Tratamiento multimodal en los schwanomas vestibulares grandes / Multimodal Treatment for Large Vestibular Schwannomas

Introducción: Los schwanomas vestibulares son lesiones clasificadas como grado I por la Organización Mundial de la Salud. Las opciones conductuales incluyen la observación, la radiocirugía o la resección microquirúrgica endoscópica. Objetivo: Describir el rol del tratamiento multimodal en los schwanomas vestibulares. Métodos: Se realizó una búsqueda sistemática de literatura médica para la identificación e inclusión de artículos en las siguientes bases de datos: PubMed/Medline, SciELO y Google Scholar, además de los servidores de preprints BioRvix y MedRvix. Se usaron los siguientes descriptores: vestibular schwanoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Se excluyeron editoriales, cartas al editor, libros, revisiones, metaanálisis y aquellos artículos con método deficiente de más de 20 años de publicación o sin mención al tema de interés. Fueron incluidos 16 artículos. Desarrollo: Los artículos incluyeron un total de 699 pacientes, de los cuales solo 228 (32,6 porciento) recibieron radioterapia en el período posoperatorio. El período de seguimiento promedió 49,6 meses. La preservación de la función facial se logró en el 88,3 porciento de los casos y el control tumoral en el 80,7 porciento. El estado de la preservación auditiva solo fue informado en el 50 porciento de los estudios. El uso de la radioterapia adyuvante mostró gran variabilidad. Conclusiones: El tratamiento multimodal para los schwanomas vestibulares grandes -compuesto por la resección subtotal, vaciamiento intratumoral, seguido de radiocirugía- se ha convertido en una opción plausible. Se necesita la publicación de mayor cantidad de reportes para ofrecer recomendaciones y estratificar la conducta(AU)

Introduction: Vestibular schwannomas are lesions classified as grade I by the World Health Organization. Behavioral options include observation, radiosurgery or endoscopic microsurgical resection. Objective: To describe the role of multimodal treatment for vestibular schwannomas. Methods: A systematic search of medical literature, for the identification and inclusion of articles, was carried out in the databases PubMed/Medline, SciELO and Google Scholar, as well as in the preprint servers BioRvix and MedRvix. The following descriptors were used: vestibular schwannoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Editorials, letters to the editor, books, reviews and metaanalyses were excluded, as well as articles with deficient method of more than 20 years of publication or not mentioning the topic of interest. Sixteen articles were included. Development: The articles included a total of 699 patients, of which only 228 (32.6 ) received radiotherapy in the postoperative period. The average follow-up period was 49.6 months. Preservation of facial function was achieved in 88.3 percent of cases; and tumor control, in 80.7 percent. Hearing preservation status was reported in only 50 percent of the studies. The usage of adjuvant radiotherapy showed great variability. Conclusions: Multimodal treatment for large vestibular schwannomas -composed of subtotal resection and intratumoral draining, followed by radiosurgery- has become a plausible option. The publication of a greater amount of reports is necessary to provide recommendations and stratify the behavior(AU)

Schwanoma palatino en paciente pediátrico de 12 años: una rara presentación clínica

El schwanoma es un tumor neural que se origina en las células de Schwann presentes en la vaina nerviosa cuya localización más frecuente a nivel oral es el dorso de lengua, siendo mucho más frecuente en adultos. En población pediátrica la presentación es muy rara siendo lengua su ubicación más frecuente, mientras que a nivel palatino solo se conocen 11 reportes en menores de 16 años. El objetivo de este reporte es presentar un caso clínico inusual de schwanoma palatino en un niño chileno de 12 años de edad, quien fue derivado con diagnóstico clínico presuntivo de carcinoma mucoepidermoide. El caso se complementó con radiografía panorámica, Conebeam, y luego biopsia incisional. Finalmente, se diagnosticó schwanoma palatino variedad sólida. Es importante considerar al schwanoma como diagnóstico diferencial en casos de tumores palatinos, en especial en casos pediátricos ya que se pueden presentar similares características clínicas a tumores de glándula salival.

The Schwannoma or neurilemmoma tumor originates from Schwann cells present in nerve sheaths. At oral level, its most frequent location is at the back of the tongue, being much more frequent in adults. In the pediatric population, this occurrence is rare and most frequently found in the tongue. At palatal level, only 11 reports are known in children under 16 years of age. The aim of this report is to present an unusual clinical case of palatal schwannoma in a 12-year-old Chilean boy, referred with a clinical diagnosis of mucoepidermoid carcinoma. The case was complemented with panoramic radiography and Cone beam, for its subsequent incisional biopsy. Finally, a solid palatal schwannoma was diagnosed. It is important to consider schwannoma as a differential diagnosis in cases of palatal tumors, especially in pediatric cases, since they can present similar clinical characteristics to salivary gland tumors.

Schwanoma Maligno de Nervo Periférico Metastático para Pulmão e Glândula Adrenal: Relato de Caso / Metastatic Peripheral Nerve Malignant Schwannoma of Lungs and Adrenal Gland: Case Report / Schwannoma Maligno Metastásico de Nervio Periférico para Pulmones y Glándula Suprarrenal: Reporte de Caso

Introdução: O schwanoma é um tumor pouco frequente que se origina das células de Schwann que recobrem os prolongamentos nervosos. A malignidade desses tumores é ainda mais rara, correspondendo a 5-10% de todos os sarcomas. O prognóstico é reservado, mesmo após completa ressecção cirúrgica. Relato do caso: Paciente do sexo masculino, 79 anos, apresentou-se com lesão crostosa em perna esquerda com dois meses de evolução. A biópsia da lesão inicialmente revelou sarcoma pleomórfico, mas seu padrão imuno-histoquímico confirmou tumor maligno da bainha do nervo periférico. O tumor desenvolveu metástase linfonodal, pulmonar e adrenal dentro de poucos anos após o diagnóstico inicial. A ressecção do tumor adrenal não foi possível. O paciente desenvolveu síndrome paraneoplásica e teve piora do estado clínico, evoluindo a óbito. Conclusão: Tendo em vista a forma inespecífica do aparecimento do tumor e sua agressividade, é importante ressaltar o papel da ressecção cirúrgica ampla no seu tratamento. Além disso, fica evidente a necessidade de novos relatos desse tipo de neoplasia para melhor definição de uma conduta mais apropriada, principalmente em casos de recorrência ou disseminação metastática

Introduction: Schwannoma is a rare type of tumor which grows from Schwann cells, that protect nerve extensions. The malignancy of these tumors is even rarer, corresponding to 5-10% of all sarcomas. The prognosis is dismal, even after complete surgical resection. Case report: A 79-year-old male patient presented with a crusted lesion in his left leg with two months of evolution. Initially, the biopsy of the lesion revealed pleomorphic sarcoma, but its immunohistochemical pattern was peripheral nerve sheath malignant tumor. The tumor developed lymph node, lung, and adrenal metastasis within a few years after the first diagnosis. Adrenal tumor resection was not possible. The patient developed paraneoplastic syndrome and his clinical status worsened, progressing to death. Conclusion: In view of the nonspecific form of tumor appearance and its aggressiveness, it is important to highlight the role of wide surgical resection in its treatment. In addition, it is clear that new case reports of this type of neoplasia are necessary for better definition of more appropriate conducts, especially in recurrence or metastatic cases

Introducción: El schwannoma es un poco común, el cual se origina en las células de Schwann, que cubren las extensiones nerviosas. La malignidad de estos tumores es aún más rara, y representa 5-10% de todos los sarcomas. El pronóstico es reservado, incluso después de una resección quirúrgica completa. Relato del caso: Varón de 79 años que presenta una lesión costrosa en la pierna izquierda a los dos meses de evolución. La biopsia de la lesión reveló inicialmente un sarcoma pleomórfico, pero su patrón inmunohistoquímico era un tumor maligno de la vaina del nervio periférico. El tumor desarrolló metástasis en los ganglios linfáticos, pulmones y suprarrenales pocos años después del diagnóstico. La resección del tumor suprarrenal no fue posible. El paciente desarrolló síndrome paraneoplásico y empeoró el estado clínico, progresando hasta la muerte. Conclusión: Dada la forma inespecífica de aparición del tumor y su agresividad, es importante destacar el papel de la resección quirúrgica amplia en su tratamiento. Además, existe una clara necesidad de nuevos informes de este tipo de neoplasias para definir mejor un abordaje más adecuado, especialmente en casos de recurrencia o diseminación metastásica

A giant retroperitoneal schwannoma in pregnancy: a rare case report and review of literature

Retroperitoneal schwannomas are though more common among females, are very much rare tumors arising from sheath of nerves. Also, extremely rare during pregnancy. Very few articles till date are there regarding retroperitoneal schwannomas that’s too in pregnant female. Usually these are symptomatic unless causing some compression to other intra-abdominal structures like vessels or bowel and usually malignant. As its retroperitoneal very difficult to diagnose it. So, with proper investigations and radiological imaging, first have to find out the differential diagnosis prior to planning for surgery.

Schwannoma em cavidade oral: relato de caso / Schwannoma in oral cavit: case report

Introdução: O schwannoma é uma neoplasia benigna que se origina nas células de Schwann da bainha de mielina dos nervos periféricos motores, sensitivos, simpáticos e dos pares cranianos. Apresenta etiologia desconhecida, e raramente ocorre em região buco-maxilo-facial (aproximadamente 1% dos casos), necessitando de tratamento cirúrgico da lesão. Objetivo: Relatar o caso de uma paciente com schwannoma benigno em cavidade oral, com tratamento cirúrgico para remoção da lesão. Relato de Caso: Paciente do gênero feminino, 18 anos, compareceu ao ambulatório do Serviço de Cirurgia e Traumatologia Buco-Maxilo-Facial da Universidade Federal da Bahia com a seguinte queixa principal: "tem uma lesão na minha boca". Ao exame clínico foi possível observar aumento de volume de consistência firme, séssil, limites bem definidos, de coloração semelhante à mucosa, em região de palato duro. Após biópsia incisional, o laudo concluiu compatibilidade com schwannoma benigno. O plano de tratamento incluiu a abordagem cirúrgica da lesão e posterior análise histopatológica, confirmando o diagnóstico de schwannoma benigno. Considerações finais: A excisão cirúrgica do schwannoma benigno com margem de segurança favorece um bom prognóstico para o paciente, bem como menor chance de recidivas e de transformação maligna da lesão(AU)

Introduction: Schwannoma is a benign neoplasm that originates in the Schwann cells of the myelin sheath of peripheral motor, sensory, sympathetic nerves and cranial nerves. It presents unknown etiology, and rarely occurs in the maxillofacial region (approximately 1% of cases), requiring surgical treatment of the lesion. Objective: To report the case of a patient with benign schwannoma in the oral cavity, with surgical treatment to remove the lesion. Case Report: Patient V.M.S., female, 18 years old, attended the clinic of the Department of Oral and Maxillofacial Surgery and Traumatology of the Federal University of Bahia with the following main complaint: "there is a lesion in my mouth". At the clinical examination, it was possible to observe an increase in the volume of firm consistency, sessile, well-defined limits of mucosa-like staining in the hard palate region. Absence of painful complaints on palpation. After an incisional biopsy, the report concluded compatibility with benign schwannoma. The treatment plan included the surgical approach of the lesion under general anesthesia. The lesion was excised (with a safety margin) and histopathological analysis was performed, confirming the diagnosis of benign schwannoma. Final considerations: Surgical excision of the benign schwannoma with margin of safety favors a good prognosis for the patient, as well as a lower chance of relapse and malignant transformation of the lesion(AU)

Sacral cellular schwannoma: a clinicopathologic analysis of eight cases / 临床与实验病理学杂志

Purpose To investigate clinicopathologic fea-tures of sacral cellular schwanoma and the difference from sacral conventional schwanoma.Methods Eight cases of sacral cellular schwanoma were collected.Microscopic examination and immunohistochemistry were performed for studying the clinical feature,radiologic appearance,pathologic characteristic,immunophenotyping,differential diagnosis and postoperative prognosis.Results There were 5 females and 3 males,whose mean age was 46.4 years.The majority of patients complained of pain in sacrococcygeal region.Radiographically,there was an endosacral or endosacral and presacal mass.Histologically,cellular schwannoma was composed of spindle cells,arranged in interlacing fascicles without nuclear palisading and Verocay bodies.Antoni A and Antoni B were not seen overtly.The destruction of bone was found.Immunohistochemically,tumor cells were diffusely and strongly positive for S-100 protein and vimentin.The mean of Ki-67 index was 6％.Tumor recurrence of 4 cases occurred several years after initial surgical resection.The mean interval to recurrence was 6.5 years.Conclusion Sacral cellular schwanoma is a rare tumor.Compared with sacral conventional schwanoma,it shows different growth pattern and pathologic features.So pathological diagnosis of the tumor should be noted for clinical follow-up and treatment.

Schwanoma gigante do VIII nervo craniano de evolução subaguda: relato de caso / Subacute evolution of giant schwannoma of VIII cranial nerve: case report

O schwanoma vestibular é uma neoplasia que se origina das células de Schwann da porção vestibular do VIII nervo craniano e que corres- ponde a cerca de 10% dos tumores intracranianos e a 80% a 90% daqueles localizados no ângulo pontocerebelar. Apesar do crescimento lento e evolução em geral benigna, estes tumores podem evoluir de maneira desfavorável devido à localização em compartimento anatômico relativamente reduzido no crânio (fossa posterior), o que pode levar a compressão de estruturas nobres (como o tronco encefálico) e a desenvolvimento de hidrocefalia obstrutiva, especialmente nos raros casos de schawanomas gigantes (> 40 mm). Apresentamos relato de caso de paciente com quadro de tinnitus e hipoacusia à esquerda que evoluiu de forma subaguda com alterações do humor, da cognição e da marcha cujo diagnóstico final foi de schwanoma gigante do VIII nervo craniano.

Vestibular schwannoma is a Schwann cells neoplasia of the vestibular branch of the VIII cranial nerve and it accounts for 10% of intracranial tumours and to 80%-90% of those located at the cerebellopontine angle. Despite slow growth and usual benign evolution, unfavourable outcomes may occur, especially in the rare cases of giant schwanno- mas (> 40mm). Owing the fact that this tumours are located in a relatively narrow space (posterior fossa), giant schwannomas may compress noble areas (as the brainstem), which can also cause obstructive hydrocephalus. We discuss a case report of a 48 years old woman complaining of left-sided hearing loss and tinnitus that developed a subacute cognitive impairment, mood disorder and gait disturbance, diagnosed with giant schwannoma of the VIII nerve.

Emergency embolization after resection of a laryngeal Schwannoma / Embolizacao de urgencia apos resseccao de schwanoma de laringe

Schwannoma is a rare cause of benign tumors of the larynx. The first-choice treatment is surgical resection. The objective of this paper is to report on a rare case of a young female patient who suffered severe intraoperative hemorrhaging during surgical resection of a laryngeal Schwannoma and needed emergency embolization.

O schwanoma representa etiologia rara de tumor benigno de laringe, tendo como principal tratamento a ressecção cirúrgica. O objetivo deste trabalho é relatar um caso raro de paciente jovem submetido à ressecção cirúrgica de schwanoma laríngeo, evoluindo, no transperatório, para complicação hemorrágica grave e necessitando de embolização de urgência.

Relato de dois casos de tumores retro-retais e revisão da literatura / Report of two cases of retrorectal tumors and literature review

Os tumores retro-retais são neoplasias raras no adulto. No espaço retro-retal, existem diversas estruturas embriológicas, que podem desenvolver grupos heterogêneos de tumores benignos e malignos. A apresentação clínica desses tumores é inespecífica, variando de queixas urinárias ou intestinais a ausência de sintomas, e a via de acesso cirúrgica ainda é alvo de discussão na literatura. Os autores relatam dois casos de tumores retro-retais, suas manifestações clínicas, diagnóstico e aspectos cirúrgicos

The retro-rectal tumors are rare neoplasms in adults. In the retro-rectal space, there are various embryological structures, which can develop a heterogeneous group of benign and malignant tumors. The clinical presentation of these tumors is nonspecific, ranging from urinary or intestinal complaints to absence of symptoms, and the surgical approach is still a matter of discussion in the literature. The authors report two cases of retro-rectal tumors, their clinical manifestations, diagnosis and surgical aspects

Schwanoma Lingual en un Adolescente: Revisión de la Literatura a Propósito de un Caso / Schwannoma of the Tongue in an Adolescent: A Short Literature Review Apropos of a Case

El Schwanoma corresponde a un tumor neurogénico benigno, derivado de las células de la cresta neural, específicamente de las células de la vaina periférica nerviosa llamadas schwannocitos. El 25% de éstos, se presentan en cabeza y cuello, y sólo un 1% se presentan en la cavidad oral, principalmente en la lengua. Presentamos un caso de una niña de 13 años de edad con diagnóstico de Schwannoma de lengua. El propósito de este reporte es enfatizar que el schwanoma lingual es un diagnóstico diferencial a considerar, pese a su baja prevalencia, entre todas las otras lesiones linguales en niños o adolescentes.

A schwannoma is a benign neurogenic tumour originating from schwannocyte of the nerve sheat. A 25% of all schwannomas are seen in the tissues of the head and neck and only a 1% of them are located in oral cavity, especially in tongue. We report the case of a 13 year old girl diagnosed with a schwannoma of the tongue. The purpose of this report is to empathize that schwannoma of the tongue is a differential diagnostic to consider among all other lingual lesions in children or adolescents.

Schwanoma lingual: presentación de un caso / Lingual schwannoma: case presentation

Introducción:el Schwanoma forma parte de los tumores que derivan de la vaina nerviosa, de crecimiento lento, asintomático en una parte de los casos, raro en la cavidad bucal y de etiología desconocida, con lo que su diagnóstico definitivo es anatomopatológico. No encontramos ningún reporte en las revistas electrónicas cubanas ni yemenitas sobre esta afección, lo cual sumado a su comportamiento no patognomónico y su rareza, nos motivó a reportar el caso. Objetivo: presentar un caso de un tumor bucal raro, con localización lingual poco frecuente. Presentación del caso: paciente masculino de 26 años de edad, procedente de Yemen, quien acude al Servicio de Cirugía Maxilofacial de la Universidad de Adén, por presentar aumento de volumen en la lengua de un año de evolución, asintomático hasta 10 días antes que se lo traumatizó y comenzó con molestias; se interpretó inicialmente como un granuloma piógeno; se decidió su escisión y biopsia para el diagnóstico definitivo, la cual se realizó bajo anestesia local sin complicaciones, con una evolución postoperatoria favorable. El resultado del examen histopatológico fue Schwanoma lingual. Discusión: se analizan los diferentes tumores benignos y malignos que en la lengua pueden confundir el diagnóstico, se valora el comportamiento no patognomónico de la entidad y a diferencia de la localización posterior reportada por muchos autores, la punta lingual fue la afectada en nuestro caso. Conclusiones: el Schwanoma es una rara afección de la cavidad bucal, que requiere el análisis histopatológico para su diagnóstico definitivo y con un buen pronóstico postquirúrgico.

Introduction: schwannoma is one of the tumors derivates from neural sheet, characterized by a slow growing, symptomless in some cases, rare in the oral cavity, unknown etiology which definitive diagnosis should be made by the anatomopathologic analysis. Not any reports at the Cubans or Yemenis electronic journals were founded. This observation in conjunction with the not patognomonic way of presentation and rareness of the lingual Schwannoma, motivated us to report this case. Objective: to present a case of rare oral tumor, with uncommon tongue location. Case presentation: a 26 years old male patient, from Yemen was attended at the Maxillofacial Service of Aden University, complaining about a mass on the tongue since one year ago which was asymptomatic, but about ten days ago the patient suffered from a masticatory trauma that produced some discomfort. Initial diagnosis was as pyogenic granuloma; excision and biopsy were made under local anesthesia to achieve the final diagnosis. Not any complications and a favorable postsurgical evolution were observed. The conclusion of the histopathological study was lingual Schwannoma. Discussion: different benign and malignant tumors of the tongue that make confusion in the diagnosis of this disease were analyzed and the not patognomonic behavior of this affection was taking into account. Many authors report about the posterior localization of this tumor, but in our case was localized at the tip of the tongue. Conclusions: Schwannoma is a rare disease of the oral cavity that requires histopathological analysis for the final diagnosis and has a good postsurgical prognosis.

Schwanoma del facial gigante: preservación auditiva y facial mediante decompresión nerviosa / Giant facial shwannoma: hearing and facial function preservation with nerve decompression

Se reporta el caso de un schwanoma del nervio facial que compromete el nervio desde la porción laberíntica hasta la intraparotídea, con erosión de la cápsula ótica a nivel de la cóclea y función facial normal. Mediante decompresión nerviosa se logró preservación de las funciones facial y auditiva y a los 20 meses posoperatorios no hay evidencia de crecimiento tumoral. Se discuten las diferentes alternativas terapéuticas para aquellos casos con función facial y coclear normales.

This report describes a patient with a large facial nerve schwannoma, which involves the facial nerve from its labyrinthine portion until its intra parotid portion with normal facial function. There was also a mild erosion of the otic capsule. It was treated with nerve decompression with facial function andhearing preservation after two years of follow up.

Tumores de nervios periféricos de la cavidad bucal: esudio epidemiológico / peripheral nerve tumors of the oral cavity: epidemiological study

Los tumores neurales son lesiones oriundas de los nervios periféricos, siendo raros en la cavidad bucal. Estas lesiones incluyen el neurofibroma, neuroma traumático, schwanoma tumor de células granulares y el neuroma encapsulado. Objetivo: El presente trabajo se propuso a estudiar la prevalencia de estos tumores en dos servicios de diagnostico oral. Material y Método: fue realizado, a través de análisis de informes histopatológicos, un estudio retrospectivo de los casos de tumores neurales solitarios en la cavidad bucal diagnosticados en el laboratorio de Patología de la Universidad de Pernambuco, en el período de 1.992 a 2.007, y en el servicio de Patología de la Universidad Federal de Sergipe, en el periodo de 2.000 a 2.007. Resultados: Fueron encontrados 28 tumores neurales benignos, de un total de 4,485 informes analizados. De una manera general, el género femenino fue el más afectado y la lengua el sitio de mayor predilección. Hubo una discreta tendencia del surgimiento de las lesiones en la 1ª, 2ª e 3ª Décadas de vida. Conclusión: el conocimiento de estas lesiones, aunque raras en la cavidad bucal, se torna imprescindible para los profesionales del área odontológica, visto que las estrategias de diagnostico y tratamiento son dependientes de los datos epidemiológicos encontrados en la literatura especializada

Peripheral nerve sheath tumors rarely occur in the oral cavity and include neurofibroma, traumatic neuroma, schwannoma, granular cell tumor and palisaded encapsulated neuroma. Aim: The purpose of the present study was to determine the prevalence of these tumors in patients referred in two oral diagnostic services. Material and Method: Specimens diagnosed as oral peripheral nerve sheath tumors archived in the oral pathology services of Universidade de Pernambuco, from 1992 to 2007, and Universidade Federal de Sergipe, from 2000 to 2007, were evaluated. Results: Twentyeight peripheral nerve sheath tumors were diagnosed. Neurofibroma was the tumor more prevalent, including 40% of the cases. In general, females were more affected than males and the most frequent oral site was the tongue. Children and young people were more prevalent. Conclusion: The knowledge of these lesions, even though rare in oral cavity, it is essential for dentistry professionals, since that diagnosis and treatment strategies are dependents to epidemiological data founded in the literature

Neurilemoma intramamario: reporte de un caso / Intramammary neurilemoma: a case report

Reportamos el caso de una mujer de 74 años que se presenta en nuestro servicio para realizar el control de una lesión nodular preexistente. El tumor estaba localizado en el cuadrante súpero externo de la mama izquierda. Si bien los hallazgos imagenológicos sugerían benignidad, el diagnóstico de certeza se realizó mediante anatomía patológica y técnicas inmunohistoquímicas.

We report on 74 year-old woman who presented to our service for a control. The tumor was located in the upper outer quadrant of the left breast. Although imaging findings changes suggested benignity, an accurate diagnosis was made by histopathology and inmunohistochemistry.