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Related Concept Videos

Increased Intracranial Pressure l: Introduction01:14

Increased Intracranial Pressure l: Introduction

Intracranial hypertension is a sustained elevation of intracranial pressure (ICP) above 22 mm Hg. In supine adults, normal ICP is ~7–15 mm Hg.The rigid, nonexpandable cranium contains three components—brain tissue, blood, and cerebrospinal fluid (CSF)—that total ~1,700 mL in a typical adult: 1,400 mL brain (~80%), 150 mL blood (~10%), and 150 mL CSF (~10%). According to the Monro–Kellie doctrine, total intracranial volume is effectively fixed. When one component expands, CSF and venous blood...
Increased Intracranial Pressure ll: Pathophysiology01:29

Increased Intracranial Pressure ll: Pathophysiology

Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...

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Rethinking the role of surgery and radiotherapy in BRAF-mutant papillary craniopharyngioma: a position statement from the neurosurgical perspective.

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Related Experiment Video

Updated: Jun 20, 2026

Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
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Papillary Craniopharyngioma: An Integrative and Comprehensive Review.

Ruth Prieto1, Tareq A Juratli2,3,4, Evan D Bander5

  • 1Department of Neurosurgery, Puerta de Hierro University Hospital, 28222 Madrid, Spain.

Endocrine Reviews
|October 1, 2024
PubMed
Summary

Papillary craniopharyngioma (PCP) is a rare, benign WHO grade 1 tumor. Over 90% harbor the BRAFV600E mutation, responding to targeted inhibitors, offering new treatment avenues.

Keywords:
BRAF mutationcraniopharyngiomahypothalamuspapillary craniopharyngiomapituitary surgerythird ventricle

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Area of Science:

  • Neuro-oncology
  • Pathology
  • Genetics

Background:

  • Papillary craniopharyngioma (PCP) is a rare tumor subtype, distinct from adamantinomatous craniopharyngioma.
  • WHO grade 1 benign tumors, PCPs feature nonkeratinizing squamous epithelium and occur mainly in adults.
  • Predominantly located in the third ventricle, PCPs affect the hypothalamus, causing visual and psychiatric issues.

Purpose of the Study:

  • To provide a comprehensive review of papillary craniopharyngioma (PCP).
  • Synthesize current knowledge on PCP epidemiology, clinical presentation, diagnosis, and treatment.
  • Highlight recent advances in understanding PCP pathogenesis and therapeutic strategies.

Main Methods:

  • Systematic review of published literature from 1856 to 2023.
  • Analysis of a cohort of 560 well-described PCPs and 99 craniopharyngioma series.
  • Synthesis of data on tumor characteristics, patient demographics, and treatment outcomes.

Main Results:

  • PCPs comprise ~20% of craniopharyngiomas, predominantly affecting adult males and the hypothalamus.
  • BRAFV600E mutation is present in over 90% of PCPs, activating the MAPK pathway.
  • Endoscopic endonasal approach offers favorable outcomes for surgical resection.

Conclusions:

  • PCP is a distinct pathological entity with specific molecular drivers.
  • Targeted therapies inhibiting the BRAFV600E-driven MAPK pathway show promise.
  • Minimally invasive surgical techniques improve patient outcomes in managing this challenging tumor.