Tumor glómico del septum nasal / Glomic tumor of the nasal septum

Presentamos el caso clínico de una mujer de 58 años que consulta por episodios de epis-taxis autolimitadas por fosa nasal derecha asociados a hipersensibilidad al manipular dicha fosa nasal. A la exploración se evidenció en el septum nasal una lesión mucosa nodular, de consistencia dura y aspecto violáceo. Se llevó a cabo exéresis de la misma por vía endonasal, con resultado histopatológico de tumor glómico (TG) del septum nasal. El TG es una neoplasia benigna perivascular, derivada de las células musculares lisas presentes en el cuerpo glómico; estructura con función termorreguladora, formada por un shunt entre arteriolas y vénulas, sin presencia de red capilar. Los cuerpos glómicos están presentes, mayoritariamente, en extremidades (lecho ungueal). Los tumores glómicos son, verdaderamente, excepcionales en el área ORL, existiendo sólo una treintena de casos publicados hasta la fecha a nivel nasosinusal. La mayoría de casos se presentan entre la 5ᵃ y 6ᵃ década de la vida y más, frecuentemente, en mujeres. Plantea diagnóstico diferencial, fundamentalmente, con dos entidades: el paraganglioma (con el que no debe ser confundido desde el punto de vista terminológico ni histológico), y con el tumor fibroso solitario (antiguo hemangiopericitoma). El comportamiento es benigno y el tratamiento es la cirugía

We present the clinical case of a 58-year-old woman who consulted for episodes of self-limited epistaxis in the right nostril associated with hypersensitivity to manipulation of this nostril. Examination revealed a nodular mucosal lesion in the nasal septum, with a hard consistency and purplish appearance. The lesion was excised endonasally, with histopatho-logical result of a glomus tumor (GT) of the nasal septum. The GT is a benign perivascular neoplasm, derived from smooth muscle cells present in the glomus body; structure with thermoregulatory function, formed by a shunt between arterioles and venules, without the presence of capillary network. Glomus bodies are mostly present in the extremities (nail bed). Glomus tumors are truly exceptional in the ENT area, with only about thirty cases published to date at the nasosinusal level. Most cases occur between the 5th and 6th decade of life and more frequently in women. It raises differential diagnosis fundamentally with two entities: paraganglioma (with which it should not be confused from the terminological or histological point of view) and with solitary fibrous tumor (former hemangiopericytoma). The behavior is benign and the treatment is surgery.

Plasmocitoma extramedular solitario rinosinusal: reporte de caso y revisión de literatura / Solitary extramedullary plasmacytoma of the rhinosinusal cavity: a case report and literature review

Resumen El plasmocitoma extramedular solitario (PES) es una neooplasia maligna infrecuente caracterizada por una proliferación aislada de células plasmáticas monoclonales en tejido extramedular. La localización más frecuente es en cabeza y cuello con predominio en el territorio rinosinusal, sin embargo, estas lesiones malignas representan menos del 1% de los tumores de esta región anatómica. El diagnostico requiere una alta sospecha clínica, análisis histopatológico acucioso, estudios serológicos y exámenes radiológicos sistémicos de acuerdo a los criterios diagnósticos establecidos en la literatura internacional. Se analiza el caso de un paciente masculino con un PES que se presentó como un tumor de fosa nasal derecha y obstrucción nasal de meses de evolución con hallazgos clínicos e imagenológicos inespecíficos. El diagnóstico definitivo se realizó mediante biopsia endoscópica nasal y estudio histopatológico. El tratamiento fue abordado de manera multidisciplinaria entre otorrinolaringología, hematología y radiooncología. De acuerdo a las guías internacionales, se decidió realizar radioterapia localizada con buen resultado clínico precoz. El PES requiere un abordaje multidisciplinario para lograr un diagnóstico y tratamiento oportuno, siendo imprescindible la exclusión del mieloma múltiple debido a las diferencias terapéuticas y en pronóstico clínico. El tratamiento puede realizarse con radioterapia y/o cirugía, siendo la radioterapia el pilar de tratamiento.

Abstract Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm characterized by isolated proliferation of monoclonal plasma cells in extramedullary tissue. The most frequent location is in the head and neck with a predominance in the rhinosinusal territory; however, these malignant lesions represent less than 1% of the tumors in this anatomical region. The diagnosis requires a high clinical suspicion, careful histopathological analysis, serological studies and systemic radiological examinations according to the diagnostic criteria established in the international literature. We analyze the case of a male patient with SEP that presented as a tumor of the right nostril and nasal obstruction of months of evolution with nonspecific clinical and imaging findings. The definitive diagnosis was made by nasal endoscopic biopsy and histopathological study. The treatment was approached by multidisciplinary teamwork. According to international guidelines, it was decided to perform localized radiotherapy with good early clinical results. SEP requires a multidisciplinary approach to achieve a timely diagnosis and treatment, being essential exclusion of multiple myeloma due to the therapeutic differences and prognosis. Treatment can be done with radiation therapy and/or surgery; radiation therapy is the mainstay of treatment.

Unique volatile metabolite signature of sinonasal inverted papilloma detectable in plasma and nasal secretions.

BACKGROUND: Sinonasal inverted papilloma (SNIP) is a benign neoplasm with aggressive features, including a high recurrence rate and a propensity for malignant transformation. Accurate diagnosis with complete resection and the need for close long-term surveillance is widely accepted as standard management. In this study, we investigate whether SNIP produces a unique volatile metabolite signature, which may ultimately lead to a novel approach to diagnose and monitor SNIP. METHODS: Whole blood and nasal secretions from patients with SNIP and healthy age-, sex-, and smoking-status-matched controls, were collected. There were 56 blood samples and 42 nasal secretion samples collected. The volatile metabolite signature of SNIP plasma and nasal secretion samples were compared with those of healthy controls using chromatography. RESULTS: Seventy-two volatiles were identified in plasma samples. Multivariate analysis of variance results, even when controlled for smoking status, indicated toluene as a significant univariate result with lower levels of toluene identified in SNIP plasma samples than healthy control plasma samples. A linear discriminant analysis (LDA) model for plasma volatiles correctly classified 23 of 24 SNIP patients and 26 of 27 control patients, with a cross-validation error rate of 6.02%. Sixty-nine volatiles were identified in nasal samples. For nasal secretion samples, no single univariate response was significant. The LDA model correctly classified 21 of 21 SNIP patients and 11 of 12 control patients, with a cross-validation error rate of 6.55%. CONCLUSION: This study suggests that SNIP produces a unique, detectable volatile metabolite signature. With further investigation, this can have dramatic clinical implications for diagnosis and monitoring. Although most volatile metabolite studies have investigated solid-organ malignancy, this novel study addresses a benign sinonasal neoplasm by using nasal secretions and plasma as an analysis medium, representing the first such study.

Excisional biopsy and radiotherapy for management of an olfactory neuroblastoma in an axolotl (Ambystoma mexicanum).

CASE DESCRIPTION: A 4-year-old sexually intact male leucistic axolotl (Ambystoma mexicanum) was presented with a 2-week history of dysrexia and difficulty swallowing. CLINICAL FINDINGS: Physical examination revealed a 1-cm-diameter intraoral mass on the rostral aspect of the palate and swelling of the left nasal fossa. Local invasion into the left nasal fossa was suspected during oral examination. The lesion was marginally excised, and an incompletely excised olfactory neuroblastoma was diagnosed histologically. Five weeks later, physical examination revealed persistent erythema, delayed healing of the rostral portion of the palate, and a mild facial deformity associated with a white mass in the nasal cavity. TREATMENT AND OUTCOME: 6 weeks after excision, adjuvant electron (6-MeV) beam radiotherapy was initiated for treatment of the incompletely excised olfactory neuroblastoma and likely presence of a recurrent mass. The protocol consisted of 4 weekly fractions of 8 Gy each (total, 32 Gy) with the axolotl under anesthesia. No acute adverse radiation effects were noted following radiotherapy. The oral erythema resolved after the third session. No recurrence was observed 2 months after treatment, and the owners reported no abnormal signs at home. The axolotl died 3.5 months after radiotherapy was completed (8 months after marginal excision of the tumor) secondary to an environmental management failure. Postmortem histologic evaluation showed no evidence of neoplasia. CLINICAL RELEVANCE: In axolotls, olfactory neuroblastoma should be considered in the differential diagnosis of intraoral palatal masses. This report describes the first application of radiotherapy for treatment of an olfactory neuroblastoma in an axolotl.

Clinical, anatomopathological, and immunohistochemical findings of a transitional cell carcinoma from nasal cavity, frontal and ethmoidal sinus with meningoencephalic invasion in a dog.

Background: Primary neoplasms of the nasal cavity and sinuses are uncommon in domestic animals, most of which are of epithelial origin, being adenocarcinoma the most common tumor diagnosed in this region. Some malignant nasal cavity neoplasms may invade the brain causing clinical neurological signs, as well as purulent nasal secretion and epistaxis. Case Description: A case of neoplasm is reported in a 14-year-old pincher presenting dyspnea, epistaxis, and neurological alterations. Necropsy revealed the presence of a mass in the oral cavity vestibule, and another in the whole nasal cavity with invasion of the cribiform plate, meninges and brain. Squamous cells carcinoma was diagnosed in the oral cavity and transitional carcinoma in the nasal cavity. The immunohistochemistry confirmed that the brain infiltration was of the same origin as the nasal cavity neoplasm. Conclusion: The present report describes a rare case of transitional carcinoma of the nasal cavity as well as the frontal and ethmoidal sinuses with brain invasion, confirmed by immunohistochemistry. It is extremely important for veterinarians to include neoplasms in their differential diagnoses, when these animals show chronic respiratory signs and neurological alterations that do not improve with appropriate treatment, always associating with complementary exams, for correct diagnosis establishment and prognosis formulation.

Quiste dermoide nasofrontal: presentación de un caso y revisión narrativa / Nasofrontal dermoid cyst: case presentation

Las lesiones de la región nasofrontal en los niños son un reto diagnóstico debido a su rareza, y su potencial comunicación con el sistema nervioso central también aumenta su complicaciones. Dentro de las principales entidades de esta región se encuentran los quistes dermoides, los gliomas nasales y los encefaloceles. Un abordaje diagnóstico y terapéutico inapropiado podría generar desde simples recurrencias hasta fistulas e infecciones en el sistema nervioso central, que podrían contribuir a mayores complicaciones o incluso, poner en riesgo la vida de los pacientes.

Injuries to the naso-frontal region in children are a diagnostic challenge, associated with their rarity, their complexity also implies their potential communication with the central nervous system. Dermoid cysts, nasal gliomas, and encephaloceles are among the main entities in this region. An inappropriate diagnostic and therapeutic approach could generate from simple recurrences (as in our case), to fistulas and infections of the central nervous system that could contribute to greater complications or even put the lives of patients at risk.

Anaplastic extraosseous (extramedullary) plasmacytoma in the right nostril.

Extraosseous (extramedullary) plasmacytomas are rare plasma cell neoplasms that can result in an erroneous and/or delayed diagnosis as often they are not considered in the differential diagnosis due to their rarity. Furthermore, the anaplastic type is one of the most difficult to recognize in biopsies. We report the case of a patient with an extraosseous plasmacytoma occluded in the right nostril. Its prompt and accurate diagnosis resulted in early treatment and a good outcome, despite the tumour being anaplastic with the risk of progressing into a plasma cell myeloma. The patient shows no recurrence or disease progression after 10 years of follow-up. Our case highlights the clinical and pathological characteristics of this rare disorder that should be considered in order to improve diagnostic criteria and thus early treatment. We also reviewed the pertinent literature.

Angiofibroma nasoseptal: reporte de un caso y revisión / Nasal septal angiofibroma: case report and review

RESUMEN Paciente de 32 años que acudió a consultas por epistaxis intermitente. A la exploración, se evidenció una masa proveniente del septum en fosa nasal izquierda no sospechosa, que a la biopsia demostró ser un angiofibroma. Se describe el abordaje terapéutico de un angiofibroma extranasofaríngeo septal y una revisión de la literatura.

ABSTRACT A 32-year-old patient attended our department for intermittent epistaxis. Upon examination, a non-suspicious mass coming from the septum was found in the left nostril, which at biopsy proved to be an angiofibroma. We describe the therapeutic approach of a septal extranasopharyngeal angiofibroma and a review of the literature.

Adenoma pleomorfo originado en pared lateral nasal: reporte de un caso clínico / Pleomorphic adenoma of lateral nasal wall: a case report

RESUMEN El adenoma pleomorfo constituye la neoplasia benigna más frecuente de las glándulas salivales mayores, y puede también presentarse en otros sitios con mucha menor frecuencia como orofaringe, hipofaringe y nasofaringe. El adenoma pleomorfo intranasal es muy infrecuente y los casos descritos en la literatura local se refieren a tumores septales. Se presenta un caso clínico de una paciente que consulta por obstrucción nasal unilateral a derecha asociado a, epistaxis y epífora ipsilateral con estudio imagenológico y biopsia que sugiere adenoma pleomorfo de la pared lateral nasal. Se realiza revisión bibliográfica al respecto.

ABSTRACT The pleomorphic adenoma is the most frequent benign neoplasm of the major salivary glands. It can also present itself in other places with much less frequency such as oropharynx, hypopharynx and nasopharynx. The intranasal pleomorphic adenoma is very unusual and the cases described in the local literature address septal tumors. A clinical case is presented of a patient who consulted for unilateral right nasal obstruction associated with epistaxis and ipsilateral epiphora with imaging study and biopsy suggesting pleomorphic adenoma of the lateral nasal wall. A bibliographic review is made in this regard.

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.

Detection of enzootic nasal tumor virus (ENTV) in a sheep flock in southern Brazil.

Enzootic nasal tumor (ENT) is a contagious neoplasm associated with enzootic nasal tumor virus (ENTV), which may induce disease in sheep (ENTV-1) and goats (ENTV-2). This study aimed to describe the occurrence of ENT in two Texel sheep (Ovis aries) from a 75-sheep flock, located in the city of Gravataí, southern Brazil. Animals used to be purchased from different origins, and no specific tests for disease monitoring or quarantine procedure were performed. Affected animals presented respiratory distress, anorexia with severe weight loss, and mucopurulent unilateral nasal discharge. Necropsy was performed in both animals and nasal cavity masses were observed. Histopathological analysis demonstrated an epithelial neoplasm compatible with nasal adenocarcinoma. PCR using a protocol that amplifies a 591 bp sequence of 5'LTR-gag region of ENTV1 was performed followed by DNA sequencing. Both samples were positive, and the sequences obtained presented highest identity (97%) with ENTV strain TN28 (GenBank accession number MH899613) detected in a Texel sheep from Scotland. This is the first report of ENTV-1 leading to enzootic nasal tumor in sheep in Latin America, which confirms the presence of the retrovirus in sheep flocks in the Brazilian territory.

Angiocentric Centrofacial Lymphoma as a Challenging Diagnosis in an Elderly Man.

BACKGROUND Angiocentric centrofacial lymphomas, now known as nasal-type extranodal natural killer T-cell lymphomas, are neoplasms of highly destructive characteristics that mainly affect the nasal cavity and palate. The most frequent clinical presentation includes fever, weight loss, nasal obstruction, epistaxis, nasal or facial edema, as well as necrotic ulcers in the nasal cavity, gums, and palate. It has been found to have an important association with the Epstein-Barr virus. Diagnostic pathology could be difficult due to the typical widespread tissue necrosis. CASE REPORT A 72-year-old Caucasian male sought medical attention with a chief complaint of nasal obstruction for the past 3 years, which only responded partially to unspecific treatment. He also presented with intermittent fever and nocturnal hyperhidrosis. Physical examination with rhinoscopy demonstrated a deviated septum, congestive turbines, and fragile and pale mucous membrane with yellowish, thick mucus. The pathology report described an angiocentric centrofacial lymphoma and a positive serology for Epstein-Barr virus. CONCLUSIONS The objective of this case report was to show that this illness represents a diagnostic challenge for the treating physician. It may be concluded that despite the poor prognosis of the disease, this particular case showed slower evolution and the patient remained stable despite multiple consecutive complications.

Nasal-type extranodal T-cell/NK lymphoma in association with hemophagocytic syndrome.

Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.

Hamartoma condromesenquimal nasoetmoidal con extensión intracraneal: a propósito de un caso / Nasoethmoidal condromesenchymal hamartoma with intracranial extension: a case report

El Hamartoma Condromesenquimal Nasal (HCMN) es una neoplasia descrita por McDermott et al en 1998 en pacientes pediátricos, con pocos casos reportados en adultos. Este tumor está constituido por tejido condroide o cartilaginoso así como por tejidos mesenquimales. Es una lesión expansiva, localmente destructiva y en algunos casos está descrita la extensión intracraneal. El comportamiento es habitualmente benigno y el tratamiento consiste en la resección quirurgica. Se presenta un caso de un paciente masculino de 8 meses de edad, quien presentaba aumento de volumen en región periorbitaria derecha, con pseudoptosis palpebral, paresia del nervio oculomotor derecho y rinorrea hialina. Mediante la Tomografia Computarizada (TC) craneal y la Resonancia Magnética (RM) cerebral se observó lesión ocupante de espacio naso fronto etmoidal. El estudio histopatológico e inmunohistoquimico determinó la presencia de tumor mesenquimal fibrocartilaginoso osificante: Hamartoma Condromesenquimal. Se revisan las características y el tratamiento de este tumor poco frecuente(AU)

The Chondromesenchymal Nasal Hamartoma (CMNH) is a rare neoplasm described in 1998 by McDermott et al in the pediatric age, with few cases reported in adults. This tumor is composed by chondroid or cartilaginous tissue as well as mesenchymal tissues. It is an expansive lesion, locally destructive and in some cases intracranial extension is described. Its behavior is usually benign and its treatment consists of surgical resection. The authors present an 8-month-old male patient with increase volume in the right periorbital region, palpebral ptosis, paresis of the right oculomotor nerve and hyaline rhinorrhea. Computed Tomography (CT) and Cerebral Magnetic Resonance Imaging (MRI) were perfomed and revealed a nasofrontoethmoidal space occupying lesion; a histopathological and inmunohistochemical study resulted in osseous fibrocartilaginous mesenchymal tumor: chondromesenchymal hamartoma. We review the characteristics and treatment of this rare tumor(AU)

Nasal-type extranodal T-cell/NK lymphoma in association with hemophagocytic syndrome

Abstract: Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.

Angiomiolipoma de cavidad nasal: reporte de un caso y revisión de la literatura / Angiomyolipoma of the nasal cavity: case report and literature review

El angiomiolipoma de cavidad nasal es un tumor hamartomatoso extremadamente infrecuente compuesto por adipocitos maduros, espacios vasculares con escaso tejido elástico y la presencia de haces de células musculares lisas maduras. Se manifiesta principalmente por obstrucción nasal unilateral y epistaxis recurrente. Se presenta el caso de una paciente de 73 años con historia crónica de obstrucción nasal y epistaxis recurrente izquierda asociada a rinorrea y descarga posterior intermitente. La tomografía computarizada (TC) y resonancia nuclear magnética (RNM) demuestran una lesión vascular circunscrita en fosa nasal izquierda. La angiografía demostró irrigación exclusiva de la arteria esfenopalatina izquierda. Se realizó extirpación de la lesión por abordaje endonasal endoscópico previa embolización arterial. La revisión de la literatura mundial muestra que es el duodécimo caso de angiomiolipoma de cavidad nasal reportado.

Angiomyolipoma of nasal cavity is an extremely rare hamartoma tumor composed of mature adipocytes, vascular spaces with lack of elastic tissue, and the presence of bundles of mature smooth muscle cells. It manifests itself mainly by unilateral nasal obstruction and recurrent epistaxis. We present the case of a 73-years-old woman with chronic history of left-sided nasal obstruction and recurrent epistaxis associated with rhinorrhea and intermittent post nasal drip. Computed tomography and magnetic resonance imaging demonstrate a vascular lesion inside the left nasal cavity. Angiography showed irrigation exclusively by the left sphenopalatine artery Surgical excision was performed by endoscopic endonasal approach after arterial embolization. World literature review showed that this is the twelfth reported case of angiomyolipoma of the nasal cavity.

[Extranodal NK-T-cell lymphoma, nasal type in granulomatosis with polyangiitis. A case report]. / Linfoma T/NK nasal en granulomatosis con poliangeítis. Reporte de un caso.

BACKGROUND: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. CLINICAL CASE: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection. It was performed an oropharynx lesion biopsy and the diagnosis was extranodal NK-T-cell lymphoma, nasal type, and positive for CD56 and granzyme. CONCLUSION: Extranodal NK-T-cell lymphoma, nasal type is a rare entity, of poor prognosis, that should be considered as a diagnosis in patients with GP unresponsive to steroid. That is the reason why biopsy of the lesion and immunohistochemistry are required.

Introducción: la granulomatosis con poliangeítis (GP) es una vasculitis sistémica necrosante con afección multiorgánica que afecta principalmente el tracto respiratorio y los riñones. La fiebre en estos pacientes se considera indicador de actividad, pero si se presenta de forma aislada, deben descartarse otras causas. Caso clínico: paciente de sexo masculino que ingresó por fiebre de origen desconocido y pérdida de peso; se le diagnosticó granulomatosis con poliangeítis por antecedente de glomerulonefritis pauciinmune, nódulos pulmonares fijos y sinusitis crónica de cuatro años de evolución; sin embargo, la fiebre persistió a pesar del tratamiento y en ausencia de infección. Se realizó biopsia de úlcera faríngea que reportó linfoma de células T/NK de tipo nasal ulcerado positivo para CD56 y granzima. Conclusión: el linfoma T/NK nasal es una rara entidad, de mal pronóstico, que debe considerarse en pacientes con GP que no responden a esteroide, por lo que requieren biopsia de la lesión e inmunohistoquímica.

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.

Schwannoma nasal / Nasal Schwannoma

Introdução: O Schwannoma é uma neoplasia benigna com origem nas células de Schwann presentes na bainha de nervos autossômicos periféricos. Localiza-se cranial ou extracranial. Na cabeça e pescoço constitui 30 a 45% dos casos extracraniais, sendo que 4% deles estão localizados no nariz e seios paranasais. Relato do caso: Paciente feminina, 37 anos, realizou tomografia de crânio após queda para investigar trauma cranioencefálico. Esta identificou velamento de seios paranasais à esquerda. O achado motivou a investigação da hiposmia e obstrução nasal à esquerda de longa data. À rinoscopia anterior, observou-se lesão expansiva polipoide ocupando a fossa nasal, não permitindo a passagem do nasofibroscópio. Tomografia computadorizada evidenciou lesão ocupando totalmente a fossa nasal esquerda, velamento do seio maxilar e células etmoidais anteriores e posteriores à esquerda. Realizou-se exérese endoscópica da lesão, que tinha provável origem no recesso esfenoetmoidal. A biópsia transoperatória com congelação observou lesão inflamatória. A confirmação do diagnóstico de Schwannoma foi feita pelo anatomopatológico e imuno-histoquímica, que evidenciou alta reatividade à proteína S-100. Discussão: As lesões intranasais unilaterais apresentam inúmeras possibilidades diagnósticas. Os sintomas são inespecíficos e os exames de imagem pouco esclarecedores. Neste relato, a tomografia sugeriu pólipo de Killian ou papiloma invertido pela unilateralidade da lesão. Porém, as características histopatológicas e a imunorreatividade à proteína S-100 auxiliaram na confirmação diagnóstica. Conclusão: O Schwannoma intranasal faz parte do diagnóstico diferencial das lesões intranasais. O exame histopatológico e a imunohistoquímica são de extrema importância para confirmação diagnóstica frente à inespecificidade dos sintomas e achados radiológicos (AU)

Introduction: Schwannoma is a benign neoplasm originating in the Schwann cells present in the sheath of peripheral autosomal nerves. It is located cranially or extracranially. In the head and neck it comprises 30 to 45% of extracranial cases, 4% of which located in the nose and paranasal sinuses. Case report: A 37-year-old female patient underwent skull tomography after a fall to investigate cranioencephalic trauma. This identified veiling of paranasal sinuses on the left. The finding motivated the investigation of long-standing hyposmia and nasal obstruction on the left. At anterior rhinoscopy, an expansive polypoid lesion occupying the nasal fossa was observed, not allowing the nasofibroscope to pass. Computed tomography revealed a lesion totally occupying the left nasal fossa, maxillary sinus veil, and anterior and posterior ethmoid cells on the left. Endoscopic excision of the lesion that was likely to originate in the sphenoethmoidal recess was performed. Intraoperative biopsy with freezing showed an inflammatory lesion. Confirmation of the Schwannoma diagnosis was made by pathology and immunohistochemistry that showed high reactivity to S-100 protein. Discussion: Unilateral intranasal lesions present numerous diagnostic possibilities. The symptoms are nonspecific and imaging exams are not very elucidating. In this report, CT scan suggested a Killian polyp or inverted papilloma due to the unilaterality of the lesion. However, the histopathological characteristics and immunoreactivity to the S-100 protein aided in the diagnostic confirmation. Conclusion: Intranasal Schwannoma is part of the differential diagnosis of intranasal lesions. Histopathological examination and immunohistochemistry are extremely important for diagnostic confirmation in the face of the non-specificity of symptoms and radiological findings (AU)