Application of peroral endoscopic-assisted laryngeal microsurgery in children with laryngeal neurofibromas / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the clinical manifestations and the effect of peroral endoscopic-assisted laryngeal microsurgery for children with laryngeal neurofibroma, and to provide clinical reference for the diagnosis and treatment of this disease. Methods:The clinical data of 4 children with laryngeal tumors admitted to the Department of Otorhinolaryngology, Children's Hospital of Chongqing Medical University from January 2021 to June 2023 were retrospectively analyzed. Laryngeal tumors were removed by peroral endoscopic-assisted laryngeal microsurgery. One case underwent tracheotomy at the same time, and one case was simultaneously performed with laryngeal T tube placement and tracheotomy. Results:Surgical resection is the best treatment for laryngeal neurofibroma, and laryngeal microsurgery should be actively used for patients with surgical indications.This surgical method has the advantages of good efficacy, minimal invasion, aesthetics and preservation of laryngeal function, which not only ensures safety, but also improves the quality of life after surgery, and has the value of development and promotion.

Solitary circumscribed neuroma of the conjunctiva: Differential diagnosis from neurofibroma is a must? / Neuroma circunscrito solitário da conjuntiva: diagnóstico diferencial de neurofibroma obrigatório?

ABSTRACT A 42-year-old male presented with a 4-week history of a mass in the right inferior palpebral conjunctiva close to the punctum. An excisional biopsy of the lesion and histopathological examination revealed that the mass was composed of Schwann cells with thin conical nuclei, fine chromatin, and unnoticeable nucleoli. Immunohistochemically, the spindle cells were diffusely and strongly positive for S100 protein. Neurofilament immunostaining was also positive, which highlighted axons. In light of these findings, the tumor was diagnosed as solitary circumscribed neuroma. A comprehensive evaluation for multiple endocrine neoplasia type 2b was performed. However, no multiple endocrine neoplasia type 2b stigmata and no family history were detected. The diagnosis was therefore finalized as solitary circumscribed neuroma, which is considered as a rare condition. The differential diagnosis is based on the histopathological examination and immunohistochemical evaluation. As the tumor can be related with multiple endocrine neoplasia type 2b, it is essential to systematically investigate for multiple endocrine neoplasia type 2b in such cases.

RESUMO Um homem de 42 anos apresentou uma massa na conjuntiva palpebral inferior direita, próxima ao punctum, com evolução de 4 semanas. Uma biópsia excisional da lesão e o subsequente exame anatomopatológico revelaram que a massa era composta de células de Schwann com núcleos cônicos, croma­tina fina e nucléolos não visíveis. Ao exame imuno-histoquímico, as células fusiformes mostraram-se difusa e fortemente positivas para a proteína S100. A imunocoloração também foi positiva para neurofilamentos e evidenciou os axônios. Considerando esses achados, o tumor foi diagnosticado como um neuroma circunscrito solitário. Procedeu-se uma investigação completa para neoplasia endócrina múltipla tipo 2b, entretanto, estigmas característicos e história familiar não foram detectados. Assim, o diagnóstico foi firmado como neuroma circunscrito solitário, condição rara cujo diagnóstico diferencial baseia-se no exame anatomopatológico e na avaliação imuno-histoquímica. Já que esse tumor pode estar relacionado à neoplasia endócrina múltipla tipo 2b, torna-se essencial, nesses casos, a investigação da neoplasia de forma sistemática.

Neurofibroma cervicodorsal en una niña con neurofibromatosis tipo 1 / Cervical dorsal neurofibroma in a girl with neurofibromatosis type 1 neurofibroma / Neurofibroma cervicodorsal em uma criança com neurofibromatose tipo 1

La neurofibromatosis es el síndrome neurocutáneo más frecuente, una enfermedad hereditaria con afectación multisistémica. Se distinguen tres formas clínicas de la enfermedad; la neurofibromatosis tipo 1 (NF-1) es la más frecuente. A las lesiones cutáneas, presentes en la mayoría de los casos, se pueden asociar tumores en cualquier localización. Los neurofibromas son característicos de la NF-1. El objetivo de esta comunicación es describir una complicación poco frecuente de la NF-1 que representa un desafío terapéutico debido a la topografía y frecuencia de recidivas. Se trata de una niña de 3 años con NF-1 con síntomas de mielopatía multisegmentaria de instalación progresiva de dos meses de evolución. La imagenología evidenció una tumoración cérvico-dorsal con compresión medular. La exéresis tumoral permitió confirmar neurofibroma intradural, extramedular. Los neurofibromas plexiformes se caracterizan por su comportamiento infiltrativo y diseminación paraespinal extensa. La resección de estas lesiones genera habitualmente morbilidad asociada al compromiso de estructuras críticas extraespinales y dificulta su resección completa tal como ocurrió en el caso clínico presentado. Se destaca la importancia de realizar un correcto seguimiento clínico y eventualmente imagenológico de los pacientes con NF-1 para detectar y abordar oportunamente los tumores, su complicación más importante.

Neurofibromatosis is one of the most frequent neurocutaneous syndrome. It is an inherited diseasewhich affects multiple systems. Three clinical presentations of the disease can be distinguished being type 1 neurofibromatosis the most common. Tumors at any body location can be associated to the usually presented cutaneous lesions. Neurofibromas are characteristic of type 1 neurofibromatosis. The aim of this study is to describe a less frequent complication of the disease which represents a therapeutic challenge due to its topography and frequency of recurrences. This is the case of a 3 year-old girl with type 1 neurofibromatosis with symptoms of multisegmental myelopathy. A cervical-dorsal tumor with medullary compression was shown in medical imaging. Tumor extraction allowed to confirm intradural, extramedullary neurofibroma. Plexiform neurofibromas are characterized by their infiltrative behavior and extensive paraspinal dissemination. Resection of these lesions usually generates morbidity associated with the involvement of extra-spinal critical structures and hinders their complete resection as occurred in the presented clinical case. It is important to perform a correct clinical and eventually imaging follow-up of patients with NF-1 to detect and treat tumors, its most important complication.

Neurofibromatose é a syndrome neurocutânea mais freqüente, uma doença hereditária com envolvimento multissistêmico. Três formas clínicas da doença são distinguidas. A neurofibromatose tipo 1 (NF-1) é a mais freqüente. As lesões cutâneas, presentes na maioria dos casos, podem ser associadas a tumores em qualquer localização. Neurofibromas são característicos de NF-1. O objetivo desta comunicação é descrever uma complicação rara de NF-1 que representa um desafio terapêutico devido à topografia e freqüência de recidivas. Trata-se de uma menina de 3 anos com NF-1 com sintomas de mielopatia multisegmentar de instalação progressiva de dois meses de evolução. A imagem mostrou um tumor cérvico-dorsal com compressão da medula espinhal. A excisão do tumor permitiu confirmar neurofibroma intradural, extramedular. Os neurofibromes plexiformes são caracterizados pelo seu comportamento infiltrativo e sua disseminação paraspinal extensa. A ressecção dessas lesões geralmente gera morbidade associada ao envolvimento de estruturas críticas extra-espinhais e dificulta sua ressecção completa, como ocorreu no caso clínico apresentado. Destaca-se a importância de realizar um acompanhamento clínico e, eventualmente, de imagens de pacientes com NF-1 para detectar e tratar oportunamente os tumores, sua complicação mais importante.

Neurofibroma solitario: informe de caso clínico / Solitary neurofibroma: report of a clinical case report

Objetivo: Informar, a partir de la descripción de un caso clínico, la presencia de un neurofiboma solitario, lesión poco frecuente en cavidad bucal.Caso clínico: Un paciente de sexo masculino, de 47 años de edad, reconsultó por un bulto asintomático en la zona posterior de mucosa yugal izquierda. A partir de los datos de la historia clínica y de estudios complementarios, se procedió a efectuar la extirpación quirúrgica de la lesión, para luego realizar la biopsia y la anatomía patológica. Los resultadosconfirmaron la presencia de un neurofibroma solitario en cavidad bucal, en un sitio poco frecuente, como lo es la mucosa yugal. Conclusión: Si bien el neurofibroma es una lesión poco frecuente en cavidad bucal, su diagnóstico y su tratamiento oportuno son fundamentales para evitar posibles recidivas o transformaciones malignas.

Aim: To report and describe the presence of a solitaryneurofiboma, infrequent injury in the oral cavity, from the reportof a clinical case.Case report: A 47-year-old male patient consulted froman asymptomatic lump in the posterior area of the left jugularmucosa. From the data of the clinical history and complementarystudies, the surgical excision of the lesion was carriedout, after which the biopsy and the pathological anatomystudy were performed. The results confirmed the presence ofa solitary neurofibroma in the oral cavity, in a rare site, suchas the jugular mucosa.Conclusion: Although the neurofibroma is a rare lesionin the oral cavity, its diagnosis and timely treatment are fundamentalto avoid possible recurrences or malignant transformations.

Neurofibroma del mesenterio en un paciente sin enfermedad de Von Recklinghausen / Mesenteric neurofibroma seen in a patient without Von Recklinghausen´disease

Los neurofibromas son tumores benignos del sistema nervioso que pueden ser solitarios o múltiples: la neurofibromatosis o enfermedad de Von Recklinghausen. Estos tumores en general son de consistencia blanda, frecuentes a nivel de partes blandas, su localización en el aparato digestivo es infrecuente y no son encapsulados. El objetivo del presente trabajo fue presentar el caso de una paciente con neurofibroma mesentérico sin enfermedad de Von Recklinghausen. Se presenta el caso de una paciente de 41 años que fue ingresada por dolor abdominal y una tumoración palpable en la región entre flanco e hipocondrio izquierdo. En los estudios imaginológicos realizados se informó como quiste del mesenterio, lo que fue confirmado en el acto quirúrgico. Se realizó exéresis de la tumoración informada por Anatomía Patológica como neurofibroma secundariamente enquistado benigno. El neurofibroma mesentérico es una tumoración que puede verse en pacientes sin enfermedad de Von Recklinghausen.

Neurofibromas are benign tumors of the nervous system that can be solitary or multiple, the neurofibromatosis or Recklinghausen's disease. In general, these tumors are soft, non-encapsulated, more frequent in soft parts of the body and infrequent in the digestive system. The objective of the present paper was to present a female patient with mesenteric neurofibroma and without Von Recklinghausen disease. Here is a 41 years-old female patient who was admitted to the hospital because she suffered abdominal pain and a palpable tumor at the region between left flank and hypochondrium. The imaging studies revealed a mesenteric cyst, which was confirmed in surgery. The tumor was classified as a benign cyst neurofibroma by pathological anatomy and finally removed. The mesenteric neurofibroma is a tumor that can be found in patients without Von Recklinghausen disease.

Extracranial neurogenic tumors of the head and neck, / Tumores neurogênicos extracranianos na cabeça e pescoço

ABSTRACT INTRODUCTION: Peripheric nerve tumors typically derive from Schwann cells of the peripheral nerve sheet. Since these tumors are uncommon, they should be considered in preoperative differential diagnosis. OBJECTIVE: To report the experience of a tertiary care department. METHODS: Forty-two patients with head and neck peripheral neurogenic tumors were retrospectively analyzed and evaluated from 1977 to 2013. The preoperative diagnosis was confirmed by biopsy or imaging study. RESULTS: The mean age was 41.7 and 15 patients (36%) were male. The mean size was 5.5 cm and 26 (61%) were located laterally in the neck. Most tumors (39.9%) presented as an asymptomatic neck mass. Most (39.9%) were resected through a neck approach. Cranial nerves were the commonest site of origin. CONCLUSIONS: Extracranial neurogenic tumors presented with a mean size of 5.5 cm, were located laterally in the neck, normally had their origin from cranial nerves, and their resection approach is cervical.

Resumo INTRODUÇÃO: Tumores dos nervos periféricos tipicamente derivam das células de Schwann da bainha dos nervos periféricos. Por serem incomuns, devem ser lembrados no diagnóstico diferencial pré-operatório. OBJETIVO: Relatar a experiência de serviço de referencia terciária. MÉTODO: De 1977 a 2013, 42 pacientes com tumores neurogênicos periféricos da cabeça e pescoço foram operados e analisados retrospectivamente. A confirmação diagnóstica pré-operatória deu-se por biópsia ou método de imagem. RESULTADOS: A média da idade foi de 41,7 anos, sendo 15 indivíduos (36%) do gênero masculino. O tamanho médio foi de 5,5 cm e 26 (61%) localizavam-se na face lateral do pescoço. A maior parte (39,9%) apresentou-se como tumor palpável assintomático. A maioria (39,9%) foi ressecadapor acesso cervical. A maioria originou-se de nervos cranianos. CONCLUSÕES: Tumores neurogênicos extracranianos apresentam-se com tamanho médio de 5,5 cm, na face lateral do pescoço, costumam originar-se de nervos cranianos e ser ressecados por via cervical.

Oral Manifestations of Neurofibromatosis: A Rare Case Report.

Neurofibroma (NF) is a rare benign non-odontogenic tumor of the oral cavity. NF may present either as solitary lesions or as part of the generalized syndrome of NF or von Recklinghausen’s disease of the skin. The heterogeneous nature of NF was established by Riccardi et al. and he recognized at least seven types of NF. Among these proposed classifications of the disease, two distinct forms are generally accepted namely, a peripheral form known as NF Type I (NF-I), and a central form known as NF-II. NF-I represents the classic form of this disease, described by Recklinghawsen in 1882. Clinically, oral NF usually appears as slow growing, painless, pedunculated or sessile nodules. For illustration, a case of a NF with oral findings is been presented.

Neurofibroma arising from phrenic nerve.

We report a case of neurofibroma arising from the left phrenic nerve and not associated with neurofibromatosis in a 46-year-old male. The patient presented with gradually progressive breathlessness and pain in chest for six months. Radiological investigations revealed a posterior mediastinal mass on the left side of the chest. On postero-lateral thoracotomy, the resected tumour was found to be arising from the phrenic nerve. Histopathological examination confirmed it to be a neurofibroma. Absence of any other distinctive lesions of neurofibromatosis makes this an unusual presentation.

Fibrosarcoma en región geniana / Fibrosarcoma in the genial region

El fibrosarcoma es la neoplasia maligna mesodérmica de más incidencia.Los autores se vieron motivados a presentar un caso clínico con esta entidad con el objetivo de enfatizar en la importancia del correcto manejo diagnóstico y terapéutico en función de la rehabilitación de estos pacientes. Se presenta un caso clínico de un paciente de 54 años de edad con antecedentes de HTA controlada, que 23 años atrás comenzó con aumento de volumen en región geniana izquierda, motivo por el que fue intervenido quirúrgicamente en los años 1987, 1991 y 2009, en otros servicios de Cirugía Máxilofacial con diagnóstico histopatológico de neurofibroma, que acudió a consulta presentando una lesión de 5 cm de diámetro, con patrón de crecimiento rápido, que sangraba espontáneamente, la cual le comprimía el globo ocular izquierdo limitándole la apertura palpebral. Luego de realizársele el estudio preoperatorio correspondiente, se decidió tratamiento quirúrgico, se obtuvo un diagnóstico concluyente a través del estudio inmunohistoquímico de fibrosarcoma de grado intermedio de diferenciación, de marcada inmunorreactividad CD34 (+, focal), Vimentina (+, focal). Se concluye que el tratamiento de esta entidad es quirúrgico, teniendo en cuenta las normas oncológicas actuales, y su enfoque terapéutico debe ser multidisciplinario en aras de lograr una correcta rehabilitación funcional y estética del paciente. La inmunohistoquímica constituye un arma diagnóstica concluyente que contribuye a instaurar tratamiento adecuado en estas lesiones.(AU)

The fibrosarcoma is the more incident mesodermic malignant neoplasm. Authors present this clinical case presenting with this entity to emphasize on the significance of the appropriate diagnostic and therapeutical management for rehabilitation of these patients. This patient is aged 54 with a history of controlled high blood pressure (HBT) who over the 23 past years begun to show an increase of volume in left genial region thus its surgical intervention in 1987, 1991 and 2009 years and came to consultation presenting with a 5 cm diameter lesion with a fast growth pattern bleeding spontaneously compressing the left ocular globe restricting the palpebral opening. After the corresponding preoperative study authors prescribed the surgical treatment obtaining a conclusive diagnosis according to a immunohistochemical study of the fibrosarcoma of intermediate degree of differentiation with a marked CD34 (+, focal) immunoreactivity, Vimentin (+,focal). We conclude that the treatment of this entity is of surgical type, taking into account the current oncology guidelines and its therapeutical approach must to be multidisciplinary to achieve the functional and aesthetic rehabilitation of the patient. The immunohistochemistry is a conclusive diagnostic tool contributing to apply the appropriate treatment in this type of lesion(AU)

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.

Neurofibromatose tipo 1 com manifestação oral: relato de caso e revisão da literatura / Neurofibromatosis type 1 with oral manifestation: a case report and literature review

Neurofibromatosis type 1 is an autosomal dominant condition characterized by the presence of benign skin tumors, alterations in the nervous system, bones and endocrine glands. It may be followed by mild mental retardation, convulsions, malignant tumors, osseous dysplasia and abnormal pigmentation. The aim of this paper is not only to report the case of a patient with neurofibromatosis type 1 with manifestation in the oral cavity, represented by the presence of a large nodular mass in the tongue, but also to undertake a brief review of this condition. The work concluded that, despite the fact that neurofibromatosis type 1 is a rare condition, which may present manifestations in the oral cavity, its study is of paramount importance to dental surgeons and multidisciplinary groups.

A neurofibromatose tipo 1 é uma condição hereditária autossômica dominante caracterizada pela presença de tumores benignos na pele, alterações em sistema nervoso, ossos e glândulas endócrinas, e que pode ser acompanhada de leve retardo mental, convulsões, tumores malignos, displasia esquelética e desordem da pigmentação. O trabalho tem como objetivo relatar o caso de uma paciente portadora de neurofibromatose tipo 1 com manifestação na cavidade oral, representada pela presença de extensa massa nodular na língua, além de fazer uma breve revisão dessa condição. Concluiu-se que, apesar de a neurofibromatose tipo 1 ser uma condição rara que pode apresentar manifestação na cavidade oral, é de extrema importância o seu conhecimento por parte dos cirurgiões-dentistas e da atuação de uma equipe multidisciplinar.

[Solitary neurofibroma of mandible in a 10 years old girl; report of a case]

Neurofibroma is the most common type of peripheral nerve neoplasm. It arises from a mixture of cell types including schwann cells and perineural fibroblasts. It can arise as solitary tumor or may be a component of neurofibromatosis. The skin is the most frequent location for neurofibroma. Lesions of the oral cavity are uncommon, but when occur, the tongue and buccal mucosa are the most common intraoral site. On rare occasions, the tumor can arise centrally within bone, either as a well demarcated or poorly defined unilucular or multilucular radiolucency. In this article, a case of solitary intraosseous neurofibroma of mandible in a 10 years old girl with a discussion on its clinical, radiological, and histopathological characteristics is reported

Dermatological manifestations of epilepsy among adult Sundanese epileptic patients

To study the pattern of dermatological changes associated with epilepsy among adult Sudanese epileptic patients. This non-interventional descriptive study included 360 adult Sudanese epileptic patients and was conducted at the El Shaab Teaching Hospital, Khartoum, Sudan, from February 2004 to August 2007. All patients had full detailed history and clinical examination. A dermatologist assessed the dermatological changes. Investigations carried out included EEG, CT brain, and serial of drug serum levels. Out of 360 patients, 31 were found to have scars due to repeated attacks of convulsions, one patient was found to have neurofibromatoma, one had tuberous-sclerosis, one had Sturge-Weber syndrome, one had Kaposi sarcoma, one had systemic lupus erythematosus, one diabetic patient had skin atrophy, one patient taking phenobarbitone had skin eruption, one patient on carbamazepine had skin changes, while 5 patients on phenytoin had skin manifestations. Skin changes can occur in epileptic patients as part of drug toxicity, or as part of the clinical manifestations of certain diseases that can cause secondary epilepsy, for example, neurofibroma

Neurofibroma central de la mandíbula: informe de un caso / Central neurofibroma of the mandible: report of a case

El neurofibroma raramente aparece como una lesión solitaria, excepto cuando está presente en los huesos maxilares. Por el contrario, múltiples lesiones se observan en la neurofibromatosis de Von Recklinhausen. El neurofibroma central ha sido informado tanto en mandíbula como en maxilar; sin embargo, esta última ubicación es la más común. Presentamos un caso de neurofibroma central de la mandíbula.

Neurofibroma solitario de la región preauricular: presentación de un caso clínico / Solitary neurofibroma of the preauricular region: report of a clinical case

EL neurofibroma es un tumor benigno que deriva de las células de Schwann y de los fibroblastos perineurales de los nervios periféricos. Dentro de los tumores de cabeza y cuello, presenta una frecuencia muy baja, pero es el tumor más frecuente de origen neurógeno. Se presentan solitarios o formando parte de la neurofibromatosis múltiples tipo 1 (enfermedad de Von Recklinghausen). El diagnóstico prequirúrgico es dificultoso. Se llega ala diagnóstico definitivo tras el estudio histopatológico de la pieza quirúrgica. Se presenta un caso clínico y se realiza la revisión bibliográfica correspondiente

Neurofibroma subglótico: diagnóstico y tratamiento quirúrgico / Subglotic neurofibroma: diagnostic and surgical treatment

El neurofibroma de laringe es una patología muy poco frecuente, clínicamente se caracteriza por una sintomatología asociada a la obstrucción de la vía aérea superior. Se presenta el caso de un paciente masculino de 54 años de edad quien consulta por estridor y disnea, se le practica una fibrobroncoscopia flexible que reporta la presencia de una tumoración subglótica de tamaño 3x3 cm. Se realiza como procedimiento quirúrgico: una cervicotomía y laringofisura a través del cartílago tiroides con exposición del área subglótica y resección completa de la tumoración, cuyo resultado histológico reportó un neurofibroma. La evolución posoperatoria fue satisfactoria, actualmente el paciente se encuentra sin recidiva de la tumoración posterior a cinco años de evaluación continua.

Solitary intraosseous neurofibroma of mandible.

Solitary intraosseous neurofibroma is a rare benign non-odontogenic tumor. Although neurofibromas occur predominantly as a feature of neurofibromatosis affecting the soft tissue, a few cases of solitary intraosseous neurofibromas of the jaw have been reported. We herewith report a case of solitary intraosseous neurofibroma of mandible in a middle-aged woman with a discussion on its clinical, radiological, and histopathological presentation along with review of cases.

Malignant peripheral nerve sheath tumor of breast in patient without Von Recklinghausen's neurofibromatosis: a case report.

Malignant peripheral nerve sheath tumor (MPNST) of the breast without Von Recklinghausen's neurofibromatosis (VRN) is extremely rare. The authors report a 19 year-old woman who presented with a 12 month history of a painless mass of the left breast. Tissue biopsy was performed. The histologic diagnosis was made with immunohistochemical study in which the tumor showed positivity of vimentin, S-100 protein, neuron-specific protein(NSE), neurofilament protein(NF) and glial fibrillary acidic protein(GFAP). The patient was referred for radiation therapy after simple mastectomy.

Giant ocular nerve neurofibroma of the cavernous sinus: a series of 5 cases.

Five cases of giant cavernous sinus neurofibroma arising from the ocular cranial nerves are reported. These patients collected over a period of 5 years consisted of 3 males and 2 females with an age range of 9 to 40 years and a mean of 20.6 years. Clinically, all patients presented with ocular palsies over a long period (mean 3.4 years). All of them underwent a frontotemporal craniotomy along with an orbito-zygomatic osteotomy and excision of the tumour. In patients with extension of the tumour into the orbit, the superior orbital fissure was drilled, the tenon's capsule was cut and the intraorbital portion was excised. The management of these tumours is discussed and the literature reviewed.