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)

Schwannoma septal, una causa infrecuente de obstrucción nasal / Septal schwannoma, an uncommon cause of nasal obstruction

Resumen Los schwannomas son neoplasias derivadas de las células de Schwann de la cubierta de los nervios periféricos. Su desarrollo en la región nasosinusal es poco frecuente, especialmente a nivel septal. Su diagnóstico diferencial es variado y debe establecerse con otras causas más habituales de masa nasal unilateral. Su tratamiento es quirúrgico. Describimos el caso de un varón de 47 años con una masa nasal derecha intervenida mediante cirugía endoscópica nasosinusal y con diagnóstico anatomopatológico de schwannoma septal.

Abstract Schwannomas are tumors that proceed from Schwann cells in the cover of peripheral nerves. It is uncommon in the sinonasal area, especially in the nasal septum. The differential diagnosis is extensive and requires contemplating other more frequent causes of unilateral nasal mass. The current treatment of septal schwannoma is surgical. We report a 47-year-old male with a right nasal mass operated by endoscopic sinonasal surgery with an anatomopathological diagnosis of a nasal septal schwannoma.

Enfrentamiento de pacientes Testigo de Jehová en cirugía otorrinolaringológica: a propósito de un caso de resección de tumor rinosinusal / Jehovah's Witnesses patients in otorhinolaryngological surgery: a case regarding a sinonasal tumor resection

Las cirugías de cabeza y cuello presentan un alto riesgo de sangrado que puede significar el uso de transfusiones sanguíneas. Existen casos en las que éstas son rechazadas, como sucede con los Testigos de Jehová. Se expone el caso de una paciente Testigo de Jehová con un tumor rinosinusal con alto riesgo de sangrado que consultó por epistaxis recurrente. Se evidencia un tumor ocluyendo la fosa nasal derecha de aspecto vascular a la rinoscopía y la tomografía computarizada. Múltiples aferencias de la arteria esfenopalatina y etmoidales se observaron en una angiografía cerebral. Previo a la resección, se embolizó la arteria maxilar. Durante la cirugía, se contó con un sistema de recuperación de sangre autóloga, hemodilución e infusión de ácido tranexámico. Se ligó la arteria etmoidal anterior derecha vía externa con apoyo endoscópico y luego se resecó el tumor vía endoscópica. La biopsia reveló un carcinoma sinonasal escamoso. Existen alternativas terapéuticas en pacientes que rechacen el uso de hemoderivados. Destacan medidas preoperatorias como la embolización endovascular, intraoperatorias como el uso de agentes hemostáticos, técnicas quirúrgicas y anestésicas. Es importante analizar todas las opciones disponibles de forma multidisciplinara y junto con el paciente, para determinar la conducta más adecuada a seguir.

Head and neck surgeries have a high risk of bleeding, and therefore could require the use of blood transfusions. There are cases for which blood transfusions are not an acceptable option, as is the case for Jehovah's Witnesses. We present the case of a Jehovah's Witness with a sinonasal tumor with a high risk of bleeding, who presented with recurrent epistaxis. Rhinoscopy and computed tomography revealed a vascular-like tumor occluding the right nasal cavity. Cerebral angiography showed afferents of the sphenopalatine and ethmoidal arteries leading to the tumor. Prior to the resection, the maxillary artery was embolized. During surgery, we relied on an autologous blood recovery system, hemodilution and tranexamic acid. Right anterior ethmoidal artery ligation was performed by an endoscopic assisted external approach. The tumor was resected endoscopically The biopsy revealed a squamous sinonasal carcinoma. There are therapeutic alternatives for patients who cannot receive blood products. There are preoperative measures such as endovascular embolization, intraoperative measures such as the use of hemostatic agents and specific surgical or anesthetic techniques. It's important to analyze all of the available options in a multidisciplinary team approach, and to take into consideration the patient's preferences, in order to determine the best surgical conduct.

Leiomiosarcoma primario de cornete nasal inferior: reporte de caso clínico y revisión de la literatura / Primary leiomyosarcoma of inferior nasal turbinate: clinical case report and literature review

Los leiomiosarcomas de la cavidad nasal y senos paranasales son neoplasias malignas de baja frecuencia, localmente agresivas. Presentan una alta tendencia a la recurrencia de aproximadamente 55% en nariz y senos paranasales. Están conformados por células musculares lisas. Según diversos autores, aproximadamente el 50% de los pacientes mueren antes del primer año y la supervivencia a los 5 años es del 20%. Anteriormente se pensaba que no poseían potencial metastásico, sin embargo, en series actuales se ha visto que presentan un alto poder metastásico de hasta el 50%. Las metástasis se presentan de forma tardía. El tratamiento recomendado consiste en la resección radical del tumor primario con un amplio margen de tejido normal y la radioterapia es de uso controversial en el manejo.

Leiomyosarcoma of the nasal cavity and paranasal sinuses are malignant, low frequency, locally aggressive neoplasm. They present a high tendency to recurrence of approximately 55% in the nose and paranasal sinuses. They are made up of smooth muscle cells. According to different authors, approximately 50% of patients die before the first year and survival at 5 years is 20%. Previously it was thought that they did not possess metastatic potential, however in current series it has been seen that they have a high metastatic power of up to 50%. Metastases present late. The recommended treatment consists of radical resection of the primary tumor with a wide margin of normal tissue.

Sinonasal organising haematoma - a little known entity / Hematoma nasossinusal em organização - uma entidade pouco conhecida

Abstract Introduction: Sinonasal organising haematoma is a recently described, rare, benign inflammatory condition, which closely resembles malignancy in its clinical presentation. Objective: To describe the clinical features of organising haematoma and to review the evolution of surgical options successfully used. Methods: A retrospective review of charts of all patients with a histopathological diagnosis of sinonasal organising haematoma was performed. Results: Six (60%) of the 10 patients were male with a mean age of 47.4 years. All patients had unilateral disease with recurrent epistaxis as the presenting symptom. Maxillary sinus was the most commonly involved sinus. There was no history of trauma in any of the patients. Hypertension (80%) was the most commonly associated comorbidity. Contrast-enhanced CT scan of the paranasal sinuses showed heterogeneous sinus opacification with/without bone erosion. Histopathological examination was diagnostic. Complete endoscopic excision was done in all patients resulting in resolution of the disease. Conclusion: Awareness of this relatively new clinical entity and its evaluation and treatment is important for otolaryngologists, maxillofacial surgeons and pathologists alike. Despite the clinical picture of malignancy, histopathological features of benign disease can safely dispel such a diagnosis.

Resumo Introdução: Hematoma nasossinusal em organização é uma condição inflamatória benigna rara, recentemente descrita, que se assemelha a lesões malignas em sua apresentação clínica. Objetivo: Descrever as características clínicas do hematoma em organização e analisar a evolução das opções cirúrgicas usadas com sucesso. Método: Foi feita a revisão retrospectiva dos prontuários de todos os pacientes com diagnóstico histopatológico de hematoma nasossinusal em organização. Resultados: Seis (60%) dos 10 pacientes eram do sexo masculino, com média de 47,4 anos. Todos os pacientes apresentavam doença unilateral com epistaxe recorrente como sintoma de apresentação. O seio maxilar era o mais comumente afetado. Não havia histórico de trauma em qualquer dos pacientes. Hipertensão (80%) foi a comorbidade mais comumente associada. A tomografia computadorizada dos seios paranasais com contraste mostrou opacificação heterogênea do seio com/sem erosão óssea. O exame histopatológico foi diagnóstico. A excisão endoscópica completa foi feita em todos os pacientes, resultou na resolução da doença. Conclusão: A conscientização a respeito dessa entidade clínica relativamente nova e sua avaliação e tratamento são importantes para os otorrinolaringologistas, cirurgiões buco-maxilo-faciais e patologistas. Apesar do quadro clínico de malignidade, as características histopatológicas da doença benigna podem descartar com segurança esse diagnóstico.

Linfoma nasal de células T/NK (granuloma letal de la línea media), una neoplasia agresiva: reporte de un caso / Nasal T/NK-cell lymphoma (lethal midline granuloma), an aggressive neoplasia: report of a case

RESUMEN El linfoma nasal de células T/NK es una neoplasia agresiva, infrecuente, con predilección por el sexo masculino. Representa el 1,5% del total de linfomas no Hodgkin, el grupo etáreo más afectado es entre 40 a 80 años. Su etiología es desconocida, pero se ha asociado con el virus de Epstein Barr. Se presenta el caso de una paciente mujer de 40 años, con dolor e induración de fosa nasal derecha asociado a secreción fétida, seropurulenta de 3 meses de evolución. Estudio histopatológico, reporta linfoma nasal de células T/NK. La paciente recibe quimioterapia, con mejoría clínica sustancial. El linfoma T/NK es una neoplasia con sintomatologia inespecífica, predominio que afecta nasofaringe, amígdalas y base de la lengua. Entre las manifestaciones encontramos dolor de garganta, obstrucción nasal, rinorrea, epistaxis y cefalea. El diagnóstico se sospecha con imágenes, pero es necesaria la confirmación histológica con marcadores de inmunohistoquímica CD45Ro, CD43, CD3, CD2, CD45Ro entre otros. Muchos casos suelen detectarse en fase tardía, cuando ya son evidentes los signos radiológicos de destrucción ósea. El diagnóstico diferencial incluye lesiones infecciosas o inflamatorias, la bola fúngica fue el diagnóstico inicial realizado en esta paciente. El tratamiento suele realizarse con radioterapia y quimioterapia.

ABSTRACT Nasal T-cell / NK lymphoma is an uncommon aggressive neoplasm with male predilection. It represents 1.5% of the total number of non-Hodgkin's lymphomas, the most affected age group is 40-80 years. Its etiology is unknown but has been associated with the Epstein Barr virus. We present the case of a female patient of 40 years, with pain and induration of the right nostril associated with fetid secretion, seropurulent of 3 months of evolution. Histopathological study, reports T-cell / NK nasal lymphoma. The patient receives chemotherapy, with substantial clinical improvement. T / NK lymphoma is a neoplasm with predominant non-specific symptomatology, affecting the nasopharynx, tonsils and base of the tongue. Among the manifestations we find sore throat, nasal obstruction, rhinorrhea, epistaxis and headache. Diagnosis is suspected with imaging, but histological confirmation with immunohistochemical markers CD45Ro, CD43, CD3, CD2, CD45Ro, among others is required. Many cases are usually detected in the late phase, when radiological signs of bone destruction are already visible. The differential diagnosis includes infectious or inflammatory lesions, the fungal ball was the initial diagnosis made in this patient. Treatment is usually done with radiotherapy and chemotherapy.

Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

Endoscopic endonasal approach for mass resection of the pterygopalatine fossa

OBJECTIVES: Access to the pterygopalatine fossa is very difficult due to its complex anatomy. Therefore, an open approach is traditionally used, but morbidity is unavoidable. To overcome this problem, an endoscopic endonasal approach was developed as a minimally invasive procedure. The surgical aim of the present study was to evaluate the utility of the endoscopic endonasal approach for the management of both benign and malignant tumors of the pterygopalatine fossa. METHOD: We report our experience with the endoscopic endonasal approach for the management of both benign and malignant tumors and summarize recent recommendations. A total of 13 patients underwent surgery via the endoscopic endonasal approach for pterygopalatine fossa masses from 2014 to 2016. This case group consisted of 12 benign tumors (10 juvenile nasopharyngeal angiofibromas and two schwannomas) and one malignant tumor. RESULTS: No recurrent tumor developed during the follow-up period. One residual tumor (juvenile nasopharyngeal angiofibroma) that remained in the cavernous sinus was stable. There were no significant complications. Typical sequelae included hypesthesia of the maxillary nerve, trismus, and dry eye syndrome. CONCLUSION: The low frequency of complications together with the high efficacy of resection support the use of the endoscopic endonasal approach as a feasible, safe, and beneficial technique for the management of masses in the pterygopalatine fossa.

Linfoma T/NK extraganglionar tipo nasal: caso clínico / Extranodal T/NK-Cell lymphoma nasal type: clinical case

El linfoma T/NK extraganglionar tipo nasal es un linfoma extraganglionar, habitualmente expresa el fenotipo NK y VEB positivo. Cursa ocasionando necrosis y angioinvasión afectando de manera preferente estructuras mediofaciales. Característicamente es muy agresivo. Presentamos un caso con una sobrevida de siete meses a partir de los primeros síntomas y realizamos revisión de la literatura.

Extranodal NK/T-cell lymphoma nasal type, is an extranodal lymphoma, usually with an NK-cell phenotype and EBV possitive. It causes necrosis and angioinvasion, and it is most commonly presenting in the midfacial area. Characteristically it is very aggressive. A case with survival of seven months from the first symptoms is reported and a review of the literature is made.

Condrosarcoma del septum nasal: reporte de un caso y revisión de la literatura / Chondrosarcoma of the nasal septum: a case report and literature review

El condrosarcoma del tabique nasal es una neoplasia poco frecuente. Cuando esto ocurre, el diagnóstico precoz es difícil porque los pacientes generalmente presentan síntomas rinosinusales inespecíficos y en forma tardía. Se presenta un caso clínico de una mujer de 37 años de edad que se presentó con una historia de exoftalmo sin síntomas rinosinusales agregados. El estudio imagenológico mostró una masa de aspecto neoplásico en el tabique nasal con extensión al seno maxilar, celdillas etmoidales, cavidad orbitaria y hacia posterior a la fosa pterigopalatina. Se consideró un tumor naso-orbitario por lo que se complementó estudio con nasofibroscopía flexible que mostró una masa nasal de aspecto liso, brillante, rosada que ocupaba piso, tabique y pared lateral de fosa nasal izquierda que no era separable del tabique nasal. Se realizó biopsia que mostró una histopatología sugestiva de condrosarcoma. Se realizó cirugía por abordaje endoscópico y con apoyo neuroquirúrgico por vía transcraneal. La presentación clínica, diagnóstico y tratamiento de este caso, así como una revisión de la literatura son discutidos.

Chondrosarcoma of the nasal septum is a rare malignancy. When this occurs, early diagnosis is difficult since patients usually present non specific symptoms rhinosinusal and late. There is a clinic case of a 37-year-old women who presented with a history of exophthalmos without added symptoms rhinosinusal. The image study showed a neoplasic mass appearance in the nasal septum with extension to the maxillary sinus, ethmoid cells, orbital cavity and post to the pterygopalatine fossa. We have considered a naso-orbital tumor which was complemented nasofibroscopy flexible nasal showed a pink mass of smooth, shiny, occupied floor, septum and lateral wall of left nasal cavity that was not separable from the nasal septum was considered. We have performed a biopsy wich shows a suggestive chondrosarcoma. Surgery was performed by endoscopic approach and via transcranial neurosurgical support. The clinical presentation, diagnosis and treatment of this case and a review of the literature are discussed.

Tumor mesenquimatoso fosfatúrico de fosa nasal con compromiso intracraneano: reporte de un caso y revisión de la literatura / Nasal fossa phosphaturic mesenchymal tumor with intracraneal compromise: case report and review of the literature

El tumor mesenquimatoso fosfatúrico (TMF) es una enfermedad extremadamente rara. Según evidencia reciente es causado por la sobreexpresión del factor de crecimiento fibroblástico 23 (FGF23), el cual genera hipofosfemia y osteomalacia. A continuación presentamos el caso de un paciente de 42 años con un tumor mesenquimatoso fosfatúrico de fosa nasal izquierda con extenso compromiso intracraneano. Cabe destacar que hasta la fecha hay 142 casos reportados de TMF en la literatura de los cuales solo 11 se ubican en fosa nasaly cavidades sinusales, y sólo dos de ellos ubicados en fosa nasal¹. El paciente tuvo una exitosa resolución quirúrgica con la consecuente normalización de parámetros analíticos (incluido el FGF23), mejoría sintomática y ausenia de recidiva hasta la fecha.

The phosphaturic mesenchymal tumor (PMT) is an extremely rare disease. According to recent evidence is caused by overexpression of fibroblast growth factor 23 (FGF23) which generates hypophosphatemia and osteomalacia. We report the case of a 42 year old patient with a left nasal fossa phosphaturic mesenchymal tumor with intracranial involvement. Should be noted that to date there are 142 reported cases of PMT in the literature of which only 11 are located in nasal fossa and sinus cavities, two of them located in nasal fossa¹. The patient had a successful surgical resolution with consequent normalization of analytical parameters (including FGF23), absence of symptoms and no recurrence to date.

Papiloma invertido nasossinusal: experiência em 30 casos / Inverted sinonasal Papilloma: 30 cases experience

O papiloma invertido nasossinusal (PINS) representa de 0,5% a 4% de todos os tumores nasossinusais, e apresenta controvérsias quanto à sua etiologia, diagnóstico e tratamento.Objetivo: Mostrar nossa experiência em 30 casos de PINS através da avaliação diagnostica, conduta, recorrência e associação com malignização, comparando nossos resultados com os da literatura. Materiais e métodos: Foi realizado um estudo retrospectivo de 30 casos de PINS tratados no período de 1985 a 1998. Resultados: As queixas mais comuns foram obstrução nasal unilateral, rinorréia mucopurulenta, epistaxe e cefaléia. Os locais de acometimento mais comuns foram a fossa nasal, seio maxilar e seio etmoidal. Conclusão: O papiloma invertido nasossinusal é infreqüente, está associado à malignidade em 10% dos casos, e a via utilizada para ressecção deve ser a sublabial com degloving médio facial, com maxilectomia medial, onde não houve recorrência

Inverted sinonasal papilloma (ISP) represents 0,5% a 4% of all sinonasal tumors and we still have controversies about your etiology, diagnoses, malignancy and surgical management. Objective: We demonstrate our experience in 30 cases of ISP by diagnostic evaluation, management, recurrence and association with squamous cell carcinome and compare our results with the literature. Material and Method: We did a retrospective study of 30 cases of ISP between 1985 and 1998. Results: The most common symtoms are unilateral nasal obstruction, purulent rhinorrhea, epistaxis and headache. The usual sites include nasal cavity, maxillary and ethmoidal sinus. Conclusions: ISP is rare, associated with malignancy in 10% of cases and the usual approach to resection must be medial maxillectomy with medio-facial degloving, which provides no recurrence in our statistics.