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.

Variétés histologiques des tumeurs bénignes du septum nasal une série documentaire de 30 cas

Contexte et objectif. Les variétés histologiques de tumeurs du septum nasal sont peu documentées dans les pays d'Afrique. La présenté étude avait pour objectif de décrire les variétés histologiques de tumeurs septales bénignes. Méthodes. C'était une analyse rétrospective de dossiers des patients admis pour tumeurs septales bénignes, entre janvier 1998 et aout 2021 ; à l'hôpital Dr Dorban en Algérie. Les variables d'intérêt englobaient les données cliniques et histopathologiques. Résultats. En 23 ans, seulement trente dossiers de tumeurs bénignes du septum nasal ont été colligés. Leur âge moyen était de 36,2 ans avec les extrêmes entre 13 et 70 ans avec une prédominance du sexe féminin (63,3 %). Les tumeurs vasculaires étaient prépondérantes (83,3 %) en particulier les hémangiomes capillaires (92 %) suivies des variétés épithéliales (13,3%) et un seul adénome pléomorphe septal. L'évolution clinique était émaillée par 3 récidives pour les hémangiomes, et absence de récidive pour l'adénome pléomorphe après 120 mois de recul. Trois autres cas étaient perdus. Conclusion: Les tumeurs bénignes du septum nasal sont rares et diversifiées sur le plan histologique. A coté des formes classiques et communes (hémangiomes, papillomes, adénomes), il existe des variétés rares et qui nécessitent une étude spécifique et une expertise dédiée.

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.

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)

Two Cases of Angioleiomyoma in the Nasal Cavity

Leiomyoma is a benign tumor arising from smooth muscle cells. They are commonly found in the uterus, skin, and gastrointestinal tract. However, it is rare in the head and neck area. Especially, only 3% of leiomyomas in the head and neck area occur in the nasal cavity. Rhinologists need to know leiomyoma as a differential diagnosis of the tumors in the nasal cavity. Herein we present two cases of angioleiomyoma in the nasal cavity and review the literature.

Two Cases of Angioleiomyoma in the Nasal Cavity

Leiomyoma is a benign tumor arising from smooth muscle cells. They are commonly found in the uterus, skin, and gastrointestinal tract. However, it is rare in the head and neck area. Especially, only 3% of leiomyomas in the head and neck area occur in the nasal cavity. Rhinologists need to know leiomyoma as a differential diagnosis of the tumors in the nasal cavity. Herein we present two cases of angioleiomyoma in the nasal cavity and review the literature.

Clinical Experience of Sinonasal Schwannomas

BACKGROUND: Schwannomas are benign tumors of ectodermal origin derived from Schwann cells of the nerve sheath. Approximately less than 4% of these tumors are found in the paranasal sinuses and there has been little information reported concerning the presentation and surgical management of sinonasal schwannomas. The purpose of this study was to analyze the clinical data, management, and long-term outcomes of sinonasal schwannomas. METHODS: Retrospective chart review of patients with sinonasal schwannomas treated from January 2001 to March 2012 was performed. Clinical data and follow-up information were obtained from a review of the patients' charts and the operative, anesthesia, and pathology reports. RESULTS: There were 4 females and 4 males included in this study. The mean age was 37.5 years (range, 22-51 years). The mean tumor size was 3.1 cm (range 1.0-6.0 cm). The origin of the tumors included: nasal septum (n = 2), nasal vestibule (n = 2), pterygopalatine fossa (n = 2), ethmoid sinus (n = 1), and inferior turbinate (n = 1). Seven patients had endoscopic resections and one patient with a schwannoma in the nasal vestibule underwent a sublabial approach. The mean follow-up was 59 months. There were no tumor recurrences during the study period. CONCLUSIONS: Schwannomas in sinonasal cavity can be treated effectively with the endoscopic approach with minimal morbidity and long-term disease control.

Nasal neuroendocrine carcinoma: a clinicopathological analysis / 解放军医学杂志

Objective To analys the clinicopathological features and pathological diagnosis and differential diagnosis of the nasal neuroendocrine carcinomas (NEC). Methods 12 cases of nasal neuroendo crine carcinomas were studied with clinical datas and light microscopic examination and electron microscopic and immunohistchemical staining. Results The majority of the patients were middleaged. The patient population was composed of 10 meles and 2 females(sex ratio,5:1) with average age 39 6 years. On pathological examination,8 of them were small cell type neuroendocrine carcinoma,3 large cell type neuroendocrine carcinoma and 1 atypical ueuroendocrine carcinoma. Electron microscopy of 8 cases showed abundant neuroendocrine granules in the tumors. Immunohistochemical analysis showed that cells expressed EMA,CK,CgA,NSE and Syn Conclusions The key point for clinicopathological features of the NEC were discussed. Its differentila diagnosis includes malignant lymphoma, undifferentiatel carcinoma, malignant melanoma and olfactory neuroblastoma. Histological type of the tumor is important for clinical treatment plan and prognostic evaluation