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Duct-like diverticulum at the base of third ventricle tumors: a morphological signature diagnostic of papillary craniopharyngioma

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Abstract

This study describes and characterizes a narrow, hollow tubular structure, termed as duct-like diverticulum (DV), found specifically at the basal midline of papillary craniopharyngiomas (PCPs) located within the third ventricle (3V). The presence of this structure was systematically investigated on autopsy studies and magnetic resonance imaging (MRI) scans of 3536 craniopharyngioma (CP) cases published in the medical literature from 1911 to 2021, as well as in other twelve 3V tumor categories (n = 1470 cases). A basal DV was observed in a total of 50 PCPs, including two of our own cases. This DV corresponds to a tubular-shaped recess invaginated at the midline bottom of the tumor, following the same angled trajectory as the pituitary stalk. It can be easily seen as a hypointense linear structure on T1- and T2-weighted MRI scans, with two main length types: long DVs (74%), which reach the tumor center, and short DVs (26%), which penetrate the tumor only a few millimeters. The DV sign identifies the papillary CP type with a specificity of 100% and a sensitivity of 33% in the overall CP population. This finding also serves to establish the strictly intra-3V location of the lesion with a 95% specificity and 42% sensitivity among papillary CPs. No similar basal DV was found in adamantinomatous CPs nor among other categories of strictly 3V tumors. Consequently, the presence of a diverticulum in a 3V tumor represents a morphological signature pathognomonic of the papillary type and a valuable sign to reliably define the strictly 3V topography.

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Fig. 1
Fig. 2

Reproduced from Lei et al. [60], with permission. C H&E-stained slice (× 20) of the tumor specimen from one author’s case showing the typical pseudo-papillary aspect of the sheets of squamous epithelium, with the generation of deep fissures or crevices (black arrow) owing to the normal dehiscence process of the epithelium (without any relation to the DV). D, E Magnetic resonance imaging (MRI) characteristics of the DV in author’s case 1 (number 49; Table 1). The DV is identified as a linear homogeneously hypointense structure which follows a diagonal trajectory along the tumor midline on the T1-weighted MRI scans after gadolinium administration. It is open to the suprasellar cistern and preserves the same signal as the cerebrospinal fluid (black arrows). F Another example of a duct-like basal diverticulum in a solid 3V CP of the squamous-papillary type (author’s case 2, number 50 in Table 1). In this case, the DV ends in a non-bifurcated manner (yellow arrow) as a “cul-de-sac” at the center of the tumor

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Acknowledgements

The authors wish to especially thank Crystal Smith and Liliya Gusakova, Reference Librarians of the National Library of Medicine, National Institutes of Health (Bethesda, MD), for their kind assistance during the process of searching and retrieving articles and monographs used in this study. We are also grateful to Melissa Grafe, Librarian for Medical History Library, Yale University (New Haven, CT), and to Lucretia MacLure, Jack Eckert, and the staff at the Francis Countway Medical Library at Harvard Medical School (Boston, MA) for their invaluable help in obtaining some of the original research material used for this study. We thank Professor Kenneth Lee, Head of Anatomical Pathology Department at Concord Repatriation General Hospital, who has gently permitted us to reproduce the autopsy image of the papillary CP shown in Figure 2A. Finally, we are grateful to George Hamilton for his critical review of the language and style of the manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, La Princesa University Hospital, C/ Diego de León 62, 28006, Madrid, Spain

    José María Pascual

  2. Department of Neurosurgery, Puerta de Hierro University Hospital, Madrid, Spain

    Ruth Prieto

  3. Department of Neurosurgery, Ramón y Cajal University Hospital, Madrid, Spain

    Rodrigo Carrasco

  4. Statistics Department, Computing Center, C.S.I.C., Madrid, Spain

    Laura Barrios

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  1. José María Pascual
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  2. Ruth Prieto
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Contributions

Conception and design: José M. Pascual. Acquisition of data: José M. Pascual, Ruth Prieto and Rodrigo Carrasco. Analysis of data: José M. Pascual, Laura Barrios; drafting the article: José M. Pascual, Ruth Prieto; critically revising the article: all authors.

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Correspondence to José María Pascual.

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Not applicable as this study consists of a review of previously published literature.

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Authors’ information

José María Pascual, M.D., Ph.D.

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

e-mail: jmpasncj@hotmail.com.

Ruth Prieto, M.D., Ph.D.

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

e-mail: rprieto29@hotmail.com.

Rodrigo Carrasco, M.D.

Department of Neurosurgery, Ramón y Cajal University Hospital, Madrid, Spain.

e-mail: rocamo@gmail.com.

Laura Barrios, B.S.

Statistics Department, Computing Center, C.S.I.C. Madrid, Spain.

e-mail: laura.barrios@csic.es.

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Pascual, J.M., Prieto, R., Carrasco, R. et al. Duct-like diverticulum at the base of third ventricle tumors: a morphological signature diagnostic of papillary craniopharyngioma. Neurosurg Rev 45, 3361–3379 (2022). https://doi.org/10.1007/s10143-022-01848-7

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