Radiat Oncol. [Medline]. This article outlines the various newer details of diagnosis, staging and treatment aspects of esthesioneuroblastoma (ENB). [Full Text]. Peripheral nerve tumors. Olfactory neuroblastoma and other round cell lesions of the sinonasal region. 112(7):628-33. De Bonnecaze G, Chaput B, Al Hawat A, Filleron T, Vairel B, Serrano E, et al. Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract. [Medline]. 2003 Apr. Oral and maxillofacial pathologists rarely encounter this tumor in their daily practice. As a result of its anatomical origin, most patient have non-specific symptoms, precluding early diagnosis. Silva EG, Butler J, MacKay B, et al. [2] Although many alterations were identified in this study, chromosomal gains in 7q11 and 20q and deletions in 2q, 5q, 6p, 6q, and 18q have been confirmed by at least 2 other studies. Collectively, NENs have an estimated incidence of 2.5 to 5 per 100,000 people per year,1with gastrointestinal NENs (GI-NENs, formerly carcinoids) comprising roughly two-thirds of the total. [Medline]. 2010 May. Constantinidis J, Steinhart H, Koch M. Olfactory neuroblastoma: the University of Erlangen-Nuremberg experience 1975-2000. [Medline]. Head Neck. 130(5):567-74. [Medline]. This website also contains material copyrighted by 3rd parties. It is thought to arise from neural tissue associated with the sense of smell. I went in for a nasal polyps removal, but when trying to remove, it was determined to be a tumor. [Medline]. 1998 Aug. 29(8):826-32. Koka VN, Julieron M, Bourhis J. Aesthesioneuroblastoma. J Neurooncol. It starts in the nerves that affect your sense of smell. Confirmed: Diet Influences Colorectal Cancer Risk. Carney ME, O''Reilly RC, Sholevar B. Theilgaard SA, Buchwald C, Ingeholm P. Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000. Herbert H Engelhard, III, MD, PhD, FACS, FAANS is a member of the following medical societies: American Association for Cancer Research, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Congress of Neurological Surgeons, Illinois State Medical SocietyDisclosure: Nothing to disclose. Drugs, encoded search term (Esthesioneuroblastoma) and Esthesioneuroblastoma, Pancreatic Neuroendocrine (Islet Cell) Tumor Imaging, Systemic Treatment of Metastatic Gastroenteropancreatic Neuroendocrine Tumors, Liver Transplant Emerges for Hepatic Mets in Colorectal Cancer, Targeting Glutamine Could Curb Castration-Resistant Prostate Cancer, Lifestyle Alone Cannot Shield Us From Cancer, On Strike or Working Overtime: 12 Endocrine Emergencies. 28(4):305-12. Head Neck. Cancer. He underwent a bifrontal craniotomy for resection of this tumor. Olfactory neuroblastoma (also known as esthesioneuroblastoma) is a very rare cancer that develops in the upper part of the nasal cavity. Cancer. [Medline]. Mod Pathol. In adults, the differential diagnosis of small round cell tumors of the head and neck region includes small cell carcinoma, lymphomas and neuroepithelial tumors, such as esthesioneuroblastoma and, as last choice, rhabdomyosarcoma. 2016 Jan. 273 (1):21-6. ISL-1, TTF-1 and PAX5 are transcription factors that are frequently upregulated in tumors showing neuroendocrine differentiation. Cancer. Mod Pathol. Recently, a tool called array comparative genomic hybridization was applied to the analysis of ENBs. It is in my right nasal cavity and ethmoid sinus, and starting to extend into dura. Arch Otolaryngol Head Neck Surg. Hyams VJ, Batsakis JG, Michaels L. Olfactory neuroblastoma. [Medline]. Mishima Y, Nagasaki E, Terui Y, Irie T, Takahashi S, Ito Y, et al. Long-term oncological outcome after endoscopic surgery for olfactory esthesioneuroblastoma. [Medline]. [7] No studies suggest a geographic variation in rates. In: Barnes L, Eveson JW, Reichart P, and Sidransky D, WHO Classification of Tumors. Int Forum Allergy Rhinol. Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory neuroepithelium. Esthesioneuroblastomas (ENBs) are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. [5] However, the current data set cannot distinguish between a rising incidence and better recognition of the disease. Pathophysiology. [Medline]. J Neurooncol. Int J Radiat Oncol Biol Phys. Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare neoplasm originating from olfactory neuroepithelium. Sterzing F, Stoiber EM, Nill S, Bauer H, Huber P, Debus J, et al. 73(2):424-32. Posttreatment prognosis of patients with esthesioneuroblastoma. Washington DC: Armed Forces Institute Press; 1988:. The following table is a timeline of my ENB journey (see progression from bottom to top): My ENB Timeline (click download to view): Download Surgery:April 21, 2010 - Day of Surgery > The tumor was removed from my head (craniotomy). 240-8. 2017 Dec 7. [Medline]. Glomangiopericytoma is a rare vascular neoplasm characterized by a pattern of prominent perivascular growth. A series of 220 patients and a systematic review. Dulguerov P, Calcaterra T. Esthesioneuroblastoma: the UCLA experience 1970-1990. Ewing sarcoma, Olfactory neuroblastoma, Hyams grade II (see comment). J Neurosurg. Patients typically present with multiple symptoms such as nasal obstruction, epistaxis, proptosis, visual disturbances, facial pain and symptoms of cranial nerve involvement. Retrospective review of adjuvant chemotherapy for esthesioneuroblastoma. 22(4):391-8. 2003 May. It is often difficult to predict which tumors will go on to metastasize. [Medline]. Glomangiopericytoma is categorized as a borderline lo… Many such lesions may include an intracranial extension or connection. They result from a failure of embryologic separation of neuroectodermal and ectodermal tissues during the development of the nose and frontobasal region. ORL J Otorhinolaryngol Relat Spec. Olfactory neuroblastomas are thought to arise from the olfactory epithelium [ 1 ]. 2009 Feb 1. Laryngoscope. Esthesioneuroblastoma (ENB) is a rare malignant tumor of neuroectodermal origin arising from neuroepithelium of olfactory mucosa. The mass filled much of the left nasal cavity, 122(1):100-5. Argani P, Perez-Ordonez B, Xiao H. Olfactory neuroblastoma is not related to the Ewing family of tumors: absence of EWS/FLI1 gene fusion and MIC2 expression. [Medline]. [Medline]. Olfactory neuroblastoma. Diagnosis and management of esthsioneuroblastoma. [Medline]. It has a bimodal peak of occurrence in the third and sixth decades of life. 385:363-9. [ 1] The tumor … Esthesioneuroblastoma: prognosis and management. Bradley PJ, Jones NS, Robertson I. KEYWORDS: esthesioneuroblastoma, sinonasal tract, chemotherapy, radiotherapy, proton therapy, surgery. Acta Otolaryngol. 2001 Dec 15. Skull Base. Laryngoscope. Background. [Medline]. 14(2):67-72. Min KW. In essence, ENBs contain variable arrangements of their small cells. Esthesioneuroblastoma: a meta-analysis and review. 72(2):113-8. [Medline]. They could only do a biopsy. Usually seen in the undifferentiated and poorly differentiated subtypes of neuroblastoma (see below) Associated with MYCN amplification and a poor prognosis. Fax: (614) 293‐7626. [Medline]. Patient, disease, and treatment factors associated with overall survival in esthesioneuroblastoma. [Medline]. [1] The tumor cells are mitotically active and are the precursor cells that develop into sustentacular and neuronal cells. The overall immunoprofile supports the diagnosis. syndrome of inappropriate antidiuretic hormone secretion (SIADH)), Typical finding is a dumbbell shaped mass centered at cribriform plate, 20% develop regional or distant metastases, usually to cervical lymph nodes and lung; late recurrence (after 10 years) is common, Grade and stage are the most important prognostic factors, Hyams histologic grade (Table 1) is the most widely used grading system which encompasses six histologic features and is an independent prognostic factor (, Involves nasal cavity and paranasal sinuses, Extends beyond the nasal cavity and paranasal sinuses, 30 year old woman and 67 year old woman with varied atypical clinical presentation (, 35 year old man with divergent differentiation (, 41 year old man with multifocal ectopic olfactory neuroblastoma (, 43 year old woman with a mass in the right nasal cavity (, 44 year old man with aberrant pattern of cytokeratin expression (, Complete surgical excision (may require craniofacial resection), with radiation therapy or chemotherapy, Low grade olfactory neuroblastoma usually contains nests and lobules of monotonous tumor cells with round nuclei, indistinct nucleoli and scanty cytoplasm in association with a vascular-rich to hyalinized stroma; fibrillary neural matrix may be present, High grade tumors may show solid growth, marked mitotic activity, nuclear pleomorphism, necrosis and no neuropil, Homer Wright pseudorosettes: neoplastic cells palisading around a central zone of fibrillar neural matrix; their presence in a nasal tumor is characteristic for olfactory neuroblastoma, Flexner-Wintersteiner rosettes: palisading tumor cells surrounding a true central lumen, Perivascular pseudorosettes may be present but are non-specific, Other findings: melanin pigment, neurons, divergent differentiation (e.g. 2009 Jul. I have been diagnosed with Esthesioneuroblastoma in April. Group of tumours that is typically seen in childhood and younger adults. ENB occurs in a wide range of age groups (3-90 y). Magnavita N, Sacco A, Bevilacqua L, D'Alessandris T, Bosman C. Aesthesioneuroblastoma in a woodworker. This is particularly true in poorly differentiated forms that tend to resemble sinonasal undifferentiated carcinoma (SNUC) and other small, round blue cell tumors, such as melanoma, lymphoma, neuroendocrine carcinoma, and rhabdomyosarcoma. [Full Text]. Kadish S, Goodman M, Wang CC. The aim of our study was … 2010. The Juan Rosai Collection of Pathology Slides, presented jointly by the United States Canadian Academy of Pathology and Aperio, Presented at the USCAP 100th Meeting in El Paso TExas 2011 Guy J Petruzzelli, MD, PhD, MBA, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American Association of Clinical Anatomists, American College of Surgeons, American Head and Neck Society, American Medical Association, American Society of Clinical Oncology, Chicago Medical Society, Georgia Society of Otolaryngology-Head and Neck Surgery, International Academy of Oral Oncology, International Head and Neck Scientific Group, North American Skull Base Society, Society of Surgical Oncology, Society of University Otolaryngologists-Head and Neck Surgeons, SWOGDisclosure: Nothing to disclose. Edition Publication Effective dates for cancer diagnoses 1st 1977 1978 - 1983 2nd 1983 1984 - 1988 3rd 1988 1989 - 1992 4th 1992 1993 - 1997 5th 1997 1998 - 2002 6th 2002 2003 - 2009 7th 2009 2010 - 2016 8th 2016 2017 - 2008 Sep. 18(5):327-37. The demonstration of human achaete-scute homologue (HASH1) gene expression, although still investigational, could become the diagnostic procedure of choice. [Medline]. This tumor constitutes only 3% of all intranasal neoplasms. Esthesioneuroblastoma: continued follow-up of a single institution's experience. Folbe A, Herzallah I, Duvvuri U, Bublik M, Sargi Z, Snyderman CH, et al. An olfactory neuroblastoma is a rare form of cancer. Microscopic (histologic) description. Cancer. Table. De Bonnecaze G, Lepage B, Rimmer J, Al Hawat A, Vairel B, Serrano E, et al. Nicholas C Shera, PhD Freelance Medical/Scientific WriterDisclosure: Nothing to disclose. Please confirm that you would like to log out of Medscape. Esthesioneuroblastoma is a rare malignant tumor of the sinonasal tract. AJNR Am J Neuroradiol. 98(1):131-5. Opsoclonus‐myoclonus‐ataxia (OMA) is a paraneoplastic neurologic syndrome affecting 2–3% of children with neuroblastoma. [Medline]. Common features of ENB on light microscopy include small, round-to-oval cells wi… Tseng J, Michel MA, Loehrl TA. Time is of the essence. J Laryngol Otol. Thomas C Origitano, MD, PhD, FACS is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, North American Skull Base Society, Congress of Neurological SurgeonsDisclosure: Nothing to disclose. 2009 Jan-Feb. 23(1):91-4. Odontogenic / Jaw Cysts. Armpit Swelling After COVID-19 Vaccine May Mimic Breast Cancer, Baby Gets Cancer From Mother During Birth: First Report. Esthesioneuroblastoma. 2008 Nov. 118(11):2006-10. 2009 Jun. Esthesioneuroblastoma (ENB) is a rare malignant tumor of neuroectodermal origin arising from neuroepithelium of olfactory mucosa. 9(6):658-63. 11:112-118. Head Neck. Physical examination demonstrated left-sided exophthalmos and blindness. Esthesioneuroblastoma. Esthesioneuroblastomas (ENBs) are undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium. Loy AH, Reibel JF, Read PW, Thomas CY, Newman SA, Jane JA, et al. Long-term carcinologic results of advanced esthesioneuroblastoma: a systematic review. This is partially due to the ability to analyze cancer genomes on a whole genome basis. [Medline]. 1994. Michael Somenek, MD is a member of the following medical societies: Alpha Omega Alpha, Sigma Xi, The Scientific Research Honor SocietyDisclosure: Nothing to disclose. Still, further experimentation will be required to determine the role of these genomic regions in ENB. Small round blue cell tumors. Broich G, Pagliari A, Ottaviani F. Esthesioneuroblastoma: a general review of the cases published since the discovery of the tumour in 1924. Nuclei 1.5 to 2 times larger than those of typical neuroblastoma cells. Gupta S, Husain N, Sundar S. Esthesioneuroblastoma chemotherapy and radiotherapy for extensive disease: a case report. McElroy EA Jr, Buckner JC, Lewis JE. 42(5):1023-7; discussion 1027-8. 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. Mhawech P, Berczy M, Assaly M, Herrmann F, Bouzourene H, Allal AS, et al. [Medline]. Olfactory neuroblastoma (ONB) is a rare neoplasm of the sinonasal area with neuroendocrine differentiation. A search of the National Cancer Database by Carey et al identified 1225 cases of ENB. Nasal and paranasal sinus carcinoma: how can we continue to make progress?. He had also lost his sense of smell. Cancer. [Full Text]. Revisions of the international criteria for neuroblastoma diagnosis, staging and response to treatment. All material on this website is protected by copyright, Copyright © 1994-2021 by WebMD LLC. Am J Surg Pathol. The importance of multimodality approach in management of ENB is reviewed in detail. 2004 Jul. 1976 Mar. Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region. Patterns of failure and outcome in esthesioneuroblastoma. Diagnosis of esthesioneuroblastoma is usually difficult on conventional light microscopy and almost always requires immunohistochemical staining for definitive diagnosis. Am J Clin Pathol. 2500010-overview for: Medscape. Lund VJ, Howard D, Wei W. Olfactory neuroblastoma: past, present, and future?. Low grade olfactory neuroblastoma usually contains nests and lobules of monotonous tumor cells with round nuclei, indistinct nucleoli and scanty cytoplasm in association with a vascular-rich to hyalinized stroma; fibrillary neural matrix may be present. 2006 Feb. 132(2):134-8. 37(3):1571-6. 2005. Curr OPin Otolaryngol Head Neck Surg. Skull Base. [Medline]. Vitamin D for All Over 50s to Prevent Cancer Deaths? [Medline]. 2008 Nov. 90(2):201-4. 1992 Aug. 102(8):843-9. 1986 Nov. 10(11):771-9. Peripheral cysts: a distinguishing feature of esthesioneuroblastoma with intracranial extension. Guled M, Myllykangas S, Frierson HF Jr, Mills SE, Knuutila S, Stelow EB. Results of a prospective study incorporating chemotherapy, surgery, and combined proton-photon radiotherapy. 2004 Sep 15. [Medline]. A proposed new classification. Am J Surg Pathol. [Medline]. Olfactory neuroblastomas generally grow slowly, … Nichols AC, Chan AW, Curry WT, Barker FG, Deschler DG, Lin DT. NSCLC accounts for about 85% of all lung cancers. Dulguerov P, Allal AS, Calcaterra TC. Interestingly, 80% of the esthesioneuroblastoma (ENB) cases published in the literature since Berger and Luc described the first case in 1924 have been identified in the last few decades. [Medline]. Head Neck. of Pathology, School of Veterinary Medicine, University of California, Davis, Calif. Abstract. It should be noted that precise histologic diagnosis is difficult because ENBs are often confused with other small round cell neoplasms of the nasal cavity. Developmental midline nasal masses in children are rare, with a reported annual incidence of one in every 20,000–40,000 live births (,6–,8). Thomas C Origitano, MD, PhD, FACS Professor and Chair, Department of Neurological Surgery, Medical Director, Neuroscience Service Line, Co-Director, Center for Cranial Base Surgery, Loyola University Medical Center [Medline]. Treatment of esthesioneuroblastoma: a 16-year meta-analysis of 361 patients. Zanation AM, Ferlito A, Rinaldo A, Gore MR, Lund VJ, McKinney KA, et al. 2006 Apr. 2014 Dec. 134 (12):1259-64. [Medline]. Morita A, Ebersold MJ, Olsen KD, Foote RL, Lewis JE, Quast LM. Contrast-enhanced T1-weighted MRI demonstrated a large lesion that originated in the paranasal sinuses and extended through the cribriform plate into the anterior cranial fossa. Beitler JJ, Fass DE, Brenner HA, Huvos A, Harrison LB, Leibel SA, et al. Arch Otolaryngol Head Neck Surg. Rosenthal DI, Barker JL, El-Naggar AK. 94(10):2623-34. S100 highlighted the sustentacular network. 113(3):502-7. J Neurooncol. Pancreas. Soft tissue - tumors of adipose differentiation. 101(6):1437-44. [Medline]. [Full Text]. There is bimodal age distribution with one peak in young adult patients (~2nd decade) and another peak in the 5th to 6thdecades. Rostomily RC, Elias M, Deng M, Elias P, Born DE, Muballe D, et al. An aggressive neoplasm derived from schneiderian epithelium and distinct from olfactory neuroblastoma. N euroendocrine tumors of the sinonasal tract, including olfactory neuroblastoma (ONB) and neuroendocrine carcinoma (NEC), are rare tumors that usually originate from … Carey RM, Godovchik J, Workman AD, Kuan EC, Parasher AK, Chen J, et al. INTRODUCTION Esthesioneuroblastoma (olfactory neuroblastoma) is an uncommon tumor originating in the upper nasal cavity; it was first described by Berger and Luc (3) in 1924. 2017 Dec. 7 (12):1186-1194. 32(5):706-14; discussion 714-5. A thirteen hour surgery took place with a … Diagnosis and management of esthesioneuroblastoma. Combination chemotherapy (cyclophosphamide, doxorubicin, and vincristine with continuous-infusion cisplatin and etoposide) and radiotherapy with stem cell support can be beneficial for adolescents and adults with estheisoneuroblastoma. [Medline]. Devaiah AK, Andreoli MT. 1998 Jul. [Medline]. Pathology of the polyp came back as inverted papilloma and the patient was sent to a university hospital for management. [Medline]. Castelnuovo P, Bignami M, Delù G, Battaglia P, Bignardi M, Dallan I. Endonasal endoscopic resection and radiotherapy in olfactory neuroblastoma: our experience. Approximately 1,000 cases have been described in the literature since the original description in 1924. Clinical utility of somatostatin receptor scintigraphic imaging (octreoscan) in esthesioneuroblastoma: a case study and survey of somatostatin receptor subtype expression. Diseases & Conditions, 2003 Abstract. Microscopically, closely packed cells interspersed with numerous thin-walled, branching staghorn vessels were seen. Laryngoscope. [Medline]. Department of Pathology, The Ohio State University, College of Medicine, 414 Doan Hall, 410 West 10th Ave., Columbus, OH 43210===Search for more papers by this author [Full Text]. 2005 Jul. ENB has an estimated incidence of 4 cases per 10 million individuals and accounts for approximately 5% of all sinonasal tumors. [Medline]. Additionally, there exists a variable presence (or absence) of true rosettes and neurofibrillary material. Lancet Oncol. Their differential diagnosis includes abscesses, hemangiomas, fibromas, lipomas, granulomas, and mucoceles.Neuroimaging is e… ENB does not show familial prevalence and has been reported in all races and on all continents. Acta Otolaryngol. [Medline]. [6]  Similar incidence rates have been obtained through epidemiologic studies performed in Denmark. Lancet Oncol 2001;2:683–90, 10.1016/S1470-2045(01)00558-7 Dublin AB, Bobinski M. Imaging characteristics of olfactory neuroblastoma (esthesioneuroblastoma) . We welcome suggestions or questions about using the website. Neuroblastomas and neuroendocrine carcinomas of the nasal cavity. There is no recognized gender predilection. 1995 Oct. 26(1):35-43. Laryngoscope. 2007 Sep. 29(9):845-50. rhabdomyoblastic, glandular and squamous differentiation), Area of rhabdomyoblastic differentiation if present is positive for, With the exception of olfactory neuroblastoma with rhabdomyoblastic differentiation, the tumor is negative for, Dense core neurosecretory granules, microtubules, neuritic processes, neurofilaments (, Recent next generation sequencing studies have shown that olfactory neuroblastoma has high level but heterogenous chromosomal instability and copy number alterations (, Absence of characteristic fusions of other small blue round cell tumors that may occur in the sinonasal tract, e.g. Madani I, Bonte K, Vakaet L, Boterberg T, De Neve W. Intensity-modulated radiotherapy for sinonasal tumors: Ghent University Hospital update. When, how and why to treat the neck in patients with esthesioneuroblastoma: a review. Department of Pathology, The Ohio State University College of Medicine, Columbus, Ohio. Share cases and questions with Physicians on Medscape consult. ENB affects males and females with similar frequency. 1995 Sep-Oct. 19(5):347-63. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjc4MDQ3LW92ZXJ2aWV3. 9:118. 1998 Apr. [Medline]. Olfactory neuroblastomas (or esthesioneuroblastomas) are most frequently staged using a system proposed by Kadish et al. Occup Med (Lond). Ultrastruct Pathol. [Medline]. Prostate. Michael Somenek, MD Physician, Facial Plastic and Reconstructive Surgery, SomenekMD Advanced Facial Plastic Surgery in 1976 1.. group a: limited to the nasal cavity group b: limited to the nasal cavity and paranasal sinuses group c: extends beyond the nasal cavity and paranasal sinuses: base of skull; intracranial compartment; orbit; distant metastatic disease 2006 Feb. 16(1):25-30. A clinical analysis of 17 cases. 2006. 2002 Image courtesy of Michael Lev, MD. Sinonasal malignancies with neuroendocrine differentiation: patterns of failure according to histologic phenotype. Chemotherapy for advanced esthesioneuroblastoma: the Mayo Clinic experience. ENB does not show a predilection toward any individual race. Sinonasal undifferentiated carcinoma. Coronal CT scan of the orbits and sinuses shows a large, enhancing, and expansile mass occupying the ethmoid air cells that is invading the cribriform plate and breaking through to the left anterior cranial fossa. [Medline]. 27(7):575-84. Initial immunoprofiling may not include myogenic markers. 1993 May. Herbert H Engelhard, III, MD, PhD, FACS, FAANS Affiliated Professor of Bioengineering, University of Illinois at Chicago 2001 Nov. 2(11):683-90. Due to the rare and complex nature of ENB, multiple opinions exist regarding the etiology, optimal staging system, and treatment modalities. 119(7):1412-6. Esthesioneuroblastoma: endonasal endoscopic treatment. /viewarticle/941888 Perez-Ordonez B, Caruana SM, Huvos AG. Cancer. Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical SocietyDisclosure: Nothing to disclose. Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug ReferenceDisclosure: Received salary from Medscape for employment. Frierson HF Jr, Mills SE, Fechner RE. 2010 Aug. 113(2):340-51. [Medline]. In: Tumors of the Upper Respiratory Tract and Ear, Atlas of Tumor Pathology. © Copyright PathologyOutlines.com, Inc. Click, El-Naggar: WHO Classification of Head and Neck Tumours, 4th Edition, 2017, Malignant neuroectodermal tumor commonly located at superior aspect of nasal cavity showing neuroblastic differentiation (, Thought to arise from olfactory membrane or olfactory placode (plate-like thickening of embryonic ectoderm from which a nerve ganglion or sensory organ will develop) which extends from roof of nasal cavity in fetus to midnasal septum and superior turbinate, Not related to neuroblastoma elsewhere in body, Originated and centered around cribriform plate of nasal cavity, Small blue round cell tumor with lobulated growth pattern and abundant neuropil, Homer Wright pseudorosettes and Flexner-Wintersteiner rosettes may be seen, The most important prognostic factors are Hyams grade and Kadish stage, Also called esthesioneuroblastoma, esthesioneuroepithelioma or olfactory placode tumor, No obvious gender, ethnic or familial predilection, Usually confined to the cribriform plate and upper nasal vault; rarely in nasopharynx, maxillary or ethmoid sinus (, May become locally invasive into paranasal sinuses, nasopharynx, palate, orbit, skull base, brain, Theorized to originate from specialized sensory neuroepithelium located in superior nasal cavity, including cribriform plate of ethmoid, nasal roof, superior nasal concha and superior nasal septum (, Usually presents with nonspecific syndromes of nasal obstruction, epistaxis, headache and rhinorrhea (, Rare symptoms include anosmia, visual disturbance, paraneoplastic syndromes (e.g.

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