Adult Pineal Region Atypical Teratoid Rhabdoid Tumor: A Case for Aggressive Surgical and Chemoradiation Management with Comprehensive Literature Review

Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant central nervous system neoplasm classified as an embryonal grade IV neoplasm by the World Health Organization. ATRT generally occurs in children younger than 3 years, with 85 pathologically confirmed cases reported in adults. It is...

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Bibliographic Details
Published in:World neurosurgery 2020-10, Vol.142, p.117-127
Main Authors: Mathkour, Mansour, Carsky, Katie, Chabot, A. Bert, Werner, Cassidy, Berry, John F., Carr, Christopher, Lockwood, Joseph D., Keen, Joseph R., Bui, Cuong J., Biro, Erin E.
Format: Article
Language:English
Subjects:
Adult
Atypical teratoid rhabdoid tumor
Chemoradiotherapy - methods
Humans
Male
Neurosurgical Procedures - methods
Pineal gland
Rhabdoid Tumor - diagnostic imaging
Rhabdoid Tumor - drug therapy
Rhabdoid Tumor - radiotherapy
Rhabdoid Tumor - surgery
Teratoma - diagnostic imaging
Teratoma - drug therapy
Teratoma - radiotherapy
Teratoma - surgery
Tumor
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Summary:Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant central nervous system neoplasm classified as an embryonal grade IV neoplasm by the World Health Organization. ATRT generally occurs in children younger than 3 years, with 85 pathologically confirmed cases reported in adults. It is most commonly supratentorial, with only 9 confirmed adult cases localized to the pineal region. The case is described of a 29-year-old man with a history of chronic migraines and a previously negative computed tomography scan of the head presenting with worsening headaches and new-onset diplopia with upward gaze palsy. Computed tomography and magnetic resonance imaging showed a hemorrhagic pineal mass with extension into the right thalamus. After resection, the immunohistochemical staining and cytogenetic profile proved consistent with ATRT, making it the ninth reported case of pineal ATRT in an adult, which was treated aggressively with good outcome. Adult ATRT is rare, especially in the pineal region, with only 9 cases reported. Because of the aggressiveness, ATRT must be considered in the differential diagnosis of pineal region lesions because early diagnosis and aggressive treatment are key to prolonged survival.
ISSN:1878-8750
1878-8769
DOI:10.1016/j.wneu.2020.06.144