Case Of Olfactory Neuroblastoma Research Articles

Ectopic Olfactory Neuroblastoma Arising in the Nasopharynx.

Olfactory neuroblastoma (ONB) is an uncommon malignant tumor typically located in the upper nasal cavity. Olfactory neuroblastoma originating in the nasopharynx is extremely rare and tends to be misdiagnosed. The authors describe a rare case of ONB arising ectopically in the nasopharynx. The patient was a 65-year-old woman with recurrent epistaxis and a feeling of fullness in the right ear. After evaluation, endoscopic surgery was performed. The pathological result proved to be ONB. Postoperative magnetic resonance imaging showed that the tumor was completely resected. The patient proceeded to have 66 Gy of postoperative intensity-modulated radiotherapy and was followed for 36 months without tumor recurrence. Olfactory neuroblastoma originating from the nasopharynx is more rare condition compared with ONB located in other areas in the literature. The symptoms of ONB ectopic to the nasopharynx are similar to those of other nasopharyngeal tumors, which were likely to be misdiagnosed. The treatment principle is the same as that of nonectopic ONB, which is surgery combined with radiotherapy. Surgery can be performed using an endoscopic transnasal approach.

Assessing the Histological Malignancy Grade of Olfactory Neuroblastoma Using the Apparent Diffusion Coefficient Histogram Analysis.

Introduction Olfactory neuroblastoma (ONB) is a rare malignant tumor of the upper nasal cavity. The Hyams classification is an important histological grading system for diagnosing recurrence and predicting survival in ONB. This study aimed to evaluate the utility of apparent diffusion coefficient (ADC) histogram analysis in distinguishing between high-grade and low-grade ONB based on the Hyams classification system. Methods This retrospective study included 17 patients (11 males, six females; mean age 54 years, range 29-84) diagnosed with ONB who underwent pretreatment magnetic resonance imaging (MRI) including diffusion-weighted imaging between December 2017 and September 2022. Two board-certified radiologists outlined the regions of interest on ADC maps of the tumors. Mean, minimum, maximum ADC, standard deviation, skewness, kurtosis, and entropy were calculated from the ADC histograms. Patients were divided into low-grade (Hyams I-II) and high-grade (Hyams III-IV) groups based on histopathological evaluation by a board-certified pathologist. ADC histogram parameters were compared between the two groups using Mann-Whitney U tests. Two-sided p-values of < 0.05 were considered statistically significant. Results The study included 10 low-grade (two grade I, eight grade II) and seven high-grade (five grade III, one grade III/IV, one grade IV) ONB cases. Comparison between the low-grade and high-grade groups showed no statistically significant differences in any of the ADC histogram parameters analyzed: mean ADC (median 1.02 vs 0.95; p = 0.591), minimum ADC (0.84 vs 0.78; p = 0.494), maximum ADC (1.06 vs 1.19; p = 0.625), standard deviation (0.09 vs 0.14; p = 0.433), skewness (-0.48 vs -0.75; p = 0.133), kurtosis (2.79 vs 3.12; p = 0.161), and entropy (4.69 vs 5.06; p = 0.315). Conclusion This study demonstrated that ADC histogram analysis was unable to differentiate between high-grade and low-grade ONB based on the Hyams classification. The findings suggest that preoperative grading of ONB malignancy using ADC histogram parameters is challenging. Thus, grading based on preoperative imaging evaluation is difficult.

Analysis of imaging features of rare tumors in nasal cavity and paranasal sinus

Objective:To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods:The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results:Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion:Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry.

Recurrence morbidity of olfactory neuroblastoma.

With modern treatment paradigms, olfactory neuroblastoma (ONB) has favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this study were to delineate the prognosis of recurrence of ONB and explore how recurrence subsites are associated with OS, disease-specific survival (DSS), and further recurrence. A retrospective chart review of ONB cases from nine academic centers between 2005 and 2021 was completed. Tumor characteristics, recurrence subsites, timelines to recurrence, additional recurrences, and survival estimates were determined using descriptive and time-to-event analyses. A final cohort of 233 patients was identified, with 70 (30.0%) patients recurring within 50.4 (standard deviation±40.9) months of diagnosis on average, consisting of local (50%), neck (36%), intracranial (9%), and distant (6%) recurrence. Compared with subjects without recurrence, patients with recurrence had significantly different primary American Joint Committee on Cancer T stage (p<0.001), overall stage (p<0.001), and modified Kadish scores (p<0.001). Histopathology identified that dural involvement and positive margins were significantly greater in recurrent cases. First recurrence was significantly associated with worse 5-year DSS (hazard ratio=5.62; p=0.003), and subjects with neck or local recurrence had a significantly better DSS compared to intracranial or distant recurrence. Recurrent cases of ONB have significantly different stages and preoperative imaging factors. Patients with local or neck recurrence, however, have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyams grade. Identifying specific factors that confer an increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning.

The role of insulinoma-associated protein 1 in predicting the progression and prognosis of human olfactory neuroblastoma in China

ObjectiveRecent studies have suggested that insulinoma-associated protein 1 (INSM1) is a useful marker for pathological diagnosis of neuroendocrine tumors. In the present study, we investigated the association between INSM1 expression and prognosis in patients with olfactory neuroblastoma (ONB) and assessed the usefulness of INSM1 as a prognostic biomarker in these patients. MethodImmunohistochemistry was performed on 109 ONB patients who underwent endoscopic surgery at Beijing Tong Ren Hospital (Beijing, China) between June 2006 and November 2021 Patient age at the time of surgery ranged from 10 months to 72 years (mean age, 43.55 ± 13.47 years). In total, 63 (57.8%) and 46 (42.2%) tumors occurred in male and female patients, respectively. The percentages of grade I–IV cases were 13.8% (15/109), 36.7% (40/109), 29.4% (32/109) and 20.2% (22/109), respectively. ResultsThe expression rate (moderately/strongly positive) of INSM1 was significantly higher in high-grade (Ⅲ/Ⅳ; 83%; 45/54) than low-grade (Ⅰ/Ⅱ; 27%; 15/55) ONB cases. High expression levels of INSM1 were significantly positively associated with high pathological stage (p < 0.001), local recurrence, and death. Kaplan‑Meier analysis revealed that patients with high INSM1 expression had significantly shorter disease‑free survival (DFS) and mean survival (75.01 ± 10.71 vs. 158.56 ± 10.32) times, and shorter overall survival (OS). Multivariate Cox regression analysis revealed that INSM1 was an independent prognostic factor for DFS (HR: 4.963, 95%CI [2.11–10.84] p < 0.001) and OS (HR: 4.791, 95%CI [2.117–10.485], p < 0.001) after adjusting for sex, age, and tumor grade. In addition, INSM1 was an independent prognostic factor for DFS in patients treated with surgery (HR: 3.714, 95%CI [1.267–10.889], p = 0.017) and chemotherapy (HR: 5.574, 95%CI [1.584–19.612], p = 0.007). ConclusionINSM1 expression had a positive association with the prognosis of patients with ONB and could serve as a prognostic biomarker in these patients.

Genomic profiling and immune landscape of olfactory neuroblastoma in China.

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of the olfactory mucosa. The paucity of genomic data has prevented the development of individualized ONB treatments. Here, we investigated the genomic and immune landscape of ONB in Chinese patients. Whole exome sequencing (WES) and multiplex immunofluorescence (MIF) analysis were performed on tissue samples from 19 Chinese ONB patients. Patients were divided into low- and high-grade groups. Overall, 929 nonsynonymous alterations were identified in 18 (94.74%) ONB cases. The most prevalent altered cancer-related genes were CTNNB1 (16%) and ZNRF3 (16%). The most mutated oncogenic pathways were the WNT and RAS pathways. The median tumor mutation burden (TMB) was 0.45, ranging from 0 to 3.25. Only one case expressed PD-L1 (> 1%) in the tumor region. The percentage of CD8+ tumor-infiltrating lymphocytes (TILs) in the tumor region ranged from 0.03% to 84.9%, with a median of 1.08%. No significant differences were observed between the low- and high-grade groups for clinicopathological features, mutant genes, mutant pathways, TMB, tumor neoantigen burden (TNB), mutant-allele tumor heterogeneity (MATH), PD-L1 expression levels, or CD8+ TIL percentage. However, the low-grade group showed significantly more CD68+ macrophages in both the tumor and total region than the high-grade group. Notably, CD68+CD163- macrophages accounted for an average of 80.5% of CD68+ macrophages. This study presents data on the genomic and immune landscape of ONB cases in China. CTNNB1 and ZNRF3 were the most prevalent altered cancer-related genes. The results of TMB, PD-L1, and CD8+ Tils suggest that ONB may be insensitive to immunotherapy. M1 macrophages may be positively associated with the prognosis of ONB. In this study, the most prevalent altered cancer-related genes were CTNNB1 (16%) and ZNRF3 (16%). The most mutated oncogenic pathways were the WNT and RAS pathways. The median tumor mutation burden (TMB) was 0.45, ranging from 0 to 3.25. Only one (1/15) case expressed PD-L1 (> 1%) in the tumor region. However, the low-grade group showed significantly more CD68+ macrophages in both the tumor and total region than the high-grade group. The higher level of CD68-related macrophages indicates that M1 macrophages potentially play an important role in ONB development that is possibly associated with prognosis.

Sox2 and βIII-Tubulin as Biomarkers of Drug Resistance in Poorly Differentiated Sinonasal Carcinomas.

Poorly differentiated sinonasal carcinomas (PDCs) are tumors that have a poor prognosis despite advances in classical treatment. Predictive and prognostic markers and new personalized treatments could improve the oncological outcomes of patients. In this study, we analyzed SOX2 and βIII-tubulin as biomarkers that could have prognostic and therapeutic impacts on these tumors. The cohort included 57 cases of PDCs: 36 sinonasal undifferentiated carcinoma (SNUC) cases, 13 olfactory neuroblastoma (ONB) cases, and 8 sinonasal neuroendocrine carcinoma (SNEC) cases. Clinical follow-up data were available for 26 of these cases. Sox2 expression was detected using immunohistochemistry in 6 (75%) SNEC cases, 19 (53%) SNUC cases, and 6 (46%) ONB cases. The absence of Sox2 staining correlated with a higher rate of recurrence (p = 0.015), especially distant recurrence. The majority of cases showed βIII-tubulin expression, with strong positivity in 85%, 75%, and 64% of SNEC, ONB, and SNUC cases, respectively. Tumors with stronger βIII-tubulin expression demonstrated longer disease-free survival than those with no expression or low expression (p = 0.049). Sox2 and βIII-tubulin expression is common in poorly differentiated sinonasal tumors and has prognostic and therapeutic utility.

Treatment and outcome in 12 cases of olfactory neuroblastoma at Mexico´s National Cancer Institute: A retrospective clinical analysis and literature review

Treatment and outcome in 12 cases of olfactory neuroblastoma at Mexico´s National Cancer Institute: A retrospective clinical analysis and literature review

Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature

Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.

Transcription Factor Expression in Sinonasal Neuroendocrine Neoplasms and Olfactory Neuroblastoma (ONB): Hyams’ Grades 1–3 ONBs Expand the Spectrum of SATB2 and GATA3-Positive Neoplasms

Sinonasal neuroendocrine neoplasms (SN-NENs) are rare and mostly include neuroendocrine carcinoma (NEC), whereas neuroendocrine tumor (NET) is exceptional in this site. Olfactory neuroblastoma (ONB) is a malignant neuroectodermal neoplasm arising in the nasal cavity. Albeit crucial for correct patients’ management, the distinction of high grade ONB from NEC is challenging and requires additional diagnostic markers. The transcription factor SATB2 has been recently introduced in routine diagnostics as an immunohistochemical marker of distal intestine differentiation. No specific data are available about SATB2 and GATA3 expression in SN-NENs. GATA3, SATB2, and, for comparison, CDX2 expression were investigated in a series of epithelial and non-epithelial SN-NENs. We collected 26 cases of ONB and 7 cases of epithelial SN-NENs diagnosed and treated in our Institution. ONBs were graded according to Hyams’ system and epithelial NENs were reclassified into 5 NECs, 1 MiNEN, and 1 amphicrine carcinoma. Immunohistochemistry was performed using standard automated protocols. Hyams’ grades 1–3 ONBs stained diffusely and intensely for SATB2, whereas grade 4 ONBs and NECs were globally negative. The non-neuroendocrine component of MiNEN and the amphicrine carcinoma were strongly positive. GATA3 was heterogeneously and unpredictably expressed in Hyams’ grades 1–3 ONBs, whereas grade 4 ONBs and NECs were completely negative. CDX2 was negative in all cases. Our study identifies, for the first time, SATB2 and GATA3 expression as features of Hyams’ grades 1–3 ONBs, expands the spectrum of SATB2 and GATA3-positive neoplasms, and suggests that Hyams’ grade 4 ONBs are not only clinically but also biologically different from low graded ONBs.

Prognostic value of microvessel density and its correlation with clinicopathological features in human olfactory neuroblastoma.

Although angiogenesis plays an important role in tumor growth and invasion, its role in the progression of olfactory neuroblastoma (ONB) has rarely been published. The aim of the present research was to analyze the prognostic role of microvessel density (MVD) in ONB and its association with clinicopathological parameters. 70 ONB cases were assessed for immunohistochemical expression of CD31, CD34, CD105, VEGF, and VEGFR2. The expression of CD105-MVD was negatively associated with histological grade and tumor Kadish stage, and its expression was positively correlated with the expression of VEGF/VEGFR2. Low expression of CD105-MVD and high tumor histological grade were strongly associated with poor survival. Thus, CD105-MVD was demonstrated to be a valuable independent prognostic indicator for ONB. MVD is expected to be useful as an important marker to distinguish tumor histological grade.

Olfactory neuroblastoma: case report and focus on cancerogenesis

Background: Neuroendocrine neoplasms (NENs) of the nasal cavity, paranasal sinuses and nasopharynx account for a wide spectrum of histotypes. They can range from indolent form to highly aggressive tumors. Olfactory neuroblastoma, like most sino-nasal NENs, represents a rare neoplasm that prompts diagnostic pitfalls. From a morphological perspective, olfactory neuroblastoma can mimic many nasal neoplasms but a proper recognition is mandatory for its aggressive behaviour. Case presentation: A case of olfactory neuroblastoma in a 85 years old woman is reported. The neoplasm has been surgical removed from the nasal cavity with a clinical and radiographic diagnosis of nasal polyp. Correct diagnostic definition has needed a complete histological and immunohistochemical characterization of the tumoral tissue in surgical pathology laboratory. Conclusion: The correct diagnosis of olfactory neuroblastoma directs the clinical management that is unique for this neoplasm in comparison to other nasal ones. Bicranial-facial surgery or a trephination procedure represent the most common approach for the treatment of this malignancy. Their success determine disease prognosis.

SSTR2 Expression in Olfactory Neuroblastoma: Clinical and Therapeutic Implications.

Somatostatin receptor 2 (SSTR2) expression has previously been documented in olfactory neuroblastoma (ONB). Here, we fully characterize SSTR2 expression in ONB and correlate staining results with clinicopathologic parameters including Hyams grade.We also assess SSTR2 immunohistochemistry expression in various histologic mimics of ONB to assess its diagnostic functionality. 78 ONBs (51 primary biopsies/excisions and 27 recurrences/metastases) from 58 patients were stained for SSTR2. H-scores based on intensity (0-3 +) and percentage of tumor cells staining were assigned to all cases. 51 histologic mimics were stained and scored in an identical fashion. 77/78 (99%) ONB cases demonstrated SSTR2 staining (mean H-score: 189, range: 0-290). There were no significant differences in staining between primary tumors and recurrences/metastases (mean H-score: 185 vs 198). Primary low-grade ONB had somewhat stronger staining than high-grade tumors (mean H-score: 200 vs 174). SSTR2 expression had no prognostic value when considering disease-free or disease-specific survival. SSTR2 staining is significantly higher in ONB than its histologic mimics (mean H-score: 189 vs 12.9, p < 0.001) suggesting a potential use of the marker in diagnosis of ONB. In conclusion, SSTR2 is consistently expressed in ONB suggesting a role for somatostatin-analog based imaging and therapy in this disease. More generally, SSTR2 may be another marker of neuroendocrine differentiation in ONB.

Olfactory neuroblastoma of the nasal cavity managed with modified draf III and adjunctive radiotherapy: Case report and literature review

Olfactory neuroblastoma (ONB) is a rare neoplasm that grows in epithelial cells of olfactory tissue. It is related to many tumor oncogenes such as MYC. We present and discuss a rare case of ONB on the elderly with typical symptoms but different treatment approaches. It was treated with endoscopy and adjuvant radiotherapy.

A Clinicopathological Study of Olfactory Neuroblastoma

Background: Olfactory neuroblastoma, also known as Esthesioneuroblastoma, is a malignant neoplasm of neuroectodermal origin. In view of its rarity, a clinicopathological study of esthesioneuroblastoma is presented.Aim: Study Olfactory neuroblastoma cases diagnosed in the past ten years in patients attending Otorhinolaryngology outpatient.Patients and Methods: A retrospective study was conducted at Kasturba Medical College, Mangalore from 2009-18. Patients who attended Otorhinolaryngology outpatient and proven histopathologically to have Olfactory neuroblastoma are included in the study with the exclusion criteria being metastasis of Neuroblastoma to Head and Neck. It is a time-bound study and cases during the study period fulfilling the inclusion criteria are included.Results: A total of six cases are taken up for the study. These include one female and five male patients. Epistaxis was the most common presenting complaint. All the patients had nasal mass and local spread was seen most commonly to paranasal sinuses, orbit and anterior cranial fossa. Level two lymph nodes were most commonly involved. Overall they were staged as Kadish C in three, A in two and D in one. Treatments included surgical resection and radiation therapy. Two patients presented with recurrence during follow-up out of which one was local and another nodal recurrence.Conclusion: Although rare, any patient in the sixth decade presenting with epistaxis must be evaluated for Olfactory neuroblastoma. Better results are obtained with the combined modality of treatment. Also, the patients must be followed-up for an extended period for the timely detection of recurrences.

Computed Tomography Image Analysis and Clinical Diagnosis Value of Nasal Olfactory Neuroblastoma

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. 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. 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.

Lobular to Lobule: Metastatic Breast Carcinoma to Olfactory Neuroblastoma.

Tumor-to-tumor metastasis (TTM) is a rare, but well-described phenomenon occurring in patients with multiple synchronous or metachronous primary malignancies. Olfactory neuroblastoma (ONB) is a rare malignant, neuroectodermal sinonasal tract tumor that occurs within the ethmoid sinus involving the cribriform plate. Very few cases of ONB have been documented to metastasize to other primary malignancies, but the reverse scenario is exceptional. During an evaluation for anosmia, a right nasal polyp was identified on imaging and endoscopy in a 66-year-old woman, with a polypectomy performed. Histologic examination showed classical features of a low-grade olfactory neuroblastoma, but within the tumor wereisolated epithelioid cells which were strongly pancytokeratin immunoreactive. Review of the clinical history revealed lobular breast carcinoma treated 10years earlier. Further evaluation with immunohistochemistry showed strong and diffuse nuclear estrogen and progesterone receptor reactivity, along with GATA3. These results confirmed TTM of an invasive lobular breast carcinoma to ONB. By employing a limited immunohistochemistry panel for all small round blue cell tumors that includes pancytokeratin, p40, S100 protein, SOX10, synaptophysin, desmin, CD99, and CD45, one is able to more accurately diagnose the classical tumor types, while also showing potentially unusual tumor features or exceptionally rare events like metastatic lobular breast carcinoma to ONB.

Targeted 595-gene genomic profiling demonstrates low tumor mutational burden in olfactory neuroblastoma.

Olfactory neuroblastoma (ONB) is a rare skull-base malignancy associated with delayed local recurrence. Treatment options in recurrent disease are few and unreliable. We undertook analysis of the ONB exome and immune environment in order to identify potential future immunotherapy treatment options. Retrospective chart review and next-generation targeted 595-gene genomic profiling was performed on a cohort of 14 ONB cases utilizing Tempus proprietary DNA and RNA sequencing technology. Tempus analysis provided a measurement of tumor mutational burden (TMB) and composition of the immune cell infiltrate present in tumor samples. Clinically relevant genomic alterations and associated targeted therapies were identified using cancer.gov and clinicaltrials.gov. TMB was tested by univariate analysis against clinical stage, pathologic grade, recurrence risk, and immune cell infiltration. The mean age for the subjects was 50 years (range, 13 to 76 years) with a male:female ratio of 1:1. TMB for ONB samples ranged from 1.3 to 9.6 mutations/megabase (Mb) with mean of 3.8 mutations/Mb. Univariate analysis showed no association between TMB and tumor stage, pathologic grade, risk of recurrence, or immune cell infiltration. Genomic profile revealed that 6 of 13 tumors had genetic alterations with targeted therapies in clinical trials, whereas 1 tumor demonstrated KRAS Q61R mutation with U.S. Food and Drug Administration (FDA)-approved targeted therapies. TMB is a novel biomarker guiding the classification of neoplasms in the emerging era of immunotherapy. The characterization of ONB as a low-TMB pathology contributes to the overall taxonomy of all cancers and suggests limited utility of immunotherapy treatment.

Treatment and outcome in 12 cases of olfactory neuroblastoma at Mexico´s National Cancer Institute: A retrospective clinical analysis and literature review.

Olfactory neuroblastoma (ONB) is a malignant neoplasm that arises from the upper nasal vault. We present a retrospective case series and clinical analysis of 12 ONB cases. Patients with ONB treated at Mexico´s National Cancer Institute between 2011 and 2018. 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. 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.

Olfactory neuroblastoma

The article describes a classic case of olfactory neuroblastoma in a 33-year-old female patient diagnosed with «nasal cavity&#x0D; neoplasm (polyp?)» who was referred to the National Centre for Neurosurgery JSC from the ENT Department of the City Hospital No. 1. The first symptoms of the tumor occurred during her pregnancy. The aggressive tumor has invaded the anterior&#x0D; cranial fossa and spread into the right half of the ethmoid sinuses and the orbit. The tumor diagnostics was complicated by the&#x0D; prevalence of symptoms of a nasal cavity neoplasm. The “olfactory neuroblastoma” diagnosis was verified by clinical observations, MRI, and pathomorphological studies. Histological examination of the tumor showed a typical structure of olfactory&#x0D; neuroblastoma. IHC examination showed an expression of neuronal differentiation markers. The degree of malignancy was&#x0D; determined by Hyams grading.