Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor

Background: Esthesioneuroblastoma is a slow-growing rare malignant neuroectodermal tumor arising from the olfactory epithelium. It has a bimodal incidence with peaks in the second and third decades of life and the sixth and seventh decades of life with equal incidence among men and women. Prognostic...

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Main Authors: Geetha Vasudevan, Srilatha Parampalli Srinivas, Padmapriya Jaiprakash, Kailesh Pujary
Format: Article
Language:English
Published: Association of Clinical Pathologists of Nepal 2023-07-01
Series:Journal of Pathology of Nepal
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Online Access:https://www.nepjol.info/index.php/JPN/article/view/42524
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author Geetha Vasudevan
Srilatha Parampalli Srinivas
Padmapriya Jaiprakash
Kailesh Pujary
author_facet Geetha Vasudevan
Srilatha Parampalli Srinivas
Padmapriya Jaiprakash
Kailesh Pujary
author_sort Geetha Vasudevan
collection DOAJ
description Background: Esthesioneuroblastoma is a slow-growing rare malignant neuroectodermal tumor arising from the olfactory epithelium. It has a bimodal incidence with peaks in the second and third decades of life and the sixth and seventh decades of life with equal incidence among men and women. Prognostic factors include the Hyams grade and modified Kadish stage. This study analyzed the clinicopathological features of esthesioneuroblastoma. Materials and methods- Retrospective study of patients who were diagnosed with esthesioneuroblastoma at a tertiary care hospital between January 2012 and May 2019 was conducted.  The research was performed according to the World Medical Association Declaration of Helsinki. Institutional research committee approval (IEC: 473/2019) was obtained. Results: Clinicopathological profiles of 8 patients who were diagnosed with esthesioneuroblastoma during the period were included. Among the eight patients, five were female and three were male.  Common presenting complaints were nasal obstruction, difficulty in breathing, epistaxis, local pain, and anosmia. The average age of presentation was 52 years, ranging from 14 to 73 years. Anterior rhinoscopic examination in these cases showed a polypoid mass located in the nasal cavity. According to the Hyams grading system, three of the eight cases were grade-2 and the remaining five cases were grade-3 Hyams histological grade. Three cases exhibited foci of ganglioneuroblastic transformation. Three patients had metastases to cervical lymph nodes and three patients had recurrence. Conclusions: Esthesioneuroblastoma is a rare aggressive malignant tumor. It can exhibit divergent epithelial or ganglionic differentiation. A proper diagnosis should be made, graded and staged before proceeding to treatment.
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spelling doaj-art-ccc55e64b6a04a639bc0ca6440f5c8092025-08-21T07:00:52ZengAssociation of Clinical Pathologists of NepalJournal of Pathology of Nepal2091-07972091-09082023-07-011311970197510.3126/jpn.v13i1.4252482303Esthesioneuroblastoma: an institutional based descriptive study of a rare tumorGeetha Vasudevan0https://orcid.org/0000-0003-4558-5797Srilatha Parampalli Srinivas1https://orcid.org/0000-0001-6419-333XPadmapriya Jaiprakash2https://orcid.org/0000-0001-5309-9540Kailesh Pujary3https://orcid.org/0000-0001-5200-6680Kasturba medical college, Manipal Academy of Higher Education, Manipal, IndiaManipal Academy of Higher Education, Manipal, IndiaKasturba medical college, Manipal Academy of Higher Education, Manipal, IndiaKasturba medical college, Manipal Academy of Higher Education, Manipal, IndiaBackground: Esthesioneuroblastoma is a slow-growing rare malignant neuroectodermal tumor arising from the olfactory epithelium. It has a bimodal incidence with peaks in the second and third decades of life and the sixth and seventh decades of life with equal incidence among men and women. Prognostic factors include the Hyams grade and modified Kadish stage. This study analyzed the clinicopathological features of esthesioneuroblastoma. Materials and methods- Retrospective study of patients who were diagnosed with esthesioneuroblastoma at a tertiary care hospital between January 2012 and May 2019 was conducted.  The research was performed according to the World Medical Association Declaration of Helsinki. Institutional research committee approval (IEC: 473/2019) was obtained. Results: Clinicopathological profiles of 8 patients who were diagnosed with esthesioneuroblastoma during the period were included. Among the eight patients, five were female and three were male.  Common presenting complaints were nasal obstruction, difficulty in breathing, epistaxis, local pain, and anosmia. The average age of presentation was 52 years, ranging from 14 to 73 years. Anterior rhinoscopic examination in these cases showed a polypoid mass located in the nasal cavity. According to the Hyams grading system, three of the eight cases were grade-2 and the remaining five cases were grade-3 Hyams histological grade. Three cases exhibited foci of ganglioneuroblastic transformation. Three patients had metastases to cervical lymph nodes and three patients had recurrence. Conclusions: Esthesioneuroblastoma is a rare aggressive malignant tumor. It can exhibit divergent epithelial or ganglionic differentiation. A proper diagnosis should be made, graded and staged before proceeding to treatment.https://www.nepjol.info/index.php/JPN/article/view/42524esthesioneuroblatoma flexner-wintersteinerhomer wright pseudorosettesolfactory neuroblastoma pseudorosetterosettesinonasal tract
spellingShingle Geetha Vasudevan
Srilatha Parampalli Srinivas
Padmapriya Jaiprakash
Kailesh Pujary
Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
Journal of Pathology of Nepal
esthesioneuroblatoma
flexner-wintersteiner
homer wright pseudorosettes
olfactory neuroblastoma
pseudorosette
rosette
sinonasal tract
title Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
title_full Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
title_fullStr Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
title_full_unstemmed Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
title_short Esthesioneuroblastoma: an institutional based descriptive study of a rare tumor
title_sort esthesioneuroblastoma an institutional based descriptive study of a rare tumor
topic esthesioneuroblatoma
flexner-wintersteiner
homer wright pseudorosettes
olfactory neuroblastoma
pseudorosette
rosette
sinonasal tract
url https://www.nepjol.info/index.php/JPN/article/view/42524
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AT padmapriyajaiprakash esthesioneuroblastomaaninstitutionalbaseddescriptivestudyofararetumor
AT kaileshpujary esthesioneuroblastomaaninstitutionalbaseddescriptivestudyofararetumor