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The Laryngoscope Feb 2022Olfactory neuroblastoma (ONB) is a rare sinonasal malignant neoplasm that is known to develop late recurrence. The aim of this study is to evaluate the long-term... (Review)
Review
OBJECTIVES/HYPOTHESIS
Olfactory neuroblastoma (ONB) is a rare sinonasal malignant neoplasm that is known to develop late recurrence. The aim of this study is to evaluate the long-term outcomes of patients with ONB and to determine the factors associated with prognosis.
STUDY DESIGN
Retrospective study.
METHODS
A retrospective review of the medical records of 139 patients diagnosed with ONB at MD Anderson Cancer Center was performed between 1991 and 2016. Descriptive statistics were calculated, and Kaplan-Meier curves were utilized to assess survival.
RESULTS
Median follow-up time was 75 months. Overall, 129 patients (92.8%) had surgery as part of their treatment and 82 (58.9%) patients received postoperative radiation therapy (PORT) or concurrent chemoradiotherapy. Endoscopic approaches were utilized for 72 patients, 69.4% of whom had pure endoscopic endonasal approaches. Five-year overall survival and disease-specific survival were 85.6% and 93.4%, respectively. Recurrence rate was 39.6% with a median time to recurrence of 42 months. Among the 31 patients who received elective nodal irradiation (ENI), two patients developed neck recurrence (6.4%) compared with 20 who developed neck recurrence when ENI was omitted (34.4%) (P = .003). Advanced Kadish stage, orbital invasion, intracranial invasion, and presence of cervical lymphadenopathy at the time of presentation were significantly associated with poor survival.
CONCLUSION
ONB has an excellent survival. Surgical resection with PORT when indicated is the mainstay of treatment. Endoscopic approaches can be used as a good tool. Elective neck irradiation reduces the risk of nodal recurrence among patients with clinically N0 neck. Despite the excellent survival, recurrence rate remains high and delayed, highlighting the need for long-term surveillance.
LEVEL OF EVIDENCE
Level 4 Laryngoscope, 132:290-297, 2022.
Topics: Esthesioneuroblastoma, Olfactory; Humans; Nasal Cavity; Nose Neoplasms; Prognosis; Retrospective Studies; Survival Rate; Time Factors; Treatment Outcome
PubMed: 34272876
DOI: 10.1002/lary.29732 -
Annals of Diagnostic Pathology Oct 2019Beyond squamous carcinoma, a variety of diagnostically challenging neoplasms arise within various head and neck sites. This is particularly the situation with neoplasms... (Review)
Review
CONTEXT
Beyond squamous carcinoma, a variety of diagnostically challenging neoplasms arise within various head and neck sites. This is particularly the situation with neoplasms where little cellular cytoplasm is present to assist the pathologist in categorizing such lesions.
OBJECTIVE
To highlight diagnostic pitfalls that accompanying neoplasms composed primarily of 'cytoplasmically-poor' cells. These pitfalls include morphologic and immunohistochemical traps that emerge from this class of neoplasms.
DATA SOURCES
Selection of pathologic specimens from the author's personal files, and literature review.
CONCLUSIONS
Interpretative pitfalls regarding the histopathology and immunophenotype of small 'cytoplasmically-poor' neoplasms are a diagnostic hazard in head and neck surgical pathology practice, and require knowledge of histomorphologic plasticity and aberrant immunophenotyping.
Topics: Head and Neck Neoplasms; Humans; Pathologists; Pathology, Clinical
PubMed: 31382078
DOI: 10.1016/j.anndiagpath.2019.07.005 -
Oral Oncology Apr 2020The clinical data on olfactory neuroblastomas (ONBs) are scarce owing to their rarity. This study aimed to assess the potential prognostic factors, outcomes, and optimal...
OBJECTIVE
The clinical data on olfactory neuroblastomas (ONBs) are scarce owing to their rarity. This study aimed to assess the potential prognostic factors, outcomes, and optimal treatment strategies in patients with ONB.
METHODS AND MATERIALS
The data of 217 patients with ONB between 1991 and 2019 were retrospectively reviewed. Long-term survival, potential prognostic factors, and outcomes with combined treatment strategies were analyzed.
RESULTS
All patients received radiotherapy (RT); 185 patients underwent surgery, and 139 patients received chemotherapy. The 5-year overall survival (OS), progression-free survival (PFS), locoregional failure-free survival (LRFS), and distant metastasis-free survival (DMFS) of the entire cohort were 80.0%, 79.0%, 79.3%, and 80%, respectively. On univariate analyses, R0/R1 resection, early Kadish stage, negative lymph nodes, absence of orbital invasion, and administration of surgery with RT were found to be favorable factors. Conversely, combined sequential treatment with surgery, RT, and chemotherapy was not associated with survival. Multivariate analysis demonstrated lymph node status, orbital invasion, and the combination of surgery and RT to be independent prognostic factors.
CONCLUSIONS
Patients with ONB, who had lymph node metastases, orbital invasion diseases, advanced Kadish stages, R2 resection margins, and received RT alone, had poor outcomes. Combined administration of surgery and RT may be a potentially useful strategy in patients with advanced Kadish stages; the role of chemotherapy in these stages requires further evaluation.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Combined Modality Therapy; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Middle Aged; Prognosis; Treatment Outcome; Young Adult
PubMed: 32126517
DOI: 10.1016/j.oraloncology.2020.104618 -
Der Pathologe Feb 2018Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck... (Review)
Review
Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms. The neural-neuroectodermal-type NENs comprise olfactory neuroblastoma and paraganglioma, each of them with distinct clinicopathological characteristics. Olfactory neuroblastomas show a spectrum of histologic differentiation and are prognostically classified by Hyams grading. Paragangliomas often occur multiple and show a familial background. Most head and neck NENs occur in the upper respiratory system. Their diagnosis follows the general guidelines for NENs, focusing on immunohistochemical profiling. Molecular examinations are so far only required in individual cases.
Topics: Carcinoid Tumor; Chromogranin A; Head and Neck Neoplasms; Humans; Lung Neoplasms; Neuroendocrine Tumors
PubMed: 29313083
DOI: 10.1007/s00292-017-0404-8 -
World Neurosurgery May 2021This article retrospectively analyzed the data of all patients diagnosed with malignant tumors of the nasal cavity and sinuses by pathologic section in all patients...
BACKGROUND
This article retrospectively analyzed the data of all patients diagnosed with malignant tumors of the nasal cavity and sinuses by pathologic section in all patients (including outpatients and inpatients) in our hospital from January 2008 to December 2017 (10 years) to discuss the nasal cavity The clinical characteristics of sinus malignant tumors and the clinical characteristics (symptoms, signs, imaging examinations) of 35 cases of olfactory neuroblastoma with high incidence were analyzed, and their treatment methods and prognosis were summarized for future nasal tumors.
CLINICAL BASIS FOR PREVENTION AND TREATMENT
In our department, 300 cases of malignant tumors of nasal cavity and sinuses were treated within 10 years. AGE: Eighty-six cases (28.6%) were in the age group 50-59 years old, occupying first place, and 55 cases (18.3%) were in the age group 60-69 years old, occupying second place. By comparing groups A and B with malignant tumors of the nasal cavity and sinuses, the difference in age distribution of olfactory neuroblastoma is statistically significant. Of the 35 cases of olfactory neuroblastoma, 23 cases were diagnosed with nasal tumors on admission, 12 cases were initially diagnosed with nasal polyps (including 7 cases with hemorrhagic and necrotic nasal polyps), and 20 cases underwent rapid pathologic definite diagnosis during the operation.
DIAGNOSIS FOR POSTOPERATIVE PATHOLOGIC RETURNS
On the basis of our analysis, the following conclusions can be drawn: Of the 300 cases of malignant tumors of the nasal cavity and paranasal sinuses, age distribution tends to be younger, the overall incidence of males is higher than that of females, but the proportion of male and female olfactory neuroblastoma is equal, and the incidence of adenoid cystic carcinoma and sarcoma. The morphology of cancer can be divided as follows: the tissue source is epithelial tissue, the cancer is harder, paler, drier, and often forms cancer nests, the parenchymal and interstitial boundaries are clear, and the fibrous tissue is hyperplastic. The incidence rate of females is higher than that of males, with the primary origin in the sinuses. Among them, the maxillary sinus is more common. Olfactory neuroblastoma, lymphoma, and sarcoma are gradually increasing in malignant tumors of the nasal cavity and sinuses.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Middle Aged; Nasal Cavity; Nose Neoplasms; Retrospective Studies; Tomography, X-Ray Computed; Young Adult
PubMed: 33039569
DOI: 10.1016/j.wneu.2020.09.124 -
Pathology, Research and Practice Aug 2018Olfactory neuroblastoma (ONB) is a kind of rare and complex head and neck tumor. The reports on this field are very scarce due to the low morbidity. Here, we summarized...
Olfactory neuroblastoma (ONB) is a kind of rare and complex head and neck tumor. The reports on this field are very scarce due to the low morbidity. Here, we summarized the clinical features and prognosis of ONB through analysis of 10 cases, and determined the phosphorylation status of some molecules known to be involved in carcinogesis such as Akt, Erk, Stat3 and Stat5 in ONB tissue. Ten ONB patients were recruited in this study, 6 male and 4 female, ranging from 26 to 66 years old. In the 10 cases, 6 were diagnosed as late T stage (T/T), 6 were at late Kadish stage (C/D) and 3 were at high Hyams grade (Ⅲ), which indicated a poorer prognosis. Patient characteristics-gender and tumor features were evaluated with respect to the overall survival (OS) through univariate analysis. The result indicated that the OS of male is obviously higher than that of female after a series of combined treatment. The OS of ONB patients in the late stage or high grade is lower than those in early stages or low grade. Moreover, p-Akt, p-Erk, p-Stat3 and p-Stat5 was detected in 5 (50%), 9 (90%), 7 (70%) and 0 patients (0%), respectively, suggesting the former 3 molecules might be potential biomarkers for diagnosis of ONB.
Topics: Adult; Aged; Biomarkers, Tumor; Disease-Free Survival; Esthesioneuroblastoma, Olfactory; Female; Humans; Kaplan-Meier Estimate; Male; Middle Aged; Nasal Cavity; Nose Neoplasms; Prognosis
PubMed: 29921494
DOI: 10.1016/j.prp.2018.06.002 -
Clinical Neuropathology 2023There are limited data regarding immune surveillance mechanisms in olfactory neuroblastoma. We investigated the expression of programmed cell death protein 1 (PD-1),...
There are limited data regarding immune surveillance mechanisms in olfactory neuroblastoma. We investigated the expression of programmed cell death protein 1 (PD-1), cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), CD4, and CD8 in olfactory neuroblastoma to identify potential therapeutic targets. Immunohistochemistry was used to detect PD-1 and CTLA-4 and measure the numbers of CD4 and CD8 T cells in 56 patients with olfactory neuroblastoma. The relationships between these molecules in tumor microenvironment, clinicopathological features, and survival were analyzed. The prevalence of PD-1 in Kadish C stage was 24.14%, significantly greater than in Kadish A and B stage. CD4 T-cell and CD8 T-cell levels correlated with higher Hyams histological grade and Kadish stage. In addition, PD-1 was related positively with CTLA-4, CD4 T cells, and CD8 T cells in olfactory neuroblastoma. Univariate survival analysis showed that higher PD-1 positivity, CD8 T cells, and Hyams grade correlated with worse clinical outcome. Multivariate analysis showed that the expression of PD-1 was an independent parameter for poor prognosis. In conclusion, olfactory neuroblastoma with PD-1 expression had more aggressive clinicopathological features and worse prognosis. PD-1 may potentially predict the outcome of olfactory neuroblastoma patients.
Topics: Humans; CD8-Positive T-Lymphocytes; Prognosis; Esthesioneuroblastoma, Olfactory; CTLA-4 Antigen; Programmed Cell Death 1 Receptor; Nasal Cavity; Nose Neoplasms; B7-H1 Antigen; Tumor Microenvironment
PubMed: 36708210
DOI: 10.5414/NP301519 -
Journal of Neurological Surgery Reports Jul 2023Esthesioneuroblastoma is a rare sinonasal malignancy that arises from the olfactory epithelium. The overall incidence of lymph node metastases is 25%. However, neck...
Esthesioneuroblastoma is a rare sinonasal malignancy that arises from the olfactory epithelium. The overall incidence of lymph node metastases is 25%. However, neck disease can present in a delayed fashion. As such, management of the clinically negative neck is controversial, with some advocating for elective neck treatment and others recommending observation with salvage treatment if necessary. At this time, no prospective head-to-head comparisons of elective versus salvage treatment have been performed.
PubMed: 37564630
DOI: 10.1055/s-0043-1770965 -
The Laryngoscope Jun 2018Compare outcomes of patients with olfactory neuroblastoma (ONB) without skull base involvement treated with and without resection of the dura and olfactory bulb. (Comparative Study)
Comparative Study
OBJECTIVE
Compare outcomes of patients with olfactory neuroblastoma (ONB) without skull base involvement treated with and without resection of the dura and olfactory bulb.
METHODS
Retrospective review of ONB patients treated from 1992 to 2013 at the MD Anderson Cancer Center (The University of Texas, Houston, Texas, U.S.A.). Primary outcomes were overall and disease-free survival.
RESULTS
Thirty-five patients were identified. Most patients had Kadish A/B. tumors (97%), Hyams grade 2 (70%), with unilateral involvement (91%), and arising from the nasal cavity (68%). Tumor involved the mucosa abutting the skull base in 42% of patients. Twenty-five patients (71%) received surgery and radiation, whereas the remainder had surgery alone. Five patients (14%) had bony skull base resection, and eight patients (23%) had resection of bony skull base, dura, and olfactory bulb. Surgical margins were grossly positive in one patient (3%) and microscopically positive in four patients (12%). The 5- and 10-year overall survival were 93% and 81%, respectively. The 5- and 10-year disease-free survival (DFS) were 89% and 78%, respectively. Bony cribriform plate resection was associated with better DFS (P = 0.05), but dura and olfactory bulb resection was not (P = 0.11). There was a trend toward improved DFS in patients with negative resection margins (P = 0.19). Surgical modality (open vs. endoscopic) and postoperative radiotherapy did not impact DFS.
CONCLUSION
Most Kadish A/B ONB tumors have low Hyams grade, unilateral involvement, and favorable survival outcomes. Resection of the dura and olfactory bulb is not oncologically advantageous in patients without skull base involvement who are surgically treated with negative resection margins and cribriform resection.
LEVEL OF EVIDENCE
4. Laryngoscope, 128:1274-1280, 2018.
Topics: Adolescent; Adult; Aged; Dura Mater; Esthesioneuroblastoma, Olfactory; Female; Humans; Male; Middle Aged; Nasal Cavity; Nose Neoplasms; Olfactory Bulb; Retrospective Studies; Skull Base; Survival Analysis; Young Adult
PubMed: 29226334
DOI: 10.1002/lary.26908 -
JAMA Otolaryngology-- Head & Neck... Sep 2023
Topics: Humans; Esthesioneuroblastoma, Olfactory; Nasal Cavity; Nose Neoplasms
PubMed: 37535387
DOI: 10.1001/jamaoto.2023.1943