Paediatric Tumours of Neuroendocrine/Peripheral Neuroectodermal Origin

  • Roberto Luksch
  • Carlo Chiesa
  • Ettore Seregni
  • Carlo Morosi
  • Marta Podda
  • Davide Biasoni
  • Gemma Gatta
  • Lorenza Gandola
  • Paola Collini
  • Paolo Scanagatta
  • Giovanna Riccipetitoni
  • Nadia Puma
  • Maria Rita Castellani
Chapter

Abstract

The neoplasms arising from the neural crest-derived cells in the adrenal medulla or extra-adrenal paraganglia include peripheral neuroblastic tumours, pheochromocytoma and paraganglioma.

Peripheral neuroblastic tumours (neuroblastoma, ganglioneuroblastoma and ganglioneuroma) account for 7–10% of all tumours in childhood. In these tumours, the MYCN oncogene status, extension of the disease and age are the most relevant prognostic factors. The 5-year survival probability ranges from >95% for patients with nonmetastatic resectable tumour and absence of MYCN amplification to <20% for patients with metastatic disease and MYCN-amplified tumour.

Pheochromocytoma and paraganglioma are very rare. Functioning tumours are associated with symptoms related to catecholamine secretion, whereas nonfunctioning tumours present with symptoms related to tumour mass effect, or present incidentally. These tumours are generally benign, but a small percentage of them are high-grade malignancies requiring multimodality therapies. Ninety percent of the cases are sporadic, while 10% are familial, such as those that occur as part of the multiple endocrine neoplasia syndromes.

Keywords

Childhood tumours Peripheral neuroblastic tumours Neuroblastoma Pheochromocytoma Paraganglioma 

Notes

Acknowledgements

The authors want to thank for their support the Associazione Bianca Garavaglia ONLUS, Busto Arsizio (Varese) and the Associazione UNA-Milano, Milan, Italy.

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Roberto Luksch
    • 1
  • Carlo Chiesa
    • 2
  • Ettore Seregni
    • 3
  • Carlo Morosi
    • 4
  • Marta Podda
    • 1
  • Davide Biasoni
    • 5
  • Gemma Gatta
    • 6
  • Lorenza Gandola
    • 7
  • Paola Collini
    • 8
  • Paolo Scanagatta
    • 9
  • Giovanna Riccipetitoni
    • 10
  • Nadia Puma
    • 1
  • Maria Rita Castellani
    • 2
  1. 1.SC Pediatria OncologicaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  2. 2.SC Medicina NucleareFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  3. 3.SS Terapia Medico-Nucleare ed EndocrinologiaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  4. 4.SS Radiologia PaediatricaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  5. 5.SC Chirurgia UrologicaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  6. 6.SSD Epidemiologia ValutativaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  7. 7.SS Radioterapia PaediatricaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  8. 8.SS Patologia dei tessuti molli e dell’osso e diagnostica generale e dell’età evolutivaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  9. 9.SC Chirurgia ToracicaFondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  10. 10.Dip. Chirurgia PaediatricaOspedale dei Bambini V. Buzzi, ASST Fatebenefratelli SaccoMilanItaly

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