Materiały źródłowe

• #1 Esthesioneuroblastoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK539694/

  Esthesioneuroblastoma accounts for 2% to 6% of nasal cavity and paranasal sinus cancer cases and 0.3% of all upper aerodigestive tract malignancies. The incidence of esthesioneuroblastoma seems to have increased in the last decade. Esthesioneuroblastoma may occur at any age, although it occurs predominantly in young adults. The mean age at presentation is 40 to 70 years. There is a second peak of incidence in later adult life. Men and women are affected equally. […] Esthesioneuroblastoma is a rare disease that requires multi-institutional and international collaboration. An interprofessional team approach provides the best outcomes. Centralized processing of esthesioneuroblastoma cases would offer the most favorable ground to accrue more patients and use a uniform database in collecting information and reporting outcomes. Due to the aggressive nature of esthesioneuroblastoma, prompt diagnosis is critical in determining appropriate treatment options. The primary care provider and nurse practitioner should promptly refer any patient with a suspected nasal mass to the otolaryngologist for further workup. Management of esthesioneuroblastoma is complex and needs an interprofessional approach involving a team that consists of an otolaryngologist, a neurosurgeon, a radiation oncologist, a pathologist, a radiologist, and an oncologist, with support from nursing and pharmacy, where indicated, as treatment moves forward.

• #2 Esthesioneuroblastoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/278047-overview

  Epidemiology […] Interestingly, 80% of the esthesioneuroblastoma (ENB) cases published in the literature since Berger and Luc described the first case in 1924 have been identified in the last few decades. However, the current data set cannot distinguish between a rising incidence and better recognition of the disease. […] ENB has an estimated incidence of 4 cases per 10 million individuals and accounts for approximately 5% of all sinonasal tumors. A search of the National Cancer Database by Carey et al identified 1225 cases of ENB. Similar incidence rates have been obtained through epidemiologic studies performed in Denmark. No studies suggest a geographic variation in rates. […] ENB does not show a predilection toward any individual race. ENB does not show familial prevalence and has been reported in all races and on all continents. ENB affects males and females with similar frequency. […] ENB occurs in a wide range of age groups (3-90 y). It has a bimodal peak of occurrence in the third and sixth decades of life.

• #3 Olfactory neuroblastoma (esthesioneuroblastoma) – UpToDate

  https://www.uptodate.com/contents/5183

  Olfactory neuroblastoma makes up approximately two percent of all sinonasal tumors and has an incidence of 0.4 per million population. […] In patients with olfactory neuroblastomas, the mean age at presentation is 53 years, with most cases occurring in patients between 35 and 70 years of age. […] There is a moderate male predominance with a 59:41 male:female ratio in one series from the Surveillance, Epidemiology, and End Results (SEER) database.

• #4 Esthesioneuroblastoma (Olfactory Neuroblastoma) – MD Searchlight

  https://mdsearchlight.com/cancer/esthesioneuroblastoma-olfactory-neuroblastoma/

  Esthesioneuroblastoma is a rare type of cancer that affects the nasal cavity and paranasal sinuses. It accounts for only a small percentage of all upper aerodigestive tract malignancies, which are cancers that occur in the upper part of the digestive tract and airway. However, the number of new cases has been increasing over the past decade. This disease can develop in people of any age, but its most commonly seen in young adults. Theres also a second peak occurrence in later adulthood. Interestingly, it affects both men and women evenly. […] Esthesioneuroblastoma is a rare form of cancer that takes place in the nasal cavity and paranasal sinuses. It makes up 2% to 6% of all cases of nasal and paranasal sinus cancers, and 0.3% of all upper aerodigestive tract cancers. The incidence of this cancer has been rising in the past decade. Esthesioneuroblastoma can occur at any age, but its commonly seen in young adults. Theres a second increase in incidence in later adulthood. It affects both men and women equally.

• #5 Olfactory neuroblastoma (esthesioneuroblastoma) – UpToDate

  https://www.uptodate.com/contents/olfactory-neuroblastoma-esthesioneuroblastoma

  Olfactory neuroblastoma makes up approximately two percent of all sinonasal tumors and has an incidence of 0.4 per million population. In patients with olfactory neuroblastomas, the mean age at presentation is 53 years, with most cases occurring in patients between 35 and 70 years of age. There is a moderate male predominance with a 59:41 male:female ratio in one series from the Surveillance, Epidemiology, and End Results (SEER) database.

• #6 Olfactory neuroblastoma

  https://www.oaepublish.com/articles/2574-1225.2023.128

  Originating from the olfactory neuroepithelium, olfactory neuroblastoma is a rare malignant tumor of the nasal cavity that typically affects adults between the ages of 35 and 70. […] It accounts for 2% to 6% of nasal cavity and paranasal sinus cancer cases with an incidence rate of 0.4 per million population. […] Most cases occur in individuals aged between 35 and 70 years with a mean age of presentation of 53 years. […] There is a moderate male predominance with a male-to-female ratio of 59 to 41. […] Currently, treatment involves surgery, radiation therapy, and/or chemotherapy followed by long-term surveillance to monitor treatment outcomes and recurrences. […] Given the risk of recurrence, extended post-treatment surveillance remains necessary. […] Overall incidence of nodal metastasis is around 30% of cases, with 5%-8% of patients presenting with nodal metastasis at the time of diagnosis.

• #7 Childhood Esthesioneuroblastoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/head-and-neck/hp/child/esthesioneuroblastoma-treatment-pdq

  Esthesioneuroblastoma (also called olfactory neuroblastoma) is a very rare small round cell tumor arising from the nasal neuroepithelium. Less than 10% of cases occur in children and adolescents. The estimated incidence of esthesioneuroblastoma is 0.1 cases per 100,000 people per year in children younger than 15 years. In the pediatric population, the median age is 10 years, and there are no gender or racial predilections. […] Despite its rarity, esthesioneuroblastoma is the most common cancer of the nasal cavity in pediatric patients, accounting for 28% of cases in a Surveillance, Epidemiology, and End Results (SEER) Program study. […] Reports of metastatic disease (Kadish stage D) vary among studies and is described at rates of 20% to 30%. Reports suggest that positron emission tomography computed tomography (PET-CT) may aid in staging the disease.

• #8 Esthesioneuroblastoma in pediatric and adolescent age. A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft Tissue Sarcoma Committees

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3368746/

  Esthesioneuroblastoma (ENB) is a rare, aggressive tumor with no established treatment in children. […] In pediatric age, the estimated incidence of ENB is 0.1/100,000 children up to 15 years of age, but it is the most common cancer of the nasal cavity, accounting for 28% of a series of 47 cases registered in the Surveillance, Epidemiology and End Results (SEER) database from 1973 to 2002. […] Our findings confirm that ENB in children has an aggressive presentation, but multimodal therapy can cure most patients. […] Our results are encouraging but future strategies must optimize treatment in terms of survival and related morbidities. […] The survival results reported here are higher than those described in previously-published series. This may be due to the systematic use of a multidisciplinary approach in all the patients concerned. […] In conclusion, our findings confirm that ENB has aggressive features in children, but a multimodal approach – relying mainly on chemotherapy and radiotherapy – can cure most patients.

• #9 Esthesioneuroblastoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/esthesioneuroblastoma

  Esthesioneuroblastoma often comes back, so you’ll need annual checkups and tests to be sure the tumor hasn’t returned. […] Esthesioneuroblastoma may grow back (recur), so healthcare providers recommend people have regular checkups to confirm it hasn’t come back. […] It’s rare, affecting about 1 in 2.5 million people worldwide each year. […] Esthesioneuroblastoma is the most common cancer of the nasal cavity in children and adolescents. […] Studies show esthesioneuroblastoma most often spreads to your sinuses, but it can spread to lymph nodes in your neck, your lungs and your bones. […] Healthcare providers estimate survival rates by looking at the experiences of people who have the condition. In this case, 50% to 90% of people with this condition were alive five years after diagnosis.

• #10 Esthesioneuroblastoma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Esthesioneuroblastoma_epidemiology_and_demographics

  The incidence of esthesioneuroblastoma is approximately 0.4 per 100,000 individuals worldwide. Esthesioneuroblastoma is noted in 4 out of 10 million individuals, accounting for 5% of sinonasal and 3% of intracranial tumors. […] According to the BBC, only 201 cases of the disease have been recorded worldwide in the past two decades. A 1997 literature search identified 1,457 cases in the published literature since its discovery in 1924, however 487 were cited in more than one paper, bringing the total of reported cases to 945. […] The majority of cases of esthesioneuroblastoma occur in patients between 40 and 70 years of age; the median age at diagnosis is 53 years. […] There is no racial predilection to esthesioneuroblastoma. […] Males are more commonly affected with esthesioneuroblastoma than females. The male to female ratio is approximately 1.2 to 1.

• #11 Esthesioneuroblastoma | Neupsy Key

  https://neupsykey.com/esthesioneuroblastoma-3/

  Esthesioneuroblastoma represents 3% to 6% of sinonasal malignancies and has an estimated incidence of 0.4 cases per 1 million people. […] No environmental, geographic, or lifestyle risk factors have been clearly associated with an increased risk of esthesioneuroblastoma. […] Current treatment guidelines recommend complete surgical resection and adjuvant radiotherapy based on improved overall survival with this regimen. […] For this reason, lifelong surveillance is recommended. […] Local recurrence has been reported in up to 60% of patients treated with surgery and radiation, with the majority of recurrences being seen within the first 12 to 24 months. […] Because of esthesioneuroblastomas propensity for late yet often treatable recurrent disease, lifelong imaging surveillance is advised.

• #12 Esthesioneuroblastoma

  https://med.uth.edu/orl/texas-sinus-institute/patient-education/esthesioneuroblastoma/

  Esthesioneuroblastoma (ENB) is now thought to represent a few percent of all malignant nasal tumors. ENB occurs over a broad age range and has a bimodal age distribution in the second and fifth decade, with most patients in their fifties and sixties. […] There are no known specific causes or risk factors for developing this tumor. […] In most studies, the mean time from onset of symptoms to diagnosis of ENB ranges between 612 months. For this reason, many patients have advanced stage at the time of diagnosis. A high index of suspicion is therefore critical to achieve an accurate and timely diagnosis. […] Due to the relatively high rate of neck involvement at presentation, a CT neck with intravenous contrast is routinely obtained to evaluate for occult metastasis. Metastatic workup also includes chest and abdomen CT, as well as liver function tests. Alternatively a Positron Emission Tomography (PET) scan could be obtained to evaluate for regional and distant metastases.

• #13 Esthesioneuroblastoma | SpringerLink

  https://link.springer.com/10.1007/978-3-642-23499-6_89

  Esthesioneuroblastoma (ENB) is a relatively rare malignancy accounting for only 6% of nasal cavity and paranasal sinus cancers (McLean et al. 2007). Approximately 1,000 cases have been reported since its initial discovery in 1924 (Gabory et al. 2010). ENB is typically identified at an advanced stage, primarily because of its typical presentation with benign and nonspecific symptoms. In a cohort of 311 patients diagnosed with ENB, approximately 17% of patients presented with a Kadish A stage tumor, 50% presented with Kadish stage B, and 34% with more advanced stage Kadish C or D tumors (Jethanamest et al. 2007). ENB has a bimodal age distribution between 10-20 years and 50-60 years. It also appears to have no predilection based on sex. No identifiable genetic or environmental…

• #14 Esthesioneuroblastoma – Wikipedia

  https://en.wikipedia.org/wiki/Esthesioneuroblastoma

  Esthesioneuroblastoma accounts for 2% of all intranasal tumors with an incidence of 0.4 cases per million people. Fewer than 700 cases have been documented in the United States. Fewer than 400 unique cases have been reported globally. Esthesioneuroblastoma can occur at any time, with peak occurrence reported in the second and sixth decades of life. […] The Kadish classification is used for clinical classification of sinonasal tumors including esthesioneuroblastoma. Subsequent research articles have been published to determine prognosis based on tumor grade. […] Esthesioneuroblastoma is characterized by neurofibrillary stroma and neurosecretary granules that are not seen concurrently by any other pathologies in the region. Histological tests such as keratin, CK5/6, S-100 protein or NSE can be run to further differentiate esthesioneuroblastoma from other tumors.

• #15 Esthesioneuroblastoma: About 12 Cases

  https://www.jscimedcentral.com/jounal-article-info/annals-of-otolaryngology-and-Rhinology/Esthesioneuroblastoma:-About-12-Cases-11845

  A well-conducted clinical examination of the ENT sphere is required to establish an early diagnosis. […] The CT/MRI pair allows the precise assessment of tumour extension. […] Based on the extension, Kadish proposed (in 1976) a 3-stage clinical classification. […] This classification was modified by Morita in 1993. […] The histopathological aspect of esthesioneuroblastoma may be confusing with some small cell naso-sinusal tumors, especially in less differentiated forms. […] Due to the rarity of esthesioneuroblastomas, the main source of assistance in making therapeutic decisions is the analysis of the results obtained by the authors who published their cases. […] There is no standardized management of these tumors. […] The difficulty in establishing a better therapeutic strategy lies mainly in the small number of patients treated in the same institution, and in the dispersion of these cases over time (often over a long period of more than 20 years), resulting in a heterogeneity of the results. […] Despite treatment, esthesioneuroblastoma remains a malignant tumour with a dark prognosis, since the overall survival at 5 years is of the order of 50%, and local or locoregional recurrences are very common.

• #16 Treatment outcomes and prognostic factors of esthesioneuroblastoma— a retrospective study from South India – ecancer

  https://ecancer.org/en/journal/article/1584-treatment-outcomes-and-prognostic-factors-of-esthesioneuroblastoma-a-retrospective-study-from-south-india

  Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare malignant neoplasm arising from the neural crest cells of the olfactory epithelium. The optimum treatment for this rare disease is still unclear. Most of the available literature on this rare head and neck tumour is limited to small retrospective series and single institutional reports. […] We aimed to investigate the clinical profile, treatment outcomes and prognostic factors of patients with ENB treated at a tertiary cancer centre in south India. […] The estimated 4-year OS for modified Kadish A, B, C and D stages was 75.0%, 90.9%, 56.4% and 0%, respectively. Advanced modified Kadish stage, lymph node involvement and orbital invasion are associated with poor outcomes. […] The majority of patients with ENB present in advanced stages. Surgery is the mainstay of treatment. Adjuvant RT may improve local control and survival in advanced cases. Advanced modified Kadish stage, lymph node involvement and orbital invasion have prognostic importance.

• #17

  https://med.uth.edu/orl/2012/09/07/esthesioneuroblastoma/

  Esthesioneuroblastoma (ENB) is also known as Olfactory Neuroblastoma. […] ENB was initially described by Berger and Luc in 1924, and is now thought to represent a few percent of all malignant nasal tumors. […] ENB occurs over a broad age range and has a bimodal age distribution in the second and fifth decade, with most patients in their fifties and sixties. […] There are no known specific causes or risk factors for developing this tumor. […] In most studies, the mean time from onset of symptoms to diagnosis of ENB ranges between 612 months. […] A high index of suspicion is therefore critical to achieve an accurate and timely diagnosis. […] Due to the relatively high rate of neck involvement at presentation, a CT neck with intravenous contrast is routinely obtained to evaluate for occult metastasis.

• #18 [68Ga]-DOTATATE PET/CT and PET/MRI in the diagnosis and management of esthesioneuroblastoma: illustrative cases in: Journal of Neurosurgery: Case Lessons Volume 1 Issue 2 (2021) Journals

  https://thejns.org/caselessons/view/journals/j-neurosurg-case-lessons/1/2/article-CASE2058.xml

  Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare sinonasal neuroectodermal malignancy with a slow onset of symptoms, favorable 5-year survival, and a propensity for delayed locoregional recurrence. Current treatment options include resection, adjuvant radiotherapy, and/or chemotherapy; however, because of its rarity and location, determining the optimal treatment for ENB has been challenging. […] ENB most commonly presents with nasal obstruction, recurrent epistaxis, headache, facial pain, sinusitis, or anosmia with unilateral symptoms occurring more commonly than bilateral symptoms. […] Staging is determined by the criteria described by Kadish et al.: stage A, tumor restricted to the nasal cavity; stage B, tumor involving the nasal cavity and at least one sinus; stage C, tumor extending beyond the paranasal cavities; and a modified stage D, cervical lymph node metastases.

• #19

  https://med.uth.edu/orl/2012/09/07/esthesioneuroblastoma/

  Metastatic workup also includes chest and abdomen CT, as well as liver function tests. […] There is no consensus on the best staging system for ENB. […] The goal of establishing a staging system for malignant tumors is to: 1) correlate disease extent and burden with prognosis; 2) guide treatment regimens. […] Unfortunately, there is no universally accepted staging system for ENB that has provided consistent information on prognosis, including overall survival. […] All these staging systems are based on CT, MR, and/or PET findings.

• #20 Esthesioneuroblastoma – EyeWiki

  https://eyewiki.org/Esthesioneuroblastoma

  Esthesioneuroblastoma (ENB) is a rare malignant tumor of the upper nasal cavity and anterior skull base first described in 1924 by Berger et al. ENB has an incidence of 0.4 per million and accounts for 3% of all sinonasal malignant tumors. […] Because of its rare occurrence, there is debate amongst grading system, prognosis and clinical management. […] Medical follow-up consists of a multidisciplinary team. Surveillance imaging is required as recurrence is common. In addition, there is a lifelong risk of lymph node metastasis so frequent clinical exams are important. […] Esthesioneuroblastoma requires lifelong surveillance for recurrence and metastasis. Most surgeons obtain regular imaging such as CT/MRI and PET scan to help monitor disease progression. […] Because recurrence can occur after 5 or even 10 years, long-term follow-up is mandatory.

• #21 Esthesioneuroblastoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/esthesioneuroblastoma

  That’s why regular checkups are important so your provider can do imaging and other tests that may detect cancer early on. […] You should plan on seeing your care team at least once a year for the rest of your life. Your team will check on your overall health. They may do imaging tests to look for signs of recurring esthesioneuroblastoma.

• #22 :: YMJ :: Yonsei Medical Journal

  https://eymj.org/DOIx.php?id=10.3349/ymj.2020.61.3.257

  Esthesioneuroblastoma as a source of ectopic Cushing’s syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. […] Esthesioneuroblastoma (olfactory neuroblastoma) is a rare sinonasal tumor originating from the olfactory neuroepithelium. […] Ectopic ACTH syndrome comprises 520% of Cushing’s syndrome. […] Esthesioneuroblastoma as a hormone-secreting tissue was first identified in 1967 as a syndrome of inappropriate antidiuretic hormone presentation, and ACTH-secreting esthesioneuroblastoma was first described in 1987. […] Without question, careful surveillance is mandatory in advanced esthesioneuroblastoma. Not only regular MRI checkup, but also 68Ga-DOTA-conjugated PET is useful for surveillance. […] Furthermore, measuring serum cortisol and plasma ACTH directly provides helpful information.

• #23 :: YMJ :: Yonsei Medical Journal

  https://eymj.org/DOIx.php?id=10.3349/ymj.2020.61.3.257

  Kanno, et al. has proposed that measurement of tumor-associated hormones is important for surveillance. […] We emphasize that using 68Ga-DOTA-conjugated PET and monitoring ACTH and cortisol levels comprise a reliable and convenient way through which to check for recurrence in ectopic ACTH-secreting esthesioneuroblastoma.

• #24 Esthesioneuroblastoma | Neupsy Key

  https://neupsykey.com/esthesioneuroblastoma-3/

  Cervical nodal metastases are present in 5% to 10% of patients at presentation and confer a significantly worse prognosis. […] Cervical nodal metastases will ultimately develop in 10% to 44% of patients over the course of their disease. […] Distant metastases have been reported in up to 7% of patients at the time of initial presentation and occur in up to 25% of patients over the course of the disease.

• #25 Patterns of Regional Spread for Esthesioneuroblastoma | American Journal of Neuroradiology

  http://www.ajnr.org/content/32/5/929

  ENB is a rare malignant neoplasm that affects the anterior skull base. […] Disease stage is a significant predictor of survival, in particular the presence or absence of lymph node metastases. […] Multiple studies have identified the cervical lymph nodes as the most frequent site of spread; however, no studies have attempted to characterize the radiographic appearance of metastatic lymph nodes or identify the primary nodal drainage for these tumors. […] Spread of ENB to cervical lymph nodes was discovered in 14/48 patients (29%). […] Of the 14 patients, 5 (36%) were discovered to have lymph node involvement at initial staging, while 9 (64%) showed development of disease in the cervical nodes on surveillance examinations. […] ENB exhibits a predictable pattern of metastasis to cervical lymph nodes, typically spreading first to level II nodes, with frequent involvement of level I and III nodes and RPNs.

• #26 Patterns of Regional Spread for Esthesioneuroblastoma | American Journal of Neuroradiology

  http://www.ajnr.org/content/32/5/929

  Disease stage on diagnosis, in particular the presence or absence of lymphatic spread, has been shown to be a significant predictor of survival. […] Multiple studies have identified the cervical lymph nodes as the most common site of spread, but no studies have attempted to characterize lymphatic spread in the neck. […] The patterns of nodal metastasis are well-understood in many head and neck cancers, but to our knowledge, no previous study has sought to describe the specific route of nodal spread in ENB. […] Our study suggested some predictable patterns of cervical node metastases. […] The involvement of level II nodes is not surprising given their known drainage of the nasal cavity and nasopharynx and frequent involvement in other carcinomas from this region. […] Our study discovered RPN involvement in 43% of patients with nodal disease.

• #27 Esthesioneuroblastoma (Olfactory Neuroblastoma) – MD Searchlight

  https://mdsearchlight.com/cancer/esthesioneuroblastoma-olfactory-neuroblastoma/

  The prognosis for Esthesioneuroblastoma (Olfactory Neuroblastoma) depends on the stage and grade of the cancer. The 5-year survival rate ranges from 75% to 90% for stage A tumors (very early stage) and drops to 45% for stage C tumors (more advanced stage). High-grade tumors have a 25% 5-year survival rate, while low-grade tumors have an 80% survival rate. Factors that could lead to a worse outcome include metastases, high stage or high-grade tumor, age above 50 or below 20 at diagnosis, intracranial spread, high proliferation, being female, or having abnormal chromosomes. […] Esthesioneuroblastoma, a type of cancer, commonly returns within two years of first appearing, affecting about 30% of patients. Additionally, the disease spreads in 35% of cases, frequently targeting the cervical lymph nodes. About 10% of patients experience distant metastasis, in which the cancer travels to distant parts of the body such as the lungs, bone, liver, or skin.

• #28 < ?php wp_title( '|', true, 'right' ); ?>

  https://surgicalneurologyint.com/surgicalint-articles/metastatic-esthesioneuroblastoma-with-widespread-recurrence-to-the-central-nervous-system/

  Esthesioneuroblastomas (ENBs) are rare malignancies of the upper digestive tract, often demonstrating local metastasis to the intracranial space through the cribriform plate. […] Around 5060% of patients experience local recurrence. […] Central nervous system (CNS) recurrence is common, but widespread dissemination involving both the intracranial space and spine without evidence of local recurrence is exceedingly rare. […] Early workup of unusual symptoms following ENB treatment should be pursued to limit advanced presentation of recurrent disease. While local surveillance is often performed, clinicians must be cognizant that ENB has demonstrated the potential for widespread CNS dissemination without evidence of recurrent local disease. […] Here, we report a rare case of ENB initially treated using a combination of resection, chemotherapy, and radiation with recurrent widespread metastasis to the CNS without local recurrence. Clinicians evaluating patients with previously diagnosed ENB should entertain suspicion for metastatic spread to the distal CNS and intracranial space.

• #29 Intracranial Metastasis of Esthesioneuroblastoma May Occur 19 Years After Remission – Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/reports/intra-cranial-metastasis-esthesioneuroblastoma-brain-cancer-treatment-risk/

  Recurrence of esthesioneuroblastoma (ENB) with non-contiguous intracranial metastasis is possible 19 years after remission, according to a poster presented at the Society for Neuro-Oncology 27th Annual Meeting. […] Based on this finding and the poor survival associated with intracranial involvement, researchers recommended that lifetime surveillance should be considered in all patients with ENB. […] ENB is a highly recurrent tumor and harbors the potential to involve the intracranial space even years after remission, the researchers wrote. Intracranial involvement entails poor overall survival. Lifetime radiographic follow-up should be considered in all patients with ENB.

• #30 The Added Value of 18F-FDG PET/CT for Evaluation of Patients with Esthesioneuroblastoma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/53/8/1200

  The purpose of this study was to evaluate the clinical utility of 18F-FDG PET/CT in esthesioneuroblastoma staging and restaging and quantify the additional benefit of PET/CT to conventional imaging. […] The overall incidence of cervical lymph node metastasis is between 20% and 25%, but approximately only 5% of patients present with positive nodal disease, signifying the tendency toward delayed nodal metastasis. […] The tendency toward late metastasis necessitates continued long-term surveillance. […] For these reasons, imaging is critical for medical management of patients with esthesioneuroblastoma. […] In our study, the additional information provided by PET/CT changed the disease stage or altered clinical management in 11 (39%) of 28 esthesioneuroblastoma patients. […] The addition of PET/CT conferred an advantage over MRI alone for several reasons.

• #31 The Added Value of 18F-FDG PET/CT for Evaluation of Patients with Esthesioneuroblastoma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/53/8/1200

  Given these findings, we suggest that the optimal imaging algorithm for the staging and restaging of esthesioneuroblastoma patients consists of combination PET/CT and contrast-enhanced MRI. […] This study demonstrated that PET/CT is a useful adjunct to conventional imaging in the initial staging and restaging of esthesioneuroblastoma.

• #32 [68Ga]-DOTATATE PET/CT and PET/MRI in the diagnosis and management of esthesioneuroblastoma: illustrative cases in: Journal of Neurosurgery: Case Lessons Volume 1 Issue 2 (2021) Journals

  https://thejns.org/caselessons/view/journals/j-neurosurg-case-lessons/1/2/article-CASE2058.xml

  [68Ga]-DOTATATE PET allows whole-body imaging of SSTR expression, revolutionizing the detection and characterization of malignancies that overexpress SSTRs, such as ENB. […] The clinical relevance and potential application of SSTR-targeted imaging for ENB diagnosis, staging, and surveillance was evaluated in 2005 utilizing [111In]-DTPA-D-Pheoctreotide ([111In]-pentetreotide) with histological correlation. […] Recent advances have allowed for the transition from scintigraphy to PET imaging with [68Ga]-labeled SSTR-targeted radiotracers. […] Within our cohort, [68Ga]-DOTATATE PET demonstrated utility in its ability to evaluate treatment response, identify the osseous and soft tissue metastatic burden, and distinguish metastatic from inflammatory adenopathy. […] Case 2 highlights the importance of continued clinical and imaging follow-up because ENB has the potential for an aggressive course, particularly those of higher grade and stage.

• #33 Olfactory neuroblastoma

  https://www.oaepublish.com/articles/2574-1225.2023.128

  Ultimately, long-term surveillance is crucial for all cases of olfactory neuroblastoma due to its tendency for late recurrences which can occur up to 15 years after initial treatment. […] It is recommended that close monitoring should involve ongoing physical and endoscopic imaging examinations as well as imaging evaluations for at least ten years after treatment. […] Nonetheless, long-term surveillance with [68Ga]-DOTATATE PET should be highly considered given its documented superiority in detection of tumors compared to that of PET/CT.

• #34 The value of elective neck irradiation in management of esthesioneuroblastoma: a retrospective study based on propensity score matching | Radiation Oncology | Full Text

  https://ro-journal.biomedcentral.com/articles/10.1186/s13014-024-02539-x

  To our knowledge, this is the first study that utilizes propensity score matching (PSM) and real-world data to analyze the clinical benefit of ENI in lymph node negative ENB patients. […] Our study showed a recurrence rate of 31.5% in the entire cohort, including 31.5% in N0 patients and 38.5% in lymph node positive patients. […] While ENI is a standard treatment for lymph node positive patients, its benefits for N0 patients remain unclear. […] Our results indicated that after PSM, ENI significantly improved LRFS and distant metastasis-free survival (DMFS) in N0 patients. […] The target volume of the ENI was controversial. […] For the locally advanced ENB invades beyond the midline patients of node negative, we recommend prophylactic irradiation to the upper neck on the bilateral side of the lesion, mainly including Ib, VIIa and II according to this real-world retrospective data. […] These findings provide valuable insights for tailoring treatment strategies for ENB patients.

• #35 Olfactory Neuroblastoma/Esthesioneuroblastoma | Skull Base Surgery | Stanford Medicine

  https://med.stanford.edu/skullbasesurgery/conditions-we-treat/Olfactory-Neuroblastoma_Esthesioneuroblastoma.html

  Olfactory neuroblastoma (or esthesioneuroblastoma) accounts for fewer than 5% of sinonasal malignancies. […] There are no known causes or risk factors. […] Spread to other sites is uncommon, but the neck should be evaluated by imaging as about 15% of patients develop involved neck nodes over time. […] Most patients with an esthesioneuroblastoma are cured: the 5-year local control rate is 75%, possibly 90 to 95%. […] Although metastases are uncommon at presentation, ~10 to 15% will develop cervical nodal metastases. […] Long-term follow-up hence is necessary.

• #36 Esthesioneuroblastoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/21373

  Esthesioneuroblastoma accounts for 2% to 6% of nasal cavity and paranasal sinus cancer cases and 0.3% of all upper aerodigestive tract malignancies. The incidence of esthesioneuroblastoma seems to have increased in the last decade. Esthesioneuroblastoma may occur at any age, although it occurs predominantly in young adults. The mean age at presentation is 40 to 70 years. There is a second peak of incidence in later adult life. Men and women are affected equally. […] Patients and their families require education on how to recognize the early signs and symptoms of sinonasal tumors. Patients with signs of nasal obstruction, epistaxis, or anosmia, should seek early intervention. Health education promoting prompt presentation to hospitals, and efforts at early detection of esthesioneuroblastoma are necessary to achieve the cure.

• #37 Endoscopic treatment of esthesioneuroblastoma | Brazilian Journal of Otorhinolaryngology

  https://www.bjorl.org/en-endoscopic-treatment-esthesioneuroblastoma-articulo-S1808869415308065

  Management of this tumor is uncertain because of the paucity of cases and advances in diagnostic and treatment methods. Current approaches include en bloc surgery, single or combined endoscopic procedures, radiotherapy, and chemotherapy. […] The purpose of this study was to report the experience of our institution in the treatment of this malignancy with endoscopic techniques. […] The postoperative morbidity related to endoscopy was minor. There were no significant complications such as bleeding, meningitis, cerebrospinal fluid leak, or altered vision. […] Esthesioneuroblastoma is a potentially curable malignancy with surgery and radiotherapy. Endoscopic techniques result in significant esthetic and functional gains, reduced recovery times, lower costs, and less morbidity and mortality compared to the conventional approach.

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