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First trace in 1924 , olfactory neuroblastoma is an uncommon malignant tumor pretend the superior os nasale pit . It accounts for about 2 - 3 % of all pinched cavity tumors and occurs in about 0.4 people per million per year . It is thought to be derived from basal cell of olfactory neuroepithelium owing to its anatomic location and certain morphologic characteristics and expression of protein exclusive of the olfactory epithelium.(1 )
Olfactory neuroblastoma can occur at any long time ranging from 2 - 94 years . It unremarkably has a bimodal age of occurrent and occurs at 2nd and 6th decades of life . It can occur in males and females at a similar ratio . The most common presenting symptom are a unilateral nasal impedimenta ( 70 % ) and epistaxis ( 50 % ) . Other less rough-cut mark and symptoms include headache , botheration , rhinorrhea , undue lacrimation , anosmia , and visual psychological disorder . It is worth noting that though the tumor come up from the olfactory neuroepithelium , anosmia ( deprivation of sense of tone ) is not a plebeian complaint and only occur in about 5 % of the patient . There have been reports of isolated cases of olfactory neuroblastoma that secrete vasopressin that can precede to high blood pressure and hyponatremia . There is a delay in diagnosing of the tumor due to its non - specific presentation and delayed growth.(2 )
Is Olfactory Neuroblastoma Hereditary?
The aetiology of olfactory neuroblastoma is poorly realize . It originates from immature neural tip cells as the tumour utter the Drosophila achaete - scute cistron ( hASH1 ) in a modify contrary - transcription polymerase mountain range reaction ( RT - PCR ) . Various researcher have shown chromosome change at various factor location that are come to to pitiable prognosis ( 4p / q , 5p / q , 6q ) , high - risk of infection relapse and metastasis ( 5p / q , 6q ) ; however , their clinical usefulness in malignant neoplastic disease therapy is yet to be determine . Although genetic alteration are noted in olfactory neuroblastoma , especially for recurrent and metastatic stages , there is no link between the patrimonial and familial occurrence of the disease.(1 )
Diagnosis Of Tumor
The imaging studies employed in the diagnosing of olfactory neuroblastoma includeCT scanandMRIimaging ( with or without contrast medium ) that show ‘ booby - shaped ’ mass expand across the cribriform plate into the cranium.(2 )
The symptomatic verification is done by biopsy of the neoplasm that testify lobular architecture comprising of primitive cellphone . These limit lobule or nests of tumor cells are present below an integral mucosa that is separated by vascularized fibrous stroma . There is the comportment of pleomorphism , rosette , mitoses , necrosis , secreter , and calcification.(2 )
Staging Of Tumor
The staging system proposed by Kadish et al . in 1976 is still used . The staging include :
degree A : tumor limited to the nasal cavity
Stage B : neoplasm involving the nasal enclosed space and one or more paranasal fistula
degree C : extension of the tumour beyond the pinched cavity and paranasal sinuses
Due to hold up in demonstration and diagnosis , most of the tumors ( about 50 % ) are found in stage C. The survival rate for stage A , phase B , and stage C is 75 - 91 % , 68 - 71 % , and 41 - 47 % , severally . The overall , 5 - year natural selection for olfactory neuroblastoma is 60 - 80 % . While low - grade tumors have a 5 - year survival of 80 % , high - score tumors have a 5 - year natural selection of 40%.(2 )
Management Of Olfactory Neuroblastoma
The intimately long term results are achieved by staring operative remotion of neuroblastoma by using bi - cranial - facial coming that transfer the cribriform home base stick with by radiation therapy . In some case , endoscopic resection can be execute for a modified tumor . For advanced tumors that are a non - resectable or circulate disease , palliative chemotherapy is the treatment of pick . Autologous osseous tissue bone marrow organ transplant has been used to attain prospicient full term survival in modified cases.(2 )
Recurrence is coarse for olfactory neuroblastoma , which come in about 30 % cases ( ranges from 15 - 70 % ) within the first 2 eld after initial direction . Cervical lymph thickening metastasis ( 25 % ) and distant metastasis ( 10 % ) is noted in all the cases of olfactive neuroblastoma . The most common internet site of metastases is lung and bones . The selection is perplex by distaff sex , age < 20 year or > 50 year , eminent grade of the tumor , all-embracing intracranial spread , remote metastasis , return of tumour , and high proliferation indicator of the tumor.(2 )
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