High-Grade Atypical Teratoid/Rhabdoid Tumor in the Pituitary Region

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Abstract

Atypical teratoid/rhabdoid tumors (AT/RTs) are embryological tumors of the central nervous system (CNS). They are typically found in children, with rare presentations in adults. We describe the presentation of an AT/RT in the pituitary region of a 37-year-old female. The patient presented with a two-week history of intractable cephalgia with sudden onset of monocular diplopia and left-sided cranial nerve VI palsy. The patient underwent transsphenoidal resection of their mass, which revealed the diagnosis. She then underwent systemic therapy with chemotherapy as well as radiation. She ultimately died 14 months after treatment completion due to unrelated events. The case highlights the rarity of AT/RT in adults, emphasizing the challenge of establishing standardized treatment protocols due to its rarity in adult presentations.

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The 2021 WHO Classification of Tumors of the Central Nervous System: a summary

David N Louis, Arie Perry, Pieter Wesseling … (2021)

The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors. Building on the 2016 updated fourth edition and the work of the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy, the 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification. At the same time, it remains wedded to other established approaches to tumor diagnosis such as histology and immunohistochemistry. In doing so, the fifth edition establishes some different approaches to both CNS tumor nomenclature and grading and it emphasizes the importance of integrated diagnoses and layered reports. New tumor types and subtypes are introduced, some based on novel diagnostic technologies such as DNA methylome profiling. The present review summarizes the major general changes in the 2021 fifth edition classification and the specific changes in each taxonomic category. It is hoped that this summary provides an overview to facilitate more in-depth exploration of the entire fifth edition of the WHO Classification of Tumors of the Central Nervous System.

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CBTRUS Statistical Report: Pediatric Brain Tumor Foundation Childhood and Adolescent Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014–2018

Quinn Ostrom, Mackenzie Price, Katherine Ryan … (2022)

The CBTRUS Statistical Report: Pediatric Brain Tumor Foundation Childhood and Adolescent Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014–2018 comprehensively describes the current population-based incidence of primary malignant and non-malignant brain and other CNS tumors in children and adolescents ages 0–19 years, collected and reported by central cancer registries covering approximately 100% of the United States population. Overall, brain and other CNS tumors are the most common solid tumor, the most common cancer, and the most common cause of cancer death in children and adolescents ages 0–19 years. This report aims to serve as a useful resource for researchers, clinicians, patients, and families.

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Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992–2012

Irene Slavc, Monika Chocholous, Ulrike Leiss … (2013)

Atypical teratoid rhabdoid tumors (ATRTs) are recently defined highly aggressive embryonal central nervous system tumors with a poor prognosis and no definitive guidelines for treatment. We report on the importance of an initial correct diagnosis and disease-specific therapy on outcome in 22 consecutive patients and propose a new treatment strategy. From 1992 to 2012, nine patients initially diagnosed correctly as ATRT (cohort A, median age 24 months) were treated according to an intensive multimodal regimen (MUV-ATRT) consisting of three 9-week courses of a dose-dense regimen including doxorubicin, cyclophosphamide, vincristine, ifosfamide, cisplatin, etoposide, and methotrexate augmented with intrathecal therapy, followed by high-dose chemotherapy (HDCT) and completed with local radiotherapy. Thirteen patients were treated differently (cohort B, median age 30 months) most of whom according to protocols in use for their respective diagnoses. As of July 2013, 5-year overall survival (OS) and event-free survival (EFS) for all 22 consecutive patients was 56.3 ± 11.3% and 52.9 ± 11.0%, respectively. For MUV-ATRT regimen-treated patients (cohort A) 5-year OS was 100% and EFS was 88.9 ± 10.5%. For patients treated differently (cohort B) 5-year OS and EFS were 28.8 ± 13.1%. All nine MUV-ATRT regimen-treated patients are alive for a median of 76 months (range: 16–197), eight in first complete remission. Our results compare favorably to previously published data. The drug combination and sequence used in the proposed MUV-ATRT regimen appear to be efficacious in preventing early relapses also in young children with M1–M3 stage disease allowing postponement of radiotherapy until after HDCT.

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Journal

Journal ID (nlm-ta): Cureus

Journal ID (iso-abbrev): Cureus

Journal ID (issn): 2168-8184

Title: Cureus

Publisher: Cureus (Palo Alto (CA) )

ISSN (Electronic): 2168-8184

Publication date (Electronic, pub): 12 February 2024

Publication date (Electronic, collection): February 2024

Volume: 16

Issue: 2

Electronic Location Identifier: e54097

Affiliations

[1 ] Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA

[2 ] Department of Head and Neck Surgery, Orlando Health Cancer Institute, Orlando, USA

[3 ] Department of Pathology, Orlando Health Cancer Institute, Orlando, USA

[4 ] Department of Radiation Oncology, Orlando Health Cancer Institute, Orlando, USA

[5 ] Department of Neurological Surgery, Orlando Health Cancer Institute, Orlando, USA

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Vincent S. Alexander alexanderv@ 123456acom.edu

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DOI: 10.7759/cureus.54097

PMC ID: 10938043

PubMed ID: 38487122

SO-VID: c7e2a8bc-11be-454c-aeca-c245d09afada

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This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

High-Grade Atypical Teratoid/Rhabdoid Tumor in the Pituitary Region

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Most cited references 11

The 2021 WHO Classification of Tumors of the Central Nervous System: a summary

CBTRUS Statistical Report: Pediatric Brain Tumor Foundation Childhood and Adolescent Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014–2018

Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992–2012

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