Tumor nasal de músculo liso de comportamiento maligno incierto: informe de un caso / Nasal smooth muscle tumor of uncertain malignant potential: A case report

Los tumores de músculo liso que no pueden ser clasificados según su histología como leiomiomas o leiomiosarcomas se denominan tumores de músculo liso de comportamiento maligno incierto. La localización nasal de estos tumores es muy infrecuente y la extensión adecuada de la cirugía para tratar estas neoplasias no está bien definida. Se describe el caso clínico de una adolescente de 16 años, que consultó por padecer un tumor de aspecto vascular en la cavidad nasal derecha y que fue tratada con éxito mediante cirugía intranasal. El diagnóstico histológico fue tumor de músculo liso de comportamiento maligno incierto. Por la rareza de estas neoplasias, su infrecuente localización nasal y la falta de evidencia que soporte cuál debe ser la extensión de la cirugía, es relevante la descripción y discusión del caso clínico.

Smooth muscle tumors that cannot be histologically classified as leiomyomas or leiomyosarcomas are defined as smooth muscle tumors of uncertain malignant potential. The location of these tumors in the nose is very rare, and the appropriate surgical extent to manage these neoplasms has not been adequately defined. Here we describe the case of a 16-year-old female adolescent who consulted due to a vascular-like tumor in the right nasal cavity who was successfully treated with intranasal surgery. The histological diagnosis was smooth muscle tumor of uncertain malignant potential. Given that these neoplasms are rare, the uncommon location in the nose, and the lack of evidence indicating the extent of surgery, it is relevant to describe and discuss this clinical case.

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.

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.

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)