Abstract
1496 Malignant Craniopharyngioma: Institutional Experience and Literature Review
Laboratory investigation, Vol.105(3 Supplement), 103737
03/2025
DOI: 10.1016/j.labinv.2024.103737
Abstract
Background
Malignant craniopharyngiomas, de novo or via malignant transformation, are exceedingly rare, with a dismal prognosis and unclear treatment standards. Little is known about the factors involved in its development. In this study, we seek to better understand the pathogenesis of malignant craniopharyngiomas.
Design
A natural language search was performed in CoPath to identify all craniopharyngioma cases at our institution over the past 35 years. In addition, a literature review was conducted to identify all previously published malignant craniopharyngioma cases.
Results
Seventy-eight craniopharyngiomas were diagnosed in 69 patients. Fifty-six (81%) patients had adamantinomatous-type (25 males, 31 females; median age at initial diagnosis = 22 years) and 13 (19%) had papillary-type (8 males, 5 females; median age at initial diagnosis = 47 years). Among those, a unique case of malignant craniopharyngioma was identified in a 36-year-old male who was initially diagnosed with a benign adamantinomatous craniopharyngioma 16 years prior. He unfortunately passed away shortly after being placed on hospice 2 months after the malignant diagnosis. A literature review identified 44 cases of malignant craniopharyngiomas (our case included) with a median age of 28 years and a median overall survival of 6 months. Of those, 17 cases (39%; 11 de novo and 6 via malignant transformation) occurred in patients without a history of prior radiation. Since a BAP1 mutation has recently been reported in a case of malignant craniopharyngioma, we performed BAP1 immunohistochemistry in our malignant craniopharyngioma case, which demonstrated complete loss of nuclear expression in the tumor, suggesting a potential BAP1 mutation. BAP1 immunohistochemistry was further performed in 10 most recent cases of benign craniopharyngiomas, all of which demonstrated normal retained nuclear expression.
Conclusions
Our literature review demonstrated that malignant craniopharyngioma tends to occur in young adults with a median overall survival of only 6 months. Since a large percentage (41%) of malignant craniopharyngiomas occurred in patients without prior radiation, other mechanisms might have contributed to their development in these patients. In addition, our malignant craniopharyngioma is the second case to show a potential BAP1 mutation. BAP1 mutation may play a role in the pathogenesis of malignant craniopharyngioma and may serve as a potential target for therapeutic intervention.
Details
- Title: Subtitle
- 1496 Malignant Craniopharyngioma: Institutional Experience and Literature Review
- Creators
- Thomas Auen - University of Nebraska Medical CenterIsabella Zhang - Boys TownJordan Burr - University of Nebraska Medical CenterMatthew Carda - University of Nebraska Medical CenterWeiwei Zhang - University of Nebraska Medical CenterJesse Cox - University of Nebraska Medical CenterAllison Cushman-Vokoun - Nebraska Medical CenterSahara Cathcart - University of Nebraska Medical CenterJie Chen - University of Nebraska Medical Center
- Resource Type
- Abstract
- Publication Details
- Laboratory investigation, Vol.105(3 Supplement), 103737
- DOI
- 10.1016/j.labinv.2024.103737
- ISSN
- 0023-6837
- eISSN
- 1530-0307
- Publisher
- Elsevier Inc
- Language
- English
- Date published
- 03/2025
- Academic Unit
- Pathology
- Record Identifier
- 9985134647302771
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