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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.

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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)

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Olfactory neuroblastoma, or esthesioneuroblastoma, is an uncommon malignant tumor originating from the neural crest that commonly occurs in the upper nasal cavity. Its ectopic origin is extremely rare, especially when located in the optical pathways. This paper reports the case of a giant ectopic esthesioneuroblastoma of the optic pathways that were surgically treated through a cranio-orbital-zygomatic (COZ) craniotomy with extensive resection, in addition to a literature review. The patient is a 46-year-old female presenting with a 4-month history of visual loss in the left eye. Since she was previously blind in the right eye from a traumatic injury, it was evolving to loss of bilateral vision. Imaging depicted an expansive infiltrating lesion involving the entire path of the right optic nerve, extending to the optic chiasm, cisternal portion of the left optic nerve, bilateral optic tract, and hypothalamus. Investigation of pituitary function was unremarkable. Esthesioneuroblastoma is a rare tumor with poorly defined standard clinical management. Its ectopic presentation makes the diagnosis even more challenging, making it difficult to manage these cases properly. Surgeons should be aware of this rare possibility, as early aggressive treatment is likely to be associated with better results.

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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.

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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.

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BACKGROUND: Endoscopic skull-base surgery (ESBS) is employed in the management of diverse skull-base pathologies. Paralleling the increased utilization of ESBS, the literature in this field has expanded rapidly. However, the rarity of these diseases, the inherent challenges of surgical studies, and the continued learning curve in ESBS have resulted in significant variability in the quality of the literature. To consolidate and critically appraise the available literature, experts in skull-base surgery have produced the International Consensus Statement on Endoscopic Skull-Base Surgery (ICAR:ESBS). METHODS: Using previously described methodology, topics spanning the breadth of ESBS were identified and assigned a literature review, evidence-based review or evidence-based review with recommendations format. Subsequently, each topic was written and then reviewed by skull-base surgeons in both neurosurgery and otolaryngology. Following this iterative review process, the ICAR:ESBS document was synthesized and reviewed by all authors for consensus. RESULTS: The ICAR:ESBS document addresses the role of ESBS in primary cerebrospinal fluid (CSF) rhinorrhea, intradural tumors, benign skull-base and orbital pathology, sinonasal malignancies, and clival lesions. Additionally, specific challenges in ESBS including endoscopic reconstruction and complication management were evaluated. CONCLUSION: A critical review of the literature in ESBS demonstrates at least the equivalency of ESBS with alternative approaches in pathologies such as CSF rhinorrhea and pituitary adenoma as well as improved reconstructive techniques in reducing CSF leaks. Evidence-based recommendations are limited in other pathologies and these significant knowledge gaps call upon the skull-base community to embrace these opportunities and collaboratively address these shortcomings.

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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.

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BACKGROUND: Endoscopic skull-base surgery (ESBS) is a rapidly growing discipline that often combines the expertise of otolaryngology-head and neck surgeons and neurosurgeons to provide care for these challenging conditions. As the field has grown, so too has the literature evaluating this surgical approach and the challenges and complications associated with these interventions. An international, multidisciplinary effort to critically evaluate and grade the current literature on ESBS has resulted in the International Consensus Statement on Endoscopic Skull Base Surgery (ICAR:SB). This Executive Summary highlights the key findings of the ICAR:SB document. METHODS: Following the prior methodology of the ICAR statements, the ICAR:SB document consists of 98 topics in ESBS that underwent a systematic review. When sufficient evidence exists, an evidence-based review with recommendation (EBRR) or evidence-based review (EBR) was created and then underwent an iterative review process until consensus was achieved. This Executive Summary recapitulates these findings. RESULTS: This summary complies the EBR and EBRR from the ICAR:SB document in the management of numerous skull-base pathologies including cerebrospinal fluid rhinorrhea, intradural tumors, sinonasal malignancies, and clival tumors, as well as the key issues of reconstruction after and the complications associated with ESBS. CONCLUSION: The ICAR:SB Executive Summary recaps the evidenced-based recommendations concerning the advantages, limitations and challenges of ESBS in the management of diverse skull-base pathologies. Although multifactorial in nature, the overall level of evidence in ESBS is modest. This represents an opportunity to address these knowledge gaps moving forward.

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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.

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BACKGROUND: Esthesioneuroblastomas (ENB) are uncommon and data regarding outcomes are often limited to single-institution series. The National Cancer Database (NCDB), which contains outcomes information from treatment centers across the United States, represents an opportunity to evaluate outcomes for rare diseases such as ENB across multiple institutions. METHODS: The NCDB was queried for location codes corresponding to the nasal cavity and paranasal sinuses and the histology code for ENB. Multivariate analyses were performed to evaluate for contributing factors to overall survival. RESULTS: A total of 1225 patients with ENB met the inclusion criteria. The 5-year overall survival was 76.2% (95% confidence interval [CI], 73.4-79.0%). Overall survival was associated with Kadish stage, grade, treatment sequence, margin status, Charlson/Deyo score, age, and gender (p < 0.05). Multivariate analysis demonstrated that, compared with surgery alone, surgery followed by radiation without chemotherapy had improved all-cause mortality (odds ratio [OR], 0.61; 95% CI, 0.40-0.95). Surgery with chemotherapy alone was associated with increased odds of all-cause mortality (OR, 4.86; 95% CI, 2.31-10.25). Multivariate subanalysis for Kadish stages A and B demonstrated no difference in survival between surgery and surgery followed by radiation, but surgery followed by chemoradiation had worse overall survival (OR, 3.03; 95% CI, 1.07-8.56). For Kadish stage C, surgery followed by radiation had improved overall survival compared with surgery alone (OR, 0.44; 95% CI, 0.24-0.81). CONCLUSION: The most common treatment for ENB is surgery followed by radiation, which is associated with the highest overall survival. The role of adjunctive chemotherapy needs to be re-evaluated in further studies.

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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.

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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.

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Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, is a rare malignant tumor that accounts for 3% of all tumors of the nasal cavity. The incidence of ENB is 0.4 cases per million in the general population, and the most common symptoms are nasal obstruction and epistaxis. Previous studies have indicated the presence of somatostatin receptors in this tumor type. Common treatment strategies for ENB include resection and adjuvant radiotherapy and/or chemotherapy (combined treatment); however, the rate of recurrence is high. Treatment of neuroendocrine tumors using radionuclides bound to somatostatin analogues is well established in clinical practice. However, a standard and effective therapeutic approach has not been reported for ENB. The current study described the case of a 74-year-old female with numerous recurrences of ENB following multiple treatments and without possibility of resection. The patient was treated with the radiolabeled-somatostatin analogue, 177Lutetium-DOTA-octreotate (177Lu-DOTA-TATE), which successfully controlled the disease. This suggests that 177Lu-DOTA-TATE is a potential treatment for ENB and may represent an effective alternative and novel therapeutic strategy for this disease.

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BACKGROUND: The gold standard of treatment for esthesioneuroblastoma consists of en bloc craniofacial resection with postoperative therapy dictated by histology and tumor extent. Numerous studies have shown fully endoscopic approaches to provide comparable survival and recurrence rates with decreased patient morbidity. Here we report the first multi-institutional series assessing smell outcomes of patients who underwent unilateral endoscopic resection of esthesioneuroblastoma with preservation of the contralateral olfactory bulb. METHODS: A multi-institutional retrospective review was performed identifying patients who underwent endoscopic unilateral resection of esthesioneuroblastoma with preservation of 1 olfactory bulb between 2003 and 2015. After completion of postoperative radiation, patients were administered the University of Pennsylvania Smell Identification Test (UPSIT) to assess olfactory function. RESULTS: Fourteen patients (7 males, 7 females) were identified and tested for posttreatment olfactory function. All 14 patients received postoperative radiotherapy and 4 patients received additional chemotherapy. Mean follow-up time was 51.7 months. There was no disease recurrence. Six patients (43%) were found to have residual smell function with 2 patients (14%) having normal or mildly reduced smell function. CONCLUSION: Here we report the first multi-institutional series demonstrating smell preservation after unilateral endoscopic resection of esthesioneuroblastoma. In carefully selected patients, this approach can yield comparable survival with decreased patient morbidity.

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BACKGROUND: Current guidelines have identified 10 to 12 weeks posttreatment as the ideal time-point for improved diagnostic accuracy of positron emission tomography/computed tomography (PET/CT) for deep tissue sites of the head and neck. After treatment, the sinonasal skull base is predisposed to prolonged inflammation that may render this time-point inappropriate for initial posttreatment imaging surveillance for sinonasal malignancies. The purpose of this study is to evaluate temporal trends in 18 F-fluorodeoxyglucose (18 FDG) sinonasal uptake after treatment for sinonasal malignancies to better elucidate the optimal time-point for initial PET/CT posttreatment evaluation in this patient population. METHODS: A retrospective analysis of all successfully treated and non-locally recurrent sinonasal malignancies over a 15-year study period (2000 to 2015) was performed at our institution. Posttreatment 18 FDG PET/CT standardized uptake value data were collected and compared between various time-points (2 to 4 months, 5 to 12 months, 5 to 24 months, and 13 to 24 months) using an independent-samples t test. RESULTS: A statistically significant difference was noted between the posttreatment time windows 2 to 4 and 5 to 12 months (p = 0.048) as well as 2 to 4 and 5 to 24 months (p = 0.02). A trend toward significance was seen when comparing 2 to 4 and 13 to 24 months (p = 0.083). CONCLUSION: Our analysis of PET/CT in patients previously treated for sinonasal malignancy suggests that the posttreatment sinonasal skull base is characterized by a prolonged period of hypermetabolism that endures beyond the period previously described for deep tissue sites of the head and neck. These findings prompt a reevaluation of the previously described 10- to 12-week cutoff point for initial posttreatment PET/CT for head and neck squamous cell carcinoma as applied to sinonasal malignancies.

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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.

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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.

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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.

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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.

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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...

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