Neuroblastoma is defined as a solid malignant tumor, which develops by multiplication of cancer cells in one or more tissues of the body. Solid tumors are different from the liquid tumors, which in turn develop in the blood or lymphatic circulation. Neuroblastoma is an extra-cerebral malignant tumor. It develops from the cells of the sympathetic nervous system and it originates in body tissues outside the brain. It is mainly found in the abdomen, spine or the adrenal glands.(1)
How Long It May Take To Recover From The Disease & How Long Does The Symptoms Last?
Prognosis of olfactory neuroblastoma and its recovery time are hard to predict as with any other type of cancer. This varies according to the disease stage, early detection and onset of therapy as well as the treatment procedure. It requires a thorough follow-up for a long period with the average time for a follow-up about 95 months to even 173 months. The 5-year survival rate for this disease is 88%.(5)
Prevent Olfactory Neuroblastoma
The origin of olfactory neuroblastoma is still poorly understood and no preventive measures could be identified. The prevention of complications is based on early diagnosis. It is sometimes possible to identify neuroblastoma during the antenatal ultrasound. Otherwise, alertness is essential after birth. Regular medical monitoring of the child is essential.
Types Of Olfactory Neuroblastomas
Neuroblastomas are currently classified from stage 1 to 4, and sometimes clinicians add stage 4s designating a particular tumor form. They are defined as follows:
- Stage 1, 2 and 3 neuroblastomas are localized forms of the tumor;
- Stage 4 neuroblastomas include the metastatic forms of neuroblastoma, which have given rise to secondary cancers, or metastases;
- Stage 4s neuroblastomas are characterized by the presence of significant metastases in the liver and skin.
Neuroblastoma accounts for 10% of solid tumors in children under 15 years old with a prevalence of 1 case in 70,000 children in this age group. It affects infants most frequently with 50% of affected children are under 2 years of age.(2)
Cause Of Olfactory Neuroblastoma
The cause of olfactory neuroblastoma remains unknown and unexplained to date. Nevertheless, researchers suggest that certain syndromes could predispose to the appearance of neuroblastoma such as Ondine syndrome, Hirschsprung’s disease or neurofibromatosis type 1.
This type of cancer can be suspected in the face of certain clinical signs. The diagnosis of olfactory neuroblastoma can be confirmed by:
- A urinalysis that assesses the levels of certain metabolites whose excretion increases during neuroblastoma (HVA, vanillylmandelic acid, dopamine)
- Imaging of the primary tumor by ultrasound, MRI or CT
- MIBG (metaiodobenzylguanidine) scintigraphy which corresponds to an imaging examination in nuclear medicine
- A biopsy by taking a fragment of tissue for analysis purposes, in particular in the case of suspected cancer
These examinations can be used to confirm the diagnosis of neuroblastoma, to measure its extent and to verify the presence or absence of metastases.
Neuroblastomas most often occur in infants and young children. They represent 10% of childhood cancer cases and 15% of malignant tumors in children under the age of 5 years.(3)
Symptoms Of Olfactory Neuroblastoma
Asymptomatic: An olfactory neuroblastoma can go unnoticed, especially during its early stages. The first symptoms of neuroblastoma most often appear when the tumor spreads.
Localized Pain: The development of neuroblastoma is often accompanied by pain in the affected area.
Local Swelling: A lump or swelling may appear in the affected area.
Altered General Condition: A neuroblastoma disrupts the proper functioning of the nervous system, which can result in loss of appetite, weight loss, slowing of growth.(4)
What Is The Management Of Olfactory Neuroblastoma?
The management of olfactory neuroblastoma depends on different parameters including the type of tumor diagnosed, its evolution and the patient’s condition. Indeed, the course of neuroblastoma can be very different from one patient to another, hence the importance of medical care and follow-up.
Generally, localized stages 1, 2 and 3 tumors are treated by surgery. Surgery may be preceded by chemotherapy. The treatment of these forms of neuroblastoma is currently showing very positive results.
Regarding stage 4 and 4s neuroblastomas, the management of metastatic forms is more complicated. These forms of neuroblastomas require more aggressive treatments than for localized neuroblastomas. Research is currently underway to improve the effectiveness of these treatments, which may include chemotherapy, stem cell transplantation, or local radiotherapy.
- Abdelmeguid AS, Teeramatwanich W, Roberts D, et al. Long-Term Outcomes of Management of Olfactory Neuroblastoma: MD Anderson Experience. Journal of Neurological Surgery Part B: Skull Base. 2018;79(S 01):A184.
- Uccella S, Ottini G, Facco C, et al. Neuroendocrine neoplasms of the head and neck and olfactory neuroblastoma. Diagnosis and classification. Pathologica. 2017;109(1):14-30.
- Yin Z, Wang Y, Wu Y, et al. Age distribution and age-related outcomes of olfactory neuroblastoma: a population-based analysis. Cancer management and research. 2018;10:1359.
- Harvey RJ, Nalavenkata S, Sacks R, et al. Survival outcomes for stage‐matched endoscopic and open resection of olfactory neuroblastoma. Head & neck. 2017;39(12):2425-2432.
- Wu J-Y, Chiou J-F, Ting L-L. Chemo-radiotherapy for olfactory neuroblastoma: cases report and literature review. 2019.
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