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.

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.

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.

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.

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...

Estesioneuroblastoma. Presentación de un caso / Esthesioneuroblastoma. A case report

El neuroblastoma olfatorio o "estesioneuroblastoma" es un tumor embrionario, infrecuente, derivado de neuroblastos de las ramificaciones nasales del sistema sensorial olfatorio, cuyo asiento es el epitelio respiratorio neuroectodérmico; fue descrito originalmente en 1924. La presentación más común es la de un crecimiento polipoide que causa epistaxis u obstrucción nasal con anosmia de larga evolución. Es localmente muy recidivante, aunque se han reportado metástasis regionales y a distancia. El objetivo de este trabajo es caracterizar desde el punto de vista patológico un paciente masculino, atendido en el servicio de Cirugía Maxilofacial del Hospital General Docente "Abel Santamaría Cuadrado" de la provincia de Pinar del Río, con un aumento de volumen en la hemicara en relación al seno maxilar izquierdo. Este paciente también tenía un tratamiento por una sinusitis maxilar, al cual se le hizo una biopsia insicional y se obtuvo como resultado un estesioneuroblastoma con metástasis frontal. Se expone por tratarse de una enfermedad poco frecuente en nuestro medio.

Olfactory neuroblastoma or "esthesioneuroblastoma" is an infrequent embryonate tumor, derived from the neuroblasts belonging to the nasal ramification of sensory-olfactory system, which seat is the neuroectodermic respiratory epithelium that was described in 1924 originally. The most common presentation is a polypoid growth that provokes epistaxis or nasal obstruction with a large natural history of anosmia. It is very recurrent locally, thought regional or distant metastases have been reported. This work was aimed at characterizing a male patient from the pathologic view point who attended to the Maxillofacial Surgery Service at "Abel Santamaria Cuadrado" University Hospital, Pinar del Rio, presenting an increase of volume on the hemiface in relation to the left maxillary sinus. This patient was under treatment because of a maxillary sinusitis, the incisional biopsy showed an esthesioneuroblastoma with frontal metastasis. Since this is a rare entity, it is described.

Esthesioneuroblastoma: a case report and review of the literature / Estesioneuroblastoma: reporte de un caso y revisión de la literatura

We report on a patient who presented to our clinic with a one-year history of diplopia in the left eye, epistaxis for one month and pain in the occipital area of the head. A biopsy of the area was performed and the lesion was diagnosed as Esthesioneuroblastoma also known olfactory neuroblastoma. Due to the size of the tumor and involvement of the vital intracranial structures it was decided to prescribe a palliative care program and palliative radiotherapy...

Se presenta el caso de una paciente que acudió a nuestra consulta con historia de un año de diplopía en el ojo izquierdo, epistaxis durante un mes y dolor en la zona occipital de la cabeza. Se realizó una biopsia del área y la lesión fue diagnosticada como estesioneuroblastoma también conocido neuroblastoma olfatorio. Debido al tamaño del tumor y la participación de las estructuras vitales intracraneales, se decidió prescribir un programa de cuidados paliativos y radioterapia paliativa...

Endoscopic treatment of esthesioneuroblastoma / Abordagem endoscópica de estesioneuroblastoma

Esthesioneuroblastoma is an uncommon malignant tumor of the nasal vault. Treatment consists of craniofacial resection. As endoscopic techniques have advanced, this approach has been recommended to avoid morbidity and to reduce costs. AIM: To evaluate outcomes in patients with esthesioneuroblastoma treated by an endoscopic technique. METHODS: A prospective study of patients diagnosed with esthesioneuroblatoma and treated by an endoscopic technique. The literature over the past 20 years was reviewed for an update on the pathology. RESULTS: We present 4 patients, 3 males and 1 female, staged according to Kadish and Dulguerov. All were treated surgically with endoscopic techniques, followed by radiotherapy. One patient was also submitted to neck dissection and chemotherapy because of regional metastasis. There were no significant postoperative complications. The mean hospital stay was 3 days; one patient stayed in the ICU for 24 hours after surgery. Follow-up is recent; so far there are no recurrences. CONCLUSION: Esthesioneuroblastoma is a potentially curable malignancy. Endoscopic techniques help reduce hospital costs and decrease the morbidity. Adequate margins of healthy tissue are obtained with endoscopic resection, as with craniofacial resection. The literature suggests that outcomes after endoscopic resection are similar to those of the conventional external approach.

Estesioneuroblastoma é uma neoplasia incomum de linhagem epitelial. Seu tratamento convencional baseia-se na ressecção craniofacial. Técnicas endoscópicas tem sido preconizadas em sua terapia com o intuito de diminuir morbidades e custos. OBJETIVO: Relatar a experiência na abordagem terapêutica do tumor com uso de endoscópio. MATERIAL E MÉTODO: Estudo prospectivo de pacientes com diagnóstico de Estesioneuroblastoma submetidos a tratamento endoscópico. Fez-se revisão de publicações relacionadas ao tumor. RESULTADOS: Apresentamos quatro pacientes, sendo 3 homens e 1 mulher, estadiados de acordo com Kadish e Dulguerov. Foram submetidos a ressecção endoscópica com margem e terapia adjuvante (radio ou quimioterapia). Não se observaram complicações pós-operatórias significativas. O tempo médio de internação foi 3 dias e um paciente permaneceu em observação no CTI por 24 horas. O seguimento é recente e não há relato de recidivas. CONCLUSÃO: Estesioneuroblastoma é um tumor maligno potencialmente curável. O uso da técnica cirúrgica endoscópica nasal traz consigo menor morbidade ao paciente (período de internação e tempo cirúrgico menor) e custos hospitalares diminuídos. Possibilita a ressecção da neoplasia com margens comparáveis à cirurgia convencional. Os resultados em longo prazo descritos na literatura são semelhantes ao tratamento convencional. Nosso seguimento ainda é recente para concluir algo sobre prognóstico.

Estesioneurablastoma y panhipopituitarismo: presentación de un caso / Esthesionerublastoma and panhypopituitarysm: case report

Objetivos: presentar un caso de estesioneuroblastoma que desarrolla hipopituitarismo como complicación grave de su tratamiento. Realizar una revisión de la literatura reciente sobre el tratamiento y pronóstico de estos tumores. Presentar una sistemática de pesquisa de hipipituitarismo en estos pacientes. Descripción: paciente masculino de 68 años que se presenta con metástasis submaxilar de estesioneuroblastoma. La RMN mostraba una masa neoplásica de gran tamaño centrada sobre el etmoides, que comprometía base de cráneo y corteza fronto-orbitaria izquierda. Intervención: se realizó tratamiento combinado de radio y quimioterapia preoperatorios, y cirugía por vía craniofacial. A los 20 meses ingresa con un cuadro convulsivo e hiponatremia grave. Se hace diagnóstico de panhipopituitarismo y el cuadro revierte con la administración de hidrocortisona de 10 mg vía oral. Conclusión: el estesioneuroblastoma es un tumor infrecuente cuyo tratamiento incluye cirugía, radioterapia y quimioterapia. Es necesario un alto índice de sospecha y el seguimiento de por vida para pesquisar la instalación del hipopituitarismo como complicación grave de su tratamiento.

Objectives. To report a case of esthesioneuroblastoma associated to hypopituitarism as a late complication of its treatment, to review the literature regarding treatment and prognosis and to resent a guideline for the diagnosis of hypopituitarism in these patients. Description. A 68-year-old male patient presented with a submaxilar metastasis of a very large esthesioneuroblastoma that involved both ethmoidal bones and invaded left cribiform plate and frontal lobe. Intervention. The patient underwent combined modality treatment with preoperative radio and chemotherapy, and craniofacial sugery of the tumor. Twenty months later he was admitted with a seizure and severe hyponatremia. A diagnosis of panhypopituitarism was made, and his sodium reverted to normal with de administration of 10 mg of hydrocortisone p.o. Conclusions Esthesioneuroblastomas are rare tumours that require multimodality treatment with surgery, radiotherapy and chemotherapy. Hypopituitarism is a late and potentially fatal complication that must be suspected and tested during the patients‘ whole lifespan.

Estesioneuroblastoma - análise dos casos tratados no Hospital Erasto Gaertner no período de 1973 a 2004 / Esthesioneuroblastoma - Cases treated at Hospital Erasto Gaertner between 1973 and 2004

Introdução: O estesioneuroblastoma é um tumor maligno raro originado das células do epitélio olfativo e freqüentemente é confundido com outras neoplasias da cavidade nasal. Devido à raridade desses tumores, há controvérsia quanto ao diagnóstico e tratamento da doença. Objetivo: Rever os casos tratados no Hospital Erasto Gaertner. Métodos: Análise dos pacientes tratados por estesioneuroblastoma no período de 1973 a 2004. Resultados: Foram tratados sete pacientes (três mulheres e quatro homens), com idade média de 45,6 anos. A apresentação clínica mais freqüente foi a obstrução nasal unilateral e, na maioria dos pacientes, mais de uma manifestação clínica estava presente. Nenhum paciente apresentava metástases a distância ao diagnóstico e um paciente, que se encontra vivo, apresentou metástases linfonodais no seguimento. A sobrevida foi de 43%. Conclusão: A maioria dos pacientes apresenta doença avançada e a sobrevida é pobre.

Introduction: Esthesioneuroblastoma (ENB) is an uncommon malignant tumor believed to arise from the olfactory epithelium and is often confused with other neoplasms of the nasal cavity. Because of the rarity of this unusual tumor there are many controversy related to diagnosis and treatment of this neoplasms. Objective: To review our experience on treatment. Methods: Records of patients with esthesioneuroblastoma treated between 1973 and 2004 were reviewed. Results: Seven consecutive patients diagnosed as having esthesioneuroblastoma (3 women and 4 men), with an average age of 45.6 years. The most common clinical presentation was nasal obstruction and most of patients had more than one clinical feature. None of patients had distant metastasis at the moment of the diagnosis, and one patient, who is alive, presented with metastatic disease on cervical lymph nodes during follow up. Conclusion: Most of patients presented with advanced disease and in this group the survival was very poor.

Estesioneuroblastoma / Estesioneuroblastoma

Se expuso el caso de una paciente, de 73 años de edad, que asiste a consulta de Medicina Interna del Hospital Hermanos Ameijeiras refiriendo obstrucción de la fosa nasal derecha desde hace aproximadamente 1 año, asociada a intensa cefalea. Se realizó examen físico que sólo mostró tumoración en la fosa nasal derecha. Se indicó rinoscopia anterior donde apareció secreción nasal serohemática, cornetes congestivos en la fosa nasal derecha y se observó tumoración de color rosa pálido. Se estudió el caso, con el planteamiento de una entidad que no es frecuente en la práctica cotidiana. Se emitió diagnóstico histológico por resección completa del tumor y, posteriormente, se envió al servicio de Oncología para tratamiento radiante posquirúrgico y con quimioterapia.

Coexistencia de neuroblastoma olfatorio y aneurisma de la arteria carótida interna: presentación de un caso / Coexistence of an olfactory neuroblastoma and internal carotid artery aneurism: case report

El neuroblastoma olfatorio es una neoplasia neuroectodérmica maligna rara que afecta a la bóveda nasal y que tiende a invadir las estructuras vecinas, entre estas a la cavidad intracraneal. Su asociación con aneurismas intracraneales nunca ha sido descrita. Presentamos a una paciente con la coexistencia de un aneurisma del segmento oftálmico de la arteria carótida interna y un neuroblastoma olfatorio con invasión intracraneal. La coexistencia entre neoplasias del sistema nervioso central y aneurismas intracraneales es infrecuente. En este artículo analizamos las posibles causas de la asociación entre neoplasias y aneurismas intracraneales.

The olfactory neuroblastoma is a rare malignant neuroectodermal neoplasia of the nasal roof. It has a highly tendency of invades near structures including intracranial compartment. Associations between olfactory neuroblastoma with intracranial aneurysms have been never described. We show a case of a female with coexistence of an internal carotid artery aneurysm and olfactory neuroblastoma. The coexistence between nervous system tumors with intracranial aneurysms is not common. In this paper we analyze the association between intracranial neoplasia and aneurysms.

Estesioneuroblastoma / Esthesioneuroblastoma

O estesioneuroblastoma é uma neoplasia maligna incomum, que tem origem no epitélio olfatório e corresponde de 4% a 6% dos tumores malignos dos seios paranasais. A última grande revisão sobre esses tumores mostra que, desde sua descoberta, há oitenta anos, menos de mil casos foram publicados na literatura mundial. As manifestações clínicas usuais constituem-se por anosmia, obstrução nasal e epistaxe. Devido às características microscópicas serem bastante variadas e inespecíficas, é importante que o diagnóstico seja bem estabelecido pela histoquímica e imunoistoquímica, pois disso dependerá o prognóstico do paciente. Por se tratar de uma doença rara, ainda não há consenso quanto ao melhor tipo de tratamento, mas os relatos de casos bem sucedidos incluíram tratamento multidisciplinar com quimioterapia, ressecção cirúrgica e radioterapia. Em um período de seis anos (janeiro/ 2000 a janeiro/2006), ocorreram quatro casos no serviço do Hospital e Maternidade Celso Pierro, que são aqui reportados

Esthesioneuroblastoma is an uncommon malignant neoplasm that arises from the olfactory epithelium and corresponds to 4% - 6% of all malignant tumors in the paranasal sinuses. The last extensive review on these tumors reveals that less than one thousand cases have been published in the literature since its discovery eighty years ago. The common clinical manifestations are anosmia, nasal obstruction and epistaxis. Since its microscopic characteristics vary greatly and are unspecific, a precise diagnosis using histochemical and immunohistochemical techniques cannot be overemphasized as the patient?s prognosis depends on the diagnosis. As it is a rare disease, there is no consensus regarding the best treatment, but reports of cases treated successfully cite a multidisciplinary approach, which includes chemotherapy, surgical resection and radiation therapy. Our service has treated four cases over a period of six years (January 2000 - January 2006) that are reported here