Olfactory Esthesioneuroblastoma (Esthesioneuroblastoma)

A malignant olfactory neuroblastoma arising from the olfactory epithelium of the superior nasal cavity and cribriform plate. It is uncommon (3% of nasal tumors) and rarely is associated with the production of excess hormones (e.g., SIADH, Cushing Syndrome). It has a high propensity for multiple local recurrences and bony metastases. (From Holland et al., Cancer Medicine, 3rd ed, p1245; J Laryngol Otol 1998 Jul;112(7):628-33)

Also Known As:

Esthesioneuroblastoma; Esthesioneuroblastoma, Olfactory; Esthesioneuroblastoma, Paranasal Sinus-Nasal Cavity; Aesthesioneuroblastomas; Esthesioneuroblastoma, Paranasal Sinus Nasal Cavity; Esthesioneuroblastomas; Esthesioneuroblastomas, Olfactory; Neuroblastomas, Olfactory; Olfactory Esthesioneuroblastomas; Olfactory Neuroblastomas; Paranasal Sinus Nasal Cavity Esthesioneuroblastoma; Aesthesioneuroblastoma; Neuroblastoma, Olfactory; Olfactory Neuroblastoma; Paranasal Sinus-Nasal Cavity Esthesioneuroblastoma

Networked: 393 relevant articles (24 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Neoplasm Metastasis (Metastasis)
3.	Melanoma (Melanoma, Malignant)
4.	Infections
5.	Nasopharyngeal Carcinoma

Experts

1.	Castelnuovo, Paolo: 6 articles (06/2022 - 09/2007)
2.	Foote, Robert L: 6 articles (07/2021 - 11/2008)
3.	Link, Michael J: 6 articles (07/2021 - 11/2008)
4.	Gallia, Gary L: 5 articles (01/2022 - 06/2015)
5.	Van Gompel, Jamie J: 5 articles (07/2021 - 03/2013)
6.	Curry, William T: 5 articles (02/2014 - 09/2008)
7.	Lin, Derrick T: 5 articles (02/2014 - 09/2008)
8.	Turri-Zanoni, Mario: 4 articles (06/2022 - 11/2017)
9.	Ishii, Masaru: 4 articles (01/2022 - 06/2015)
10.	La Rosa, Stefano: 3 articles (06/2022 - 11/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Olfactory Esthesioneuroblastoma:

1.	Etoposide (VP 16)FDA LinkGeneric 03/15/1994 - "A 3-year-old boy had localized esthesioneuroblastoma and received 2 courses of carboplatin (200 mg/m2/day during a 1-hour infusion for 3 consecutive days) associated with etoposide (150 mg/m2/day after carboplatin). " 11/15/2004 - "Eleven consecutive patients with newly diagnosed olfactory neuroblastoma were treated with etoposide (75 mg/m2), ifosfamide (1000 mg/m2), and cisplatin (20 mg/m2) all administered intravenously on Days 1-5. Cycles were repeated every 21 days. " 11/15/2004 - "Neoadjuvant etoposide, ifosfamide, and cisplatin for the treatment of olfactory neuroblastoma." 11/15/2004 - "The purpose of the current study was to evaluate the efficacy of neoadjuvant chemotherapy with the combination of etoposide, ifosfamide, and cisplatin (VIP) for patients with olfactory neuroblastoma. " 07/01/2016 - "We report a case of esthesioneuroblastoma with intracranial extension treated with Vincristine, Adriamycin, Cyclophosphamide, Ifosfamide, Etoposide protocol followed by radiation with 5 years of follow-up. "
2.	Cisplatin (Platino)FDA LinkGeneric 09/01/2000 - "We recommend systemic control using cisplatin-based chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic olfactory neuroblastoma." 05/01/1998 - "This series suggests that cisplatin-based chemotherapy is active in advanced, high-grade esthesioneuroblastoma and is a reasonable choice in the systemic treatment of these patients." 11/15/2004 - "Eleven consecutive patients with newly diagnosed olfactory neuroblastoma were treated with etoposide (75 mg/m2), ifosfamide (1000 mg/m2), and cisplatin (20 mg/m2) all administered intravenously on Days 1-5. Cycles were repeated every 21 days. " 11/15/2004 - "Neoadjuvant etoposide, ifosfamide, and cisplatin for the treatment of olfactory neuroblastoma." 11/15/2004 - "The purpose of the current study was to evaluate the efficacy of neoadjuvant chemotherapy with the combination of etoposide, ifosfamide, and cisplatin (VIP) for patients with olfactory neuroblastoma. "
3.	Ifosfamide FDA LinkGeneric 11/15/2004 - "Eleven consecutive patients with newly diagnosed olfactory neuroblastoma were treated with etoposide (75 mg/m2), ifosfamide (1000 mg/m2), and cisplatin (20 mg/m2) all administered intravenously on Days 1-5. Cycles were repeated every 21 days. " 11/15/2004 - "Neoadjuvant etoposide, ifosfamide, and cisplatin for the treatment of olfactory neuroblastoma." 11/15/2004 - "The purpose of the current study was to evaluate the efficacy of neoadjuvant chemotherapy with the combination of etoposide, ifosfamide, and cisplatin (VIP) for patients with olfactory neuroblastoma. " 07/01/2016 - "We report a case of esthesioneuroblastoma with intracranial extension treated with Vincristine, Adriamycin, Cyclophosphamide, Ifosfamide, Etoposide protocol followed by radiation with 5 years of follow-up. " 05/01/2010 - "Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine."
4.	Radioisotopes (Radionuclides)IBA 12/01/2023 - "Further case accrual of peputide receptor radionuclide therapy is required to develop a treatment for unresectable olfactory neuroblastoma." 01/01/2020 - "Efficacy of Peptide Receptor Radionuclide Therapy for Esthesioneuroblastoma." 12/01/1996 - "The accuracy of labelled octreotide in the diagnosis of olfactory neuroblastoma indicates that it might be useful in radionuclide therapy of patients with advanced disease, when no other treatment modalities are available." 12/01/2023 - "Peptide Receptor Radionuclide Therapy for Recurrent Olfactory Neuroblastoma After Cranioplasty for Surgical Infection: A Case Report." 04/01/2015 - "A 51-year-old man with a recurrent metastatic esthesioneuroblastoma (olfactory neuroblastoma) was referred for peptide receptor radionuclide therapy (PRRT). "
5.	Monoclonal AntibodiesIBA 07/15/1982 - "Neuronal origin of human esthesioneuroblastoma demonstrated with anti-neurofilament monoclonal antibodies." 09/01/1995 - "Immunohistochemical analyses with MIB 1 monoclonal antibodies, directed against recombinant parts of Ki-67 antigen, were performed to estimate the proliferative potential of the esthesioneuroblastomas. " 06/01/1988 - "The efficacy of two new monoclonal antibodies with cell lineage-restricted reactivity (HMB-45 [melanocytes] and anti-synaptophysin [neuroepithelial cells]) was compared with that of "traditional" antibody panels in the delineation of malignant melanoma (MM) of the sinonasal region, nasopharyngeal carcinoma (NPC), and olfactory neuroblastoma (ONBL). " 08/01/1991 - "The authors used a monoclonal antibody (MAb), 5.1H11, to examine NCAM immunoreactivity in frozen sections of more than 450 tumors, including more than 80 small round cell tumors (SRCT) of childhood and adolescence (neuroblastomas, Ewing's sarcomas [ES], peripheral neuroepitheliomas [PN], primitive neuroectodermal tumors [PNET], esthesioneuroblastomas, malignant ectomesenchymoma, medulloblastomas, small cell osteosarcomas, mesenchymal chondrosarcomas, embryonal rhabdomyosarcomas, and lymphomas). " 01/01/1986 - "The immunoreactivity of the anti-Leu 7 (HNK-1) monoclonal antibody, a marker for natural killer cells, was evaluated with the peroxidase-anti-peroxidase (PAP) method on sections of human paraffin-embedded tissues from 135 tumors of the central nervous system and five esthesioneuroblastomas. "
6.	Peptide Receptors (Peptide Receptor)IBA 01/01/2020 - "Efficacy of Peptide Receptor Radionuclide Therapy for Esthesioneuroblastoma." 12/01/2023 - "Peptide Receptor Radionuclide Therapy for Recurrent Olfactory Neuroblastoma After Cranioplasty for Surgical Infection: A Case Report." 04/01/2015 - "A 51-year-old man with a recurrent metastatic esthesioneuroblastoma (olfactory neuroblastoma) was referred for peptide receptor radionuclide therapy (PRRT). " 01/01/2020 - "Esthesioneuroblastoma is rare, with limited therapeutic options when unresectable or metastatic; however, expression of somatostatin receptors qualifies it for peptide receptor radionuclide therapy (PRRT). " 12/01/2018 - "Neuroradiological and Neuropathological Changes After 177Lu-Octreotate Peptide Receptor Radionuclide Therapy of Refractory Esthesioneuroblastoma."
7.	Protons (Proton)IBA 09/01/2008 - "To determine the efficacy of craniofacial resection and proton radiation for the management of esthesioneuroblastoma (ENB). " 02/01/2014 - "Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy." 09/01/2008 - "Esthesioneuroblastoma: the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy." 01/01/1997 - "Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation. "
8.	ChromograninsIBA 03/01/1989 - "However, immunohistochemical stains such as NSE, S-100, and chromogranin immunostain can be very helpful in diagnosing olfactory neuroblastoma. " 03/01/1998 - "Negative staining of B- and T-cell markers, LCA, neuroendocrine markers such as NSE, chromogranin and synaptophysin, and CK-negativity excluded olfactory neuroblastoma, small cell undifferentiated carcinoma, and lymphoma. " 01/01/2017 - "Histopathological diagnosis was "mixed olfactory neuroblastoma-carcinoma (squamous and glandular differentiation) Hyams grade IV." On immunohistochemistry, the tumor cells were positive for neuron specific enolase (NSE), synaptophysin, chromogranin, and CD56 and peripherally for S100. "
9.	anti-synaptophysinIBA 06/01/1988 - "The efficacy of two new monoclonal antibodies with cell lineage-restricted reactivity (HMB-45 [melanocytes] and anti-synaptophysin [neuroepithelial cells]) was compared with that of "traditional" antibody panels in the delineation of malignant melanoma (MM) of the sinonasal region, nasopharyngeal carcinoma (NPC), and olfactory neuroblastoma (ONBL). "
10.	S100 Proteins (S 100 Protein)IBA 09/01/1986 - "Olfactory neuroblastoma: an immuno-electron microscopic study of S-100 protein-positive cells." 09/18/2022 - "To evaluate the prognostic value of S-100 protein and Ki-67 labeling index in olfactory neuroblastomas. " 09/18/2022 - "The prognostic value of S-100 protein and Ki-67 index in olfactory neuroblastoma." 09/01/1986 - "No S-100 protein-reactive cells were observed within the olfactory epithelium of the two patients with olfactory neuroblastoma. " 07/01/1995 - "The present study indicates that (1) although typical olfactory neuroblastomas exhibit PG/NB differentiation, they more closely resemble PG, (2) occasional tumors show GNB and/or epithelial differentiation, and (3) survival rates may correlate with S-100 protein immunoreactivity and Ki-67 LI. "

Therapies and Procedures

1.	Radiotherapy 01/01/2023 - "For esthesioneuroblastoma, surgical resection with negative margins and adjuvant radiation therapy remains the gold standard. " 04/25/2011 - "Improved survival following surgery and radiation therapy for olfactory neuroblastoma: analysis of the SEER database." 09/01/2007 - "Our aim was to evaluate the efficacy of a bimodal method of treatment consisting in endoscopic resection followed by radiotherapy in patients with olfactory neuroblastoma (ON). " 11/01/2017 - "This study indicates surgery and combined radiotherapy may currently be the optimal treatment for esthesioneuroblastoma." 04/01/2002 - "The purpose of this study was to compare dose distribution of inverse planned intensity modulated radiation therapy (IMRT) with that of conformal radiation therapy (SCRT) in the treatment of esthesioneuroblastoma, and to report initial clinical results. "
2.	Therapeutics 04/01/2017 - "Introduction Advanced Kadish stage esthesioneuroblastoma requires more extensive resections and aggressive adjuvant therapy to obtain adequate disease-free control, which can lead to higher complication rates. " 04/01/2003 - "To evaluate the efficacy of multimodality therapy in patients with esthesioneuroblastoma (ENB). " 01/01/2023 - "The purpose of review is to provide a comprehensive review of the literature focusing on the recent advances in the diagnosis, molecular underpinning, and targeted therapy of olfactory neuroblastoma (ONB). " 06/13/2022 - "Targeted therapies may play a vital role in the management of refractory olfactory neuroblastoma." 06/13/2022 - "They highlighted prior uses of targeted therapy in the management of refractory olfactory neuroblastoma within the context of current standard treatment regimens. "
3.	Drug Therapy (Chemotherapy) 05/01/1998 - "A retrospective review of patients with recurrent esthesioneuroblastoma treated with chemotherapy between 1970 and 1995 at the Mayo Clinic was undertaken to gain more information regarding the efficacy of chemotherapy treatment for these patients. " 06/01/2013 - "Objectives Surgical resection in addition to adjuvant radiation with or without chemotherapy is the mainstay of treatment for esthesioneuroblastoma (ENB). " 06/01/2021 - "The role of chemotherapy in the management of olfactory neuroblastoma: A 40-year surveillance, epidemiology, and end results registry study." 08/01/2023 - "The clinical value of different treatment modalities, especially systemic chemotherapy (CT) in patients with locoregionally advanced olfactory neuroblastoma (LA ONB) remains unclear. " 07/07/2021 - "Objective: To summarize the preliminary experience in the treatment of esthesioneuroblastoma (ENB) and to explore the effect of age, chemotherapy, modified Kadish stage and pathological grade on the prognosis of ENB. "
4.	Neoadjuvant Therapy 05/07/2023 - "Objective: To evaluate the efficacy of neoadjuvant chemotherapy (NACT) in the treatment of locally advanced olfactory neuroblastoma (ONB), and to explore the factors related to the efficacy of NACT. " 12/01/2022 - "To investigate the efficacy of pretreatment ADC and Ki-67 index in the prediction of the response to neoadjuvant chemotherapy (NACT) in advanced olfactory neuroblastoma (ONB) patients. " 05/07/2023 - "[Neoadjuvant chemotherapy in the treatment of locally advanced olfactory neuroblastoma in 25 cases]." 12/01/2022 - "The role of ADC value and Ki-67 index in predicting the response to neoadjuvant chemotherapy in advanced stages of olfactory neuroblastoma." 01/01/2020 - "We report the case of a 15-year-old female with a diagnosis of Kadish stage C esthesioneuroblastoma who was treated with neoadjuvant chemotherapy and surgical resection. "
5.	Combined Modality Therapy 10/05/2011 - "This case report demonstrates the potential efficacy of planned combined modality therapy, including limited surgery and early chemoradiation in the control of locally advanced olfactory neuroblastoma." 02/01/2022 - "A 66-year-old man underwent multimodal treatment for olfactory neuroblastoma (ONB). " 08/01/2013 - "Multimodal treatment and long-term outcome of patients with esthesioneuroblastoma." 06/01/2007 - "Combined modality therapy of esthesioneuroblastoma." 07/01/2003 - "Combined modality therapy is preferred over RT alone in advanced-stage esthesioneuroblastoma. "