scholarly journals Atypical teratoid/rhabdoid tumor arising from the trigeminal nerve in an adult

2016 ◽  
Vol 29 (6) ◽  
pp. 447-449 ◽  
Author(s):  
Fang Yu ◽  
Florence Chiang ◽  
Carlos Bazan
Keyword(s):  
Trigeminal Nerve ◽  
Diagnostic Testing ◽  
Rhabdoid Tumor ◽  
Imaging Features ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Immunohistochemistry Staining ◽  
Bony Destruction ◽  
Atypical Teratoid ◽  
Appropriate Therapy

A 30-year-old male presented with left facial pain and numbness. Initial MRI demonstrated an enhancing mass involving the left trigeminal nerve. Follow-up imaging showed interval growth with erosion of the sphenoid body. Surgical resection was performed and immunohistochemistry staining was consistent with an atypical teratoid/rhabdoid tumor. Awareness of this entity and its imaging features such as diffusion restriction, intratumoral hemorrhage, and bony destruction, can help guide confirmatory diagnostic testing and appropriate therapy.

Atypical Teratoid-Rhabdoid Tumor Spreading along the Trigeminal Nerve

2006 ◽  
Vol 42 (4) ◽  
pp. 258-263 ◽  
Author(s):  
R. Beschorner ◽  
M. Mittelbronn ◽  
A. Koerbel ◽  
U. Ernemann ◽  
D.R. Thal ◽  
...  
Keyword(s):  
Trigeminal Nerve ◽  
Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Atypical Teratoid

Rare intraparenchymal choroid plexus carcinoma resembling atypical teratoid/rhabdoid tumor diagnosed by immunostaining for INI1 protein

2009 ◽  
Vol 4 (4) ◽  
pp. 368-371 ◽  
Author(s):  
E. Andrew Stevens ◽  
Constance A. Stanton ◽  
Kyle Nichols ◽  
Thomas L. Ellis
Keyword(s):  
Choroid Plexus ◽  
Rhabdoid Tumor ◽  
Choroid Plexus Carcinoma ◽  
Nuclear Staining ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Imaging Studies ◽  
Atypical Teratoid ◽  
History Of ◽  
The Right

The authors present the case of a rare extraventricular, intraparenchymal choroid plexus carcinoma (CPC). This 6-year-old girl presented to the emergency department with a 1-week history of headaches, nausea, and vomiting. Imaging studies revealed an intraaxial cystic and solid mass located in the right frontal lobe with central nodular enhancement and minimally enhancing cyst walls. Gross-total resection was accomplished via craniotomy without complications. The initial pathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT); however, immunostaining for INI1 protein (using the BAF47/SNF5 antibody) showed retention of nuclear staining in the tumor cells, resulting in a change in the diagnosis to CPC. There was no evidence of recurrence at the last follow-up 2.5 years after treatment, which supports the diagnosis of CPC over AT/RT. This case emphasizes the importance of immunostaining for INI1 protein for distinguishing CPC from AT/RT in cases with atypical or indeterminate features.

scholarly journals Laser interstitial thermal therapy for pediatric atypical teratoid/rhabdoid tumor: case report

2020 ◽  
Vol 48 (1) ◽  
pp. E11
Author(s):  
Abdullah H. Feroze ◽  
Margaret McGrath ◽  
John R. Williams ◽  
Christopher C. Young ◽  
Chibawanye I. Ene ◽  
...  
Keyword(s):  
Rhabdoid Tumor ◽  
Thermal Therapy ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Laser Interstitial Thermal Therapy ◽  
Published Report ◽  
Atypical Teratoid ◽  
Left Caudate ◽  
The Right ◽  
Radiographic Improvement

Herein, the authors describe the successful use of laser interstitial thermal therapy (LITT) for management of metastatic craniospinal disease for biopsy-proven atypical teratoid/rhabdoid tumor in a 16-month-old boy presenting to their care. Specifically, LITT was administered to lesions of the right insula and left caudate. The patient tolerated 2 stages of LITT to the aforementioned lesions without complication and with evidence of radiographic improvement of lesions at the 2- and 6-month follow-up appointments. To the authors’ knowledge, this represents the first such published report of LITT for management of atypical teratoid/rhabdoid tumor.

Central Nervous System Atypical Teratoid/Rhabdoid Tumor: Results of Therapy in Children Enrolled in a Registry

2004 ◽  
Vol 22 (14) ◽  
pp. 2877-2884 ◽  
Author(s):  
Joanne M. Hilden ◽  
Sharon Meerbaum ◽  
Peter Burger ◽  
Jonathan Finlay ◽  
Anna Janss ◽  
...  
Keyword(s):  
Treatment Guidelines ◽  
Tumor Therapy ◽  
Intrathecal Chemotherapy ◽  
Rhabdoid Tumor ◽  
Primary Therapy ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Stem Cell Rescue ◽  
Atypical Teratoid ◽  
Disseminated Disease

Purpose Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor. Materials and Methods A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. Follow-up information was sought twice yearly. Results Information was complete for 42 patients. Median age at diagnosis was 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen tumors were infratentorial; 26 were supratentorial. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (3 to 62 months from diagnosis) and one patient died of toxicity. Fourteen patients (33%) show no evidence of disease (9.5 to 96 months from diagnosis). The median survival is 16.75 months and the median event-free survival is 10 months. Conclusion Aggressive therapy has prolonged the natural history in a subset of children. Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Enrollment onto the AT/RT registry should be continued.

scholarly journals ATRT-03. IDENTIFICATION OF microRNA-BASED PROGNOSTIC BIOMARKERS AND CANDIDATE THERAPEUTIC AGENTS FOR ATYPICAL TERATOID/RHABDOID TUMOR

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii275-iii276
Author(s):  
Yang Zhang ◽  
Jianguo Xu
Keyword(s):  
Target Genes ◽  
Kinase Inhibitor ◽  
Therapeutic Agents ◽  
Rhabdoid Tumor ◽  
Janus Kinase ◽  
Prognostic Biomarkers ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Overlapping Genes ◽  
Hub Genes ◽  
Atypical Teratoid

Abstract BACKGROUND MicroRNA (miRNA) has been found to be involved in development of many malignant pediatric brain tumors, including atypical teratoid/rhabdoid tumor (AT/RT) that is highly aggressive and carries a dismal prognosis. The current study investigated the potential value of miRNAs and pivotal genes associated with AT/RT using bioinformatics analysis, aiming to identify new prognostic biomarkers and candidate drugs for AT/RT patients. METHODS Differentially expressed miRNAs (DEMs) and genes (DEGs) between AT/RT and normal control samples were obtained from GEO database. The target genes of DEMs were predicted via TargetScanHuman7.2 and miRDB, and then intersected with DEGs. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes analyses of overlapping genes were conducted, followed by construction of protein-protein interaction network. Hub genes were determined by Cytoscape software, and their prognostic values were evaluated using Kaplan-Meier analysis. Connectivity Map database was used to identify latent therapeutic agents. RESULTS A total of 11 DEMs (hsa-miR-1224-5p, hsa-miR-128-3p, hsa-miR-17-5p, hsa-miR-18b-5p, hsa-miR-29c-5p, hsa-miR-329-3p, hsa-miR-379-5p, hsa-miR-433-3p, hsa-miR-488-5p, hsa-miR-656-3p and hsa-miR-885-5p) were screened. By intersecting 3275 predicted target genes and 925 DEGs, we finally identified 226 overlapping genes that were enriched in pathways in cancer and MAPK signaling pathway. Four hub genes (GRIA2, NRXN1, SLC6A1 and SYT1) were significantly associated with the overall survival of AT/RT patients. Candidate drugs included histone deacetylase inhibitor (givinostat), DNA synthesis inhibitor (floxuridine), cyclin-dependent kinase inhibitor (purvalanol) and janus kinase inhibitor (lestaurtinib). CONCLUSION In summary, this study systematically analyzed AT/RT-related miRNAs and pivotal genes to provide novel prognostic biomarkers and potential therapeutic agents.

Atypical Teratoid Rhabdoid Tumor in a Newborn: Can IVF Be a Risk Factor?

2021 ◽  
pp. 1-5
Author(s):  
Turkay Rzayev ◽  
Kubra Gokce ◽  
Safak Gucyetmez ◽  
Suheyla Bozkurt ◽  
Adnan Dagcinar ◽  
...  
Keyword(s):  
Risk Factor ◽  
Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Atypical Teratoid

Epithelioid Sarcoma Arising in a Long-Term Survivor of an Atypical Teratoid/Rhabdoid Tumor in a Patient With Rhabdoid Tumor Predisposition Syndrome

2021 ◽  
pp. 109352662098649
Author(s):  
Tiffany G Baker ◽  
Michael J Lyons ◽  
Lee Leddy ◽  
David M Parham ◽  
Cynthia T Welsh
Keyword(s):  
Epithelioid Sarcoma ◽  
Rhabdoid Tumor ◽  
Malignant Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Diagnostic Challenge ◽  
Patient Demographics ◽  
Immunohistochemical Markers ◽  
Genetic Aberration ◽  
Atypical Teratoid ◽  
Rhabdoid Tumors

Rhabdoid tumor predisposition syndrome (RTPS) is defined as the presence of a SMARCB1 or SMARCA4 genetic aberration in a patient with malignant rhabdoid tumor. Patients with RTPS are more likely to present with synchronous or metachronous rhabdoid tumors. Based on the current state of rhabdoid tumor taxonomy, these diagnoses are based largely on patient demographics, anatomic location of disease, and immunohistochemistry, despite their nearly identical histologic and immunohistochemical profiles. Thus, the true distinction between such tumors remains a diagnostic challenge. Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) is a rare, aggressive, primarily pediatric malignancy with variable histologic features and a well documented association with loss of SMARCB1 expression. Epithelioid sarcoma (ES) is a rare soft tissue tumor arising in patients of all ages and characteristically staining for both mesenchymal and epithelial immunohistochemical markers while usually demonstrating loss of SMARCB1 expression. To our knowledge we herein present the first documented case of a patient with RTPS who presented with metachronous AT/RT and ES.

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