Presentación de un caso y revisión de literatura: anestesia total intravenosa en cirugía de estesioneuroblastoma / Presentation of a case and literature review: total intravenous anesthesia in estesioneuroblastoma surgery

Resumen: El estesioneuroblastoma es una neoplasia de las células del neuroepitelio olfatorio. Tiene una baja incidencia de 0.4 por cada millón de habitantes. Se caracteriza por síntomas como sinusitis, epistaxis, cefalea, discapacidad visual, obstrucción nasal, hiposmia y rinorrea. En este reporte se presenta el caso de un paciente masculino de 29 años con estesioneuroblastoma estadio Kadish C, quien fue programado para resección quirúrgica bajo anestesia total intravenosa con resultados satisfactorios durante el procedimiento quirúrgico.

Abstract: Esthesioneuroblastoma is a neoplasm of the olfactory neuroepithelium cells, it has a low incidence of 0.4 per million inhabitants. It is characterized by symptoms such as sinusitis, epistaxis, headache, visual impairment, nasal obstruction, hyposmia, and rhinorrhea. We present the case of a 29-year-old male patient with Kadish C stage esthesioneuroblastoma, who was scheduled for surgical resection under total intravenous anesthesia with satisfactory results during the surgical procedure.

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)

Estesioneuroblastoma: el impacto de la enfermedad oncológica cerebral en los cambios neuropsicológicos - Estudio de Caso Clínico

Introducción: La enfermedad oncológica cerebral de tipo estesioneuroblastoma es un tumor poco frecuente, pero con extensiones que pueden llegar hasta la base del cráneo y, en ocasiones, puede presentar metástasis a grandes distancias que comúnmente llegan hasta los pulmones. Esta investigación tuvo como propósito, identificar y analizar los cambios neuropsicológicos antes y después de la intervención quirúrgica. Método: Se consideró apenas un participante para este estudio de caso, un hombre con 53 años que residía en la ciudad de Lisboa. Se aplicó un screening constituido por diversas subpruebas de la WISC y la figura compleja de Rey, además se evaluó la regulación emocional a partir de la prueba EADS. Resultados: En la evaluación preoperatoria se verificaron alteraciones significativas para la atención, memoria, funciones ejecutivas y todos los ámbitos de la regulación emocional. Una vez extirpado el tumor, apenas se verificaron alteraciones en las funciones ejecutivas y sintomatología depresiva. Conclusiones: Se verifican mejorías importantes debido a la estimulación natural, sin embargo, las funciones ejecutivas tendrán que seguir un programa de rehabilitación neuropsicológico para minimizar el daño.

Background: Esthesioneuroblastoma-type brain oncology is a rare tumor, but with extensions that can reach the base of the skull and, occasionally, can present long-distance metastases that commonly reach the lungs. We aimed to identify and analyze the neuropsychological changes before and after the surgical intervention. Methods: Only one participant was considered for this case study, a 53-year-old man who lived in the city of Lisbon. A screening consisting of various subtests of the WISC and the complex figure of Rey was applied. In addition, we used the EADS test to assess emotional regulation. Results: In the preoperative evaluation, significant alterations were verified for attention, memory, executive functions and all areas of emotional regulation. Once the tumor was removed, hardly any alterations were verified in executive functions and depressive symptomatology. Conclusions: Significant improvements are verified due to natural stimulation, however, executive functions will have to follow a neuropsychological rehabilitation program to minimize the damage.

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.

Analysis of the effect of endoscopic surgery combined with conventional surgery in Neuroblastoma / 临床耳鼻咽喉头颈外科杂志

The aim of this study is to evaluate the efficacy of endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma. Forty-three patients with olfactory neuroblastoma undergoing surgery combined with radiotherapy were retrospectively analyzed. The patients were divided into endoscopic surgery and conventional surgery. All patients received postoperative radiotherapy at a dose of 60-70 Gy, the 5-year survival rate and local recurrence time of the two groups were compared, and the therapeutic effects of endoscopic surgery and traditional surgery were compared. Through survival analysis, the 5-year overall survival rates of the traditional surgery group and the endoscopic surgery group were 50% and 58% （=0.560）, the local recurrence rates were 44% and 48% （=0.288）, and the mean recurrence time was 5.6 months and 12.5 months （=0.032）. There was no difference between endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma, and the time of local recurrence was significantly prolonged. In early Neuroblastoma, endoscopic sinus surgery may be superior to open surgery in terms of efficacy and patient survival.

Analysis of the effect of endoscopic surgery combined with conventional surgery in Neuroblastoma / 临床耳鼻咽喉头颈外科杂志

Objective@#The aim of this study is to evaluate the efficacy of endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma. @*Method@#Forty-three patients with olfactory neuroblastoma undergoing surgery combined with radiotherapy were retrospectively analyzed. The patients were divided into endoscopic surgery and conventional surgery. All patients received postoperative radiotherapy at a dose of 60-70 Gy, the 5-year survival rate and local recurrence time of the two groups were compared, and the therapeutic effects of endoscopic surgery and traditional surgery were compared.@*Result@#Through survival analysis, the 5-year overall survival rates of the traditional surgery group and the endoscopic surgery group were 50% and 58% （P=0.560）, the local recurrence rates were 44% and 48% （P=0.288）, and the mean recurrence time was 5.6 months and 12.5 months （P=0.032）.@*Conclusion@#There was no difference between endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma, and the time of local recurrence was significantly prolonged. In early Neuroblastoma, endoscopic sinus surgery may be superior to open surgery in terms of efficacy and patient survival.

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.

Estesioneuroblastoma Olfatorio: Presentacion De Un Caso / Oligatory Esthesioneuroblastoma: Presentation of a Case

Se presenta un caso de una paciente con pérdida de peso, congestión nasal epistaxis, aumento de volumen en cuello con disfagia a sólidos y líquidos de 1 mes de evolución. La tomografía de cuello muestra una masa de tejidos blandos en la base de cuello con erosión del esfenoides con extensión a la fosa craneal media, con erosión del clivus, el esfenoides y la si­lla turca. El diagnostico histopatológico es un estesioneuroblastoma.

We present a case of a patient with weight loss, nasal congestion, epistaxis, increase neck volu­ me with dysphagia to solids and liquids of 1 month of evolution. The neck tomography shows a soft tissue mass at the base of the neck with erosion of the sphe­ noid with extension to the middle cranial fossa, with erosion of the clivus, the sphenoid and the sella turcica. The histopathological diagnosis is an esthesioneuroblastoma.

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.

Esthesioneuroblastoma - A Clinicopathological Study

Background: Esthesioneuroblastoma is a rare tumour accounting for only 3% of all intranasal tumors. Its proximity with important structures and aggressive behavior presents a diagnostic and therapeutic challenge. CT, MRI and histopathology forms the basic pillars of management. Methods: A prospective study was conducted between Feb 1998 to Feb 2004 in the Department of ENT, JNMC, AMU Aligarh. All the five patients taken up for study proved histopathologically to be cases of esthesioneuroblastoma. Results: Of the five patients, three presented with unilateral nasal obstruction (60%) and two with epistaxis (40%). Proptosis was present in one patient. Anosmia, headache and pain were other associated symptoms. Conclusions: hepatic and renal involvement occurs in patients suffering from dengue fever. Hence, multidisciplinary approach should be carried out while treating such patients.

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.

Clinical decision on a patient with esthesioneuroblastoma / 中国肿瘤临床

Olfactory neuroblastoma is a rare malignant tumor. Although multiple therapeutic modalities including surgery, radio-therapy, or chemotherapy could be used in patients with olfactory neuroblastoma, no standardized treatment has been achieved. This re-view introduces a case of adult olfactory neuroblastoma treated by a multiple disciplinary team in Tianjin Medical University Cancer In-stitute and Hospital. This review also aims to explore a complete set of diagnostic and treatment practices for the benefit of future pa-tients.

Secreción ectópica de ACTH: Presentación infrecuente de un estesioneuroblastoma / An Infrequent Presentation of Esthesioneuroblastoma with Ectopic ACTH Syndrome

Introducción: El estesioneuroblastoma es un tumor derivado de los neuroblastos del epitelio respiratorio neuroectodérmico. Representa entre el 3 al 6 % de los tumores intranasales. La secreción ectópica de Hormona Adrenocorticotrofina (ACTH) es la causa del 10 al 20 % de Síndrome de Cushing dependiente de ACTH, siendo el estesioneuroblastoma una rara causa de secreción ectópica de ACTH. Presentación del caso: Paciente de sexo femenino de 63 años que consulta por mareos, náuseas, debilidad muscular, y rinorrea de dos años de evolución tratada con corticoides tópicos en forma prolongada. Al examen físico mostraba fenotipo Cushing característico. El laboratorio confirmó valores de ACTH veinte veces por encima de los rangos normales. En IRM cerebral se evidencia lesión expansiva en región etmoidal con compromiso de órbita y base de cráneo. Se resuelve tratamiento quirúrgico ante la necesidad de descompresión tumoral y tratamiento médico para hipercortisolismo en el período pre y posquirúrgico. La anatomía patológica informó proliferación neoplásica maligna de origen neurogénico y las pruebas inmunohistoquímicas confirmaron positividad para ACTH. Comentario: El Síndrome de Cushing por secreción ectópica de ACTH a partir de estesioneuroblastoma es muy poco frecuente, reportándose solo once casos a la fecha en la literatura médica indexada. El tratamiento es complejo y requiere de equipo interdisciplinario. Rev Argent Endocrinol Metab 51:192-196, 2014 Los autores declaran no poseer conflictos de interés.

Introduction: Esthesioneuroblastoma is a tumor originating from the neuroblasts in the neuroectodermal olfactory epithelium. It accounts for 3 % to 6 % of nasal tumors. Adrenocorticotropic hormone (ACTH) ectopic secretion is the cause for 10 % to 20 % of ACTH-dependent Cushing Syndrome cases. Esthesioneuroblastoma constitutes a rare cause of ectopic ACTH secretion. Case presentation: A 63 year-old female patient presented with dizziness, nausea, muscle weakness, and rhinorrhea of two-year duration under long-term treatment with topical corticosteroids. On physical examination, she showed typical Cushingoid phenotype. Brain MRI revealed an expanding lesion in the ethmoid area involving the orbit and the cranial base. Sub­maxillary metastasis was also detected. Lab tests confirmed ACTH values 20-fold above normal. The patient underwent treatment for hypercortisolism prior to surgery. Surgical treatment was prompted by the need to decompress the tumor. Comment: Esthesioneuroblastoma is a rare tumor. Although approximately 1,000 cases have been published in the last 20 years, only eleven of them were due to ACTH secretion. Treatment is complex and requires an interdisciplinary team. Rev Argent Endocrinol Metab 51:192-196, 2014 No financial conflicts of interest exist.

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...

Aspergilosis nasosinusal con extensión intracraneal en una paciente inmunocompetente: una entidad infrecuente con características radiológicas típicas / Sinonasal aspergillosis with intracranial extension in an immunocompetent patient: a rare disease with typical imaging features

El aspergilo es un hongo ubicuo. Las localizaciones de infección primaria más comunes son el tracto respiratorio y los senos paranasales. La afectación intracraneal es rara y conlleva una alta mortalidad. Ocurre mayoritariamente por extensión hematógena desde el pulmón, pero en pacientes inmunocompetentes, la extensión directa desde los senos paranasales es más común. Describimos el caso de una mujer de 25 años originaria de India que se presentó en el servicio de urgencia de nuestro centro hospitalario con cefalea frontal crónica y progresiva. Los hallazgos en los estudios de imágenes sugirieron el diagnóstico de sinusitis fúngica con extensión intracraneal, siendo el patógeno más frecuente el aspergilo. El diagnóstico fue confirmado anátomo-patológicamente. Revisamos los hallazgos radiológicos típicos que deben ayudar al diagnóstico precoz de esta entidad, rara, pero potencialmente mortal.

Aspergillus is a ubiquitous fungus. The most common primary sites of infection are the respiratory tract and sinuses. Intracranial infection is rare and implies a high mortality. It occurs mainly by hematogenous extension from the lung, but in immunocompetent patients, direct extension from the sinuses is more common. We describe the case of a 25 year old woman from India who consulted in the emergency room of our hospital with chronic and progressive frontal headache. The findings in imaging studies suggested the diagnosis of fungal sinusitis with intracranial extension, being the most common pathogen of Aspergillus. The diagnosis was anatomically-pathologically confirmed. We review the typical radiological findings which should help in the early diagnosis of this rare but potentially fatal disease.

Hyperostotic Esthesioneuroblastoma: Rare Variant and Fibrous Dysplasia Mimicker

A 65-year-old male presented with a 3-year history of orbital symptoms. An imaging-based diagnosis of fibrous dysplasia involving the skull base was made at another institution. CT showed a diffuse sinonasal mass and ground-glass appearance of the bones of the anterior skull base with bony defects and mucocele formation. MRI demonstrated an accompanying intracranial and orbital rind of soft tissue mass along the hyperostotic bones. FDG-PET showed corresponding intense hypermetabolism. Small cysts were observed at the tumor-brain interface. Biopsy revealed esthesioneuroblastoma with bone infiltration that is compatible with the hyperostotic variant of esthesioneuroblastoma. There are a few cases of hyperostotic esthesioneuroblastoma reported in the literature.

Neuroblastoma olfatório ectópico: estudo de caso e revisão da literatura / Ectopic olfactory neuroblastoma: case study and literature review

O neuroblastoma olfatório, também conhecido como estesioneuroblastoma (ENB), é tumor maligno de crescimento lento, com origem principalmente na neuroectoderme da cavidade nasal. O pico de incidência do ENB é aos 53 anos, com a maioria dos casos ocorrendo entre os 40 e 70 anos, principalmente em homens. Há poucos relatos de ENB nos seios etmoidal, maxilar e esfenoidal, hipófise e nasofaringe. Esse tumor geralmenteapresenta-se como doença invasiva e tendência a recorrer tardiamente. A disseminação mais frequente do ENB é para os linfonodos cervicais e, se presente, significa pior prognóstico. Seus sintomas são inespecíficos, sendo a obstrução nasal o mais comum. Os exames de imagem são importantes no diagnóstico do ENB, juntamente com o histopatológico e o imuno-histoquímico, que também auxiliam no diagnóstico diferencial. O tratamento cirúrgico de ressecção craniofacial associado à radioterapia apresenta os melhores resultados na sobrevida. Descreve-se seguidamente um caso clínico ilustrativo dessa doença em paciente masculino de oito meses, faixa etária incomum da ENB, com queixa familiar inicial de obstrução nasal e coriza hialina com rajas de sangue...

Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a slow-growing malignant tumor of that originates mainly in the neuroectodermal cells in the nasal cavity. The peak incidence of ENB is at age 53, with the majority of cases occurring between 40 and 70 years, and mostly among men. There are a few reports of ENB in the ethmoid, maxillary, and sphenoid sinuses, or in the pituitary and nasopharynx. This tumor usually presents as an invasive disease with a tendency for later recurrence. The most frequent dissemination of ENB is to the cervical lymph nodes, which represents a worse prognosis. Its symptoms are nonspecific and nasal obstruction is the most common complaint. Imaging tests are important for the diagnosis of ENB, along with histopathological and immunohistochemical tests to help in the differential diagnosis. Surgical treatment of craniofacial resection associated with radiotherapycan achieve the best results for survival. We present a discussion of an illustrative clinical case of the disease in a male patient aged eight months, an unusual age for ENB, brought to attention by an initial family complaint of nasal obstruction and bloodstained hyaline rhinorrhea...