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Craniopharyngiomas

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Atlas of Sellar and Parasellar Lesions

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Abstract

Craniopharyngiomas (CP) are benign, yet locally invasive, neoplasms typically arising as suprasellar and sellar lesions.

CPs can arise anywhere along the vestiges of the stomodeal diverticulum and craniopharyngeal duct, most frequently in the region of the infundibulum.

In rare cases, CPs have been reported to arise in less typical locations along the remnants of the primitive craniopharyngeal duct, including the nasopharynx or sphenoid bone, or as primary intraventricular lesions.

Overall, CPs comprise approximately 3 % of all intracranial tumors, but this proportion is notably higher in the pediatric population (8–10 % of all pediatric brain tumors).

The estimated incidence of craniopharyngiomas is 0.13 per 100,000 persons per year.

CPs comprise approximately 3 % of surgically resected lesions in major transsphenoidal series.

Historically, CPs were said to present in a bimodal age distribution with peak ages at 5–14 years and then 50–74 years, but they could present in patients of any age. These statistics are outmoded, distorted by reports of pediatric data. Age-specific incidence in adults increases with age.

The clinical presentation of CPs at any age frequently includes headache, visual loss, and hypopituitarism.

In children and adolescents, growth and sexual retardation, obesity, and hydrocephalus are frequently observed.

Many patients with CPs suffer from chronic obesity, which is thought to develop secondary to hypothalamic dysfunction.

Memory loss and cognitive deficits are more common findings in older patients.

Diabetes insipidus occurs on presentation in 6–38 % of new cases.

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Author information

Authors and Affiliations

  1. Department of Neurological Surgery, Keck School of Medicine, University of Southern California, 1200 N State Street, #3300, Los Angeles, CA, 90033, USA

    Gabriel Zada MD, MS

  2. Department of Pathology (Neuropathology), University of Virginia School of Medicine, 1215 Lee Street, Room 3060–HEP, Charlottesville, VA, 22908, USA

    M. Beatriz S. Lopes MD, PhD

  3. Department of Radiology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Srinivasan Mukundan Jr PhD, MD

  4. Department of Neurosurgery, Brigham and Women’s Hospital, 15 Francis Street, Boston, MA, 02115, USA

    Edward Laws Jr MD

Authors
  1. Gabriel Zada MD, MS
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  2. M. Beatriz S. Lopes MD, PhD
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  3. Srinivasan Mukundan Jr PhD, MD
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  4. Edward Laws Jr MD
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Corresponding author

Correspondence to Gabriel Zada MD, MS .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, University of Southern California, LOS ANGELES, California, USA

    Gabriel Zada

  2. Department of Pathology, University of Virginia, CHARLOTTESVILLE, Virginia, USA

    M. Beatriz S. Lopes

  3. Department of Radiology, Brigham and Women's Hospital, BOSTON, Massachusetts, USA

    Srinivasan Mukundan Jr.

  4. Department of Neurosurgery, Brigham and Women’s Hospital, BOSTON, Massachusetts, USA

    Edward R. Laws Jr.

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Zada, G., Lopes, M.B.S., Mukundan, S., Laws, E. (2016). Craniopharyngiomas. In: Zada, G., Lopes, M., Mukundan Jr., S., Laws Jr., E. (eds) Atlas of Sellar and Parasellar Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-22855-6_21

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  • DOI: https://doi.org/10.1007/978-3-319-22855-6_21

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-22854-9

  • Online ISBN: 978-3-319-22855-6

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