abstract

Pancreatic tumors in infancy and childhood are rare, and diagnosis can be challenging. The differential diagnosis for malignant pancreatic tumors includes benign tumors, tumors infiltrating pancreas from adjacent organs, like neuroblastoma, Wilms’ tumor, and hepatoblastoma, as well as involvement of the pancreas by leukemia, lymphoma, or lymphoproliferative disorders. In case of a primary pancreatic tumor, pancreatoblastoma has to be considered especially in a child under the age of 10 years, while a solid-pseudopapillary neoplasm (SPN) - in female adolescents. Because of similar morphology, SPN might easily be confused with neuroendocrine tumors. Acinar cell carcinoma (ACC) and ductal adenocarcinoma are extremely rarely seen in children.

Complete resection is the mainstay of treatment as most pancreatic tumors are little radio- and chemosensitive. However, patients often present with metastases and/or unresectable tumor at the time of diagnosis. For pancreatoblastoma, which shows chemosensitivity, the PLADO regime (cisplatin and doxorubicin), as recommended for hepatoblastoma in the SIOPEL study, is the most widely used protocol. Overall survival (OS) for pancreatoblastoma is therefore more favorable (about 80%). In case of complete resection, SPN shows an OS of 95%. Carcinoma on the other hand has a poor prognosis (OS 23%).

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Authors and Affiliations

  1. Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdansk, Poland

    Ewa Bien

  2. Pediatric Surgery, University Medicine Greifswald, Greifswald, Germany

    Winfried Barthlen

  3. Department of Pediatric Hematology/Oncology, University Children’s Hospital Tuebingen, Tuebingen, Germany

    Ines B. Brecht & Bence Sipos

  4. Internal Medicine VIII, University Hospital Tuebingen, Tuebingen, Germany

    Bence Sipos

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  1. Ewa Bien
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  2. Winfried Barthlen
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  3. Ines B. Brecht
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  4. Bence Sipos
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Correspondence to Ewa Bien .

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Editors and Affiliations

  1. Clinic of Pediatrics, Municipal Hospital Dortmund, University Witten-Herdecke, Dortmund, Germany

    Dominik T. Schneider

  2. Department of Hematology and Oncology, University Children’s Hospital, Tübingen, Baden-Württemberg, Germany

    Ines B. Brecht

  3. Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta and Emory University, Atlanta, GA, USA

    Thomas A. Olson

  4. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Milano, Italy

    Andrea Ferrari

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Bien, E., Barthlen, W., Brecht, I.B., Sipos, B. (2022). Pancreatic Tumors. In: Schneider, D.T., Brecht, I.B., Olson, T.A., Ferrari, A. (eds) Rare Tumors in Children and Adolescents. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-92071-5_28

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