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Olfactory neuroblastoma — Clinical Guidelines

Overview

Olfactory neuroblastoma is a rare neurogenic tumor arising in the nasal cavity or paranasal sinuses, often presenting with nonspecific symptoms due to its location and modest clinical presentation. Accurate diagnosis frequently requires histopathological and immunohistochemical studies beyond routine clinical evaluation. 3 17

Diagnosis

Clinical Presentation: Often subtle, including nasal obstruction, epistaxis, and facial pain. 3

Imaging: CT and MRI are essential for assessing tumor extent and local invasion. 3

Biopsy and Histopathology: Essential for definitive diagnosis. 3 17

Immunohistochemistry: Useful markers include neuron-specific enolase (NSE), S-100 protein, and glial fibrillary acidic protein (GFAP). 17

Endoscopic Examination: Can aid in initial suspicion and planning for biopsy. 3

Management

Surgical Resection: Primary treatment, often requiring specialized techniques due to tumor location. 3

Radiation Therapy: Used postoperatively for residual or recurrent disease. 3

Chemotherapy: Considered in advanced or metastatic cases, though specific regimens are not detailed in provided abstracts. 3

Follow-Up: Regular imaging and clinical assessments to monitor for recurrence or metastasis. 3

Special Populations

Elderly: Polypharmacy may influence olfactory function, complicating diagnosis and management. 2 6

Comorbidities: No specific guidance provided in abstracts; individualized care is recommended. 3

Key Recommendations

Definitive Diagnosis Requires Histopathological Examination with Immunohistochemistry: Essential for distinguishing olfactory neuroblastoma from other tumors. (Evidence: Moderate 17)

Surgical Resection Should Be the Primary Treatment Approach: Tailored to the extent of disease and anatomical constraints. (Evidence: Expert opinion 3)

Postoperative Radiation Therapy Is Indicated for Residual or Recurrent Disease: To improve outcomes and reduce recurrence rates. (Evidence: Moderate 3)

Regular Follow-Up Monitoring Is Crucial: Including imaging and clinical evaluations to detect early recurrence or metastasis. (Evidence: Expert opinion 3)

References

1 César CPHAR, Lima KM, Oliveira OMS, Santos LMN, Dantas VS, Domenis DR et al.. Speech therapy program for the rehabilitation of smell. CoDAS 2026. link 2 Izco-Cubero M, Zambom-Ferraresi F, Zambom-Ferraresi F, de la Riva MLFG, Santamaría E, Fernandez-Irigoyen J et al.. Impact of medication use on olfactory performance in older adults. Frontiers in public health 2025. link 3 Marina TC, Constantin BN, Flavia B, Silvana SO, Marioara P, Sarau CA. Olfactory Neuroblastoma-A Challenging Fine Line between Metastasis and Hematology. Medicina (Kaunas, Lithuania) 2023. link 4 Yoshino A, Goektas G, Mahmut MK, Zhu Y, Goektas O, Komachi T et al.. A New Method for Assessment of Retronasal Olfactory Function. The Laryngoscope 2021. link 5 Miwa T, Ikeda K, Ishibashi T, Kobayashi M, Kondo K, Matsuwaki Y et al.. Clinical practice guidelines for the management of olfactory dysfunction - Secondary publication. Auris, nasus, larynx 2019. link 6 Ottaviano G, Savietto E, Scarpa B, Bertocco A, Maculan P, Sergi G et al.. Influence of number of drugs on olfaction in the elderly. Rhinology 2018. link 7 Sanmartí-Espinal M, Galve R, Iavicoli P, Persuy MA, Pajot-Augy E, Marco MP et al.. Immunochemical strategy for quantification of G-coupled olfactory receptor proteins on natural nanovesicles. Colloids and surfaces. B, Biointerfaces 2016. link 8 Bazáes A, Schmachtenberg O. Odorant tuning of olfactory crypt cells from juvenile and adult rainbow trout. The Journal of experimental biology 2012. link 9 Williams GS, Ramachandran K, Nall L, Fong J, Mathews J. Management of post-infectious olfactory dysfunction: a nationwide survey of UK ENT consultants. B-ENT 2009. link 10 Shu CH, Yuan BC. Assessment of odor identification function in Asia using a modified "Sniffin' Stick" odor identification test. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2008. link 11 . Medical devices; ear, nose, and throat devices; classification of olfactory test device. Final rule. Federal register 2006. link 12 Hornung DE. Nasal anatomy and the sense of smell. Advances in oto-rhino-laryngology 2006. link 13 Germanà A, Montalbano G, Laurà R, Ciriaco E, del Valle ME, Vega JA. S100 protein-like immunoreactivity in the crypt olfactory neurons of the adult zebrafish. Neuroscience letters 2004. link 14 Andressen C, Arnhold S, Mai JK. Differential expression of lactoseries carbohydrate epitopes HNK-1, CD15, and NALA by olfactory receptor neurons in the developing chick. Anatomy and embryology 1998. link 15 Sandmark B, Broms I, Löfgren L, Ohlson CG. Olfactory function in painters exposed to organic solvents. Scandinavian journal of work, environment & health 1989. link 16 Blakely RD, Ory-Lavollée L, Grzanna R, Koller KJ, Coyle JT. Selective immunocytochemical staining of mitral cells in rat olfactory bulb with affinity purified antibodies against N-acetyl-aspartyl-glutamate. Brain research 1987. link90049-7) 17 Choi HS, Anderson PJ. Immunohistochemical diagnosis of olfactory neuroblastoma. Journal of neuropathology and experimental neurology 1985. link 18 Hempstead JL, Morgan JI. Monoclonal antibodies reveal novel aspects of the biochemistry and organization of olfactory neurons following unilateral olfactory bulbectomy. The Journal of neuroscience : the official journal of the Society for Neuroscience 1985. link

Olfactory neuroblastoma