Olfactory neuroblastoma: A rare sinonasal malignancy.

Olfactory neuroblastoma is a rare malignant tumour arising from the olfactory nerve and extending into the nasal cavity. In this case report, the case of a 42-year-old male is presented. The patient had a two-month history of progressive nasal blockage and episodes of epistaxis. No complaint of anosmia or facial pain was reported. All the necessary examinations were performed. Upon investigation, the CT scan and MRI showed a polypoid mass involving the right maxillary sinus, eroding the medial wall and expanding into the osteo-meatal complex. The diagnosis of olfactory neuroblastoma was confirmed through histopathological examination and further validated by immunohistochemistry as it was positive for synaptophysin, chromogranin, gamma enolase, and neurofilament. On staging, the tumour was Kadish B. The mass was excised by lateral rhinotomy. The patient was kept on radiotherapy and was free from recurrence upon follow-up 10 months later. It was concluded that based on the analysis of findings related to olfactory neuroblastomas, clinicians should contemplate the possibility of an ONB when radiographic images depict a dumbbell-shaped mass within the nasal cavity, accompanied by peritumoural cysts. Using a multimodal treatment approach is advisable.

Endoscopy-assisted medial canthus incision for olfactory neuroblastoma: a case report.

Sinonasal malignant tumors are a group of uncommon malignancies that account for less than 1% of all tumors. These tumors often involve the maxillary sinus and nasal cavity, with less cumulative incidence in the ethmoidal sinus, sphenoidal sinus, and frontal sinus. The lack of consensus on the management of sinonasal malignancies is due to their rarity, diagnostic challenges, and the heterogeneity of treatments. In this paper, we present a case of endoscopic-assisted medial canthus incision combined with radiotherapy in the treatment of sinonasal malignant tumors, with the aim of providing valuable insights to clinicians on the management of these tumors.

Operative Technique and Complication Management in a Case of Giant Esthesioneuroblastoma Resected by a Combined Transcranial and Endonasal Endoscopic Approach: Technical Case Report.

BACKGROUND AND IMPORTANCE: Esthesioneuroblastoma (ENB) is a rare anterior skull base tumor derived from olfactory epithelium. There are very few operative videos in the literature demonstrating the surgical resection techniques for giant ENB because of their rarity and complexity. In this technical report, we demonstrate the microsurgical resection of a very large and complex high-grade ENB, initially deemed unresectable, through a bifrontal craniotomy and extended subfrontal approach combined with an endonasal endoscopic approach. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, nasal congestion, and bloody nasal drainage. MRI showed a large nasal cavity mass with extension into the anterior cranial fossa and bifrontal lobes. There was significant bifrontal edema causing headaches and abutting the optic nerves. Initial management with surgical resection was offered to the patient for local tumor control and to preserve her vision. A combined bifrontal craniotomy and endonasal transsphenoidal approach was used for resecting this giant tumor. After achieving gross total resection, we reconstructed the anterior skull base in layers. She developed several postoperative complications which were appropriately managed. CONCLUSION: We demonstrate the surgical resection of a giant ENB through a combined transcranial and endonasal endoscopic approach. We discuss the several postoperative complications in this complex case and the lessons learned.

Radiographic predictors of occult intracranial involvement in olfactory neuroblastoma patients.

BACKGROUND: Traditional management of olfactory neuroblastoma (ONB) includes margin-negative resection with removal of cribriform plate, dura, and olfactory bulb, regardless of intracranial disease. This approach may be overtreating certain patients. Our investigation examines risk factors associated with occult intracranial disease to optimize therapeutic outcomes. METHODS: This retrospective, multi-institutional cohort study examined clinical covariates associated with occult intracranial involvement. Patient demographics, staging, Hyam's grade, and pathologic involvement of dura, olfactory bulb/tract, and brain were collected. Diagnostic imaging was reviewed. Positive and negative predictive value (NPV) were estimated along with effect size estimates. Cox hazard regression examined associations with overall survival (OS) and disease-free survival (DFS). RESULTS: A total of 224 subjects with new diagnoses of ONB (2005-2021) were identified. Skull base bone involvement on computed tomography (CT) had the highest NPV for pathologic dura (88.0%), olfactory bulb (88%), and brain involvement (97.3%). Hyam's grade category was significantly associated with dural involvement (φC  = 0.26; 95% confidence interval [CI]: 0.16, 0.42). Subjects without radiologic skull base involvement (n = 66) had pathologic positivity of 12.1%. Within this subgroup, Hyam's grade was clinically significant for dural positivity (φ = 0.34; 95% CI: -0.12, 0.71) with 28.6% involvement in high grade tumors. Neither clinical nor pathologic positivity of intracranial structures were associated with significantly different OS or DFS. CONCLUSIONS: Both CT and magnetic resonance imaging (MRI) had reasonably good NPV for involvement of dura and olfactory bulb. Higher Hyam's grade was associated with dural involvement. Patients with low-grade tumors not involving the skull base may be suitable for avoiding skull base resection; however, further investigation is warranted.

Magnetic resonance imaging findings in patients with idiopathic olfactory dysfunction and normal findings on nasoendoscopy.

OBJECTIVE: In presentations of anosmia or dysosmia, magnetic resonance imaging may be required to screen for intracranial pathology such as olfactory neuroblastomas and other intracranial masses impacting on the olfactory pathway. This study aimed to establish positive magnetic resonance imaging findings of anosmia or dysosmia for scans performed before the coronavirus disease 2019 pandemic. METHODS: The study examined the outcome of patients who presented with isolated olfactory dysfunction and who underwent magnetic resonance imaging between 2015 and 2019. RESULTS: Of the 131 patients, 41 (31.3 per cent) had normal scan findings, 50 (38.2 per cent) had insignificant paranasal mucosal disease and 6 (4.6 per cent) had mucosal thickening significant enough to require additional intervention. These interventions included repeat nasoendoscopy or commencement of intranasal or oral steroids. No patients had olfactory neuroblastoma. CONCLUSION: Only 4.6 per cent of the magnetic resonance imaging scans revealed abnormal findings related to anosmia or dysosmia, and none required ENT surgical intervention. None of the magnetic resonance imaging scans identified an olfactory neuroblastoma or intracranial masses impacting on the olfactory pathway.

The role of ADC value and Ki-67 index in predicting the response to neoadjuvant chemotherapy in advanced stages of olfactory neuroblastoma.

OBJECTIVES: To investigate the efficacy of pretreatment ADC and Ki-67 index in the prediction of the response to neoadjuvant chemotherapy (NACT) in advanced olfactory neuroblastoma (ONB) patients. METHODS: A total of 21 advanced ONB patients (mean 43.48 years ± 14.26; range 25-69 years; 13 men and 8 women) with diffusion-weighted imaging (DWI) before NACT between June 2015 and October 2021 were retrospectively analyzed. Patients were categorized into responders and non-responders according to RECIST 1.1 after two cycles of NACT. The clinical data, ADCmean value, and Ki-67 index were analyzed. RESULTS: Kadish stage, ADCmean value, and Ki-67 index showed statistical significance between responders and non-responders. Patients with Kadish C stage were more likely to respond to platinum-based NACT (p = 0.035). Patients with the lower ADCmean value showed response to NACT (p = 0.002) and the cutoff point was 1.04 × 10-3 mm2/s. Patients with the higher Ki-67 index showed response to NACT (p = 0.003) and the cutoff point was 17.5%. Predictive performance of Ki-67 index and ADCmean value showed no significance between responders and non-responders (p = 0.865). A significant negative correlation was found between ADCmean value and Ki-67 index (r = -0.539, p = 0.038). CONCLUSIONS: The pretreatment ADCmean value, Ki-67 index and Kadish stage have the potential to predict the response to NACT in advanced ONB patients. ADVANCES IN KNOWLEDGE: This is the first study that investigated the feasibility of DWI in predicting the response to NACT in ONB patients and showed that Kadish stage, pretreatment ADCmean and Ki-67 index may play an important role in the prediction.

Nasoethmoidal Schwannoma as a Mimicar of Esthesioneuroblastoma: A Case Report and Literature Review.

Nasoethmoidal schwannomas are rare lesions and their presentation with intracranial extension is even rarer. Here, a patient presenting with rhinorrhea, epistaxis, and proptosis of left eye was diagnosed with giant nasoethmoidal schwannoma extending to frontal lobe and orbit, which was managed with bifrontal craniotomy with endoscopic transnasal gross total excision. This being predominantly a benign lesion has good prognosis if total excision is achieved.

Cystic Intracranial Recurrence of Olfactory Neuroblastoma without Accumulation on Fluorine-18-fluorodeoxyglucose Positron Emission Tomography.

A 66-year-old man underwent multimodal treatment for olfactory neuroblastoma (ONB). When he was 72 years old, a cystic intracranial lesion without accumulation on fluorine-18-fluorodeoxyglucose positron emission tomography was detected. Surgical resection was performed when the patient was 73 years old. The pathological examination revealed recurrence of ONB, and the patient underwent focal irradiation. At age 81, he presented with a second recurrence in the right occipital lobe with radiological and pathological findings similar to the prior recurrence. This case suggests that pathological confirmation should be considered in cases with atypical radiological findings following the treatment of ONB.

Ectopic Primary Olfactory Neuroblastoma: Case Series and Literature Review.

OBJECTIVE: Olfactory neuroblastoma (ONB) is a rare malignant tumor arising in the upper nasal cleft. Rarely, ONB may originate in ectopic sites and the impact of this on prognosis and treatment strategies continues to be debated. METHODS: A retrospective analysis was undertaken of patients with ectopic ONB treated between 2000 and 2020 in a tertiary-care referral center for skull base tumors. Three patients were included in this analysis: a 37-year-old woman with ONB arising from the bulla ethmoidalis; a 28-year-old man with inappropriate secretion of antidiuretic hormone caused by a maxillary sinus ONB; and a 41-year-old man with lacrimal sac ONB. Preoperative workup, surgical approach, adjuvant treatments and postoperative surveillance were analyzed. Relevant literature published between 2000 and January 2021 was fully reviewed to investigate oncologic outcomes and delineate the standard of care for such rare tumors. RESULTS: All patients were treated via endoscopic endonasal resection with radical intent, followed by adjuvant treatments when required. No recurrences of disease were observed after a mean follow-up time of 32 months (range, 12-60 months). Data emerging from the literature suggest that a multidisciplinary treatment approach, including free-margins surgical resection followed by adjuvant radiotherapy or radiochemotherapy, is recommended. Olfactory bulb and dura preservation should be attempted whenever feasible. CONCLUSIONS: Endoscopic endonasal surgery should be preferred, when possible, to achieve complete excision to minimize patients' morbidity. The ectopic site of origin affects prognosis and should be considered when selecting the appropriate multimodal treatment strategy.

Outcomes and Quality-of-Life Measures after Endoscopic Endonasal Resection of Kadish Stage C Olfactory Neuroblastomas.

OBJECTIVE: The endoscopic endonasal approach (EEA) has been applied in the treatment of olfactory neuroblastoma (ONB). However, there is a lack of research examining the impact of EEA on locally advanced ONB. This study assessed the outcomes of EEA in patients with locally advanced ONB and its impact on the quality of life (QOL). METHODS: We retrospectively reviewed patients with Kadish stage C ONB who underwent EEA between December 2004 and October 2019 and assessed demographic data, histopathologic grade, the extent of resection, postoperative complications, and outcomes. Preoperative and postoperative QOL was assessed using the Sino-Nasal Outcome Test. RESULTS: Twenty-six patients (18 men, 8 women; aged 26-79 years) were enrolled, with 12 cases of Hyams grade II and III and 1 case of grade I and IV each. In total, 25 patients received radiotherapy and 16 patients received chemotherapy, of whom 11 received preoperative neoadjuvant chemotherapy. Postoperative nasal bleeding was observed in 2 patients. The follow-up ranged from 8 to 124 months (median, 42.3 months). The 1-year and 5-year overall survival were 96.2% and 84.8%, respectively. The 1-year and 5-year disease-free survival were 76.9% each. The analysis of the postoperative Sino-Nasal Outcome Test scores showed significant improvement in certain psychological and sleep-associated domains, compared with the preoperative scores. CONCLUSIONS: Our results showed that pure EEA followed by radiotherapy offered excellent outcomes in the management of selected patients with locally advanced ONB. The postoperative QOL was significantly improved. More research is required on neoadjuvant chemotherapy to establish its role.

Comparison of a Hybrid IMRT/VMAT technique with non-coplanar VMAT and non-coplanar IMRT for unresectable olfactory neuroblastoma using the RayStation treatment planning system-EUD, NTCP and planning study.

The purpose of this study was to compare hybrid intensity-modulated radiotherapy (IMRT) and volumetric-modulated arc therapy (Hybrid IMRT/VMAT), with non-coplanar (nc) IMRT and nc-VMAT treatment plans for unresectable olfactory neuroblastoma (ONB). Hybrid IMRT/VMAT, nc-IMRT and nc-VMAT plans were optimized for 12 patients with modified Kadish C stage ONB. Dose prescription was 65 Gy in 26 fractions. Dose-volume histogram parameters, conformation number (CN), homogeneity index (HI), integral dose and monitor units (MUs) delivered per fraction were assessed. Equivalent uniform dose (EUD) and normal tissue complication probability (NTCP) based on the EUD model (NTCPLogit) and the Lyman-Kutcher-Burman model (NTCPLKB) were also evaluated. We found that the Hybrid IMRT/VMAT plan significantly improved the CN for clinical target volume (CTV) and planning treatment volume (PTV) compared with the nc-VMAT plan. In general, sparing of organs at risk (OARs) is similar with the three techniques, although the Hybrid IMRT/VMAT plan resulted in a significantly reduced Dmax to contralateral (C/L) optic nerve compared with the nc-IMRT plan. The Hybrid IMRT/VMAT plan significantly reduce EUD to the ipsilateral (I/L) and C/L optic nerve in comparison with the nc-IMRT plan and nc-VMAT plan, but the difference in NTCP between the three technique was <1%. We concluded that the Hybrid IMRT/VMAT technique can offer improvement in terms of target conformity and EUD for optic nerves, while achieving equal or better OAR sparing compared with nc-IMRT and nc-VMAT, and can be a viable radiation technique for treating unresectable ONB. However, the clinical benefit of these small differences in dosimetric data, EUD and NTCP of optic nerves may be minimal.

Utility of CT texture analysis to differentiate olfactory neuroblastoma from sinonasal squamous cell carcinoma.

The purpose of this study was to examine differences in texture features between olfactory neuroblastoma (ONB) and sinonasal squamous cell carcinoma (SCC) on contrast-enhanced CT (CECT) images, and to evaluate the predictive accuracy of texture analysis compared to radiologists' interpretations. Forty-three patients with pathologically-diagnosed primary nasal and paranasal tumor (17 ONB and 26 SCC) were included. We extracted 42 texture features from tumor regions on CECT images obtained before treatment. In univariate analysis, each texture features were compared, with adjustment for multiple comparisons. In multivariate analysis, the elastic net was used to select useful texture features and to construct a texture-based prediction model with leave-one-out cross-validation. The prediction accuracy was compared with two radiologists' visual interpretations. In univariate analysis, significant differences were observed for 28 of 42 texture features between ONB and SCC, with areas under the receiver operating characteristic curve between 0.68 and 0.91 (median: 0.80). In multivariate analysis, the elastic net model selected 18 texture features that contributed to differentiation. It tended to show slightly higher predictive accuracy than radiologists' interpretations (86% and 74%, respectively; P = 0.096). In conclusion, several texture features contributed to differentiation of ONB from SCC, and the texture-based prediction model was considered useful.

Combined Simultaneous Multiportal Approach via Minimally Invasive Transciliary and Endoscopic Endonasal Approaches for Tumors Invading Both the Skull Base and the Sinonasal Area.

BACKGROUND: A combined transcranial and transfacial approach has long been the gold standard for surgical management of large tumors with sinonasal and skull base involvement. The extended endoscopic endonasal approach for such pathologies has its advantages, but it has flaws as well, such as anatomic limitations and more ponderous skull base reconstruction and thus higher risk of postoperative complications. Our primary technique for surgical treatment of these pathologies has been a combination of transfacial and minimally invasive transciliary supraorbital keyhole approaches. With the aim to further minimize invasiveness, potential complications, and unsatisfactory aesthetic outcomes during surgical treatment of large tumors invading both the sinonasal area and the skull base, we abandoned the transfacial approach and simultaneously combined the transciliary supraorbital keyhole approach with the endoscopic endonasal approach. METHODS: The well-known microscope-assisted minimally invasive approach via a transciliary supraorbital keyhole craniotomy was combined with the endoscopic endonasal approach. RESULTS: Six patients with different histologic types of tumors affecting the sinonasal area and the skull base were operated on. The mean operative time was 3 hours, there were no unexpected intraoperative or postoperative complications, and total tumor removal was achieved in each patient. None of the patients experienced complications associated with the surgery during follow-up. CONCLUSIONS: Our combined simultaneous multiportal approach enables total tumor eradication with reduced operative time and is associated with minimal intraoperative and postoperative complications, low mortality rate, and excellent cosmetic results.

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

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.

Occult olfactory neuroblastoma presenting with multiple bone metastases: a case report.

RATIONABLE: Olfactory neuroblastoma (ONB) is a rare malignant tumor of the nasal cavity, the primary local symptoms are usually inconspicuous. Patients are often admitted to various specialties based on different primary symptoms, which may result in delayed diagnosis and even a misdiagnosis. PATIENT CONCERNS: Here we report a case of ONB that presented initially as multiple ostealgia without any local symptoms of the tumor and primarily misdiagnosed as multiple myeloma. The patient was a 47-year-old female with bone pain at multiple sites. The initial diagnosis was considered as multiple myeloma. However, the morphologic examination of bone marrow suggested that the tumor cells originated from the nervous tissues. After the positron emission computed tomography scan, the primary lesion in the nasal cavity was located, and a biopsy was performed. DIAGNOSIS: The final diagnosis of ONB was confirmed by histopathological tests. INTERVENTIONS: The patient was treated with metronomic chemotherapy. OUTCOMES: The symptoms of bone pain were significantly relieved 3 months later. The emission computed tomography scan of the whole body bones and the magnetic resonance imaging of the head showed that the tumor size did not change significantly and proved a progression-free of the disease. LESSONS: It is a reasonable strategy to identify the original latent tumor by a prompt positron emission computed tomography scan when the primary diagnosis indicates a metastatic disease, especially for the occult malignancies like ONB.

Case report expanding the germline AXIN2- related phenotype to include olfactory neuroblastoma and gastric adenoma.

BACKGROUND: Pathogenic AXIN2 variants cause absence of permanent teeth (hypodontia), sparse hair and eye brows (ectodermal dysplasia), and gastrointestinal polyps and cancer. Inheritance is autosomal dominant with variable penetrance. Only twenty- five patients have been reported from five families. A Mayo Clinic pilot program tested 3009 newly diagnosed cancer patients for pathogenic germline variants in 83 hereditary cancer genes, including AXIN2. We found only one patient with a pathogenic AXIN2 variant. CASE PRESENTATION: The proband was a 49 year-old female who came to Otolaryngology clinic complaining of right-sided nasal obstruction. Biopsy of identified nasal polyp revealed olfactory neuroblastoma (esthesioneuroblastoma). Surgical resection with gross, total tumor resection was followed by radiation therapy. The patient enrolled in a clinical pilot of genetic testing and a pathogenic variant in AXIN2, c.1822del (p.Leu608Phefs*81) (NM_004655.3) was found. She was seen in Medical Genetics clinic and found to have a personal history of hypodontia. Her eyebrows, hair, and nails were all normal. She underwent upper endoscopy and colonoscopy. A four mm gastric adenoma was found and removed. CONCLUSIONS: This is the first case reported on a patient with a pathogenic, germline AXIN2 variant and an olfactory neuroblastoma or a gastric adenoma. We propose that these could be features of the AXIN2 phenotype. The known association between gastric adenomas and familial adenomatous polyposis, the other Wnt/beta-catenin disorder, supports the hypothesis that pathogenic AXIN2 variants increase risk as well. As the odds of a chance co-occurrence of a pathogenic AXIN2 variant and an olfactory neuroblastoma are so rare, it is worth exploring potential causation. We are building a clinical registry to expand understanding of the AXIN2 phenotype and request any clinicians caring for patients with pathogenic AXIN2 variants to contact us.

Olfactory Neuroblastoma.

Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance imaging are mandatory for staging (in association with 18F-fluorodeoxyglucose positron emission tomography) in high-grade and/or high-stage tumors. Biopsy must be representative to confirm a diagnosis and for grading purposes. Two complementary classifications are described: one (Kadish) based on clinical-radiological analysis, and the other (Hyams) on histological criteria. Based on Hyams grading, studies have pointed out that grades III-IV entail significantly different behavior and prognosis. A multimodal approach, which may combine surgery, chemotherapy, and radiotherapy, is essential to manage these tumors. Treatment schedules which include surgery seem to be superior to others. Surgery classically consisted of anterior craniofacial resection to obtain good exposure. However, the role of transnasal endoscopic surgery has expanded because of its association with fewer complications, shorter hospital stays, and comparable oncologic results to the open surgical techniques. Unilateral endoscopic craniectomy can be performed for limited lesions to avoid definitive anosmia. Treatment that includes radio- and chemotherapy is recommended for advanced and high-grade tumors. The role of neoadjuvant chemotherapy in advanced-stage lesions is emerging. The main prognostic factors associated with poor patient outcome are Hyams grade III-IV, Kadish C-D, and positive surgical margins. Lifelong follow up is recommended.

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

ANTECEDENTES: El neuroblastoma olfatorio es una neoplasia maligna que se origina en la bóveda nasal superior. OBJETIVO: Presentar una serie de casos y un análisis clínico retrospectivo. MÉTODO: Pacientes con neuroblastoma olfatorio tratados en el Instituto Nacional de Cancerología, de México, entre 2011 y 2018. RESULTADOS: La proporción de Kadish en las etapas B, C y D fue del 16, el 58 y el 25%, respectivamente. Los grados 1, 2 y 3 de Hyams fueron el 25, el 50 y el 25%, respectivamente. El abordaje quirúrgico más frecuente fue el craneofacial, en cinco casos (42%), seguido del transfacial en cuatro (33%) y del abordaje endoscópico endonasal en tres (25%). La resección total macroscópica se logró en ocho pacientes (67%). Cinco pacientes (42%) se sometieron a una segunda operación debido a enfermedad recurrente o progresiva. La tasa de complicaciones quirúrgicas fue del 8,3%. La sobrevida libre de progresión fue de 41 meses y la supervivencia media global fue de 63,6 meses. CONCLUSIONES: La resección quirúrgica seguida de radioterapia y quimioterapia para la enfermedad metastásica y recurrente proporciona el mejor resultado en términos de supervivencia y recurrencia. Hasta donde sabemos, esta es la primera serie de casos reportados en México.

68Ga-DOTATATE and 18F-FDG PET/CT for the Management of Esthesioneuroblastoma of the Sphenoclival Region.

We present a 48-year-old woman with an olfactory neuroblastoma who was referred for accurate staging using PET/CT. The Ga-DOTATATE PET/CT showed a 51 × 32-mm mass with an SUVmax of 7.59 in the sphenoidal sinuses, whereas radiotracer uptake on F-FDG PET/CT was similar to that of brain tissue. Ga-DOTATATE PET/CT might be especially useful in regions with difficult tumor visualization resulting from high background, such as brain tissue. The results of this case may suggest that somatostatin receptor imaging in patients with esthesioneuroblastoma may facilitate the potential application of radiotheranostic agents for the treatment of this aggressive subtype of tumors.