Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension

Olfactory neuroblastoma (esthesioneuro­blastoma) is a rare malignant tumor of neuroectodermal origin. With only about 1,000 cases reported, there are no clear guidelines regarding management of this disease. Intracranial extension and orbital involvement have been shown to be independent risk factor...

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Main Authors: Raid Aljumaily, John S. Nystrom, Richard O. Wein
Format: Article
Language:English
Published: SAGE Publishing 2011-01-01
Series:Rare Tumors
Subjects:
Online Access:http://www.pagepress.org/journals/index.php/rt/article/view/2180
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author Raid Aljumaily
John S. Nystrom
Richard O. Wein
author_facet Raid Aljumaily
John S. Nystrom
Richard O. Wein
author_sort Raid Aljumaily
collection DOAJ
description Olfactory neuroblastoma (esthesioneuro­blastoma) is a rare malignant tumor of neuroectodermal origin. With only about 1,000 cases reported, there are no clear guidelines regarding management of this disease. Intracranial extension and orbital involvement have been shown to be independent risk factors associated with poorer outcomes. We hereby report a case of a 46-year old male presented with an 8-month history of progressive nasal obstruction and intermittent right-sided epistaxis associated with anosmia and increased pressure sensation in and around the right eye. Further evaluation revealed a large enhancing heterogeneous cystic and solid mass in the right nasal cavity measuring 5.0¥5.3¥4.6 cm with extension superiorly into the anterior cranial fossa and frontal lobes, ethmoid and sphenoid sinuses. A biopsy of this mass confirmed high grade olfactory neuroblastoma. Because of the intra-cranial extension, a decision was made to start neoadjuvant chemotherapy with cisplatin and etoposide. The patient had very good response to this treatment on a repeat imaging study and went on to have resection of this mass. Post-operatively, he received radiation therapy to the tumor bed and 2 more cycles of chemotherapy. He has been followed now for more than 8 months with no evidence of disease recurrence.
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spelling doaj.art-96cc97bd7b9a44668490cbaabb7fcf142022-12-22T01:28:19ZengSAGE PublishingRare Tumors2036-36052036-36132011-01-0131e1e110.4081/rt.2011.e11352Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extensionRaid Aljumaily0John S. NystromRichard O. WeinTufts Medical CenterOlfactory neuroblastoma (esthesioneuro­blastoma) is a rare malignant tumor of neuroectodermal origin. With only about 1,000 cases reported, there are no clear guidelines regarding management of this disease. Intracranial extension and orbital involvement have been shown to be independent risk factors associated with poorer outcomes. We hereby report a case of a 46-year old male presented with an 8-month history of progressive nasal obstruction and intermittent right-sided epistaxis associated with anosmia and increased pressure sensation in and around the right eye. Further evaluation revealed a large enhancing heterogeneous cystic and solid mass in the right nasal cavity measuring 5.0¥5.3¥4.6 cm with extension superiorly into the anterior cranial fossa and frontal lobes, ethmoid and sphenoid sinuses. A biopsy of this mass confirmed high grade olfactory neuroblastoma. Because of the intra-cranial extension, a decision was made to start neoadjuvant chemotherapy with cisplatin and etoposide. The patient had very good response to this treatment on a repeat imaging study and went on to have resection of this mass. Post-operatively, he received radiation therapy to the tumor bed and 2 more cycles of chemotherapy. He has been followed now for more than 8 months with no evidence of disease recurrence.http://www.pagepress.org/journals/index.php/rt/article/view/2180Olfactory Neuroblastomacisplatinetoposideneoadjuvant chemotherapy
spellingShingle Raid Aljumaily
John S. Nystrom
Richard O. Wein
Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
Rare Tumors
Olfactory Neuroblastoma
cisplatin
etoposide
neoadjuvant chemotherapy
title Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
title_full Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
title_fullStr Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
title_full_unstemmed Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
title_short Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
title_sort neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension
topic Olfactory Neuroblastoma
cisplatin
etoposide
neoadjuvant chemotherapy
url http://www.pagepress.org/journals/index.php/rt/article/view/2180
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AT johnsnystrom neoadjuvantchemotherapyinthesettingoflocallyadvancedolfactoryneuroblastomawithintracranialextension
AT richardowein neoadjuvantchemotherapyinthesettingoflocallyadvancedolfactoryneuroblastomawithintracranialextension