scholarly journals Outcomes with respect to extent of surgical resection for pediatric atypical teratoid rhabdoid tumors

2019 ◽  
Vol 36 (4) ◽  
pp. 713-719
Author(s):  
Alexandra Richards ◽  
Ronak Ved ◽  
Christopher Murphy ◽  
Dawn Hennigan ◽  
John-Paul Kilday ◽  
...  
Keyword(s):  
Overall Survival ◽  
Surgical Resection ◽  
Total Resection ◽  
Term Survival ◽  
Common Location ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors ◽  
Extent Of Surgical Resection

Abstract Purpose To evaluate overall survival for atypical teratoid rhabdoid tumors (ATRTs) in relation to extent of surgical resection. Methods The neurosurgical tumor databases from three UK Pediatric centers (University Hospital of Wales, Alder Hey and Royal Manchester Children’s Hospital) were analyzed. Patients with a diagnosis of ATRT were identified between 2000 and 2018. Data was collected regarding demographics, extent of resection, complications, and overall survival. Results Twenty-four patients diagnosed with ATRT underwent thirty-eight operations. The age range was 20 days to 147 months (median 17.5 months). The most common location for the tumor was the posterior fossa (nine patients; 38%). Six patients (25%) underwent a complete total resection (CTR), seven (29%) underwent a near total resection (NTR), eight (33.3%) underwent a subtotal resection (STR), and three patients (12.5%) had biopsy only. Two-thirds of patients who underwent a CTR are still alive, as of March 2019, compared to 29% in the NTR and 12.5% in the STR groups. Out of the thirty-eight operations, there were a total of twenty-two complications, of which the most common was pseudomeningocele (27%). The extent of surgical resection (p = 0.021), age at surgery (p = 0.00015), and the presence of metastases at diagnosis (0.015) significantly affected overall survival. Conclusions Although these patients are a highly vulnerable group, maximal resection is recommended where possible, for the best chance of long-term survival. However, near total resections are likely beneficial when compared with subtotal resections and biopsy alone. Maximal surgical resection should be combined with adjuvant therapies for the best long-term outcomes.

Long-term survival following additive radiotherapy in patients with atypical teratoid rhabdoid tumors

2016 ◽  
Vol 192 (8) ◽  
pp. 569-581 ◽  
Author(s):  
Khaled Elsayad ◽  
Jan Kriz ◽  
Laith Samhouri ◽  
Uwe Haverkamp ◽  
Ronald Straeter ◽  
...  
Keyword(s):  
Term Survival ◽  
Long Term Survival ◽  
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 < 18 months was 21%; 18–36 months was 26%; and > 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<0.0001), IT (p=0.01) and AuHCR (p<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<0.0001) were independently associated with reduced risk of death.

Neurocognitive evaluation of long term survivors of atypical teratoid rhabdoid tumors (ATRT): The Canadian registry experience

2015 ◽  
Vol 62 (7) ◽  
pp. 1265-1269 ◽  
Author(s):  
Lucie Lafay-Cousin ◽  
Taryn Fay-McClymont ◽  
Donna Johnston ◽  
Chris Fryer ◽  
Katrin Scheinemann ◽  
...  
Keyword(s):  
Neurocognitive Evaluation ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors ◽  
Long Term Survivors

Does Surgical Resection Have a Role in the Treatment of Large or Multinodular Hepatocellular Carcinoma?

2010 ◽  
Vol 76 (11) ◽  
pp. 1189-1197 ◽  
Author(s):  
Giovanni Ramacciato ◽  
Paolo Mercantini ◽  
Niccolò Petrucciani ◽  
Matteo Ravaioli ◽  
Alessandro Cucchetti ◽  
...  
Keyword(s):  
Overall Survival ◽  
Liver Resection ◽  
Surgical Resection ◽  
Survival Rates ◽  
Treatment Modalities ◽  
Impaired Liver Function ◽  
Term Survival ◽  
Impaired Liver ◽  
Disease Free

Several effective treatments are available for patients with small solitary hepatocellular carcinomas (HCCs). Conversely, the management of patients with large or multinodular HCCs is controversial, and the role of surgical resection is not well defined. Between 2000 and 2006, 51 patients with large or multinodular HCC underwent liver resection. Clinicopathologic and follow-up data were prospectively collected and retrospectively reviewed. The perioperative and long-term outcomes were analyzed. Univariate and multivariate analysis of prognostic factors were conducted. Although 20 patients had multinodular HCCs, 31 had large solitary tumors. Perioperative mortality occurred in eight patients and complications in 15. In patients with large solitary tumors, 5-year disease-free and overall survival were 41.3 per cent and 56.1 per cent, respectively. Those with multinodular HCCs demonstrated 5-year disease-free and overall survival rates of 0 per cent and 33.6 per cent, respectively. Liver resection can result in long-term survival in select patients with large or multinodular HCCs, even in select patients with impaired liver function. Large solitary HCCs seem to have better prognoses than multinodular tumors, with lower recurrence and higher survival rates after surgery. Randomized controlled trials comparing resection to other treatment modalities are indicated to determine optimal patient management.

scholarly journals Clinical Predictors of Survival for Patients with Atypical Teratoid/Rhabdoid Tumors

2022 ◽  
Author(s):  
Vismaya S Bachu ◽  
Pavan Shah ◽  
Adrian E Jimenez ◽  
Adham M Khalafallah ◽  
Jignesh Tailor ◽  
...  
Keyword(s):  
Overall Survival ◽  
Combination Therapy ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Bivariate Analysis ◽  
Cell Transplant ◽  
Clinical Predictors ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract Purpose: Atypical teratoid/rhabdoid tumors (AT/RT) are malignant central nervous system (CNS) neoplasms of the young. Our study analyzed a large AT/RT cohort from the National Cancer Database (NCDB) to elucidate predictors of short-term mortality and overall survival (OS). Methods: Information was collected on patients with histologically-confirmed AT/RT using the NCDB (2004-2016). Kaplan-Meier analysis indicated OS. Prognostic factors for 30-day mortality, 90-day mortality, and OS were determined via multivariate Cox proportional-hazards (CPH) and logistic regression models. Results: Our cohort of 189 patients had a median age of 1 year (IQR [1, 4]) and tumor size of 4.7±2.0 cm at diagnosis. Seventy-two percent were under 3 years old; 55.6% were male and 71.0% were Caucasian. Fifty (27.2%) patients received only surgery (S) (OS=5.91 months), 51 (27.7%) received surgery and chemotherapy (S+CT) (OS=11.2 months), and 9 (4.89%) received surgery and radiotherapy (S+RT) (OS=10.3 months). Forty-five (24.5%) received S+CT+RT combination therapy (OS=45.4 months), 13 (17.1%) received S+CT+BMT/SCT (bone marrow or stem cell transplant) (OS=55.5 months), and 16 (8.70%) received S+CT+RT+BMT/SCT (OS=68.4 months). Bivariate analysis of dichotomized age (HR=0.550, 95% CI[0.357, 0.847], p=0.0067) demonstrated significantly increased patient survival if diagnosed at or above 1 year old. On multivariate analysis, administration of S+CT+RT, S+CT+BMT/SCT, or S+CT+RT+BMT/SCT combination therapy predicted significantly (p<0.05) increased OS compared to surgery alone. Conclusion: AT/RTs are CNS tumors where those diagnosed under 1 year old have a significantly worse prognosis. Our study demonstrates that while traditional CT, RT, and BMT/SCT combination regimens prolong life, overall survival in this population is still low.

scholarly journals Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Fahd Refai ◽  
Haneen Al-Maghrabi ◽  
Hassan Al Trabolsi ◽  
Jaudah Al-Maghrabi
Keyword(s):  
Multimodal Therapy ◽  
Tumor Suppressor Protein ◽  
Central Nervous System Tumors ◽  
Histopathological Diagnosis ◽  
Atypical Teratoid Rhabdoid Tumor ◽  
Thoracic Spinal ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1. Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy.

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