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A squamous cell carcinoma arising from an intracranial epidermoid cyst: A case report with literature review
J Korean Skull Base Soc 2024;19(1):60-65
Published online May 30, 2024
© 2024 Korean Skull Base Society.

Junmo Jeon1, Hyunsik Bae2,*, Yeon-Lim Suh2, Ho Jun Seol1

1Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
2Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Correspondence to: Ho Jun Seol

*Current affiliation: Pathology Center, Seegene Medical Foundation, Seoul, Korea
Received March 7, 2024; Revised March 9, 2024; Accepted March 11, 2024.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
The malignant transformation of benign intracranial epidermoid cysts is rare. Here, we report a case of an intracranial primary squamous cell carcinoma within an epidermoid cyst. Thus, we present this case along with a comprehensive review of the relevant literature. A 56-year-old woman presented with dizziness, tinnitus, and disequilibrium persisting for three months. Initial magnetic resonance imaging (MRI) indicated a neurogenic tumor on the right side of Meckel’s cave and bilateral vestibular schwannomas, suggesting neurofibromatosis type 2. However, follow-up MRI after two months revealed a significant increase in the size of the right Meckel’s cave tumor. Consequently, surgical resection was performed, which was initially considered a rapidly growing neurogenic tumor. Histopathological examination revealed a squamous cell carcinoma arising from an epidermoid cyst. Whole-body metastasis evaluation showed no primary tumors in other areas; however, leptomeningeal seeding at the sacral canal level was identified. Subsequently, the patient underwent craniospinal irradiation and chemotherapy, followed by radiation therapy. Although most epidermoid cysts follow a benign clinical course, we encountered an exceptionally rare case of malignant transformation originating from an epidermoid cyst. Because definitive management strategies are lacking, further clinical trials concerning the optimal treatment and outcomes of this disease are required.
Keywords : Intracranial epidermoid cyst, Malignant transformation, Primary intracranial squamous cell carcinoma

May 2024, 19 (1)
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