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Clinicopathological characteristics and outcomes in Embryonal tumor with multilayered rosettes: a decade long experience from a tertiary care centre
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  • Kirti Gupta,
  • Ridhi Sood,
  • Pravin Salunke,
  • Debajyoti Chatterjee,
  • Renu Madan,
  • Chirag Ahuja ,
  • Richa Jain,
  • Amita Trehan,
  • Bishan Radotra
Kirti Gupta
Post Graduate Institute of Medical Education and Research

Corresponding Author:kirtigupta10@yahoo.co.in

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Ridhi Sood
Post Graduate Institute of Medical Education and Research
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Pravin Salunke
Post Graduate Institute of Medical Education and Research
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Debajyoti Chatterjee
Post Graduate Institute of Medical Education and Research
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Renu Madan
Post Graduate Institute of Medical Education and Research
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Chirag Ahuja
Post Graduate Institute of Medical Education and Research
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Richa Jain
Postgraduate Institute of Medical Education & Research
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Amita Trehan
postgraduate Institute of Medical Education & Research
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Bishan Radotra
Post Graduate Institute of Medical Education and Research
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Abstract

Background: Embryonal tumor with multilayered rosettes (ETMR) are a heterogenous group clinically, pathologically and topographically. Due to limited cases, data regarding its molecular genetics, pathology and prognostic factors is evolving. We retrospectively analysed our cohort of ETMR over last decade in order to study their clinicopathological characteristics and outcome. Methods: Our cohort consisted of patients diagnosed with Embryonal tumor with abundant neuropil and true rosettes (ETANTR)/Ependymoblastoma (EBL)/ Medulloepithelioma (MEPL) over the past decade. Clinical details, including outcome and imaging data was retrieved. Histological analysis including immunohistochemical work-up was performed. Results: Cohort included 15 patients with age range between 1-28 years and M:F ratio of 1.5:1. Supratentorial location predominated in comparison to tumors arising in posterior fossa. ETANTR and EBL patterns were equally distributed (40% each), followed by one case each of mixed pattern (EBL+ETANTR), MEPL and embryonal tumor, unclassified. All tumors readily expressed LIN 28A and INI-1 was retained. Recurrence with evidence of glial and rhabdoid differentiation was noted in a single patient 9 months following resection. Follow-up period ranged from 1-31 months, with overall median survival of 6.4 months. Eight patients were planned for adjuvant treatment following surgery, of which only four could complete it. All patients, except for one, succumbed to the disease. Conclusions: ETMR have a heterogenous morphology and gathers ETANTR, EBL, MEPL within its spectrum. Following treatment, the recurrent tumor may feature glial/rhabdoid differentiation. LIN28A is expressed in all cases, however should be interpreted in context of histology. Prognosis of ETMR remains dismal despite multimodal therapy.