Clinical, Radiological, and Molecular Insights into Craniopharyngioma

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Neurobiology and Clinical Neuroscience".

Deadline for manuscript submissions: closed (31 January 2026) | Viewed by 3628

Editors


E-Mail
Guest Editor
Department of Neurosurgery and Neurooncology, Medical University of Lodz, Barlicki University Hospital, Lodz, Poland
Interests: neuro-oncology; brain tumors; skull base; microneurosurgery; neuroanatomy; neuronavigation; stereotactic surgery; intracranial aneurysm

E-Mail Website
Guest Editor
Department of Neurosurgery and Neurooncology, Medical University of Lodz, Barlicki University Hospital, Lodz, Poland
Interests: neurosurgery; neuro-oncology; cancer-predisposing syndrome; CMMRD
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

The sellar region is of particular interest to neurosurgeons and encompasses a variety of conditions, including pituitary adenomas, craniopharyngiomas, aneurysms, astrocytomas, and meningiomas. Among these, craniopharyngioma remains one of the most challenging entities. It is a slow-growing, benign intracranial tumor, but it exhibits locally invasive behavior, complicating treatment and prognosis.

One of the major difficulties with craniopharyngioma is its high recurrence rate, which can reach up to 50%. Identifying risk factors for recurrence is a significant area of current research. Surgical approach selection is critical, and preserving the pituitary stalk during surgery has been associated with better functional outcomes but can increase the risk of tumor persistence or recurrence. Gross total resection (GTR) remains the goal for reducing recurrence, although subtotal resection (STR) followed by adjuvant radiotherapy (XRT) is also considered in cases where complete resection may lead to serious complications.

In addition to surgical and radiological advances, new adjuvant therapies are being explored to address the challenging nature of craniopharyngioma. Intratumoral therapy with sclerosing agents, such as bleomycin, has been trialed, and targeted therapies, including inhibitors for BRAF, MEK, and interleukin-6, are under investigation. However, the long-term efficacy and safety of these treatments are still uncertain, highlighting the need for further studies to evaluate their potential.

Furthermore, a deeper understanding of the histopathologic subtypes of craniopharyngioma is also crucial. Current research aims to clarify how these subtypes influence tumor behavior, recurrence risk, and response to therapies. As a result, future studies are essential for improving diagnostic precision, refining therapeutic approaches, and enhancing long-term outcomes for patients with this complex tumor.

Further investigation into the clinical, radiological, molecular, and therapeutic aspects of craniopharyngioma is essential to better understand and manage this condition.

Dr. Ernest Jan Bobeff
Dr. Bartosz Szmyd
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-anonymized peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • neuro-oncology
  • brain tumors
  • skull base
  • craniopharyngioma
  • stalk

Benefits of Publishing in a Special Issue

  • Ease of navigation: Grouping papers by topic helps scholars navigate broad scope journals more efficiently.
  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
  • External promotion: Articles in Special Issues are often promoted through the journal's social media, increasing their visibility.
  • Reprint: MDPI Books provides the opportunity to republish successful Special Issues in book format, both online and in print.

Further information on MDPI's Special Issue policies can be found here.

Published Papers (2 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

22 pages, 11326 KB  
Article
Isolation and Characterization of the Adamantinomatous Craniopharyngioma Primary Cells with Cancer-Associated Fibroblast Features
by Dongting Chen, Ting Lei, Yulin Wang, Zaitao Yu, Siqi Liu, Ling Ye, Wanfang Li, Qin Yang, Hongtao Jin, Fangjun Liu and Yan Li
Biomedicines 2025, 13(4), 912; https://doi.org/10.3390/biomedicines13040912 - 9 Apr 2025
Cited by 2 | Viewed by 2165
Abstract
Backgrounds: Adamantinomatous craniopharyngiomas (ACPs) are benign intracranial tumors that behave aggressively due to their location, infiltration of the surrounding nervous tissue and high capacity for recurrence. In this study, we aimed to construct ACP primary cell models for further investigation of tumorigenic [...] Read more.
Backgrounds: Adamantinomatous craniopharyngiomas (ACPs) are benign intracranial tumors that behave aggressively due to their location, infiltration of the surrounding nervous tissue and high capacity for recurrence. In this study, we aimed to construct ACP primary cell models for further investigation of tumorigenic and recurrent mechanisms. Methods: Primary cells were isolated from primary (one case) and recurrent (one case) ACP. Short tandem repeat (STR) analysis was used to clarify the identity of the ACP primary cells we isolated. Whole exome sequencing (WES), immunofluorescence (IF) and immunohistochemistry (IHC) were performed on primary cells and corresponding ACP tissues, to determine the mutational profile and to clarify the tissue origin and phenotypic of primary cells. Transcriptome RNA-seq was performed to obtain the gene expression characteristics of ACP primary cells. Subsequently, a heterotopic ACP xenograft mouse model was established to confirm the tumorigenesis capacity of ACP primary cells. Results: ACP primary cells were successfully cultured. The genetic variants were similar to the original tumor tissue, and they owned expression of cancer-associated fibroblast (CAF) markers (FSP1/S100A4, Vimentin) and nuclear translocation β-catenin. Meanwhile, they had an high level expression of extracellular matrix components (Fibronectin). The tumor formation ability of ACP primary cells was verified. The transcriptional signatures of ACP primary cells were also explored. Conclusions: We successfully isolated and characterized ACP primary cells that acquired multiple CAF features and demonstrated stable propagation through dozens of passages. These PDC models laid the foundation for further research on ACP. Full article
(This article belongs to the Special Issue Clinical, Radiological, and Molecular Insights into Craniopharyngioma)
Show Figures

Figure 1

Review

Jump to: Research

11 pages, 252 KB  
Review
Systemic Molecularly Targeted Therapies for Neoadjuvant and Salvage Craniopharyngioma: A Contemporary Narrative Review
by Joseph J. Neubecker, Daniel W. Griepp, Jeffrey P. Turnbull, Joshua Caskey, Shivum Desai, Adam Mansour, Rabia Ahmed, Andrew Beggs, Annie T. K. Griepp, Heather Heitkotter, Chad F. Claus, Boyd F. Richards and Prashant S. Kelkar
Biomedicines 2026, 14(3), 499; https://doi.org/10.3390/biomedicines14030499 - 25 Feb 2026
Viewed by 971
Abstract
Craniopharyngiomas are rare, histologically benign but locally aggressive intracranial tumors that are associated with substantial visual, endocrine, and hypothalamic morbidity. Advances in molecular characterization have enabled the use of systemic molecularly targeted therapies, particularly in the recurrent or refractory setting, with the goal [...] Read more.
Craniopharyngiomas are rare, histologically benign but locally aggressive intracranial tumors that are associated with substantial visual, endocrine, and hypothalamic morbidity. Advances in molecular characterization have enabled the use of systemic molecularly targeted therapies, particularly in the recurrent or refractory setting, with the goal of limiting further surgical or radiation-related injury to the hypothalamic–pituitary axis. Papillary craniopharyngioma (PCP), defined by near-universal BRAF V600E mutations, exhibits profound and rapid responses to combined BRAF and MEK inhibition, with objective response rates exceeding 90% in prospective studies. These responses can facilitate less extensive surgery, enable de-escalation of radiotherapy, or allow deferral of local treatment. In contrast, adamantinomatous craniopharyngioma (ACP), characterized by CTNNB1 mutations and a cystic phenotype with a prominent inflammatory microenvironment, lacks a single actionable oncogenic driver. Early clinical experience suggests that Interleukin-6/Interleukin-6 receptor (IL-6/IL-6R) blockade, alone or in combination with bevacizumab, may stabilize or reduce cystic components in selected patients, although evidence remains limited to small case series. Other systemic approaches for ACP, including MAPK pathway inhibition and immune-directed strategies, are still under investigation. Across subtypes, adverse events have generally been class-expected and manageable, but data on long-term endocrine, hypothalamic, and neurocognitive outcomes are sparse. This review synthesizes current evidence for neoadjuvant, adjuvant, and palliative craniopharyngioma systemic targeted therapies and highlights the ongoing clinical considerations of this therapy. Full article
(This article belongs to the Special Issue Clinical, Radiological, and Molecular Insights into Craniopharyngioma)
Back to TopTop