Pineal Atypical Teratoid Rhabdoid Tumor in a 5-Month-Old Child

2020 ◽  
pp. 1-5
Author(s):  
Chandrashekhar Gendle ◽  
Madhivanan Karthigeyan ◽  
Pravin Salunke ◽  
Kirti Gupta
Keyword(s):  
Short Interval ◽  
Pineal Region ◽  
Age Group ◽  
Childhood Brain Tumors ◽  
Case Presentation ◽  
Pediatric Age Group ◽  
Very Young Children ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

<b><i>Introduction:</i></b> Atypical teratoid rhabdoid tumors (ATRT), an uncommon malignant intracranial tumor with aggressive behavior are mostly seen in posterior fossa in young pediatric age-group. <b><i>Case Presentation:</i></b> We present an infrequent location of this tumor in the pineal region in a 5-month-old infant. Also, the lesion was non-enhancing which was highly atypical of an ATRT. It was near-totally excised with the child placed in sitting position. However, within a short interval, a tumor recurrence was noted. <b><i>Conclusion:</i></b> The case possibly represents an extended spectrum of congenital childhood brain tumors. Importantly, it highlights an atypical imaging of ATRT in very young children.

Atypical teratoid/rhabdoid tumors with multilayered rosettes in the pineal region

2016 ◽  
Vol 33 (4) ◽  
pp. 261-266 ◽  
Author(s):  
Junmei Wang ◽  
Zhaoxia Liu ◽  
Jingyi Fang ◽  
Jiang Du ◽  
Yun Cui ◽  
...  
Keyword(s):  
Pineal Region ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes

2005 ◽  
Vol 18 (6) ◽  
pp. 1-5 ◽  
Author(s):  
Michael L. Chen ◽  
J. Gordon McComb ◽  
Mark D. Krieger
Keyword(s):  
Imaging Study ◽  
Signs And Symptoms ◽  
Tumor Location ◽  
Pineal Region ◽  
Rapid Progression ◽  
Atypical Teratoid ◽  
The Mean ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Object Atypical teratoid/rhabdoid tumors (ATRTs) represent a relatively newly categorized neoplastic entity. They commonly present in childhood, and have a rapidly progressive clinical course with a survival time of less than 1 year. Treatment regimens have been nonuniform. In this retrospective review of patients with ATRTs who were treated at the authors' institution according to a uniform protocol, the goal was to assess the efficacy of the treatment and its outcome. Methods Over a 7-year period, ATRT was diagnosed in 11 patients (six boys and five girls). The median age of the patients was 61 months, and their ages ranged from 3 months to 17 years. Signs and symptoms began, on average, a little more than 1 month before diagnosis and included the following: headache (36%), nausea and vomiting (46%), lethargy (18%), seizures (27%), cranial nerve findings (46%), ataxia (18%), long tract findings (18%), and hydrocephalus (46%). Tumor location was cortical in four patients, in the pineal region in four, in the posterior fossa in two, and spinal in one. In one patient disseminated disease was revealed on the initial imaging study; seven patients had disseminated tumor subsequently. Treatment consisted of chemotherapy in 11 patients, chemotherapy and local radiation in five, and chemotherapy and craniospinal radiation in three. Six patients are alive, three have died, and two were lost to follow-up review. The mean time to death was 24 months, and ranged from 2 to 67 months. Among the surviving patients the mean duration of follow up is 18.5 months and ranges from 2 to 37 months. The median time to progression was 3.5 months. Conclusions Atypical teratoid/rhabdoid tumors are malignant lesions with rapid progression. Further study is necessary to determine the efficacy of therapy.

scholarly journals ATRT-05. RESULTS OF MULTICENTER TRIAL CONCERNING THE TREATMENT OF CHILDREN WITH ATYPICAL TERATOID/RHABDOID TUMORS (ATRT) OF THE CENTRAL NERVOUS SYSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii276-iii276
Author(s):  
Olga Zheludkova ◽  
Lyudmila Olkhova ◽  
Yuri Kushel’ ◽  
Armen Melikyan ◽  
Marina Ryzhova ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Progression Free Survival ◽  
Multicenter Trial ◽  
Intrathecal Methotrexate ◽  
Subtotal Resection ◽  
Tumor Biopsy ◽  
Atypical Teratoid ◽  
The Central Nervous System ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract We analyzed 105 patients under 18 years. The median age was 21 months. There were 54 boys and 51 girls. The supratentorial tumors were in 53 patients, infratentorial in 48, and in spinal cord in 4. 60 had stage M0,29-М+and 16-Mx. All the patients got surgical treatment:total tumor removal in 34,subtotal in 37,partial in 30,and biopsy in 4;75 patients got chemoradiotherapy to ATRT-2006;6-CWS;13-EU-RHAB;5-HIT-SKK;individual schemes in 6. RESULTS: 47 are alive,1 was LFU, and 57 died. PFS was 32%±0.05; the five-year OS 40%±0.05. The median survival-30 months, the median progression-free survival-12 months, and the median of follow-up-23 months. PFS was significantly better in patients more than 12 months compared to patients younger than 12 months:40 and 12%;p=0.00161.After total resection PFS was higher compared to subtotal resection, partial resection, and tumor biopsy:48,38,0,and 0%(p=0.025). After chemoradiotherapy, PFS was higher compared to patients without radiotherapy: 49and 0%(р=0.0000000).PFS for stage M0 was higher compared to stage M+and stage Mx:41,15,and 27%,respectively(р=0.00032).PFS was better for the tumors in the spinal cord and infratentorial location compared to the supratentorial location:67,37,and 25%(p=0.0876).The survival rate was higher among the patients who got treatment according to the ATRT-2006 protocol compared to EU-RHAB, individual regimens, CWS, and HIT-SKK:39,19,17,17,and 0% respectively;p=0.00159.The survival was higher among the patients who got intraventricular/intrathecal Methotrexate,Cytarabine, Prednisolone than among the patients who got only Methotrexate or none at all:40,0,and 5%, respectively; p=0.00015. CONCLUSIONS: Survival was significantly better in patients more than 12month, without metastases, with total removal tumor, chemotheradiotherapy by ATRT-2006 protocol with i/t, i/v Methotrexate/Cytarabine/Prednisolone.

scholarly journals Histomorphology Evolution of Recurrent Atypical Teratoid/Rhabdoid Tumors

2015 ◽  
Vol 144 (suppl 2) ◽  
pp. A023-A023
Author(s):  
Esther Yoon ◽  
Taliya Farooq ◽  
Lawrence Sann ◽  
Eric Vail ◽  
George Kleinman
Keyword(s):  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals ATRT-26. META-ANALYSIS OF TREATMENT MODALITIES IN METASTATIC ATYPICAL TERATOID/RHABDOID TUMORS IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii281-iii281
Author(s):  
Reena M Underiner ◽  
Mostafa Eltobgy ◽  
Joseph R Stanek ◽  
Jonathan L Finlay ◽  
Mohamed S AbdelBaki
Keyword(s):  
Surgical Resection ◽  
Meta Analysis ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Substantial Effect ◽  
Treatment Modalities ◽  
Risk Of Death ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract BACKGROUND Metastatic atypical teratoid/rhabdoid tumors (AT/RT) are aggressive central nervous system tumors that present during infancy and are associated with dismal outcomes. Patients receive multimodal treatment including surgical resection, systemic chemotherapy and one or more of intrathecal chemotherapy (IT), marrow-ablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and radiation therapy (XRT). While data regarding treatment modalities for AT/RT patients exist, no comprehensive data have been published regarding the metastatic patient population. METHODS We performed a meta-analysis of 1,578 articles published through September 2018, including 44 studies with a total of 123 subjects. Additionally, seven patients were incorporated through chart review of patients treated at Nationwide Children’s Hospital. RESULTS Analysis of 130 patients revealed a 3-year overall survival (OS) of 25%. Age at diagnosis had a significant impact on survival (p=0.0355); 3-year OS for infants &lt; 18 months was 21%; 18–36 months was 26%; and &gt; 36 months was 36%. Location of the primary tumor, metastatic stage and extent of surgical resection did not have significant impact on OS. On univariate analysis, XRT (p&lt;0.0001), IT (p=0.01) and AuHCR (p&lt;0.0001) were found to significantly improve survival. The most substantial effect was noted in patients who received AuHCR (3-year OS of 60% versus 9% in those who did not). On multivariable analysis XRT (p=0.0006), IT (p=0.0124) and AuHCR (p&lt;0.0001) were independently associated with reduced risk of death.

scholarly journals AT-18INVESTIGATING THE BIOLOGY OF ATYPICAL TERATOID/RHABDOID TUMORS BY WHOLE GENOME CRISPR/CAS9 SCREENING

2016 ◽  
Vol 18 (suppl 3) ◽  
pp. iii5.2-iii5
Author(s):  
Matthew P. Selby ◽  
Martina A. Finetti ◽  
Matthew Bashton ◽  
Ruth E. Cranston ◽  
Alicia Del-Carpio-Pons ◽  
...  
Keyword(s):  
Whole Genome ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors
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