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Cirugia Y Cirujanos 2020Olfactory neuroblastoma (ONB) is a malignant neoplasm that arises from the upper nasal vault. (Review)
Review
INTRODUCTION
Olfactory neuroblastoma (ONB) is a malignant neoplasm that arises from the upper nasal vault.
OBJECTIVE
We present a retrospective case series and clinical analysis of 12 ONB cases.
MATERIALS AND METHODS
Patients with ONB treated at Mexico´s National Cancer Institute between 2011 and 2018.
RESULTS
The Kadish proportion of B, C, and D stage was 16%, 58%, or 25%, respectively. Hyams Grade 1, 2, or 3 was 25%, 50%, and 25%, respectively. The most common surgical approach was the craniofacial in 5 cases (42%), followed by the transfacial in 4 cases (33%), and the endonasal endoscopic approach in 3 cases (25%). Gross total resection was achieved in 8 patients (67%). Five patients (42%) underwent a second operation due to recurrent/progressive disease. The surgical complication rate was 8.3%. Progression-free survival was 41 months and the mean overall survival was 63.6 months.
CONCLUSIONS
Surgical resection followed by radiotherapy, and chemotherapy for metastatic and recurrent disease provides the best outcome in terms of survival and recurrence. To the best of our knowledge, this is the first series of cases reported in Mexico.
Topics: Academies and Institutes; Antineoplastic Agents; Chemotherapy, Adjuvant; Cisplatin; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Mexico; Middle Aged; Nasal Cavity; Neoplasm Recurrence, Local; Nose Neoplasms; Progression-Free Survival; Radiotherapy Dosage; Radiotherapy, Adjuvant; Reoperation; Retrospective Studies; Treatment Outcome
PubMed: 32567594
DOI: 10.24875/CIRU.19001111 -
Oral Oncology Feb 2020Optimal neck management in patients with olfactory neuroblastoma (ONB), a rare malignancy, remains uncertain. This study aimed to analyse patterns of cervical lymph node...
OBJECTIVE
Optimal neck management in patients with olfactory neuroblastoma (ONB), a rare malignancy, remains uncertain. This study aimed to analyse patterns of cervical lymph node metastases and corresponding clinical outcomes and to investigate the value of elective neck irradiation (ENI) in this population.
METHODS AND MATERIALS
This study retrospectively reviewed clinical records, imaging findings, nodal metastasis features and treatment data of 217 patients with ONB treated at our hospital during 1991-2019. Univariate and multivariate analyses were used to assess the influence of cervical lymph node involvement on treatment outcomes. Survival and regional failure rates were compared between patients with or without ENI.
RESULTS
Thirty-two patients (14.7%) presented initially with cervical lymph node metastases, most frequently at levels II (10.6%, 23/217) and VIIa (5.5%, 12/217). Patients with and without cervical node metastasis differed significantly in overall (OS) (41.9% vs. 86.1%, p < 0.001), progression-free (PFS) (41.9% vs. 84.8%, p < 0.001), regional failure-free (45.9% vs. 89%, p < 0.001) and distant metastasis-free survival (41.5% vs. 86.1%, p < 0.001). Cervical lymph involvement was an independent factor affecting poor OS (hazard ratio, 0.184, 95% confidence interval, 0.078-0.436, p < 0.001) and PFS (hazard ratio, 0.198, 95% confidence interval, 0.088-0.445, p < 0.001). Moreover, 43.8% patients (95/217) underwent ENI, which significantly reduced the incidence of regional recurrence from 10.7% to 3.2% (χ = 4.396, p = 0.036) but did not significantly affect other survival outcomes. Regional failures could be resolved using salvage treatment.
CONCLUSIONS
Our findings indicate the importance of systematic therapy for patients with initial cervical lymph node metastases. ENI is not recommended for N0 disease.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Combined Modality Therapy; Disease Management; Esthesioneuroblastoma, Olfactory; Female; Humans; Kaplan-Meier Estimate; Lymphatic Metastasis; Male; Middle Aged; Multimodal Imaging; Neoplasm Grading; Neoplasm Staging; Prognosis; Treatment Failure; Treatment Outcome; Young Adult
PubMed: 31835073
DOI: 10.1016/j.oraloncology.2019.104505 -
International Forum of Allergy &... Sep 2019Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, represents up to 3% of all sinonasal neoplasms. Hyams histologic grading is a promising tool in... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, represents up to 3% of all sinonasal neoplasms. Hyams histologic grading is a promising tool in predicting metastases and establishing prognoses for this complex tumor.
METHODS
A systematic literature search was performed in the PubMed, Ovid MEDLINE, and Cochrane databases. ENB patients with Hyams I-II or III-IV were categorized as low-grade Hyams (LGH) or high-grade Hyams (HGH), respectively. Binary and continuous random-effects models were applied to calculate odds ratios (ORs) for the incidences of neck and distal metastases as well as for 5- and 10-year overall survival rates.
RESULTS
Of the 57 screened articles published from 1993 to 2018, 16 (525 patients) and 21 (563 patients) provided data for tumor metastases and overall survival rates, respectively. Neck metastasis was observed in 18.2% of HGH vs 7.9% of LGH patients. Distant metastasis was noted in 20.7% of HGH vs 8.9% of LGH patients. LGH patients had 5- and 10-year overall survival rates of 81.2% and 64.0%, respectively, as compared with 60.9% and 40.6%, respectively, for HGH patients. In comparing HGHs vs LGHs, the collective ORs for neck and distant metastases were 2.08 (95% confidence interval [CI], 1.09-3.99; p = 0.03) and 2.37 (95% CI, 1.07-5.26; p = 0.03), respectively. Moreover, in comparing LGHs vs HGHs, collective ORs for 5- and 10-year overall survival rates were 3.39 (95% CI, 2.09-5.49; p < 0.001) and 3.03 (95% CI, 1.82-5.06; p < 0.001), respectively.
CONCLUSION
HGH ENBs, compared with LGH ENBs, are more likely to metastasize to neck or distal targets and to have lower overall survival rates.
Topics: Esthesioneuroblastoma, Olfactory; Humans; Incidence; Models, Statistical; Nasal Cavity; Neoplasm Metastasis; Neoplasm Staging; Nose Neoplasms; Prognosis; Survival Analysis
PubMed: 31251848
DOI: 10.1002/alr.22373 -
International Forum of Allergy &... May 2024Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory... (Review)
Review
Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory bulbs and tracts. This results in postoperative anosmia that may significantly impact a patient's quality of life without definitive added benefit in survival. The prevalence of occult intracranial disease is low, especially for Hyams grade I and II tumors. A unilateral approach sparing the contralateral cribriform plate and olfactory system can be considered for select cases of early stage, low-grade tumors when the disease does not cross midline to involve the contralateral olfactory cleft or septal mucosa and when midline dural margins can be cleared with frozen pathology. Approximately half of patients who undergo unilateral resection may have residual olfaction even with adjuvant unilateral radiation. Early data suggest favorable disease-free survival and overall survival for patients who underwent the unilateral approach; however, larger sample studies are needed to confirm comparability to bilateral resections regarding oncologic outcomes.
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity; Smell; Treatment Outcome
PubMed: 38526915
DOI: 10.1002/alr.23345 -
Ear, Nose, & Throat Journal Oct 2023Olfactory neuroblastoma (ONB) is a rare malignancy of the sinonasal cavity, originating from neuroepithelial olfactory cells. Olfactory neuroblastoma can be difficult to...
Olfactory neuroblastoma (ONB) is a rare malignancy of the sinonasal cavity, originating from neuroepithelial olfactory cells. Olfactory neuroblastoma can be difficult to diagnose due to its anatomic position and variable symptomatic presentation, leading to diagnosis at a more advanced stage. Here, we present the case of a 35-year-old man with no previous medical history who had a bicycle accident secondary to syncope. He was found to be hyponatremic and suspected to have syndrome of inappropriate antidiuretic hormone secretion (SIADH). In the workup of SIADH, a brain magnetic resonance imaging revealed a mass in the left middle meatus. The lesion secreted inappropriate amounts of ADH, resulting in symptomatic paraneoplastic SIADH. This ultimately led to the early recognition and successful resection of this rare ONB. The patient has remained disease-free for over 5 years. This case is a fortunate example of a functional malignancy of the sinonasal tract that was discovered early and successfully treated as a result of symptomatic SIADH.
Topics: Male; Humans; Adult; Inappropriate ADH Syndrome; Esthesioneuroblastoma, Olfactory; Nose Neoplasms; Nasal Cavity
PubMed: 34134537
DOI: 10.1177/01455613211022102 -
Radiotherapy and Oncology : Journal of... Mar 2017To clarify the efficacy and feasibility of proton beam therapy (PBT) for olfactory neuroblastoma (ONB).
PURPOSE
To clarify the efficacy and feasibility of proton beam therapy (PBT) for olfactory neuroblastoma (ONB).
METHODS AND MATERIALS
We retrospectively reviewed 42 consecutive patients who received PBT with curative intent for ONB at National Cancer Center Hospital East from November 1999 to March 2012.
RESULTS
Five patients (12%) had Kadish A disease, nine (21%) had Kadish B, and twenty-eight (67%) had Kadish C. All patients except one received a total dose of 65Gy (relative biological effectiveness: RBE) in 26 fractions. Twenty-four patients (57%) received induction and/or concurrent chemotherapy. The median follow-up for all eligible patients was 69months (7-186). The 5-year overall survival (OS) and progression-free survival (PFS) rates were 100% and 80% for Kadish A, 86 and 65% for Kadish B, and 76% and 39% for Kadish C, respectively. The sites of the first progression were local in six patients (30%), regional in eight (40%), distant in two (10%), local and regional in two (10%), and local and distant in two (10%). Late adverse events of grade 3-4 were seen in six patients (ipsilateral visual impairment, 3; bilateral visual impairment, 1; liquorrhea, 1; cataract, 1).
CONCLUSION
PBT was a safe and effective modality for ONB.
Topics: Adult; Aged; Disease-Free Survival; Esthesioneuroblastoma, Olfactory; Feasibility Studies; Female; Follow-Up Studies; Humans; Male; Middle Aged; Nasal Cavity; Nose Neoplasms; Proton Therapy; Retrospective Studies; Treatment Outcome
PubMed: 28062086
DOI: 10.1016/j.radonc.2016.12.020 -
Indian Journal of Otolaryngology and... Oct 2022Olfactory neuroblastoma (ONB) or esthesioneuroblastoma is a rare malignant intranasal tumor, commonly originated from upper part of nasal cavity. Majority of cases...
Olfactory neuroblastoma (ONB) or esthesioneuroblastoma is a rare malignant intranasal tumor, commonly originated from upper part of nasal cavity. Majority of cases presented with nasal obstruction or epistaxis. ONB is rarely reported in ectopic locations. Here we present the first-ever documented case of an olfactory neuroblastoma situated anterior to body of maxilla, presented as left sided facial swelling. This case report is aimed at achieving the consideration of this rare tumour as a differential diagnosis in the lesions of the anatomical region surrounding the commonly known site of origin i.e. the sinonasal cavity.
PubMed: 36452651
DOI: 10.1007/s12070-021-02595-9 -
Cancers May 2021Olfactory neuroblastoma (ONB) is a rare sinonasal neoplasm with a peculiar behavior, for which limited prognostic factors are available. Herein, we investigate the...
Olfactory neuroblastoma (ONB) is a rare sinonasal neoplasm with a peculiar behavior, for which limited prognostic factors are available. Herein, we investigate the transcriptional pathways altered in ONB and correlate them with pathological features and clinical outcomes. We analyze 32 ONB patients treated with curative intent at two independent institutions from 2001 to 2019 for whom there is available pathologic and clinical data. We perform gene expression profiling on primary ONB samples and carry out functional enrichment analysis to investigate the key pathways associated with disease-free survival (DFS). The median age is 53.5 years; all patients undergo surgery and a pure endoscopic approach is adopted in the majority of cases (81.2%). Most patients have advanced disease (stages III-IV, 81.2%) and 84.4% undergo adjuvant (chemo)radiotherapy. The median follow-up is 35 months; 11 (26.8%) patients relapse. Clinical characteristics (gender, stage and Hyams' grade) are not associated with the outcomes. In contrast, TGF-beta binding, EMT, IFN-alpha response, angiogenesis, IL2-STAT5 and IL6-JAK-STAT3 signaling pathways are enriched in patients experiencing recurrence, and significantly associated with shorter DFS. Clustering of transcriptional profiles according to pathological features indicates two distinct molecular groups, defined by either cytokeratin-positive or -negative immunostaining. Definition of the characterizing ONB transcriptomic pathways may pave the way towards tailored treatment approaches.
PubMed: 34064009
DOI: 10.3390/cancers13112527 -
Journal of Clinical Medicine Jun 2021Olfactory neuroblastoma (ONB) is a rare neuroepithelial-derived malignancy that usually presents in the nasal cavity. The rarity of ONB has led to conflicting reports...
Olfactory neuroblastoma (ONB) is a rare neuroepithelial-derived malignancy that usually presents in the nasal cavity. The rarity of ONB has led to conflicting reports regarding associations of patient age and ONB survival and outcome. Moreover, long-term outcomes of chemotherapy and other treatment modalities are speculated. Here, we aimed to compare survival outcomes across age groups through time and determine associations between treatment modality and survival. In this retrospective population-based study, we analyzed the SEER 2000-2016 Database for patients with ONB tumors. Using Kaplan-Meier survival analysis, a significant effect of age and cancer-specific survival (CSS) was observed; geriatric ONB patients had the lowest CSS overall. Generalized linear models and survival analyses demonstrated that CSS of the pediatric patient population was similar to the geriatric group through 100 months but plateaued thereafter and was the highest of all age groups. Radiation and surgery were associated with increased CSS, while chemotherapy was associated with decreased CSS. GLM results showed that tumor grade, stage and lymph node involvement had no CSS associations with age or treatment modality. Our results provide insight for future investigations of long-term outcomes associated with ONB patient age and treatment modality, and we conclude that survival statistics of ONB patients should be analyzed in terms of trends through time rather than fixed in time.
PubMed: 34207118
DOI: 10.3390/jcm10122685 -
Oncology Letters Dec 2015Olfactory neuroblastoma (ON) is a rare type of malignant neoplasm originating from the olfactory neuroepithelial cells of the nasal cavity. ON is also known as...
Olfactory neuroblastoma (ON) is a rare type of malignant neoplasm originating from the olfactory neuroepithelial cells of the nasal cavity. ON is also known as esthesioneuroblastoma or neuroendocrine carcinoma. The malignancy accounts for <3% of tumors originating in the nasal cavity. Through the nasal cavity, ON may infiltrate the sinuses, the orbit and the cranium. The tumor is characterized by a pattern of slow growth and local recurrences. Treatment options are surgical excision or surgery combined with a radiotherapy (RT) and/or chemotherapy combination treatment. The present study reports the case of a 69-year-old patient with a mass in the nasal cavity who was treated by combined surgical excision and RT. The literature for ON and the treatment of the tumor are also discussed.
PubMed: 26788185
DOI: 10.3892/ol.2015.3821