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Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare form of cancer involving nasal cavity and believed to arise from the olfactory epithelium. It can cause loss of vision, and taste.<ref name="wiki"> Esthesioneuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Esthesioneuroblastoma Accessed on January 26, 2016</ref > Esthesioneuroblastomas are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. The tumor cells are mitotically active and are the precursor cells that develop into sustentacular and neuronal cells. They usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air-cells on one side and extending through the cribriform plate into the anterior cranial fossa.<ref>Barnes, Leon. Pathology and genetics of head and neck tumours. Lyon: IARC Press, 2005. Print.</ref> Based on the duration of symptoms, esthesioneuroblastoma may be classified into two subtypes neuroblastoma proper and neuroendocrine carcinomas.<ref name="pmid7483011">{{cite journal| author=Min KW| title=Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region. | journal=Ultrastruct Pathol | year= 1995 | volume= 19 | issue= 5 | pages= 347-63 | pmid=7483011 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7483011  }} </ref> Genes involved in the pathogenesis of esthesioneuroblastoma include chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q. On gross pathology, soft and hemorrhagic, polypoid appearance, and rich and fragile vascular supply of the tumor are characteristic findings of esthesioneuroblastoma. On microscopic histopathological analysis, arrangements of cells into rosettes or pseudorosettes are characteristic findings of esthesioneuroblastoma.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref><ref name="librepathology">  Esthesioneuroblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Olfactory_neuroblastoma Accessed on January 25, 2015</ref><ref>Hyams, V. J. (1988). Tumors of the upper respiratory tract and ear. Washington, D.C.: Armed Forces Institute of Pathology.</ref><ref name="pmid8630875">{{cite journal| author=Hirose T, Scheithauer BW, Lopes MB, Gerber HA, Altermatt HJ, Harner SG et al.| title=Olfactory neuroblastoma. An immunohistochemical, ultrastructural, and flow cytometric study. | journal=Cancer | year= 1995 | volume= 76 | issue= 1 | pages= 4-19 | pmid=8630875 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8630875  }} </ref><ref name="pmid18408657">{{cite journal| author=Guled M, Myllykangas S, Frierson HF, Mills SE, Knuutila S, Stelow EB| title=Array comparative genomic hybridization analysis of olfactory neuroblastoma. | journal=Mod Pathol | year= 2008 | volume= 21 | issue= 6 | pages= 770-8 | pmid=18408657 | doi=10.1038/modpathol.2008.57 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18408657  }} </ref><ref name="pmid15272537">{{cite journal| author=Mhawech P, Berczy M, Assaly M, Herrmann F, Bouzourene H, Allal AS et al.| title=Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract. | journal=Am J Clin Pathol | year= 2004 | volume= 122 | issue= 1 | pages= 100-5 | pmid=15272537 | doi=10.1309/QD0K-9Q1J-BH6B-5GQQ | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15272537  }} </ref><ref name="pmid8583243">{{cite journal| author=Carney ME, O'Reilly RC, Sholevar B, Buiakova OI, Lowry LD, Keane WM et al.| title=Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma). | journal=J Neurooncol | year= 1995 | volume= 26 | issue= 1 | pages= 35-43 | pmid=8583243 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8583243  }} </ref> The incidence of esthesioneuroblastoma is approximately 0.4 per 100,000 individuals worldwide. Esthesioneuroblastoma can present in a wide range of age groups; however, it is mostly noted in a bimodal distribution, occurring most frequently in the second and sixth decades of life. There is no racial predilection to the esthesioneuroblastoma.<ref name="ShirzadiDrazin2013">{{cite journal|last1=Shirzadi|first1=Ali S.|last2=Drazin|first2=Doniel G.|last3=Strickland|first3=Allison S.|last4=Bannykh|first4=Serguei I.|last5=Johnson|first5=J. Patrick|title=Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup|journal=Case Reports in Surgery|volume=2013|year=2013|pages=1–8|issn=2090-6900|doi=10.1155/2013/107315}}</ref><ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref> There are no established risk factors for esthesioneuroblastoma.
Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare form of cancer involving nasal cavity and believed to arise from the olfactory epithelium. It can cause loss of vision, and taste.<ref name="wiki"> Esthesioneuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Esthesioneuroblastoma Accessed on January 26, 2016</ref > Esthesioneuroblastomas are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. The tumor cells are mitotically active and are the precursor cells that develop into sustentacular and neuronal cells. They usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air-cells on one side and extending through the cribriform plate into the anterior cranial fossa.<ref>Barnes, Leon. Pathology and genetics of head and neck tumours. Lyon: IARC Press, 2005. Print.</ref> Based on the duration of symptoms, esthesioneuroblastoma may be classified into two subtypes neuroblastoma proper and neuroendocrine carcinomas.<ref name="pmid7483011">{{cite journal| author=Min KW| title=Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region. | journal=Ultrastruct Pathol | year= 1995 | volume= 19 | issue= 5 | pages= 347-63 | pmid=7483011 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7483011  }} </ref> Genes involved in the pathogenesis of esthesioneuroblastoma include chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q. On gross pathology, soft and hemorrhagic, polypoid appearance, and rich and fragile vascular supply of the tumor are characteristic findings of esthesioneuroblastoma. On microscopic histopathological analysis, arrangements of cells into rosettes or pseudorosettes are characteristic findings of esthesioneuroblastoma.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref><ref name="librepathology">  Esthesioneuroblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Olfactory_neuroblastoma Accessed on January 25, 2015</ref><ref>Hyams, V. J. (1988). Tumors of the upper respiratory tract and ear. Washington, D.C.: Armed Forces Institute of Pathology.</ref><ref name="pmid8630875">{{cite journal| author=Hirose T, Scheithauer BW, Lopes MB, Gerber HA, Altermatt HJ, Harner SG et al.| title=Olfactory neuroblastoma. An immunohistochemical, ultrastructural, and flow cytometric study. | journal=Cancer | year= 1995 | volume= 76 | issue= 1 | pages= 4-19 | pmid=8630875 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8630875  }} </ref><ref name="pmid18408657">{{cite journal| author=Guled M, Myllykangas S, Frierson HF, Mills SE, Knuutila S, Stelow EB| title=Array comparative genomic hybridization analysis of olfactory neuroblastoma. | journal=Mod Pathol | year= 2008 | volume= 21 | issue= 6 | pages= 770-8 | pmid=18408657 | doi=10.1038/modpathol.2008.57 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18408657  }} </ref><ref name="pmid15272537">{{cite journal| author=Mhawech P, Berczy M, Assaly M, Herrmann F, Bouzourene H, Allal AS et al.| title=Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract. | journal=Am J Clin Pathol | year= 2004 | volume= 122 | issue= 1 | pages= 100-5 | pmid=15272537 | doi=10.1309/QD0K-9Q1J-BH6B-5GQQ | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15272537  }} </ref><ref name="pmid8583243">{{cite journal| author=Carney ME, O'Reilly RC, Sholevar B, Buiakova OI, Lowry LD, Keane WM et al.| title=Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma). | journal=J Neurooncol | year= 1995 | volume= 26 | issue= 1 | pages= 35-43 | pmid=8583243 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8583243  }} </ref> The incidence of esthesioneuroblastoma is approximately 0.4 per 100,000 individuals worldwide. Esthesioneuroblastoma can present in a wide range of age groups; however, it is mostly noted in a bimodal distribution, occurring most frequently in the second and sixth decades of life. There is no racial predilection to the esthesioneuroblastoma.<ref name="ShirzadiDrazin2013">{{cite journal|last1=Shirzadi|first1=Ali S.|last2=Drazin|first2=Doniel G.|last3=Strickland|first3=Allison S.|last4=Bannykh|first4=Serguei I.|last5=Johnson|first5=J. Patrick|title=Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup|journal=Case Reports in Surgery|volume=2013|year=2013|pages=1–8|issn=2090-6900|doi=10.1155/2013/107315}}</ref><ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref> There are no established risk factors for esthesioneuroblastoma.
If left untreated, 10%-62% of patients with esthesioneuroblastoma may progress to develop metastasis. Common complications of esthesioneuroblastoma include tumor recurrence and metastasis. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. The five-year mortality rate of patients with esthesioneuroblastoma is approximately 56% and 20% for grade I and II and grade III and IV tumors, respectively. The ten-year mortality rate of patients with esthesioneuroblastoma is approximately 67% and 34% for grade I and II and grade III and IV tumors, respectively.<ref>Kane, Ari J., et al. "Posttreatment prognosis of patients with esthesioneuroblastoma: clinical article." Journal of neurosurgery 113.2 (2010): 340-351.</ref><ref name="ShirzadiDrazin2013">{{cite journal|last1=Shirzadi|first1=Ali S.|last2=Drazin|first2=Doniel G.|last3=Strickland|first3=Allison S.|last4=Bannykh|first4=Serguei I.|last5=Johnson|first5=J. Patrick|title=Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup|journal=Case Reports in Surgery|volume=2013|year=2013|pages=1–8|issn=2090-6900|doi=10.1155/2013/107315}}</ref> Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, neck mass, and anosmia.<ref name="pmid19721769">{{cite journal| author=Ward PD, Heth JA, Thompson BG, Marentette LJ| title=Esthesioneuroblastoma: Results and Outcomes of a Single Institution's Experience. | journal=Skull Base | year= 2009 | volume= 19 | issue= 2 | pages= 133-40 | pmid=19721769 | doi=10.1055/s-0028-1096195 | pmc=PMC2671304 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19721769  }} </ref><ref name="pmid18469486">{{cite journal| author=Koo BK, An JH, Jeon KH, Choi SH, Cho YM, Jang HC et al.| title=Two cases of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma and literature review. | journal=Endocr J | year= 2008 | volume= 55 | issue= 3 | pages= 469-75 | pmid=18469486 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18469486  }} </ref><ref name="pmid18575019">{{cite journal| author=Sharma S, Lasheen W, Walsh D| title=Paraneoplastic refractory hypercalcemia due to advanced metastatic esthesioneuroblastoma. | journal=Rhinology | year= 2008 | volume= 46 | issue= 2 | pages= 153-5 | pmid=18575019 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18575019  }} </ref><ref name="pmid24170477">{{cite journal| author=Gabbay U, Leider-Trejo L, Marshak G, Gabbay M, Fliss DM| title=A case and a series of published cases of esthesioneuroblastoma (ENB) in which long-standing paraneoplastic SIADH had preceded ENB diagnosis. | journal=Ear Nose Throat J | year= 2013 | volume= 92 | issue= 10-11 | pages= E6 | pmid=24170477 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24170477  }} </ref><ref name="pmid26199564">{{cite journal| author=Kunc M, Gabrych A, Czapiewski P, Sworczak K| title=Paraneoplastic syndromes in olfactory neuroblastoma. | journal=Contemp Oncol (Pozn) | year= 2015 | volume= 19 | issue= 1 | pages= 6-16 | pmid=26199564 | doi=10.5114/wo.2015.46283 | pmc=PMC4507891 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26199564  }} </ref> Common physical examination findings of esthesioneuroblastoma include reddish-gray, polypoid mass located in the upper nasal fossa, epistaxis, nasal discharge, nasal pain, facial swelling, trismus, proptosis, diplopia, anosmia, serous otitis media, cervical lymphadenopathy, retropharyngeal lymphadenopathy, decreased visual acuity, extraocular movement paralysis, altered mental status, and frontal headaches. Biopsy may be helpful in the diagnosis of esthesioneuroblastoma. Findings on biopsy diagnostic of esthesioneuroblastoma include small, round-to-oval cells with coarsely granular chromatin, prominent nuclear membranes, multiple small nucleoli, and scant cytoplasm with pseudorosette or occasional Homer-Wright rosettes separated by fibrous septa. Calcification and necrosis are sometimes seen, and mitotic figures are rare.<ref name="pmid20345216">{{cite journal| author=Kane AJ, Sughrue ME, Rutkowski MJ, Aranda D, Mills SA, Buencamino R et al.| title=Posttreatment prognosis of patients with esthesioneuroblastoma. | journal=J Neurosurg | year= 2010 | volume= 113 | issue= 2 | pages= 340-51 | pmid=20345216 | doi=10.3171/2010.2.JNS091897 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20345216  }} </ref> On head and neck CT, esthesioneuroblastoma is characterized by soft tissue attenuation, with relatively homogenous enhancement.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref> Head and neck [[MRI scan]] is diagnostic of esthesioneuroblastoma. On [[head]] and [[neck]] [[MRI]], esthesioneuroblastoma is characterized by heterogenous intermediate signal on T1 and T2 weighted MRI and  variable enhancement on T1 contrast with gadolinium.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref>
If left untreated, 10%-62% of patients with esthesioneuroblastoma may progress to develop metastasis. Common complications of esthesioneuroblastoma include tumor recurrence and metastasis. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. The five-year mortality rate of patients with esthesioneuroblastoma is approximately 56% and 20% for grade I and II and grade III and IV tumors, respectively. The ten-year mortality rate of patients with esthesioneuroblastoma is approximately 67% and 34% for grade I and II and grade III and IV tumors, respectively.<ref>Kane, Ari J., et al. "Posttreatment prognosis of patients with esthesioneuroblastoma: clinical article." Journal of neurosurgery 113.2 (2010): 340-351.</ref><ref name="ShirzadiDrazin2013">{{cite journal|last1=Shirzadi|first1=Ali S.|last2=Drazin|first2=Doniel G.|last3=Strickland|first3=Allison S.|last4=Bannykh|first4=Serguei I.|last5=Johnson|first5=J. Patrick|title=Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup|journal=Case Reports in Surgery|volume=2013|year=2013|pages=1–8|issn=2090-6900|doi=10.1155/2013/107315}}</ref> Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, neck mass, and anosmia.<ref name="pmid19721769">{{cite journal| author=Ward PD, Heth JA, Thompson BG, Marentette LJ| title=Esthesioneuroblastoma: Results and Outcomes of a Single Institution's Experience. | journal=Skull Base | year= 2009 | volume= 19 | issue= 2 | pages= 133-40 | pmid=19721769 | doi=10.1055/s-0028-1096195 | pmc=PMC2671304 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19721769  }} </ref><ref name="pmid18469486">{{cite journal| author=Koo BK, An JH, Jeon KH, Choi SH, Cho YM, Jang HC et al.| title=Two cases of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma and literature review. | journal=Endocr J | year= 2008 | volume= 55 | issue= 3 | pages= 469-75 | pmid=18469486 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18469486  }} </ref><ref name="pmid18575019">{{cite journal| author=Sharma S, Lasheen W, Walsh D| title=Paraneoplastic refractory hypercalcemia due to advanced metastatic esthesioneuroblastoma. | journal=Rhinology | year= 2008 | volume= 46 | issue= 2 | pages= 153-5 | pmid=18575019 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18575019  }} </ref><ref name="pmid24170477">{{cite journal| author=Gabbay U, Leider-Trejo L, Marshak G, Gabbay M, Fliss DM| title=A case and a series of published cases of esthesioneuroblastoma (ENB) in which long-standing paraneoplastic SIADH had preceded ENB diagnosis. | journal=Ear Nose Throat J | year= 2013 | volume= 92 | issue= 10-11 | pages= E6 | pmid=24170477 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24170477  }} </ref><ref name="pmid26199564">{{cite journal| author=Kunc M, Gabrych A, Czapiewski P, Sworczak K| title=Paraneoplastic syndromes in olfactory neuroblastoma. | journal=Contemp Oncol (Pozn) | year= 2015 | volume= 19 | issue= 1 | pages= 6-16 | pmid=26199564 | doi=10.5114/wo.2015.46283 | pmc=PMC4507891 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26199564  }} </ref> Common physical examination findings of esthesioneuroblastoma include reddish-gray, polypoid mass located in the upper nasal fossa, epistaxis, nasal discharge, nasal pain, facial swelling, trismus, proptosis, diplopia, anosmia, serous otitis media, cervical lymphadenopathy, retropharyngeal lymphadenopathy, decreased visual acuity, extraocular movement paralysis, altered mental status, and frontal headaches. Biopsy may be helpful in the diagnosis of esthesioneuroblastoma. Findings on biopsy diagnostic of esthesioneuroblastoma include small, round-to-oval cells with coarsely granular chromatin, prominent nuclear membranes, multiple small nucleoli, and scant cytoplasm with pseudorosette or occasional Homer-Wright rosettes separated by fibrous septa. Calcification and necrosis are sometimes seen, and mitotic figures are rare.<ref name="pmid20345216">{{cite journal| author=Kane AJ, Sughrue ME, Rutkowski MJ, Aranda D, Mills SA, Buencamino R et al.| title=Posttreatment prognosis of patients with esthesioneuroblastoma. | journal=J Neurosurg | year= 2010 | volume= 113 | issue= 2 | pages= 340-51 | pmid=20345216 | doi=10.3171/2010.2.JNS091897 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20345216  }} </ref> On head and neck CT, esthesioneuroblastoma is characterized by soft tissue attenuation, with relatively homogenous enhancement.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref> Head and neck [[MRI scan]] is diagnostic of esthesioneuroblastoma. On [[head]] and [[neck]] [[MRI]], esthesioneuroblastoma is characterized by heterogenous intermediate signal on T1 and T2 weighted MRI and  variable enhancement on T1 contrast with gadolinium.<ref name="radio"> Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016</ref>


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare form of cancer involving nasal cavity and believed to arise from the olfactory epithelium. It can cause loss of vision, and taste.[1] Esthesioneuroblastomas are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. The tumor cells are mitotically active and are the precursor cells that develop into sustentacular and neuronal cells. They usually present as a soft tissue mass in the superior olfactory recess involving the anterior and middle ethmoid air-cells on one side and extending through the cribriform plate into the anterior cranial fossa.[2] Based on the duration of symptoms, esthesioneuroblastoma may be classified into two subtypes neuroblastoma proper and neuroendocrine carcinomas.[3] Genes involved in the pathogenesis of esthesioneuroblastoma include chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q. On gross pathology, soft and hemorrhagic, polypoid appearance, and rich and fragile vascular supply of the tumor are characteristic findings of esthesioneuroblastoma. On microscopic histopathological analysis, arrangements of cells into rosettes or pseudorosettes are characteristic findings of esthesioneuroblastoma.[4][5][6][7][8][9][10] The incidence of esthesioneuroblastoma is approximately 0.4 per 100,000 individuals worldwide. Esthesioneuroblastoma can present in a wide range of age groups; however, it is mostly noted in a bimodal distribution, occurring most frequently in the second and sixth decades of life. There is no racial predilection to the esthesioneuroblastoma.[11][4] There are no established risk factors for esthesioneuroblastoma. If left untreated, 10%-62% of patients with esthesioneuroblastoma may progress to develop metastasis. Common complications of esthesioneuroblastoma include tumor recurrence and metastasis. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. The five-year mortality rate of patients with esthesioneuroblastoma is approximately 56% and 20% for grade I and II and grade III and IV tumors, respectively. The ten-year mortality rate of patients with esthesioneuroblastoma is approximately 67% and 34% for grade I and II and grade III and IV tumors, respectively.[12][11] Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, neck mass, and anosmia.[13][14][15][16][17] Common physical examination findings of esthesioneuroblastoma include reddish-gray, polypoid mass located in the upper nasal fossa, epistaxis, nasal discharge, nasal pain, facial swelling, trismus, proptosis, diplopia, anosmia, serous otitis media, cervical lymphadenopathy, retropharyngeal lymphadenopathy, decreased visual acuity, extraocular movement paralysis, altered mental status, and frontal headaches. Biopsy may be helpful in the diagnosis of esthesioneuroblastoma. Findings on biopsy diagnostic of esthesioneuroblastoma include small, round-to-oval cells with coarsely granular chromatin, prominent nuclear membranes, multiple small nucleoli, and scant cytoplasm with pseudorosette or occasional Homer-Wright rosettes separated by fibrous septa. Calcification and necrosis are sometimes seen, and mitotic figures are rare.[18] On head and neck CT, esthesioneuroblastoma is characterized by soft tissue attenuation, with relatively homogenous enhancement.[4] Head and neck MRI scan is diagnostic of esthesioneuroblastoma. On head and neck MRI, esthesioneuroblastoma is characterized by heterogenous intermediate signal on T1 and T2 weighted MRI and variable enhancement on T1 contrast with gadolinium.[4]

Historical Perspective

Esthesioneuroblastoma was first discovered by Berger and Luc, in 1924.[19]

Classification

Based on the duration of symptoms, esthesioneuroblastoma may be classified into two subtypes neuroblastoma proper and neuroendocrine carcinomas.[3]

Pathophysiology

Genes involved in the pathogenesis of esthesioneuroblastoma include chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q. On gross pathology, soft and hemorrhagic, polypoid appearance, and rich and fragile vascular supply of the tumor are characteristic findings of esthesioneuroblastoma. On microscopic histopathological analysis, arrangements of cells into rosettes or pseudorosettes are characteristic findings of esthesioneuroblastoma.[4][5][20][7][8][9][10]

Causes

Genes involved in the pathogenesis of esthesioneuroblastoma include chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q.[8][9][10]

Differential Diagnosis

Esthesioneuroblastoma must be differentiated from other tumors with similar histological appearance, such as lymphoma, Ewing sarcoma, melanoma, olfactory/ other [rhabdomyosarcoma or Merkel cell carcinoma], neuroblastoma, and small cell carcinoma. Distinguishing esthesioneuroblastomas from the other tumors is of paramount importance because the tumors respond differently to various treatment modalities.[4][21]

Epidemiology and Demographics

The incidence of esthesioneuroblastoma is approximately 0.4 per 100,000 individuals worldwide. Esthesioneuroblastoma can present in a wide range of age groups; however, it is mostly noted in a bimodal distribution, occurring most frequently in the second and sixth decades of life. There is no racial predilection to the esthesioneuroblastoma.[11][4]

Risk Factors

There are no established risk factors for esthesioneuroblastoma.

Screening

According to the United States Preventive Services Task Force, screening for esthesioneuroblastoma is not recommended.[22]

Natural History, Complications, and Prognosis

If left untreated, 10%-62% of patients with esthesioneuroblastoma may progress to develop metastasis. Common complications of esthesioneuroblastoma include tumor recurrence and metastasis. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. The five-year mortality rate of patients with esthesioneuroblastoma is approximately 56% and 20% for grade I and II and grade III and IV tumors, respectively. The ten-year mortality rate of patients with esthesioneuroblastoma is approximately 67% and 34% for grade I and II and grade III and IV tumors, respectively.[23][11]

Staging

According to a staging system by Kadish, there are four stages of esthesioneuroblastoma based upon the extent of the primary tumor and lymph node or distant metastases. In 1992, Dulguerov and Calceterra proposed a classification based on the tumor, node, metastasis (TNM) system, predicted on CT and MRI findings that can be identified before treatment.[24][25][26]

History and Symptoms

Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, neck mass, and anosmia.[13][14][15][16][17]

Physical Examination

Common physical examination findings of esthesioneuroblastoma include reddish-gray, polypoid mass located in the upper nasal fossa, epistaxis, nasal discharge, nasal pain, facial swelling, trismus, proptosis, diplopia, anosmia, serous otitis media, cervical lymphadenopathy, retropharyngeal lymphadenopathy, decreased visual acuity, extraocular movement paralysis, altered mental status, and frontal headaches.

Laboratory Findings

There are no diagnostic lab findings associated with esthesioneuroblastoma.

CT

On head and neck CT, esthesioneuroblastoma is characterized by soft tissue attenuation, with relatively homogenous enhancement.[4]

MRI

Head and neck MRI scan is diagnostic of esthesioneuroblastoma. On head and neck MRI, esthesioneuroblastoma is characterized by heterogenous intermediate signal on T1 and T2 weighted MRI and variable enhancement on T1 contrast with gadolinium.[4]

Other Imaging Studies

Other imaging studies for esthesioneuroblastoma include angiography/digital subtraction angiography and nuclear medicine.[4]

Other Diagnostic Studies

Biopsy may be helpful in the diagnosis of esthesioneuroblastoma. Findings on biopsy diagnostic of esthesioneuroblastoma include small, round-to-oval cells with coarsely granular chromatin, prominent nuclear membranes, multiple small nucleoli, and scant cytoplasm with pseudorosette or occasional Homer-Wright rosettes separated by fibrous septa. Calcification and necrosis are sometimes seen, and mitotic figures are rare.[18]

Medical Therapy

Primary Prevention

There are no primary preventive measures available for esthesioneuroblastoma.

Secondary Prevention

Secondary prevention strategies following esthesioneuroblastoma include nasal irrigation with sterile isotonic sodium chloride begins within a few days after packing removal, removal of nasal crusting should be performed regularly during initial postoperative visits, and craniotomy sutures are removed 7-10 days after surgery.

References

  1. ↑ Esthesioneuroblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Esthesioneuroblastoma Accessed on January 26, 2016
  2. ↑ Barnes, Leon. Pathology and genetics of head and neck tumours. Lyon: IARC Press, 2005. Print.
  3. ↑ 3.0 3.1 Min KW (1995). "Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region". Ultrastruct Pathol. 19 (5): 347–63. PMID 7483011.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 4.9 Esthesioneuroblastoma. Radiopedia(2015) http://radiopaedia.org/articles/olfactory-neuroblastoma Accessed on January 25, 2016
  5. ↑ 5.0 5.1 Esthesioneuroblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Olfactory_neuroblastoma Accessed on January 25, 2015
  6. ↑ Hyams, V. J. (1988). Tumors of the upper respiratory tract and ear. Washington, D.C.: Armed Forces Institute of Pathology.
  7. ↑ 7.0 7.1 Hirose T, Scheithauer BW, Lopes MB, Gerber HA, Altermatt HJ, Harner SG; et al. (1995). "Olfactory neuroblastoma. An immunohistochemical, ultrastructural, and flow cytometric study". Cancer. 76 (1): 4–19. PMID 8630875.
  8. ↑ 8.0 8.1 8.2 Guled M, Myllykangas S, Frierson HF, Mills SE, Knuutila S, Stelow EB (2008). "Array comparative genomic hybridization analysis of olfactory neuroblastoma". Mod Pathol. 21 (6): 770–8. doi:10.1038/modpathol.2008.57. PMID 18408657.
  9. ↑ 9.0 9.1 9.2 Mhawech P, Berczy M, Assaly M, Herrmann F, Bouzourene H, Allal AS; et al. (2004). "Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract". Am J Clin Pathol. 122 (1): 100–5. doi:10.1309/QD0K-9Q1J-BH6B-5GQQ. PMID 15272537.
  10. ↑ 10.0 10.1 10.2 Carney ME, O'Reilly RC, Sholevar B, Buiakova OI, Lowry LD, Keane WM; et al. (1995). "Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma)". J Neurooncol. 26 (1): 35–43. PMID 8583243.
  11. ↑ 11.0 11.1 11.2 11.3 Shirzadi, Ali S.; Drazin, Doniel G.; Strickland, Allison S.; Bannykh, Serguei I.; Johnson, J. Patrick (2013). "Vertebral Column Metastases from an Esthesioneuroblastoma: Chemotherapy, Radiation, and Resection for Recurrence with 15-Year Followup". Case Reports in Surgery. 2013: 1–8. doi:10.1155/2013/107315. ISSN 2090-6900.
  12. ↑ Kane, Ari J., et al. "Posttreatment prognosis of patients with esthesioneuroblastoma: clinical article." Journal of neurosurgery 113.2 (2010): 340-351.
  13. ↑ 13.0 13.1 Ward PD, Heth JA, Thompson BG, Marentette LJ (2009). "Esthesioneuroblastoma: Results and Outcomes of a Single Institution's Experience". Skull Base. 19 (2): 133–40. doi:10.1055/s-0028-1096195. PMC 2671304. PMID 19721769.
  14. ↑ 14.0 14.1 Koo BK, An JH, Jeon KH, Choi SH, Cho YM, Jang HC; et al. (2008). "Two cases of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma and literature review". Endocr J. 55 (3): 469–75. PMID 18469486.
  15. ↑ 15.0 15.1 Sharma S, Lasheen W, Walsh D (2008). "Paraneoplastic refractory hypercalcemia due to advanced metastatic esthesioneuroblastoma". Rhinology. 46 (2): 153–5. PMID 18575019.
  16. ↑ 16.0 16.1 Gabbay U, Leider-Trejo L, Marshak G, Gabbay M, Fliss DM (2013). "A case and a series of published cases of esthesioneuroblastoma (ENB) in which long-standing paraneoplastic SIADH had preceded ENB diagnosis". Ear Nose Throat J. 92 (10–11): E6. PMID 24170477.
  17. ↑ 17.0 17.1 Kunc M, Gabrych A, Czapiewski P, Sworczak K (2015). "Paraneoplastic syndromes in olfactory neuroblastoma". Contemp Oncol (Pozn). 19 (1): 6–16. doi:10.5114/wo.2015.46283. PMC 4507891. PMID 26199564.
  18. ↑ 18.0 18.1 Kane AJ, Sughrue ME, Rutkowski MJ, Aranda D, Mills SA, Buencamino R; et al. (2010). "Posttreatment prognosis of patients with esthesioneuroblastoma". J Neurosurg. 113 (2): 340–51. doi:10.3171/2010.2.JNS091897. PMID 20345216.
  19. ↑ Broich G, Pagliari A, Ottaviani F (1997). "Esthesioneuroblastoma: a general review of the cases published since the discovery of the tumour in 1924". Anticancer Res. 17 (4A): 2683–706. PMID 9252701.
  20. ↑ Hyams, V. J. (1988). Tumors of the upper respiratory tract and ear. Washington, D.C.: Armed Forces Institute of Pathology.
  21. ↑ Argani P, Perez-Ordoñez B, Xiao H, Caruana SM, Huvos AG, Ladanyi M (1998). "Olfactory neuroblastoma is not related to the Ewing family of tumors: absence of EWS/FLI1 gene fusion and MIC2 expression". Am J Surg Pathol. 22 (4): 391–8. PMID 9580174.
  22. ↑ http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=esthesioneuroblastoma Accessed on December 26, 2016.
  23. ↑ Kane, Ari J., et al. "Posttreatment prognosis of patients with esthesioneuroblastoma: clinical article." Journal of neurosurgery 113.2 (2010): 340-351.
  24. ↑ Kadish S, Goodman M, Wang CC (1976). "Olfactory neuroblastoma. A clinical analysis of 17 cases". Cancer. 37 (3): 1571–6. PMID 1260676.
  25. ↑ Morita A, Ebersold MJ, Olsen KD, Foote RL, Lewis JE, Quast LM (1993). "Esthesioneuroblastoma: prognosis and management". Neurosurgery. 32 (5): 706–14, discussion 714-5. PMID 8492845.
  26. ↑ Dulguerov P, Calcaterra T (1992). "Esthesioneuroblastoma: the UCLA experience 1970-1990". Laryngoscope. 102 (8): 843–9. PMID 1495347.

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