Intracranial atypical teratoid rhabdoid tumor: current management and a single institute experience of 15 patients from north India

Biswas, Ahitagni; Julka, Pramod Kumar; Bakhshi, Sameer; Suri, Ashish; Rath, Goura Kishor

doi:10.1007/s00701-015-2355-2

Cited by 26 publications

(44 citation statements)

References 25 publications

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“…However, these authors also state that, with a postoperative mortality rate of 4%, surgery may not be beneficial above receiving chemotherapy alone. Conversely, more recent work by Biswas et al and Lafay-Cousin et al suggest that CTR correlates with an increased 2-year survival from 21.7% (STR) to 60% (CTR) [7,22]. This is similar to the findings of the present study, which identified a trend toward increased survival with maximal resection (66.6% compared to 29% and 12.5% in the NTR and STR groups, respectively; Table 1).…”

Section: Discussionsupporting

confidence: 90%

“…3 Kaplan-Meier curves comparing survival of patients after CTR, NTR, and STR. Logrank test identified that greater resection was significantly associated with increased survival (p = 0.048) resection with increased survival [7,15,17,18,22,29]. Previous studies have estimated that 30% of patients will have metastatic disease at presentation, of which there is a higher incidence in patients less than 3 years old [12,29].…”

Section: Discussionmentioning

confidence: 95%

“…Due to the lack of large prospective studies regarding ATRT, there is still uncertainty regarding the optimal treatment for these children and therefore, management strategies vary. It is, however, generally accepted that these patients need a combination of surgical resection followed by adjuvant chemotherapy (as per the European Rhabdoid Tumor Registry 2007 Protocol) plus or minus radiotherapy, according to age at presentation [7,8,27].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2019

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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.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

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

et al. 2019

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“…Patients can also present with cranial nerve palsies (cranial nerves VII and VIII) if cerebellopontine angle is involved by tumor. 28 – 30 …”

Section: Diagnosismentioning

confidence: 99%

Atypical teratoid/rhabdoid tumors: challenges and search for solutions

Biswas¹,

Kashyap²,

Kakkar³

et al. 2016

CMAR

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106

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Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%–2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myriad of diagnostic and therapeutic challenges. On the basis of radiology and histopathology alone, distinction of AT/RT from medulloblastoma or primitive neuroectodermal tumor is difficult, and hence this tumor has been commonly misdiagnosed as primitive neuroectodermal tumor for decades. Presence of a bulky heterogeneous solid-cystic mass with readily visible calcification and intratumor hemorrhage, occurring off-midline in children <3 years of age, should alert the radiologist toward the possibility of AT/RT. Presence of rhabdoid cells on histopathology and polyphenotypic immunopositivity for epithelial, mesenchymal, and neuroectodermal markers along with loss of expression of SMARCB1/INI1 or SMARCA4/BRG1 help in establishing a diagnosis of AT/RT. The optimal management comprises maximal safe resection followed by radiation therapy and multiagent intensive systemic chemotherapy. Gross total excision is difficult to achieve in view of the large tumor size and location and young age at presentation. Leptomeningeal spread is noted in 15%–30% of patients, and hence craniospinal irradiation followed by boost to tumor bed is considered standard in children older than 3 years. However, in younger children, craniospinal irradiation may lead to long-term neurocognitive and neuroendocrine sequel, and hence focal radiation therapy may be a pragmatic approach. In this age group, high-dose chemotherapy with autologous stem cell rescue may also be considered to defer radiation therapy, but this approach is also associated with significant treatment-related morbidity and mortality. Novel small molecule inhibitors hold promise in preclinical studies and should be considered in patients with relapsed or refractory tumor.

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“…An atypical teratoid/rhabdoid tumor is a rare disease with unfavorable outcome most commonly seen in children, although few cases of adult AT/RT have also been reported (20). Patient age at the time of diagnosis ranges between 7.2 months and 5 years (1,7,21,22,25,26,28); however, cases of in utero diagnosis have also been reported (4).…”

Section: █ Discussionmentioning

confidence: 99%

Survival benefit with gross total resection and adjuvant radiotherapy in childhood atypical teratoid/rhabdoid tumors: results of a single-center cohort of 27 cases

Işıkay¹,

Hanalıoğlu²,

Başar³

et al. 2019

Turkish Neurosurgery

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AIM:To share a single center experience with 27 atypical teratoid/rhabdoid tumor (AT/RT) cases, and to determine the effect of gross total tumor resection and other clinical characteristics on the overall survival rate of AT/RT. MATERIAL and METHODS:We included 27 patients-with a histopathologically confirmed primary intracranial childhood AT/ RT-who were operated in our clinic between January 2000 and December 2017. Age, sex, tumor location, disseminated disease, the presence of hydrocephalus, symptom duration till diagnosis, the extent of resection, and adjuvant radiotherapy were evaluated for their influence on overall survival. RESULTS:Median age at diagnosis for 27 patients was 19.1 months (7.2 months-5 years). Gross total resection was possible in 13 (48.72%) patients. Except for three patients who died of perioperative complications, all patients received chemotherapy and 11 received radiotherapy. In univariate analysis, male sex, older age at diagnosis (≥24 months), gross total resection, and radiotherapy were associated with overall longer survival; however, radiotherapy remained the only significant parameter in multivariate analysis. CONCLUSION:AT/RT is a rare and dreadful brain tumor that has low survival rates despite contemporary treatment. Radiotherapy seems to prolong survival; however, large-scale studies are needed to establish prognostic factors.

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Intracranial atypical teratoid rhabdoid tumor: current management and a single institute experience of 15 patients from north India

Abstract: Median survival of 10 months reflects the aggressive biology of this rare neoplasm. Maximal safe resection followed by craniospinal irradiation and systemic chemotherapy with ICE or VAC regimen is a reasonable treatment strategy in this uncommon malignancy.

Cited by 26 publications

References 25 publications

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

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

Atypical teratoid/rhabdoid tumors: challenges and search for solutions

Survival benefit with gross total resection and adjuvant radiotherapy in childhood atypical teratoid/rhabdoid tumors: results of a single-center cohort of 27 cases

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