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Pineal Region Tumors

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Textbook of Pediatric Neurosurgery

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Abstract

The pineal region is home to a group of histologically diverse tumors. These include germ cell tumors, embryonal tumors, and tumors of pineal parenchymal origin. For newly diagnosed masses, initial management is typically focused on managing the patient’s obstructive hydrocephalus, imaging the entire neuraxis, and analyzing cerebrospinal fluid (CSF) for germ cell markers. Today, magnetic resonance imaging (MRI) and computed tomography (CT) can provide such high-quality detail of the tumor that the broad list of tumor types can be substantially narrowed. Determining the metastatic disease status (cranial and spinal) is of great importance for therapy and prognosis. The result of the cerebrospinal fluid (CSF) analysis is critical, as it will determine whether surgery directed at the tumor (i.e., resection or biopsy) is needed or if the patient can be initially treated with chemotherapy and/or radiation. Elevated alpha-fetoprotein (α-FP) and/or beta-human chorionic gonadotropin (β-hCG) are diagnostic for a group of neoplasms called non-germinomatous germ cell tumors. These tumors are initially managed with chemotherapy. For all other tumors that are marker-negative, resection is eventually needed with one exception – germinoma. Germinomas are exquisitely sensitive to radiation. Pineal tumors are one of the most challenging masses to resect, owing to their deep location and intimate relationship to critical neurovascular structures. Surgical approaches can be broadly categorized as supra- or infratentorial. Tumor type has the greatest impact on survival, but other predictors include age, extent of resection, and presence or absence of metastatic disease on presentation.

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Author information

Authors and Affiliations

  1. Institute of Brain Protection Sciences, Johns Hopkins University and Medicine, Johns Hopkins All Children’s Hospital, St. Petersburg, FL, USA

    Nir Shimony

  2. Department of Radiology, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, TN, USA

    Asim F. Choudhri

  3. Department of Radiation Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA

    John T. Lucas Jr.

  4. Semmes Murphey, Memphis, TN, USA

    Paul Klimo Jr.

Authors
  1. Nir Shimony
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  2. Asim F. Choudhri
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  3. John T. Lucas Jr.
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  4. Paul Klimo Jr.
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Corresponding author

Correspondence to Paul Klimo Jr. .

Editor information

Editors and Affiliations

  1. Pediatric Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Trust, London, UK

    Dachling Pang

  3. Division of Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

Section Editor information

  1. Division of Neurosurgery, Suite 1503, The Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

  2. The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

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Shimony, N., Choudhri, A.F., Lucas, J.T., Klimo, P. (2020). Pineal Region Tumors. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-72168-2_88

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  • DOI: https://doi.org/10.1007/978-3-319-72168-2_88

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-72167-5

  • Online ISBN: 978-3-319-72168-2

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