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

  • Clinical Article - Brain Tumors
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Abstract

Objective

We intended to assess the clinicopathological features and treatment outcome in patients of intracranial atypical teratoid rhabdoid tumor (AT/RT), a rare malignant tumor of the brain.

Methods

Medical records were reviewed and clinical data collected on AT/RT in a 6-year period (2006–2012). Overall survival was analyzed by Kaplan–Meier method. Univariate analysis of factors predictive of overall survival was done by log-rank test.

Results

Fifteen patients met the study criterion (male:female = 4:1). Median age at presentation was 5 years (range, 0.8–8 years). Presenting complaints included vomiting (73.33 %), headache (46.67 %), orbital symptoms (33.33 %), motor impairment (26.67 %), gait abnormality (20 %), and seizure (20 %). Median duration of symptoms was noted to be 2 months (range, 0.5–6 months). On contrast-enhanced MRI of brain, tumor location was supratentorial in 60 % patients and infratentorial in 40 % of patients. Cystic component and hydrocephalus were noted in 73.33 % patients each, whereas contrast enhancement and calcification were discerned in 53.33 and 40 % of the patients, respectively. All patients underwent tumor resection—gross total (26.67 %), near-total (13.33 %) and subtotal (60 %). Histopathology was confirmative of AT/RT with MIB-1 labeling index varying from 11 to 85 % (median 45 %). There was a lack of immunostaining for INI-1 protein, suggesting INI-1gene mutation or deletion. Adjuvant radiation (36 Gray/20 fractions/4 weeks to entire neuraxis followed by local boost 20 Gray/10 fractions/2 weeks) was started in six patients (40 %) and completed in five patients. Young age at presentation and poor performance status precluded the use of radiation in the remainder. Systemic chemotherapy was administered in ten (66.67 %) patients. Median number of cycles given was three (range, 1–12) with ICE (ifosfamide, carboplatin, etoposide) and VAC (vincristine, dactinomycin, cyclophosphamide) being the common regimens (26.67 and 20 %, respectively). After a median follow-up of 8.33 months (mean, 12.27 months), median overall survival was noted to be 10 months. At last follow-up, two patients are in complete response, one patient is on treatment, three patients are alive with evidence of disease, and nine patients expired due to disease progression. The 1- and 2-year actuarial rate of overall survival was noted to be 48.1 and 24.1 %, respectively. On univariate analysis, extent of surgery (p = 0.0149), use of craniospinal radiation (p = 0.0087), and MIB1 labeling index (p = 0.0034) were significant predictors of overall survival while age (≥5 years versus <5 years) was of borderline significance (p = 0.08).

Conclusions

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.

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Authors and Affiliations

  1. Departments of Radiotherapy, All India Institute of Medical Sciences, New Delhi, India

    Ahitagni Biswas, Pramod Kumar Julka & Goura Kishor Rath

  2. Departments of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India

    Sameer Bakhshi

  3. Departments of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India

    Ashish Suri

Authors
  1. Ahitagni Biswas
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  2. Pramod Kumar Julka
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  3. Sameer Bakhshi
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  4. Ashish Suri
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  5. Goura Kishor Rath
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Correspondence to Ahitagni Biswas.

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Biswas, A., Julka, P.K., Bakhshi, S. et al. Intracranial atypical teratoid rhabdoid tumor: current management and a single institute experience of 15 patients from north India. Acta Neurochir 157, 589–596 (2015). https://doi.org/10.1007/s00701-015-2355-2

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  • DOI: https://doi.org/10.1007/s00701-015-2355-2

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