Orbital Schwannoma: Case Report and Review

Orbital schwannomas are rare, presenting a rate of incidence between 1 and 5% of all orbital lesions. Their most common clinical symptoms are promoted by mass effect, such as orbital pain and proptosis. The best complementary exam is the magnetic resonance imaging (MRI), which shows low signal in T1, high signal in T2, and heterogeneous contrast enhancement. The treatment of choice is surgical, with adjuvant radiotherapy if complete resection is not possible. We report the case of a 24-year-old male patient with orbital pain and proptosis, without previous history of disease. The MRI showed a superior orbital lesion compatible with schwannoma, which was confirmed by biopsy after complete resection using a fronto-orbital approach.

Schwannomas of Peripheral Nerves: Unusual Cause of Sciatic and Literature Review / Schwanomas dos nervos periféricos: causa rara de ciatalgia e revisão da literatura

Schwannomas of peripheral nerves are the most common benign tumors in this location; it has specific radiologic and histologic features that enable its fundamental distinction of neurofibromas. Given its importance in neurosurgical practice, known radiologic and histologic criteria must be updated regarding this disease entity, as well as its therapeutic strategy and results obtained with it. This article reviews this subject, followed by a case report considered out of the ordinary, in which the tumor was located in the sciatic nerve in a patient with neurofibromatosis type 1, treated by microsurgical resection, which evolved favorably.

Os schwannomas dos nervos periféricos são os tumores benignos mais comuns nesta localização. Apresentam características radiológicas e histológicas específicas que possibilitam a sua fundamental distinção dos neurofibromas. Dada a sua importância na prática neurocirúrgica, devem ser conhecidos os critérios radiológicos e histológicos atualizados à respeito desta entidade nosológica, bem como sua estratégia terapêutica e o resultado com ela obtido. Neste artigo, é feita uma revisão do tema, seguido do relato de um caso considerado fora do habitual, no qual o tumor localizava-se no nervo ciático, em uma paciente portadora de neurofibromatose tipo 1, tratada por meio de ressecção microcirúrgica, que evoluiu de forma favorável.

Neurofibromatosis: part 2 – clinical management / Neurofibromatoses: parte 2 – manejo clínico

Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.

A primeira parte desta diretriz abordou o diagnóstico diferencial das neurofibromatoses (NF): neurofibromatose do tipo 1 (NF1), neurofibromatose do tipo 2 (NF2) e schwannomatose (SCH). As NF compartilham algumas características, como a origem neural dos tumores e sinais cutâneos, e afetam cerca de 80 mil brasileiros. O aumento do conhecimento científico sobre as NF tem permitido melhor manejo clínico e redução da morbidade das complicações, resultando em melhor qualidade de vida para os pacientes com NF. A maioria dos médicos é capaz de realizar o diagnóstico das NF, mas a variedade de manifestações clínicas e a dificuldade de se prever o surgimento e a gravidade de complicações, torna o manejo da NF um desafio para o clínico e envolve diferentes especialistas para o tratamento adequado e aconselhamento genético, especialmente a NF2 e a SCH. O presente texto sugere algumas orientações para o acompanhamento dos portadores de NF, com ênfase na NF1.

Hemotórax espontáneo en paciente con schwannoma de mediastino / Spontaneous hemothorax caused by a schwannoma of the posterior mediastinum

Background: Schwannomas are the most common neurogenic tumors of the posterior mediastinum. They are usually asymptomatic and the symptoms associated with them are chest pain, cough and dyspnea. Case report: We report a 36 years old female consulting in the emergency room due to sudden onset dyspnea. The imaging study confirmed the presence of a hemothorax secondary to a tumor of the posterior mediastinum, which was surgically excised. The pathological study reported a Schwannoma.

Introducción: El mediastino es un área anatómica que contiene estructuras y células pluripotenciales que pueden originar distintos tumores. Los Schwannomas son los tumores neurogénicos más comunes del mediastino posterior, se caracterizan por tener un crecimiento lento. A menudo son asintomáticos y se diagnostican como hallazgos radiológicos. Cuando son sintomáticos, lo más común es que presenten dolor torácico, tos y disnea. El hemotórax es una rara forma de manifestación. Caso clínico: Se presenta en este trabajo el caso de una mujer de 36 años, que ingresa al servicio de urgencia por un cuadro de disnea súbita. El estudio por imágenes confirma el diagnóstico de hemotórax, secundario a un tumor de mediastino posterior, el cual requiere cirugía. Anatomía patológica corrobora el diagnóstico presuntivo de Swchannoma de mediastino posterior. Conclusión: La cirugía es necesaria para poder resecar completamente el tumor y en el Schwannoma benigno es curativa.

Schwannoma intratorácico / Intrathoracic schwannoma

El schwannoma (neurilemoma) es un tumor benigno de lento crecimiento originado en la vaina de Schwann, cuya localización en la cavidad torácica es excepcional. En general es asintomático y se descubre en forma incidental, aunque puede ocasionar síntomas cuando la lesión crece o invade estructuras subyacentes. Su importancia radica en la posibilidad de confusión con tumores malignos. Presentamos una paciente con dolor torácico de evolución prolongada causado por un schwannoma. La imagen tomográfica es sugestiva de tumor extrapulmonar, por ello el schwannoma en esta localización debe ser considerado en el diagnóstico diferencial de tumores pleurales metastásicos o primarios como el lipoma, el tumor fibroso solitario y el mesotelioma.

The schwannoma (neurilemmoma) is a slow-growing benign tumor originating from Schwann sheath whose location in the chest cavity is exceptional. It is generally asymptomatic and is discovered incidentally but can cause symptoms when the lesion grows or invade underlying structures. Its importance lies in the possibility of confusion with malignant tumors. We present a patient who complains of chest pain caused by a prolonged course schwannoma. The tomographic image is suggestive of extrapulmonary tumor, so the schwannoma in this location should be considered in the differential diagnosis of metastatic or primary pleural tumors such as lipoma, solitary fibrous tumor and mesothelioma.

Schwannoma in Head and Neck: Preoperative Imaging Study and Intracapsular Enucleation for Functional Nerve Preservation

PURPOSE: In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. MATERIALS AND METHODS: In 7 cases of schwannomas in the head and neck region, we predicted whether the tumor originated from the vagus nerve or the cervical sympathetic chain through imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI). All patients were performed intracapsular enucleation, and the function of the vagus nerve and the sympathetic nerve was evaluated preoperatively and postoperatively. RESULTS: Preoperative imaging studies showed 6 cases where the tumor was located between the carotid artery and the internal jugular vein, and 1 case where the tumor was located posteriorly, displacing the carotid artery and the internal jugular vein anteriorly. At the time of operation, we confirmed schwannoma originating from the vagus nerve on the first 6 cases, and schwannoma originating from the sympathetic nervous system on the last case. All patients went through successful intracapsular enucleation, and of the seven schwannoma cases, 6 patients maintained normal postoperative neurological function (85.7%). CONCLUSION: Preoperative imaging studies offer valuable information regarding the location and origination of the tumor, and intracapsular enucleation helped us to preserve the nerve function.

Charcot-Marie-Tooth 1A Concurrent with Schwannomas of the Spinal Cord and Median Nerve

We identified Charcot-Marie-Tooth disease type 1A (CMT1A) in a family with schwannomas in the spinal cord and median nerve. The CMT1A in this family showed an autosomal dominant pattern, like other CMT patients with PMP22 duplication, and the family also indicated a possible genetic predisposition to schwannomas by 'mother-to-son' transmission. CMT1A is mainly caused by duplication of chromosome 17p11.2-p12 (PMP22 gene duplication). A schwannoma is a benign encapsulated tumor originating from a Schwann cell. A case of hereditary neuropathy with liability to pressure palsies (HNPP) concurrent with schwannoma has been previously reported. Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background.

Tumores do plexo lombossacral. Relato de dois casos e revisão da literatura / Lumbosacral plexus tumors. Report of two cases and literature review

Introdução: Tumores do plexo lombossacral(TPLS)são raros. Entre estes, neurofibromas e schwannomas são os mais comuns. Em geral, atingem grandes proporções, sendo, na maioria das vezes, detectados incidentalmente durnate investigações de sintomas inespecíficos, tais como dor abdominal, dor lombar e constipação. Por vezes, comprometem a coluna vertebral, provocando destruição dos corpos vertebrais e/ou alargamento dos farames intervertebrais, podendo haver invasão do canal vertebral. Objetivo: Relatar os casos de dois pacientes que apresentavam volumosas massas retroperitoneais revomidas cirurgicamente. O acesso cirúrgico foi realizado pela equipe da cirurgia geral, e a equipe de neurocirurgia procedeu a ressecção de ambos os tumores por meio de dissecção microcirúrgica no interior do músculo psoas maior. o exame histopatológico diagnosticou neurofibroma e schwannoma, não relacionados à neurofibromatose tipo 1(NF1).Conclusão: Os TPLS são lesões cujo tratamento deve ser cirúrgico, realizado por equipe multidisciplinar, utilizando técnicas de microcirurgia para obtenção de um bom resultado funcional com possibilidade de ressecção total sem déficit neurológico.

Schwannoma of bile duct--a case report.

This is a case report of a 29 year old woman who presented with painless and progressive obstructive jaundice. Imaging investigations of the abdomen revealed a tumour of the common bile duct. She was treated by complete excision of the bile duct and hepaticojejunostomy. The histopathology report of the tumour read as benign schwannoma.

Cystic cervical intramedullary schwannoma with syringomyelia.

We report a case of cervical intramedullary cystic schwannoma associated with segmental syrinx in a young adult without evidence of neurofibromatosis. The relevant literature is reviewed.

Cystic olfactory groove schwannoma.

Intracranial schwannoma not related to cranial nerves are unusual and rarely found in the subfrontal region. We report a case of cystic olfactory groove schwannoma in a 55-year- old male, who presented with late onset seizure without raised intracranial pressure. The tumor was excised completely.

Malignant Transformation of Intrathoracic Ancient Neurilemmoma in a Patient without Von Recklinghausen's Disease

Malignant transformation of a neurilemmoma is an exceedingly rare event. We describe a case of intrathoracic ancient neurilemmoma undergoing a malignant change in a 39-yr-old man. The patient presented with right flank and chest pain for several months. Plain radiography and CT scan of the chest showed a soft tissue mass lesion at the extrapleural space with erosion of surrounding ribs at the right basal lung area. The excised mass was encapsulated and measured 4.5 x 3.5 x 2.3 cm. The cut surface showed grayish-white and glistening with a focal cystic change and hemorrhage. Necrosis was not seen. Histologically, the tumor showed the features of classic neurilemmoma composed of the Antoni type A and B areas with perivascular hyalinization. In addition, obviously histo-logically malignant foci manifested by presence of markedly increased cellularity with fascicular arrangement, active mitotic activity, hyperchromasia, and gradual loss of original neurilemmomatous feature were noted.

Gastric schwannoma presenting as gastric polyp with gastrointestinal bleeding.

Benign neurogenic tumors of stomach are the commonest of all nonepithelial tumors of the stomach, but solitary gastric schwannomas are rare. We report a 58-year-old man with gastric schwannoma presenting as fundal polyp and upper gastrointestinal bleeding. Endoscopic polypectomy was done.

Mixed tumour of schwannoma and meningioma in a patient with neurofibromatosis-2 : a case report.

The co-existence of schwannoma and meningioma as a mixed intracranial tumour is uncommon and so far only eight cases have been published in the literature. Because of rarity, we report a unique case of mixed tumour having schwann cell and meningeal components, in a patient with neurofibromatosis type -2 (NF-2). The possible mechanisms for the occurrence of these mixed tumours are discussed.