scholarly journals Adult Sellar Region Atypical Teratoid/Rhabdoid Tumor: A Retrospective Study and Literature Review

2020 ◽  
Vol 11 ◽  
Author(s):  
Fujun Liu ◽  
Shucai Fan ◽  
Xin Tang ◽  
Shuangmin Fan ◽  
Liangxue Zhou
Keyword(s):  
Adjuvant Radiotherapy ◽  
Rhabdoid Tumor ◽  
Subtotal Resection ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Sellar Region ◽  
Ki 67 ◽  
Duration Of Symptoms ◽  
Atypical Teratoid ◽  
Index Value ◽  
Reported Data

Background: Adult sellar region atypical teratoid/rhabdoid tumor (AT/RT) is a rare lesion. We aimed to elucidate clinical, radiologic, and pathological characteristics, treatment strategies, and outcomes of this disease.Methods: Five adult sellar AT/RT patients were retrospectively analyzed between January 2015 and December 2018. In addition, we performed a review of the reported data on adult sellar AT/RT.Results: Patients (n = 5) were female with a median age of 50 years. The mean duration of symptoms, of which headache was the most frequent, was 1.6 months (range, 2 weeks−8 months). The average tumor size was 2.82 cm (range, 1.9–4.5 cm). All lesions were irregularly shaped. MRI showed heterogeneous enhancement in three of five lesions. Four of five patients underwent subtotal resection (STR) and one gross total resection (GTR). Whereas, one patient received post-operative adjuvant radiotherapy, one patient received post-operative combination of radio- and chemotherapy. The review of the reported data showed that 39 cases of adult sellar AT/RT had been reported. The estimated median overall survival (OS) was 23 months with a 1-year survival estimate of 59.7%. The median OS for patients with GTR was 28 months and 17 months for patients with STR. Kaplan–Meier analysis showed that patients with high (≥35%) MIB-1/Ki67 index value had a significantly shorter OS compared with those with low (<35%) index value (p = 0.033), and that patients who received post-operative combination radio- and chemotherapy had longer OS than that of those who did not (p < 0.001).Conclusion: Adult sellar region AT/RT is a rapidly growing tumor with a poor prognosis. High levels of MIB1/Ki-67 on histology may indicate aggressive feature of the tumor. Maximal safe resection followed by adjuvant radiotherapy combined with chemotherapy may be the optimal therapeutic strategy for adult sellar region AT/RT.

scholarly journals Atypical Teratoid/Rhabdoid Tumor of the Sellar Region: A Case Report and Review of the Literature

2020 ◽  
Vol 10 (1) ◽  
pp. 13-16
Author(s):  
Raghad Adnan Bokhari ◽  
Mohammed Bafaqeeh ◽  
Saad Al-Obaysi ◽  
Areej Al-Aman ◽  
Wafa Alshakweer
Keyword(s):  
Case Report ◽  
Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Sellar Region ◽  
Review Of The Literature ◽  
Atypical Teratoid

scholarly journals Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult With Long Survival: Case Report and Review of the Literature

2017 ◽  
Vol 9 (3) ◽  
pp. 216-220 ◽  
Author(s):  
Mussa Hussain Almalki ◽  
Ashjan Alrogi ◽  
Abdulkarim Al-Rabie ◽  
Sadeq Al-Dandan ◽  
Abdullah Altwairgi ◽  
...  
Keyword(s):  
Case Report ◽  
Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Sellar Region ◽  
Review Of The Literature ◽  
Long Survival ◽  
Atypical Teratoid ◽  
Survival Case

Atypical teratoid/rhabdoid tumor of the sellar region in adult women: Is it a sex-related disease?

2018 ◽  
Vol 49 ◽  
pp. 16-21 ◽  
Author(s):  
Akihiro Nishikawa ◽  
Toshihiro Ogiwara ◽  
Alhusain Nagm ◽  
Kenji Sano ◽  
Maki Okada ◽  
...  
Keyword(s):  
Rhabdoid Tumor ◽  
Adult Women ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Sellar Region ◽  
Related Disease ◽  
Atypical Teratoid

Atypical teratoid/rhabdoid tumor of the pineal region in an adult

2010 ◽  
Vol 113 (2) ◽  
pp. 374-379 ◽  
Author(s):  
Hidehiro Takei ◽  
Adekunle M. Adesina ◽  
Vidya Mehta ◽  
Suzanne Z. Powell ◽  
Lauren A. Langford
Keyword(s):  
Tumor Cells ◽  
Third Ventricle ◽  
Pineal Region ◽  
Rhabdoid Tumor ◽  
Subtotal Resection ◽  
Molecular Testing ◽  
Malignant Rhabdoid Tumor ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Protein Loss ◽  
Atypical Teratoid

An atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal tumor most often occurring in the posterior fossa in children younger than 3 years of age. Adult cases of AT/RT are very rare, and 27 cases with a diagnosis of either AT/RT or (malignant) rhabdoid tumor have been reported to date. The authors report an adult case of an AT/RT occurring in the pineal region with molecular cytogenetic and immunohistochemical confirmation. A 33-year-old woman presented with a 2-month history of headache and blurred vision progressing to diplopia, and was admitted emergently due to deteriorating mental status. An MR image showed a heterogeneously enhancing mass involving the posterior third ventricle and pineal region with mild hydrocephalus. She underwent a subtotal resection of the tumor and was then treated with chemoradiation. Thirteen months after surgery, she was still alive with radiological evidence of recurrence/residual lesions. Histological sections showed epithelioid cellular sheets of rhabdoid tumor cells with scattered mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for epithelial membrane antigen and vimentin, and showed focal expression of glial fibrillary acidic protein, pancytokeratin, and neurofilament protein. Loss of nuclear immunoreactivity for INI1 protein was observed. Fluorescence in situ hybridization analysis showed monosomy 22. Histologically, this tumor consisted exclusively of epithelioid tumor cells with rhabdoid features. The differential diagnoses include rhabdoid glioblastoma, metastatic carcinoma, and rhabdoid meningioma. Molecular testing to identify monosomy 22 or deletions of the chromosome 22q11 containing the INI1/hSNF5 gene and/or immunohistochemical staining with INI1 antibody is of great importance for the diagnosis of this tumor.

scholarly journals SAT-LB304 A Rare Case of Atypical Teratoid/Rhabdoid Tumor of Sellar Region in an Adult

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Parvathy Madhavan ◽  
Paul Schwartz ◽  
Vitaly Kantorovich
Keyword(s):  
Third Ventricle ◽  
Rhabdoid Tumor ◽  
Caucasian Woman ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Sellar Region ◽  
Sellar Mass ◽  
Who Grade ◽  
Transcallosal Approach ◽  
Atypical Teratoid ◽  
Suprasellar Extension

Abstract Introduction: Atypical teratoid/rhabdoid tumor(ATRT) is an aggressive tumor of the central nervous system mostly seen in the pediatric age group. It is rare for these tumors to occur in adults, especially in the sellar region. Here we describe a case of ATRT of sellar region with rapidly progressive confusion and panhypopituitarism. Case report: 58-year-old Caucasian woman presented with complaints of headache, generalized weakness, nausea and anorexia. Patient was also noted to be confused throughout her presentation and unable to provide detailed history. She also complained of blurring of vision and was noted to have visual field loss by her ophthalmologist. CT head without contrast revealed a sellar mass with suprasellar extension which is 2.5 x 2.4 x 3.5 cm with mass-effect on the base of anterior third ventricle. Blood work showed an A.M cortisol of 0.2 mcg/dL(6.2-19.4), prolactin of 62.6 ng/mL(4.8-22.3), TSH of 2 mIU/L(0.27-4.20) and free T4 of 0.36 ng/dL [0.80-1.90]. Cosyntropin stimulation test showed a 30-minute cortisol of 2.8 mcg/dL and a 60-minute cortisol of 4 mcg/dL. She was also noted to have hypernatremia with serum sodium upto 151 mmol/L(136-145). She was started on levothyroxine 88 mcg, DDAVP 0.1 mg PO twice daily and stress dose steroids which was weaned down to prednisone 7.5 mg daily eventually. MRI revealed 2.1 cm sellar mass with 3.1 cm suprasellar extension and rapidly progressive increase in size over several weeks period. Patient initially underwent left frontal midline craniotomy via transcallosal approach for resection of third ventricular and supra sellar tumor. For the residual tumor removal, she also underwent endoscopic resection of ATRT of brain via extended transnasal craniotomy with extensive nasoseptal flap repair of the skull base. Pathology revealed atypical teratoid/rhabdoid tumor of sellar region, WHO grade IV. The neoplastic cells characteristically show loss of INI-1 (SMARCB1), retained BRG1 (SMARCA4), focal weak positivity for EMA, Vimentin, Neurofilament (NF), CD56 and Cytokeratin AE1/AE3 is strongly positive. There is patchy non-specific immunoreactivity to PAX5 and PAX8. Hospital course was complicated by respiratory failure, NSTEMI, encephalopathy and patient is currently receiving supportive care for the same. Patient will be receiving radiotherapy and chemotherapy for further management of ATRT. Conclusion:This case represents a rare occurrence of ATRT and suggests the need for suspicion in case of rapidly growing pituitary mass with progressive panhypopititarism.

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
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