Malignant transformation of an intracranial epidermoid cyst into squamous cell carcinoma: a case report and systematic review
- 1Department of Oncology, The Second Xiangya Hospital, Central South University
- 2Village Market Department, M.P. Shah Hospital
- 3Clinical Teaching and Research Section, The Second Xiangya Hospital, Central South University
Abstract
Objective: Intracranial epidermoid cysts (IECs) are rare, benign congenital lesions that occasionally undergo malignant transformation into squamous cell carcinoma (SCC). This study presents a rare case of an IEC in the anterior cranial fossa transforming into SCC and reviews existing literature to summarize clinical characteristics, treatment strategies, and survival outcomes.
Methods: We detail the clinical presentation, management and long-term follow-up of a 52-year-old female who developed SCC 75 months after initial subtotal resection of an anterior cranial fossa IEC. We also conducted a comprehensive systematic literature search across PubMed, MEDLINE and CNKI in accordance with PRISMA guidelines compiling 59 well-documented cases (including our index case) to analyze demographic features, transformation intervals, treatment modalities and overall survival.
Results: Our patient underwent repeat subtotal tumor resection followed by adjuvant intensity-modulated radiation therapy (IMRT). She maintained a disease-free survival (DFS) of 14 months post-radiotherapy (17 months post-reoperation) until July 2026, when she developed multifocal intraventricular recurrence. The literature review revealed a mean patient age of 54.7 years with an equal gender distribution (1.03:1). The cerebellopontine angle (CPA) was the most frequent tumor site (50.85%). The mean interval from IEC diagnosis to malignant transformation was 9.24 years. Prognosis for transformed SCC remains poor, with a median overall survival of 6 months. However, patients treated with surgery combined with radiotherapy demonstrated significantly improved outcomes (median survival 13 months) compared to those undergoing surgery alone (41 days).
Conclusions: Malignant transformation of IECs into SCC is a rare, aggressive complication. Long-term surveillance with contrast-enhanced MRI is vital for patients with previously resected IECs. Early detection, maximal safe surgical resection followed by adjuvant radiotherapy currently offers the most favorable survival benefit.
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BibTeX
@article{onzere2026malignant,
title = {{Malignant transformation of an intracranial epidermoid cyst into squamous cell carcinoma: a case report and systematic review}},
author = {Lorraine Edna Onzere and Jinan Ma and Elihuruma Eliufoo Stephano and Peter Mutinda Kisangai and Yunfei Li and Weini Quan and Yuanqiang Wu},
journal = {Frontiers in Oncology},
year = {2026},
month = oct,
doi = {10.3389/fonc.2026.1978480},
url = {https://www.frontiersin.org/articles/10.3389/fonc.2026.1978480/full},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Malignant transformation of an intracranial epidermoid cyst into squamous cell carcinoma: a case report and systematic review
AU - Lorraine Edna Onzere
AU - Jinan Ma
AU - Elihuruma Eliufoo Stephano
AU - Peter Mutinda Kisangai
AU - Yunfei Li
AU - Weini Quan
AU - Yuanqiang Wu
PY - 2026
DA - 2026/10/28
JO - Frontiers in Oncology
DO - 10.3389/fonc.2026.1978480
UR - https://www.frontiersin.org/articles/10.3389/fonc.2026.1978480/full
AB - Objective: Intracranial epidermoid cysts (IECs) are rare, benign congenital lesions that occasionally undergo malignant transformation into squamous cell carcinoma (SCC). This study presents a rare case of an IEC in the anterior cranial fossa transforming into SCC and reviews existing literature to summarize clinical characteristics, treatment strategies, and survival outcomes. Methods: We detail the clinical presentation, management and long-term follow-up of a 52-year-old female who developed SCC 75 months after initial subtotal resection of an anterior cranial fossa IEC. We also conducted a comprehensive systematic literature search across PubMed, MEDLINE and CNKI in accordance with PRISMA guidelines compiling 59 well-documented cases (including our index case) to analyze demographic features, transformation intervals, treatment modalities and overall survival. Results: Our patient underwent repeat subtotal tumor resection followed by adjuvant intensity-modulated radiation therapy (IMRT). She maintained a disease-free survival (DFS) of 14 months post-radiotherapy (17 months post-reoperation) until July 2026, when she developed multifocal intraventricular recurrence. The literature review revealed a mean patient age of 54.7 years with an equal gender distribution (1.03:1). The cerebellopontine angle (CPA) was the most frequent tumor site (50.85%). The mean interval from IEC diagnosis to malignant transformation was 9.24 years. Prognosis for transformed SCC remains poor, with a median overall survival of 6 months. However, patients treated with surgery combined with radiotherapy demonstrated significantly improved outcomes (median survival 13 months) compared to those undergoing surgery alone (41 days). Conclusions: Malignant transformation of IECs into SCC is a rare, aggressive complication. Long-term surveillance with contrast-enhanced MRI is vital for patients with previously resected IECs. Early detection, maximal safe surgical resection followed by adjuvant radiotherapy currently offers the most favorable survival benefit.
SN - 2234-943X
ER -