Clinicopathological characteristics and outcomes in embryonal tumor with multilayered rosettes: A decade long experience from a tertiary care centre in North India
| Autor: | Pravin Salunke, Ridhi Sood, Bishan Dass Radotra, Amita Trehan, Richa Jain, Renu Madan, Debajyoti Chatterjee, Kirti Gupta, Chirag Kamal Ahuja |
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| Rok vydání: | 2021 |
| Předmět: |
Adult
Male 0301 basic medicine Pathology medicine.medical_specialty Neuropil Adolescent India Context (language use) Tertiary care Pathology and Forensic Medicine Cohort Studies Tertiary Care Centers Young Adult 03 medical and health sciences 0302 clinical medicine medicine Humans Neuroectodermal Tumors Primitive Child Retrospective Studies Brain Neoplasms business.industry Infant RNA-Binding Proteins Cell Differentiation Histology Multimodal therapy SMARCB1 Protein General Medicine Neoplasms Germ Cell and Embryonal Prognosis medicine.disease Immunohistochemistry Survival Rate 030104 developmental biology Case-Control Studies Child Preschool 030220 oncology & carcinogenesis Cohort Neoplasm Recurrence Local Medulloepithelioma business Follow-Up Studies Ependymoblastoma |
| Zdroj: | Annals of Diagnostic Pathology. 53:151745 |
| ISSN: | 1092-9134 |
| DOI: | 10.1016/j.anndiagpath.2021.151745 |
| Popis: | 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 and 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 to 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. |
| Databáze: | OpenAIRE |
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