Neoadjuvant chemotherapy in the treatment of locally advanced olfactory neuroblastoma in 25 cases / 中华耳鼻咽喉头颈外科杂志

Objective: To evaluate the efficacy of neoadjuvant chemotherapy (NACT) in the treatment of locally advanced olfactory neuroblastoma (ONB), and to explore the factors related to the efficacy of NACT. Methods: A total of 25 patients with ONB who underwent NACT in Beijing TongRen Hospital from April 2017 to July 2022 were retrospectively analyzed. There were 16 males and 9 females, with an average age of 44.9 years (ranged 26-72 years). There were 22 cases of Kadish stage C and 3 cases of stage D. After multiple disciplinary team(MDT) discussion, all patients were treated sequentially with NACT-surgery-radiotherapy. Among them, 17 cases were treated with taxol, cis-platinum and etoposide (TEP), 4 cases with taxol, nedaplatin and ifosfamide (TPI), 3 cases with TP, while 1 case with EP. SPSS 25.0 software was used for statistical analysis, and survival analyses were calculated based on the Kaplan-Meier method. Results: The overall response rate of NACT was 32% (8/25). Subsequently, 21 patients underwent extended endoscopic surgery and 4 patients underwent combined cranial-nasal approach. Three patients with stage D disease underwent cervical lymph node dissection. All patients received postoperative radiotherapy. The mean follow-up time was 44.2 months (ranged 6-67 months). The 5-year overall survival rate was 100.0%, and the 5-year disease-free survival rates was 94.4%. Before NACT, Ki-67 index was 60% (50%, 90%), while Ki-67 index was 20% (3%, 30%) after chemotherapy [M (Q1, Q3)]. The change of Ki-67 before and after NACT was statistically significant (Z=-24.24, P<0.05). The effects of age, gender, history of surgery, Hyams grade, Ki-67 index and chemotherapy regimen to NACT were analyzed. Ki-67 index≥25% and high Hyams grade were related to the efficacy of NACT (all P<0.05). Conclusions: NACT could reduce Ki-67 index in ONBs. High Ki-67 index and Hyams grade are clinical indicators sensitive to the efficacy of NACT. NACT-surgery-radiotherapy is effective for patients with locally advanced ONB.

Hemicraniectomía endonasal por estesioneuroblastoma: informe de un caso / Endonasal hemicraniectomy by esthesioneuroblastoma: report of a case

El estesioneuroblastoma es una neoplasia maligna que se origina del epitelio olfatorio. El tratamiento se establece de acuerdo con su extensión y el grado histológico de atipia y puede incluir cirugía, cirugía más radioterapia o más radioquimioterapia. Se han utilizado diferentes abordajes quirúrgicos que incluyeron incisiones faciales y craneotomía pero, con la mayor experiencia adquirida en cirugía endoscópica de senos paranasales y el trabajo en equipo con el neurocirujano, se han desarrollado técnicas endonasales que posibilitan realizar resecciones oncológicas en pacientes seleccionados, con menos morbilidad, internación breve y sin comprometer el control local de la enfermedad. Describimos el caso clínico de una paciente con un estesioneuroblastoma con invasión intracraneal, que fue tratada con éxito mediante una hemicraniectomía endonasal preservando el bulbo olfatorio contralateral. (AU)

Esthesioneuroblastoma is a malignant neoplasm that originates from the olfactory epithelium. Treatment is established according to its extension and the histological degree of atypia and may include surgery, surgery more radiotherapy or more chemoradiation therapy. Different surgical approaches have been used, including facial incisions and craniotomy, but with the greater experience acquired with endoscopic sinus surgery and teamwork with the neurosurgeon, endonasal techniques have been developed that make it possible to perform oncological resections in selected patients, with less morbidity, brief hospitalization and without compromising local control of the disease. We describe the clinical case of a patient with an esthesioneuroblastoma with intracranial invasion who was successfully treated by endonasal hemicraniectomy preserving the contralateral olfactory bulb. (AU)

Neuroblastoma olfatorio / Olfactory neuroblastoma

El neuroblastoma olfativo, también conocido como estesioneuroblastoma, es un tumor derivado de la capa basal del epitelio olfativo. Se presenta como una masa de tejido blando en la porción superior de la cavidad nasal que involucra las células de aire etmoides anteriores y medias en un lado y se extiende a través de la placa cribiforme en la fosa craneal anterior, es poco frecuente y representa menos del 3 % de las neoplasias intranasales. De acuerdo con la evolución epidemiológica, su distribución por edades es bimodal con un pico en pacientes adultos en la segunda década de la vida y otro en los quinta y sexta, sin existir predilección por género reconocida

Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a tumor derived from the basal layer of the olfactory epithelium. It presents as a soft tissue mass in the upper portion of the nasal cavity that involves the anterior and middle ethmoid air cells on one side and extends through the cribriform plate in the anterior cranial fossa, it is rare and represents less 3% of intranasal neoplasms. According to the epidemiological evolution, its age distribution is bimodal with a peak in adult patients in the second decade of life and another in the fifth and sixth, with no recognized gender predilection

Primary experience and discussion on the treatment of esthesioneuroblastoma / 中华耳鼻咽喉头颈外科杂志

Objective: To summarize the preliminary experience in the treatment of esthesioneuroblastoma (ENB) and to explore the effect of age, chemotherapy, modified Kadish stage and pathological grade on the prognosis of ENB. Methods: The clinical data of 87 ENB patients from the First Affiliated Hospital of Sun Yat-sen University and Sun Yat-sen University Cancer Center between June 2002 and November 2017 were retrospectively analyzed. The modified Kadish stage was used to evaluate the extent of the lesions, and the Hyams grading system was used for pathological grading. The patients were followed up regularly to evaluate the recurrence and metastasis of the tumor. Cox proportional hazard model was used for univariate and multivariate analyses. Prognostic factors with P<0.05 in univariate analysis were included in multivariate analysis. After controlling the confounding factors, the model coefficients were used to calculate the hazard ratio (HR) and 95% confidence interval (CI). Results: The median follow-up time of ENB patients was 29 months, and the 5-year overall survival rate was 39.3%. In univariate analysis, age, chemotherapy, modified Kadish stage and pathology grade were independent predictors of overall survival, while gender, radiotherapy and surgery were not prognostic factors. Multivariate analysis showed that modified Kadish stage and pathology grade were independent predictors of overall survival rate after excluding confounding factors. Conclusions: Age, chemotherapy, modified Kadish stage and pathological grade are taking important role in the overall survival rate of patients with ENB. Modified Kadish stage and pathological grade are independent predictors of overall survival rate.

An olfactory neuroblastoma with intracranial extension treated with chemotherapy and radiotherapy: A case report

Background@#Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare malignant tumor that presents as a locally aggressive disease. It accounts for an incidence of 0.4 per million population affecting men and women equally in all ages. As olfactory neuroblastoma is rare, having an intracranial extension is even more unusual. Only a few cases have been reported in literature, hence, there is no widely accepted standard of care. @*Case Presentation@#This is a case of a 33-year-old female who presented with a 4-month history of nasal congestion which progressed to a rapidly growing nasal mass and bilateral proptosis. She soon became bedridden and exhibited signs of increased intracranial pressure. Imaging revealed a heterogeneously enhancing nasal cavity mass with intracranial extension. Biopsy and immunohistochemistry confirmed the olfactory neuroblastoma. She subsequently underwent chemotherapy and radiotherapy since the tumor was unresectable. In a span of three months, after only three cycles of chemotherapy with cisplatin and etoposide and thirty fractions of radiation therapy, the patient significantly improved from being completely bedridden to an ambulatory individual capable of self-care. We completed eight cycles of chemotherapy and referred to surgical specialists for possible resectability. However, the patient did not consent for surgery and opted to have palliative care.@*Conclusion@#Most cases of olfactory neuroblastoma are treated through combination therapy. The patient significantly improved from the administration of chemotherapy with cisplatin and etoposide plus radiotherapy. This case report shows the significance of chemotherapy with radiotherapy as the treatment of choice for late-stage olfactory neuroblastoma in which surgery is not amenable.

Neuroblastoma olfatorio. todo lo que el radiólogo debe saber / Olfactory Neuroblastoma. everything the Radiologist Should Know

Resumen El neuroblastoma olfatorio (NBO) es un tumor maligno poco frecuente que se origina de las células neuroepiteliales olfativas. Su diagnóstico precoz es difícil debido a la poca especificidad de los síntomas que presentan los pacientes. Las pruebas de imagen juegan un papel importante en su diagnóstico y en la planificación quirúrgica, por lo que es importante que los radiólogos conozcan sus hallazgos y las diferentes clasificaciones que ayudarán a elegir el tratamiento más adecuado para cada tumor.

Abstract Olfactory neuroblastoma (ONB) is a rare malignant tumor that originates from olfactory neuroepithelial cells. Its early diagnosis is difficult due to the low specificity of the symptoms. Imaging tests play an important role in its diagnosis and surgical planning so it is important that radiologists know their findings and the different classifications that will help to choose the most appropriate treatment for each tumor.

Adrenocorticotropic Hormone-Secreting Esthesioneuroblastoma with Ectopic Cushing's Syndrome

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. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. ⁶⁸Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.

Estesioneuroblastoma: reporte de caso y revisión bibliográfica / Esthesioneuroblastoma: case report and review

Resumen El estesioneuroblastoma (ENB), también conocido como neuroblastoma olfatorio, es una neoplasia maligna rara de la bóveda nasal que se cree que surge de las células receptoras neurosensoriales en el epitelio olfatorio. El ENB representa el 3% de todos los tumores nasales. Los tratamientos para ENB incluyen cirugía, radioterapia y/o quimioterapia, aunque a veces, es difícil lograr un tratamiento radical utilizando estas estrategias, ya que la mayoría de los pacientes se diagnostican en una etapa avanzada. Presentamos el caso de un varón de 46 años a quien se le diagnosticó ENB en el año 2016. Fue tratado inicialmente con resección endoscópica nasal, seguido de una craneotomía bifrontal. Posteriormente, recibió radioterapia adyuvante. Actualmente, su seguimiento a 3 años no muestra evidencia de metástasis locales, regionales o distantes, ni recurrencia.

Abstract Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant neoplasm of the nasal vault that is believed to arise from neurosensory receptor cells in the olfactory epithelium. ENB accounts for 3% of all nasal tumors. The treatments for ENB include surgery, radiotherapy and/or chemotherapy, though sometimes, it is difficult to achieve radical treatment using these strategies as most patients are diagnosed at a late stage. We present a case of a 46-year old male who had been diagnosed with ENB in 2016. He was initially treated with nasal endoscopic resection, followed by a bifrontal craniotomy. Afterwards, he received adjuvant radiotherapy. Currently, his 3-year follow up show no evidence of local, regional o distant metastasis, nor recurrence.

Abordaje endoscópico endonasal puro de estesioneuroblastoma / Purely endonasal endoscopic resection of esthesioneuroblastoma

Presentamos el caso de una mujer de 56 años con diagnóstico de estesioneuroblastoma Kadish C/Hyams II tratado con resección quirúrgica mediante un abordaje endoscópico endonasal puro y radioterapia adyuvante. Describimos el caso y discutimos sus aspectos relevantes.

We report the case of a 56-year-old woman with diagnosis of esthesioneuroblastoma Kadish C/Hyams II treated with a purely endonasal endoscopic resection and adjuvant radiotherapy. We described the case and discuss its relevant aspects.

Estesioneuroblastoma o neuroblastoma olfatorio / Esthesioneuroblastoma or olfactory neuroblastoma

Estesioneuroblastoma o también llamado neuroblastoma olfatorio es un tumor maligno infrecuente derivado del neuroepitelio olfatorio. La historia clínica de un paciente con un neuroblastoma olfatorio es muy inespecífica. Durante la última década, los métodos endoscópicos se han aplicado gradualmente para el tratamiento de tumores de la base del cráneo. El tratamiento puramente endoscópico endonasal del estesioneuroblastoma ha mostrado excelentes resultados de supervivencia con disminución de las complicaciones. Presentamos un paciente afectado por un neuroblastoma olfatorio tratado solo con cirugía endoscópica endonasal.

Esthesioneuroblastoma or olfactory neuroblastoma is an uncommon malignant tumor arising from the olfactory neuroepithelium. The clinical symptoms of a patient with olfactory neuroblastoma are very inespecific. During the past decade, endoscopic approaches have been gradually applied in treating skull base tumors. The purely endoscopic endonasal surgery for esthesioneuroblastoma showed successful survival results with remarkably decreased complications. We reported a patient who suffered olfactory neuroblastoma treated with only endoscopic endonasal surgery.

Olfactory neuroblastoma

@#This 57 year-old woman presented with a seizure. She had a history of attending the ENT and neurosurgical departments for more than a decade. At the time of her initial presentation many years prior, her main complaint was of nasal congestion. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Olfactory neuroblastoma is an uncommon slow growing tumour of the nasal cavity with no established etiological basis. With a neuroectodermal origin, it arises from the olfactory epithelium of the upper nasal cavity.1 Most cases arise from the cribriform plate, upper third of the nasal septum, superior turbinates or anterior ethmoidal air cells. However, it typically presents late when multiple structures are involved, which may include the orbits and intracranial compartments.2 Accounting for approximately 2% of sinonasal tumors, although often late to present, ironically only a minority of patients experience anosmia.3 The commonest complaint at initial presentation is nasal blockage accounting for nearly a quarter of cases, with headache and epistaxis the next most frequent symptoms.1 Multi-modality imaging is essential in that the most recognized management of this infrequent tumor is a combination of craniofacial surgery and radiotherapy. The imaging pathway in this case was typical, with CT and MRI complementing each other in maximizing tumor delineation. Computed Tomography has superior definition is reviewing bony involvement which is a typical finding, whereas MRI has superiority in evaluating the extent of soft tissue invasion and establishing tumor boundaries against post obstruction fluid in the paranasal sinuses.3 In this case the CT illustrates the gross destruction of the skull base, orbital and sinus margins. (Figure 1-4) The MRI outlines the extension of disease involving the pituitary fossa, brainstem and frontal sinus invasion. (Figures 5 and 6)

Esthesioneuroblastoma in a boy with 47, XYY karyotype / 소아과

Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid mass in the right nasal cavity was detected through sinuscopy. Biopsy of the right nasal polyp was performed. Based on the result, the patient was diagnosed with a high-grade esthesioneuroblastoma. Nuclear imaging revealed increased uptake in both the right posterior nasal cavity and the right cervical IB-II space, suggesting metastatic lymph nodes. Cytogenetic analysis revealed a 47, XYY karyotype. Twelve courses of concurrent chemotherapy were administered. Three years after the completion of chemotherapy, the patient had had no disease recurrence. He manifested behavioral violence and temper tantrums, so we started methylphenidate for correction of the behavior.

Estesioneuroblastoma: relatos de casos e revisão de literatura / Esthesioneuroblastoma: Case Reports and Literature Review

O estesioneuroblastoma (ENB) é uma neoplasia rara que atinge o neuroepitélio olfativo, comumente invadindo os seios paranasais, a base do crânio e a região orbitária. Apresenta metástase em 10 a 30 % dos casos. Distribui-se de forma bimodal para a idade, principalmente dos 11 aos 20 anos e dos 51 aos 60 anos, sem influência do sexo e raça do indivíduo. A ínfima quantidade de casos relatados resulta em dúvidas quanto ao melhor tratamento a ser dispensado ao paciente. Relatamos três casos, todos Kadish C, tornando o tratamento e o prognóstico um desafio à prática neurocirúrgica.

The esthesioneuroblastoma (ENB) is a rare neoplasm that affects the olfactory neuroepithelium, commonly invading the paranasal sinuses, skull base and the orbital region. Shows metastasis in 10% to 30% of cases. Distributed in a bimodal shape for the age, from 11 to 20 years and 51 to 60 years especially, with no influence of gender and race of the individual. The tiny amount of reported cases result in doubt about the best treatment to be dispensed to the patient. We report three cases, all Kadish C, making treatment and prognosis a challenge to neurosurgery.

Olfactory neuroblastoma with initial manifestations of hyponatremia: a case report / 临床耳鼻咽喉头颈外科杂志

Clinical records of a patient with olfactory neuroblastoma presented with hyponatremia as initial symptoms were analyzed and the literatures were reviewed. At initial onset, the patient presented with hyponatremia. After pathological examination, the diagnosis was olfactory neuroblastoma. The blood sodium has been normal after operation and radiotherapy. The incidence rate of olfactory neuroblastoma is low, and it is easily misdiagnosed. Its diagnosis relies on pathological examination. We should pay more attention to the unspecific symptoms of patients with hyponatremia, which can help to improve early diagnosis and the prognosis.

The long-term clinical efficacy of endoscopic surgery of primary sinonasal malignant neoplasms / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To assess the long-term clinical efficacy of endoscopic surgery of primary sinonasal malignant neoplasms and find out the potential postoperative prognostic factors.@*METHOD@#Forty-three clinical cases of primary sinonasal malignant neoplasms treated under endoscopy were analyzed retrospectively.@*RESULT@#Fifteen patients died. The 1-year, 2-year, 3-year and 5-year survival rates were 83. 7% (36/43), 74. 4% (32/43), 69. 8% (30/43) and 65. 1% (28/43), respectively. Kaplan-Meier single-variable analysis showed that gender, T grade, TNM stage and pathological types of olfactory neuroblastoma were statistically significant prognostic factors (P< 0. 05). COX Pro-Portional hazard models showed that TNM stage was an independent prognostic factors.@*CONCLUSION@#Endoscopic surgery for primary sinonasal malignant neoplasms is a safe and effective minimally invasive surgical treatment, and it is an important supplement to the traditional surgery. Gender, T grade, TNM stage and pathological types of olfactory neuroblastoma might be significant prognostic factors.

Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment

Introduction: Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferentiated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives: We will discuss diagnostic strategies, recent advances in immunohistochemistry and molecular diagnosis, and treatment strategies. Data Synthesis: These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center are given a different diagnosis on review of pathology. Correct classification is vital, as these tumors are significantly different in biological behavior and response to treatment. The past decade has witnessed advances in diagnosis and therapeutic modalities leading to improvements in survival. However, the optimal treatment for esthesioneuroblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma remain debated. We discuss advances in immunohistochemistry and molecular diagnosis, diagnostic strategies, and treatment selection. Conclusions There are significant differences in prognosis and treatment for esthesioneuroblastoma, neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. Recent advances have the potential to improve oncologic outcomes but further investigation in needed...

Sinonasal teratocarcinosarcoma: clinicopathologic study and analysis / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To study the clinicopathologic features, immunophenotype, diagnosis and differential diagnosis of Sinonasal teratocarcinosarcoma (SNTCS).@*METHOD@#The clinical findings, morphologic features and immunohistochemical markers in one case of SNTCS were studied, and the relevant literatures were reviewed.@*RESULT@#The Tumor tissue is composed of three layers, with mature and immature squamous epithelium nests, neural epithelial cells and olfactory neuroblastoma-like cells derived of ectoderm; Sarcomatoid components and bone tissue derived of mesoderm; The glandular and tubular structures part of which is adenocarcinoma and respiratory epithelium derived of endoderm; The fetal clear cell squamous epithelium is typical. In addition, diffuse large cytoplasm-with high light and cytoplasm with dark light has no obviously boundery. Immunohistochemical staining showed immune markers of different germ layers corresponding, squamous epithelium, glandular epithelium and respiratory epithelium were positive for CK and EMA, neural epithelial cells and olfactory neuroblastoma-like cells were positive for S-100, NSE and Syn, sarcomatoid area was positive for Vim, light dye area was positive for Vim, CD99 and CK, dark area was positive for NSE and GFAP.@*CONCLUSION@#SNTCS is a rare malignant tumor with the features of teratoma and carcinosarcoma, its histopathological and immunohistochemical features were typical, should be more drawn and sliced to avoid misdiagnosis and missed diagnosis.

Clinical analysis of 49 cases of esthesioneuroblastoma / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To investigate the optimal treatment and prognostic factors of esthesioneuroblastoma (ENB).</p><p><b>METHOD</b>Forty-nine cases of ENB, received therapies in Chinese people liberation army general hospital from October 2004 to June 2013, were reviewed retrospectively. The therapeutical strategies and prognostic factors were analyzed using the Kaplan-Meier method. Efficacy of different therapeutical strategies was compared.</p><p><b>RESULT</b>The 5-year overall survival (OS) in the group was 63.8%. The 5-year OS of Kadish stage A, B, C were 90.0%, 77.1%, 32.6%, respectively, with significant difference (χ(2) = 7.379, P = 0.03). The 5-year OS of the patients treated by surgery and radiotherapy, chemoradiotherapy, surgery with chemoradiotherapy were 68.6%, 0, 92.3%, respectively, with significant difference (χ(2) = 8.140, P = 0.02). The 5-year OS of the patients with transnasal endoscopic resection (or combined with transcranial resection) and lateral rhinotomy ( or combined with transcranial resection) were 63.7%, 50.0%, respectively, which had no significant difference (χ(2) = 3.818, P = 0.05).</p><p><b>CONCLUSIONS</b>ENB has a relatively good prognosis in head and neck malignancies, the prognosis is associated with clinical stages and treatment approaches. Transnasal endoscopic surgery based multimodality is the preferable choice.</p>