open access

Vol 18, No 6 (2022)
Case report
Published online: 2022-09-29
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Aggressiveness of esthesioneuroblastoma: a rare case report and review of literature

Diptajit Paul1, Sheeba Bhardwaj1, Geetanand Yadav1, Vivek Kaushal1
DOI: 10.5603/OCP.2022.0041
·
Oncol Clin Pract 2022;18(6):410-414.
Affiliations
  1. Pandit B D Sharma Post Graduate Institution of Medical Sciences, Rohtak, Haryana, India

open access

Vol 18, No 6 (2022)
CASE REPORTS
Published online: 2022-09-29

Abstract

Introduction. Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare malignant neuroecto dermal nasal tumor with distinctive clinical, histopathological, radiological, and molecular features. It arises from olfactory receptors in the nasal mucosa or the cribriform plate of the ethmoid bone. It is generally slow-growing; fast-growing tumors may lead to widespread metastasis. Here, we present an atypical case of aggressive esthesioneuroblastoma treated with a multimodality approach. 

Case description. A 28-year male, presented with a painful swelling over the right cheek lasting for 5 months. The patient underwent surgery, and histopathology of the surgical specimen revealed a small round blue cell tumor with widespread positivity for synaptophysin and CD 56. The histopathological appearance and immunohistochemical profile of the biopsy tissue confirmed esthesioneuroblastoma. Owing to subtotal resection, the patient received adjuvant radical radiotherapy to the local site and lymph nodes. Three months post-radiotherapy, a CECT scan showed a partial response, so the patient received intravenous chemotherapy. However, the patient had local disease progression; establishing the aggressivness of esthesioneuroblastoma in our patient. The patient is alive with residual stable disease after 2.5 years from the initial diagnosis and is follow up. 

Conclusion. Esthesioneuroblastomas are uncommon tumors and owing to their slow-growing nature, the patient may neglect them. There is a wide spectrum of clinical presentations and outcomes in such patients, and as the literature on esthesioneuroblastoma is scarce due to its rarity, this case report seeks to contribute to a better understanding of such uncommon malignancy in terms of its clinical presentation, behavior, and outcomes. 

Abstract

Introduction. Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare malignant neuroecto dermal nasal tumor with distinctive clinical, histopathological, radiological, and molecular features. It arises from olfactory receptors in the nasal mucosa or the cribriform plate of the ethmoid bone. It is generally slow-growing; fast-growing tumors may lead to widespread metastasis. Here, we present an atypical case of aggressive esthesioneuroblastoma treated with a multimodality approach. 

Case description. A 28-year male, presented with a painful swelling over the right cheek lasting for 5 months. The patient underwent surgery, and histopathology of the surgical specimen revealed a small round blue cell tumor with widespread positivity for synaptophysin and CD 56. The histopathological appearance and immunohistochemical profile of the biopsy tissue confirmed esthesioneuroblastoma. Owing to subtotal resection, the patient received adjuvant radical radiotherapy to the local site and lymph nodes. Three months post-radiotherapy, a CECT scan showed a partial response, so the patient received intravenous chemotherapy. However, the patient had local disease progression; establishing the aggressivness of esthesioneuroblastoma in our patient. The patient is alive with residual stable disease after 2.5 years from the initial diagnosis and is follow up. 

Conclusion. Esthesioneuroblastomas are uncommon tumors and owing to their slow-growing nature, the patient may neglect them. There is a wide spectrum of clinical presentations and outcomes in such patients, and as the literature on esthesioneuroblastoma is scarce due to its rarity, this case report seeks to contribute to a better understanding of such uncommon malignancy in terms of its clinical presentation, behavior, and outcomes. 

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Keywords

esthesioneuroblastoma; external beam radiotherapy; olfactory neuroblastoma; round blue cell tumor; VAC regimen; VIP regimen

About this article
Title

Aggressiveness of esthesioneuroblastoma: a rare case report and review of literature

Journal

Oncology in Clinical Practice

Issue

Vol 18, No 6 (2022)

Article type

Case report

Pages

410-414

Published online

2022-09-29

Page views

519

Article views/downloads

59

DOI

10.5603/OCP.2022.0041

Bibliographic record

Oncol Clin Pract 2022;18(6):410-414.

Keywords

esthesioneuroblastoma
external beam radiotherapy
olfactory neuroblastoma
round blue cell tumor
VAC regimen
VIP regimen

Authors

Diptajit Paul
Sheeba Bhardwaj
Geetanand Yadav
Vivek Kaushal

References (15)
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