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Strictly third ventricle craniopharyngiomas: pathological verification, anatomo-clinical characterization and surgical results from a comprehensive overview of 245 cases

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Abstract

The strictly third ventricle craniopharyngioma topography (strictly 3V CP) defines the subgroup of lesions developed above an anatomically intact third ventricle floor (3VF). The true existence of this exceedingly rare topographical category is highly controversial owing to the presumed embryological CP origin from Rathke’s pouch, a structure developmentally situated outside the neural tube. This study thoroughly analyzes the largest series of strictly 3V CPs ever collected. From 5346 CP reports published between 1887 and 2021, we selected 245 cases with reliable pathological, surgical, and/or neuroradiological verification of an intact 3VF beneath the tumor. This specific topography occurs predominantly in adult (92.6%), male (64.4%) patients presenting with headache (69.2%), and psychiatric disturbances (59.2%). Neuroradiological features defining strictly 3V CPs are a tumor-free chiasmatic cistern (95.9%), an entirely visible pituitary stalk (86.4%), and the hypothalamus positioned around the tumor’s lower pole (92.6%). Most are squamous papillary (82%), showing low-risk severity adhesions to the hypothalamus (74.2%). The adamantinomatous variant, however, associates a higher risk of severe hypothalamic adhesion (p < .001). High-risk attachments are also associated with psychiatric symptoms (p = .013), which represented the major predictor for unfavorable prognoses (83.3% correctly predicted) among cases operated from 2006 onwards. CP recurrence is associated with infundibulo-tuberal symptoms (p = .036) and incomplete surgical removal (p = .02). The exclusive demographic, clinico-pathological and neuroradiological characteristics of strictly 3V CPs make them a separate, unique topographical category. Accurately distinguishing strictly 3V CPs preoperatively from those tumors replacing the infundibulum and/or tuber cinereum (infundibulo-tuberal or not strictly 3V CPs) is critical for proper, judicious surgical planning.

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Abbreviations

ACP:

Adamantinomatous craniopharyngioma

CP:

Craniopharyngioma

EETS:

Endoscopic endonasal transsphenoidal

FTV:

Frontal transventricular

HICP:

High intracranial pressure

MRI:

Magnetic resonance imaging

PCP:

Papillary craniopharyngioma

Tc:

Transcallosal

TLT:

Translamina terminalis

TS:

Transsphenoidal

3V:

Third ventricle

3VF:

Third ventricle floor

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Acknowledgements

The authors wish to especially thank Crystal Smith and Liliya Gusakova, Reference Librarians at the National Library of Medicine, National Institutes of Health (Bethesda, MD), Lucretia Maclure and Jack Eckert, Librarians at the Francis Countway Medical Library at Harvard Medical School (Boston, MA), as well as Melissa Grafe, John R. Bumstead Librarian for Medical History, Head of the Medical Library of the Cushing/Whitney Medical Library and Bill Landis of the Manuscript and Archives Department of the Sterling Memorial Library, Yale University (New Haven, CT) for their invaluable assistance during the process of searching and retrieving the original works used in this study. We are very grateful to Maria Rosdolsky for assisting with the translation of German works. We are also indebted to Eduard Winter and Verena Hofecker from the Pathologisch-anatomische Sammlung im Narrenturm-NHM, Vienna, Austria for allowing us access to the collection of brain tumor specimens. Finally, we are grateful to George Hamilton for his critical review of the language and style of the manuscript. This work is dedicated in loving memory to Ricardo Prieto (1948–2021).

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Authors and Affiliations

  1. Department of Neurosurgery, Puerta de Hierro University Hospital, Manuel de Falla 1, 28222, Majadahonda, Madrid, Spain

    Ruth Prieto

  2. Statistics Department, Computing Center, CSIC, Madrid, Spain

    Laura Barrios

  3. Department of Neurosurgery, La Princesa University Hospital, Madrid, Spain

    José M. Pascual

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Ruth Prieto: conceived and designed the analysis, collected the data, performed the statistical analysis and interpretation of data, wrote the paper, and approved the final version of the manuscript; Laura Barrios: performed the statistical analysis and approved the final version of the manuscript; José M. Pascual: conceived and designed the analysis, collected the data, critically revised the article, and approved the final version of manuscript.

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Prieto, R., Barrios, L. & Pascual, J.M. Strictly third ventricle craniopharyngiomas: pathological verification, anatomo-clinical characterization and surgical results from a comprehensive overview of 245 cases. Neurosurg Rev 45, 375–394 (2022). https://doi.org/10.1007/s10143-021-01615-0

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