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.

Schwannoma pancreático como tumor sincrónico. Reporte de caso / Pancreatic schwannoma as a synchronous tumor. Case report

Los Schwannomas son tumores derivados de las células de Schwann de las vainas de los nervios periféricos. Se pueden localizar en cualquier región anatómica que contenga tejido nervioso periférico, siendo más frecuentes en la región craneofacial y las extremidades. Los Schwannomas pancreáticos son entidades sumamente infrecuentes de las cuales solo se han descrito 68 casos a nivel mundial. En el presente trabajo se presenta el caso de un paciente con hallazgo incidental de tres tumores sincrónicos dentro de los cuales se encuentra un Schwannoma pancreático.Caso clínico : Paciente femenino de 66 años de edad con antecedente de diabetes mellitus tipo 1 y enfermedad diverticular pancolónica quien acude presentando cuadro clínico compatible con absceso lumbar izquierdo. Se realiza TC de abdomen y pelvis con doble contraste que evidencia extensa área de colección heterogénea en región retroperitoneal que diseca hacia región lumbar y glútea izquierda, además de la presencia de tumor hipodenso de bordes lobulados en mesogastrio. Se realiza colonoscopia que reporta lesión exofítica ulcerada en unión rectosigmoidea. El resto de paraclínicos y estudios de extensión se encontraban dentro de límites normales. Se decide resolución quirúrgica mediante drenaje percutáneo de absceso y laparotomía exploradora. Informe histopatológico: cistoadenoma seroso microquístico de cuerpo de páncreas, Schwannoma de cola de páncreas y adenocarcinoma moderadamente diferenciado de colon sigmoides.Conclusión : Los Schwannomas pancreáticos son entidades sumamente infrecuentes que pueden presentarse con una amplia variedad de manifestaciones clínicas, sin embargo, deben tenerse en cuenta como posible diagnóstico diferencial ante el hallazgo de un tumor pancreático(AU)

Schwannomas, also called Neurilemmomas or Neurinomas, are tumors derived from Schwann cells of the peripheral nerve sheaths. They can be located in any anatomical region that contains peripheral nervous tissue, being more frequent in the craniofacial region and the extremities. Pancreatic Schwannomas are extremely rare entities of which only 68 cases have been described worldwide. In the present study we present the case of a patient with an incidental finding of three synchronous tumors, including a pancreatic Schwannoma.Clinical case : A 66-year-old female patient with a history of type 1 diabetes mellitus and pancolonic diverticular disease who presented with symptoms compatible with left lumbar abscess. A double-contrast CT of the abdomen and pelvis was performed, which revealed a large area of heterogeneous collection in the retroperitoneal region that dissected towards the left lumbar and gluteal region, in addition to the presence of a hypodense tumor with lobulated borders in the mesogastrium. A colonoscopy was performed, which reported an ulcerated exophytic lesion at the rectosigmoid junction. The rest of the paraclinical and extension studies were within normal limits. Surgical resolution is decided by percutaneous abscess drainage and exploratory laparotomy. Histopathological report: microcystic serous cystadenoma of the body of the pancreas, Schwannoma of the pancreas tail, and moderately differentiated adenocarcinoma of the sigmoid colon.Conclusion : Pancreatic Schwannomas are extremely rare entities that can present with a wide variety of clinical manifestations, however, they should be taken into account as a possible differential diagnosis when a pancreatic tumor is found(AU)

Mucosal Schwann cell hamartoma of the gallbladder

Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered.

Claudin 14/15 play important roles in early wallerian degeneration after rat sciatic nerve injury / 中华创伤杂志(英文版)

PURPOSE@#Wallerian degeneration (WD) is an antegrade degenerative process distal to peripheral nerve injury. Numerous genes are differentially regulated in response to the process. However, the underlying mechanism is unclear, especially the early response. We aimed at investigating the effects of sciatic nerve injury on WD via CLDN 14/15 interactions in vivo and in vitro.@*METHODS@#Using the methods of molecular biology and bioinformatics analysis, we investigated the molecular mechanism by which claudin 14/15 participate in WD. Our previous study showed that claudins 14 and 15 trigger the early signal flow and pathway in damaged sciatic nerves. Here, we report the effects of the interaction between claudin 14 and claudin 15 on nerve degeneration and regeneration during early WD.@*RESULTS@#It was found that claudin 14/15 were upregulated in the sciatic nerve in WD. Claudin 14/15 promoted Schwann cell proliferation, migration and anti-apoptosis in vitro. PKCα, NT3, NF2, and bFGF were significantly upregulated in transfected Schwann cells. Moreover, the expression levels of the β-catenin, p-AKT/AKT, p-c-jun/c-jun, and p-ERK/ERK signaling pathways were also significantly altered.@*CONCLUSION@#Claudin 14/15 affect Schwann cell proliferation, migration, and anti-apoptosis via the β-catenin, p-AKT/AKT, p-c-jun/c-jun, and p-ERK/ERK pathways in vitro and in vivo. The results of this study may help elucidate the molecular mechanisms of the tight junction signaling pathway underlying peripheral nerve degeneration.

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Histopathological analysis of gangliosides use in peripheral nerve regeneration after axonotmesis in rats / Análise histopatológica da regeneração nervosa periférica com uso de gangliosídeos após axonotmese em ratos

PURPOSE: To analyze the action of gangliosides in peripheral nerve regeneration in the sciatic nerve of the rat. METHODS: The sample was composed of 96 male Wistar rats. The animals were anaesthetized and, after identification of the anaesthesic plane, an incision was made in the posterior region of the thigh, followed by skin and muscle divulsion. The right sciatic nerve was isolated and compressed for 2 minutes. Continuous suture of the skin was performed. The animals were randomly divided into two groups: the experimental group (EG), which received subcutaneous injection of gangliosides, and the control group (CG), which received saline solution (0.9 percent) to mimic the effects of drug administration. RESULTS: No differences were observed between the experimental and control groups evaluated on the eighth day of observation. At 15 and 30 days the EG showed an decrease in Schwann cell activity and an apparent improvement in fibre organization; at 60 days, there was a slight presence of Schwann cells in the endoneural space and the fibres were organized, indicating nerve regeneration. At 15 and 30 days, the level of cell reaction in the CG had diminished, but there were many cells with cytoplasm in activity and in mitosis; at 60 days, hyperplastic Schwann cells and mitotic activity were again observed, as well as nerve regeneration, but to a lesser extent than in the EG. CONCLUSION: The administration of exogenous gangliosides seems to improve nerve regeneration.

OBJETIVO: Avaliar os efeitos de gangliosídeos na regeneração nervosa periférica em nervo isquiático de ratos após axonotmese. MÉTODOS: Foram utilizados 96 ratos machos albinos (Wistar). Os animais foram anestesiados e após constatação do plano anestésico, foi realizada incisão na face posterior da coxa direita do animal. Em seguida, foi realizada a dissecção cirúrgica da pele e do músculo e divulsão dos músculos. O nervo isquiático direito foi isolado e sofreu compressão por 2 minutos. Efetuou-se a sutura contínua da pele. Os animais foram distribuídos aleatoriamente em 2 grupos: experimental (GE) que receberam gangliosídeos pela via sub-cutânea e controle (GC) que receberam soro fisiológico 0,9 por cento com a finalidade de mimetizar os efeitos de administração da droga de estudo. A análise histopatológica foi realizada em 8, 15, 30 e 60 dias. RESULTADOS: Não se evidenciaram diferenças significantes entre os grupos controle e experimental avaliados com 8 dias. Nos grupos experimentais de 15 e 30 dias observou-se uma diminuição da atividade das células de Schwann e aparente melhora na organização das fibras nervosas; com 60 dias havia discreta presença células de Schwann no espaço endoneural e as fibras nervosas estavam organizadas sinalizando a regeneração nervosa. Nos grupos controles de 15 e 30 dias o padrão de reação celular diminuiu, entretanto havia muitas células com citoplasmas em atividade e mistose; com 60 dias observou-se ainda a presença de hiperplasia de células de Schwann, atividade mitótica ainda presente e regeneração nervosa presente, porém em menor grau comparando-se com aquele visto no grupo experimental. CONCLUSÃO: A administração de gangliosídeos exógenos parece incrementar a regeneração nervosa.

Intraosseous neurilemmoma of L2 vertebra--a case report.

Intraosseous neurilemmoma (schwannoma) is a rare bone tumor with incidence less than 0.2% of all primary bone tumors. It is a benign neoplasm arising from the schwann cells of the nerve sheath. When they occur, they are usually found in the mandible. This paper reports an unusual occurrence of intraosseous neurilemmoma in L2 vertebral body. This patient presented with complaints of backache and progressively increasing weakness in both lower limbs. Roetengenograms showed an osteolytic lesion of L2 vertebra localized in the left half of the body and pedicle. Computed tomograph revealed a large soft tissue component of the tumor mass with thecal sac compression. A computed tomograph assisted needle biopsy revealed the tumor to be neurilemmoma. Subsequently, during decompression, it was possible to shell out tumor from surrounding tissues. Remaining tumor was curettedfrom bone and the cavity packed with autograft. The recovery was uneventful with incorporation of graft. The aim of this article is to highlight intraosseous neurilemmoma as a possible differential diagnosis in bony tumors. Intraosseous neurilemmoma of lumbar vertebrae is an extremely rare occurrence and till date only four cases are reported in English literature.

Light and electron microscopic appearances of peripheral nerves from two lepromatous leprosy patients after 12 months of multidrug therapy and their significance.

Biopsies from radial cutaneous nerves of a lepromatous patient and one borderline lepromatous patient treated with 12 doses of multidrug regimen were studied using light and electronmicroscopes. Histopathologically both showed typical lepromatous neuritis. Electronmicroscopic examination showed demyelination, atrophy and degeneration of myelinated axons and nonmyelinated axons and a marked increase in collagen fibrils. Perineurial cells, Schwann cells and endoneurial macrophages contained numerous persisting M. leprae. Almost all the organisms in macrophages were fragmented and could be considered non-viable. A few M. leprae found in Schwann cells showed structure of viable bacilli. It is possible a few dead or dormant organisms may persist for many years in Schwann cells or in fibrous tissue without producing any ill effects, and may cause relapse only in rare instances. Since 12 months of MDT resulted in the clearance of M. leprae in course of time and the reported relapse rates after years were insignificant, implementation of MDT for a year for all MB patients is justified provided surveillance of these patients is ensured. Administration of uniform MDT for 6 months is worth a trial.

Estudo retrospectivo do schwuanoma: uma análise de vinte anos / Retrospective study of schwannoma: a twenty-year analysis

Os schwannomas, também chamados de neurilemomas são tumores de origem nervosa, encapsulados, solitários, apresentando crescimento lento e progressivo. Originam-se da bainha de Schwann e são relativamente raros no segmento cabeça e pescoço, podendo ocorrer em nervo central, periférico ou simpático. Através de estudo retrospectivo, foram analisados os indicadores sexo, faixa etária, aspectos clínicos, histopatológicos, diagnóstico, diagnóstico diferencial, tratamento, prognóstico, de pacientes portadores da referida patologia, atendidos e tratados no Serviço de Cirurgia de Cabeça e Pescoço do Centro de Oncologia (CEON) do Hospital Universitário Oswaldo Cruz (HUOC) - Universidade de Pernambuco (UPE), de Janeiro de 1975 a Janeiro de 1995 (20 anos), através do levantamento dos dados no Centro Integrado de Anatomia Patológica (CIAP) da referida instituição. Compararam-se os indicadores supracitados com os apresentados na revisão da bibliografia consultada. No total de 43 casos (100 por cento), 53,5 (por cento) eram do sexo masculino, a 3ª década de vida foi a mais prevalente (28,2 por cento), bem como o sítio topográfico da região cervical direita (10 casos) e 79,1 (por cento) eram do tipo benigno. Conclui-se que a hipótese diagnóstica de Schwannoma deve ser sempre considerada pelo cirurgião, em pacientes portadores de massas cervicais dolorosas ou não. A cirurgia é o tratamento de eleição, podendo a radioterapia ser utilizada nos casos em que o tumor é considerado tecnicamente irressecável

Neural hypertrophy in carcinoma stomach.

Neural hypertrophy with hyperplastic Schwann cells in the wall of the stomach along with enterochromaffin cell hyperplasia was incidentally observed at histology in the gastrectomy specimen of a 43-year-old man with carcinoma stomach who had presented with upper abdominal pain of one year duration. The patient had no previous abdominal surgery or evidence of gastrointestinal obstructive pathology. The significance of this neural hypertrophy is not known.

Schwannoma e neurofibroma intramedular / Intramedullary schwannoma and neurofribroma

O neurofibroma e o schwannoma são neoplasias raras que envolvem a medula e suas raízes, correspondendo, respectivamente, a 3% e 27% dos tumores espinhais. Os autores apresentam dois casos de tumores intramedulares operados no Serviço de Neurologia e Neurocirurgia da Santa Casa de Belo Horizonte. O estudo anatomopatológico mostrou tratar-se de um schwannoma e de um neurofibroma, respectivamente. A partir desses achados é feita uma discussão sobre os aspectos clínicos e patológicos desses tumores, salientado a importância do diagnóstico diferencial entre eles. Atualmente, embora ainda não seja reconhecido de maneira universal, esses tumores são considerados como unidades distintas. Além do interesse teórico em diferenciar tais lesões,existe a importância clínica do prognóstico, pois a malignidade não ocorrenos schwannomas, e é extremamente rara nos neurofibromas solitários, ecomum nos múltiplos.

Spinal neurofibroma is one of the rarest neoplasm involving the spinal cordand roots. The intramedullary localization of schwannomas is rare, corresponding to 0,3% of all intraspinal tumors. The authors report two cases of cervico-toracic intramedullary tumors, respectively a schwannoma and a neurofibroma, surgically treated. The present study analysis the clinical and pathological aspects of these tumors and the importance of its differential diagnosis.

Neuroma apendicular (obliteración fibrosa) asociado a microcarcinoide solitario / Neuroma of the appendix (fibrous obliteration) associated with solitary microcarcinoid

Informamos un caso de neuroma apendicular (obliteración fibrosa de la punta) asociado con microcarcinoide solitario del tercio medio del apéndice cecal. Esta lesión resulta de la proliferación neuromatosa con obliteración de la luz e hiperplasia de células endócrinas extraepiteliales. La inmunohitoquímica para proteína S-100, enolasa neurona específica (NSE) y factor de crecimiento epidérmico (EGF) resultaron positivos y la desmina, el antígeno epitelial de membrana (EMA) y la proteína fibrilar ácida glial (GFAP) fueron negativos. Lo anterior indica que la obliteración fibrosa del apéndice cecal es una proliferación de células de Schwann y no un proceso fibroblástico degenerativo. La hiperplasia de células endócrinas podría estimular por un lado, la proliferación de células de Schwann y por otro, inducir el crecimiento de carcinoides y así explicar esta interesante asociación

Schwannomas: estudio clínico patológico de 85 casos / Schwannomas:clinical and pathologic study of 85 cases

El shwannoma es un tumor que se origina en las células de Schwann de las vainas de los nervios periféricos. Este tipo de tumor usualmente exhibe características histológicas benignas, aunque por su localización puede comportarse clínicamente como una entidad grave sobre todo en casos con tumores de dimensiones que comprometan las funciones del tallo cerebral. Se hizo una revisión de las biopsias en el Departamento de Neuropatología del Instituto Nacional de Neurología y Neurocirugía de México, en el período comprendido entre julio de 1985 y junio de 1990. En ese período se recibieron 2447 biopsias; el diagnóstico de schwannoma se hizo en 100 biopsias, que correspondieron 85 pacientes, 47 de ellos mujeres, es decir un total de 4.08 por ciento de schwannomas. Las edades variaron de 15 a 69 años, con un promedio de 37.6. En 64 casos los schwannomas fueron intracraneales, y en 18 se localizaron en el canal espinal. El nervio acústico fue el origen del tumor en 57 casos, dos casos se originaron en el trigémino y uno en el nervio facial; en cuatro casos no se pudo precisar el nervio de origen. Los casos intrarraquídeos fueron de localización cervical en siete, torácica en nueve y lumbosacras en dos. Las manifestaciones clínicas más comunes fueron afección auditiva en 52 casos, presión intracraneal aumentada en 20, afección cerebral en 17, hipoestesia o paresia facial en 16, trastornos oculomotores en seis y paresia o parálisis morora en cinco pacientes

Ectomesenquimoma maligno; informe de un caso / Malignant ectomesen chimoma: report of a case

Se informan los hallazgos clínicos y patológicos de un ectomesenquimoma maligno de partes blandas. Los ectomesenquimomas malignos son tumores compuestos por derivados de la cresta neural y uno o más elementos mesenquimatosos malignos, por lo general rabdomiosarcoma, condrosarcoma, angiosarcoma y liposarcoma maligno y liposarcoma. En esta neoplasia, el tratamiento más adecuado es el quirúrgico, pues la quimioterapia y la radioterapia no parecen modifiar el curso de la enfermedad. En este caso el tratamiento fue quirúrgico, sin datos de actividad tumoral a los 16 meses subsecuentes a la operación.

Schwannoma cervical calcificado / Cervical calcified schwannoma

Presentamos un caso de schwannoma cervical calcificado, en un paciente de 19 años de edad, cuyo motivo de consulta fue tumor cervical, localizado en la región lateral derecha del cuello. El día 15-08-88 fue intervenido quirúrgicamente, reaizando cervicotomía lateral derecha; los hallazgos correspondieron a un tumor sólido prevertebral, con relaciones en la base del cráneo, que desplazaba el esófago, la tráquea y el paquete vasculonervioso cervical derecho; fue resecado en su totalidad. Dada la laboriosa resección, lo difícil del diagnóstico diferencial y la baja frecuencia de esta lesión consideremos su reporte y la revisión de bibliografía nacional e internacional

Schwannoma de células granulosas: presentación de un caso / Granular cell schwannomas: case report

Se presenta un caso de schwannoma de células granulosas histológicamente benigno en una mujer de 53 años de edad, con localización y tamaño no habituales, destacando la carencia de características clínicas propias identificatorias del tumor. Se adhiere a la derivación histogenética de estructuras periféricas schwannianas, remarcando la importancia diagnóstica definitoria de la histopatología, ya que el patrón histológico posee caracteres distintivos suficientes. Asimismo, la ultraestructura e inmunorreactividad con proteínas S100 avalan el origen tumoral a partir de estructuras schwannicas

Neurinoma intramedular: relato de caso / Intramedullary neurinoma: report of a case

Os autores apresentam um caso de Neurinoma Intramedular, em paciente masculino de 25 anos de idade, com boa evoluçäo pós-cirúrgica