Epidemiological Profile of 96 Intracranial Tumors Treated in a Single Reference Center

Objectives The present study aims to categorize the prevalence of intracranial tumors surgically treated at the neurosurgery service of Hospital Universitário Evangélico Mackenzie (HUEM) between 2016 and 2018. Material and Methods This survey included patients surgically treated due to primary or metastatic intracranial neoplasia between 2016 and 2018 at a referral center in the city of Curitiba. These patients were analyzed for epidemiological, histopathological, and topographic data, and they underwent an assessment of the outcome at the time of hospital discharge. Results Atotal of 96patientsmet the inclusion criteria. Themost prevalent tumorwas the glioma, with 39.6% of the sample, with glioblastoma being themost prevalent histological type. Brainmetastases andmeningiomas represented, respectively, 21.9%and 18.8%of the total. There was a predominance of supratentorial and intra-axial tumors in our sample. Conclusion Glioma was the most commonly found tumor, directly associated with high morbidity and mortality. The development of new and more effective drugs with action directed at themolecular level of intracranial tumorsmay be the path to a longer survival and improvement in the quality of life of these patients.

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

Resonancia magnética con secuencia HASTE de carcinoma epidermoide del hueso temporal / HASTE magnetic resonance imaging of a squamous cell temporal bone carcinoma

El carcinoma epidermoide del hueso temporal es una neoplasia derivada de células epidérmicas del estrato espinoso, y que dado su baja incidencia, y presentación clínica similar a un cuadro de otitis media crónica colesteatomatosa, su diagnóstico es habitualmente tardío. El estudio del carcinoma epidermoide del hueso temporal se realiza con tomografía computarizada, la que evidencia erosión ósea. Sin embargo, existen distintos diagnósticos diferenciales, tanto malignos como benignos, que pueden provocar hallazgos similares en la tomografía computarizada. La resonancia magnética (RM) es un método de exploración de gran valor complementario para el estudio del carcinoma de oído medio. La RM con secuencia de difusión HASTE ha surgido como herramienta de estudio en patología de hueso temporal, tales como colesteatomas y teratomas de oído medio, pese a esto, no existen reportes en la literatura de la presentación imagenológica del carcinoma epidermoide de hueso temporal utilizando este método. En este artículo se presenta un caso clínico de una paciente con carcinoma epidermoide de oído medio, cuyo estudio preoperatorio incluyó RM-HASTE. Aun cuando se requiere un mayor número de casos para establecer el patrón imagenológico, se concluye que esta técnica es de utilidad para diferenciar este tumor de otitis media crónica colesteatomatosa.

Squamous cell carcinoma of the temporal bone (SCC) is a malignancy originated from epidermis spinous cells. Its low incidence and similar presentation to cholesteatomas, contributes to its late diagnosis. The most widely used study for this tumor is computed tomography (CT), which mainly evidences erosion of the temporal bone. There are several other diagnoses, malignant and benign, that could provoke similar findings at the CT. Magnetic resonance imaging (MRI) is a method of great value to study SCC. MRI with HASTE diffusion sequence has recently emerged as an important tool in the study of temporal bone pathologies, such as cholesteatoma and middle ear teratoma, despite this, there are no reports in the literature of the imaging presentation of SCC using this method. This article presents a clinical case of a patient with SCC, in whose preoperative study was performed HASTE-MRI. Although a larger number of cases are required to establish an imaging pattern, it is concluded that this technique is useful to differentiate this tumor from middle ear cholesteatomas.

Malignant tumors of the temporal bone - our experience / Tumores malignos do osso temporal - a nossa experiência

ABSTRACT INTRODUCTION: Malignant tumors of the temporal bone are rare, with an estimated incidence of about 0.8-1.0 per 1,000,000 inhabitants per year. The vast majority of these tumors are squamous cell carcinomas and their treatment is eminently surgical. OBJECTIVE: This study is an attempt at systematizing the forms of clinical presentation, the therapeutic possibilities, and oncological outcomes of patients with malignant tumors of the temporal bone in a tertiary hospital in Portugal. METHODS: The authors present a retrospective study of temporal bone tumors treated and followed during otorhinolaryngology consultations between 2004 and 2014. A review of the literature is also included. RESULTS: Of the 18 patients included in the study, 16 had a primary tumor of the temporal bone, in most cases with squamous cell carcinoma histology. Of these, 13 patients were treated with curative intent that always included the surgical approach. Disease persistence was observed in one patient and local recurrence in five patients, on average 36.8 months after the initial treatment. CONCLUSIONS: The anatomical complexity of the temporal bone and the close associations with vital structures make it difficult to perform tumor resection with margins of safety and thus, tumor relapses are almost always local. A high level of suspicion is crucial for early diagnosis, and stringent and prolonged follow-up after treatment is essential for diagnosis and timely treatment of recurrances.

Resumo Introdução: Os tumores malignos do osso temporal são raros, com uma incidência estimada de cerca de 0,8-1 por milhão de habitantes por ano. A grande maioria são carcinomas espinocelulares e o seu tratamento é eminentemente cirúrgico. Objetivo: Este trabalho tem como objetivo tentar sistematizar as formas de apresentação clínica, as possibilidades terapêuticas e os resultados oncológicos de doentes com tumores malignos do osso temporal num hospital terciário em Portugal. Método: Os autores apresentam um estudo retrospectivo de tumores do osso temporal tratados e acompanhados em consultas de otorrinolaringologia entre 2004 e 2014. É também apresentada uma revisão da literatura. Resultados: Dos 18 doentes incluídos no estudo, 16 apresentavam um tumor primário do osso temporal, na maioria dos casos com histologia de carcinoma espinocelular. Destes, 13 doentes foram submetidos a tratamento com intuito curativo que incluiu sempre uma abordagem cirúrgica. Verificou-se persistência da doença em 1 doente e recidiva local em 5 doentes, em média 36,8 meses após o tratamento inicial. Conclusões: A complexidade anatómica do osso temporal e as estreitas relações com estruturas de importância vital tornam difícil a exérese tumoral com margens de segurança, pelo que as recidivas tumorais são quase sempre locais. Um nível de suspeição elevado é fundamental para um diagnóstico precoce e o seguimento rigoroso e prolongado após o tratamento é essencial para o diagnóstico e tratamento oportuno das recidivas.

Reconstruções craniofaciais: ainda há espaço para os retalhos locorregionais? / Craniofacial reconstructions: is there still room for locoregional flaps?

Introdução: Os tumores avançados de cabeça e pescoço ainda têm elevada prevalência no Brasil. A reconstrução de um defeito resultante de ressecção craniofacial é um desafio para o cirurgião plástico. Os retalhos livres são a primeira escolha para essas reconstruções e os retalhos locorregionais têm sido utilizados em casos selecionados. O objetivo deste estudo é avaliar uma série de reconstruções com retalhos locorregionais em pacientes submetidos a ressecções oncológicas craniofaciais, demonstrando a aplicabilidade desses retalhos e o resultado final das reconstruções. Métodos: Foram avaliadas, retrospectivamente, quatro reconstruções craniofaciais com retalhos locorregionais de fronte e couro cabeludo. Os pacientes foram operados no Hospital das Clínicas da Universidade Federal de Pernambuco (HC-UFPE) e assinaram termo de consentimento permitindo a publicação científica de suas fotos e casos clínicos. Resultados: Todas as reconstruções foram bem sucedidas. Não ocorreram casos de infecção local ou meningite, necrose do retalho, fístulas liquóricas ou deiscências. As reconstruções com retalhos locorregionais apresentadas foram seguras e simples para reconstruir defeitos extensos em região craniofacial. Conclusões: Os cirurgiões plásticos podem realizar reconstruções complexas com estes retalhos, demonstrando que ainda há espaço para este tipo de reconstrução.

Introduction: Advanced tumors of the head and neck still have a high prevalence in Brazil. Reconstructing a defect resulting from a craniofacial resection is a challenge for the plastic surgeon. Free flaps are the first choice for these reconstructions, and locoregional flaps have been used in selected cases. The objective of this study was to evaluate a number of reconstructions with locoregional flaps in patients undergoing craniofacial oncologic resection, to demonstrate the applicability of these flaps and the end result of the reconstructions. Methods: We retrospectively studied four craniofacial reconstructions with locoregional flaps on the forehead and scalp. The patients were operated at the Clinical Hospital of the Federal University of Pernambuco. They signed a consent form allowing the scientific publication of their photographs and clinical case records. Results: All reconstructions were successful. There were no cases of local infection or meningitis, flap necrosis, liquor fistula, or dehiscence. Reconstructions with locoregional flaps were safe and simple to perform for extensive defects in the craniofacial region. Conclusions: Plastic surgeons can perform complex reconstructions with locoregional flaps, demonstrating that there is still room for this type of reconstruction.

Lesiones etmoidales con extensión secundaria: estrategia quirúrgica y complicaciones / Ethmoidal lesions with secondary extension: surgical strategies and complications

Introduction: The removal of ethmoidal tumors with secondary extension to the cranial base and/or facial region involves a high complexity and it is associated to a high morbility. Objective: To determine the results of craniofacial surgery in patient with ethmoid extended tumors. Methods: It was carried out a traverse retrospective descriptive study. The sample was conformed by the patients intervened surgically of anterior cranial base lesions by means of a combined craneofacial surgery during the period: January of the 2009 to January of the 2012 in the National Institute of Oncology and Radiobiology with a 2 year pursuit. Descriptive statistical variables were used. Results: 20 patients were intervened. The age average was of 44,8 years. It prevail the masculine sex (65 percent). Nasal obstruction constitutes the most frequent presentation. Adenocarcinoma and epidermoid carcinoma constituted the most frequent malignant lesions. Inside the benign lesions prevail the invertedpapiloma. The techniques more employees were the bilateral frontal craneotomy and total etmoidectomy. The most frequent complication was the cerebrospinal fluid leak. The 2 years overall survival in patients with malignant lesions was 35 percent. Conclusions: A high number of complications was identified but they didn’t affect the survival neither the quality of life. An acceptable rate of survival was achieved in malignant lesions.

Introducción: La remoción de lesiones etmoidales con extensión secundaria a la base cranealy/o región facial, entraña una elevada complejidad y se encuentra asociada a una elevada morbilidad. Objetivo: Determinar los resultados de la cirugía combinada craneofacial en pacientes con neoplasias etmoidales extendidas. Métodos: Se realizó un estudio descriptivo retrospectivo transversal. La muestra estuvo conformada por los pacientes intervenidos quirúrgicamente de lesiones de base craneal anterior mediante un abordaje combinado craneofacial durante el período: enero de 2009 a enero de 2012 en el Instituto Nacional de Oncología y Radiobiología con un seguimiento de 2 años. Se emplearon variables estadísticas descriptivas. Resultados: Fueron intervenidos 20 pacientes. El promedio de edad fue de 44,8 años. Predominó el sexo masculino (65 por ciento). La obstrucción nasal constituyó la presentación más frecuente. El adenocarcinoma y el carcinoma epidermoide constituyeron las lesiones malignas más frecuentes. Dentro de las lesiones benignas predominó el papiloma invertido. Las técnicas más empleadas fueron la craneotomía frontal bilateral y la etmoidectomía total. La complicación más frecuente fue la fístula de líquido cefalorraquídeo. La supervivencia global a los 2 años en los pacientes con lesiones malignas fue del 35 por ciento. Conclusiones: Se identificó un elevado número de complicaciones pero no afectaron la supervivencia ni la calidad de vida. Se logró una aceptable tasa de supervivencia en lesiones malignas.

Carcinoma de osso temporal sem foco metastático primário: relato de caso e revisão de literatura / Carcinoma temporal bone unfocused metastatic primary: case report and literature review

Os tumores do osso temporal são raros e geralmente apresentam sintomas como otorreia, otalgia e hipoacusia, por isso podem ser facilmente confundidos com um processo infeccioso, retardando o diagnóstico e piorando o prognóstico do paciente. KS, 7 anos, masculino. Estado geral: regular. Ao exame: consciente; linfonodos cervicais palpáveis, móveis; massa palpável, imóvel e indolor em topografia retroauricular esquerda; surdez à esquerda e paralisia facial esquerda. Tomografia computadorizada de crânio evidenciou lesão expansiva do osso temporal captante de contraste com extensão para fossa média e posterior do crânio. Realizada complementação radiológica com ressonância de encéfalo, a qual apresentou imagem hipercaptante em T1 contrastado. Nas incidências T2, observa-se edema lobotemporal adjacente à lesão. Exame angiográfico cerebral apresentou obstrução tumoral do seio sigmoide esquerdo. Paciente submetido à mastoidectomia radical esquerda com ligadura e ressecçãodo seio sigmoide esquerdo resultando em ressecção completa lesional. Anatomopatológico e exame imunoistoquímico compatíveis com adenocarcinoma. Encaminhado para terapia oncológica com quimioterapia e radioterapia. Óbito após quatro meses do tratamento neurocirúrgico. O diagnóstico precoce associado com a extensão do tumor acarreta melhor ou pior prognóstico para os pacientes acometidos por essa moléstia.

Tumors of the temporal bone are rare and usually have symptoms such as otorrhea, otalgia and hearing loss and, therefore, can be easily confused with an infectious process delaying diagnosis and worse prognosis. KS, age 7, male. General condition: regular. On examination: conscious, palpable cervical lymph nodes, mobile; palpable mass, painless and property surveying retroauricular left, left deafness and facial paralysis left. Cranial computed tomography showed a lesion of the temporal bone with extensionto the middle fossa and posterior skull that enhance with contrast. Performed complementation with radiological MRI brain image which showed uptake, in contrast T1. Incidences in T2, there is edema temporal lobe adjacent to the lesion. Cerebral angiography showed a tumor obstructing the left sigmoidsinus. Patient underwent left radical mastoidectomy with ligation and resection of the left sigmoid sinus resulting in complete lesional resection. Histopathological and immunohistochemical examination compatible with adenocarcinoma. Referred for cancer therapy with chemotherapy and radiation. Death within four months after neurosurgical treatment. Early diagnosis associated with tumor extension carries a better or worse prognosis for patients affected by this disease.

Implantes extraorais em pacientes irradiados / Extraoral implants in irradiated pacients

O objetivo deste trabalho foi avaliar o resultado da osseointegração de implantes extraorais lemento de suporte e retenção de próteses nas reabilitações de deformidades faciais. MÉTODO: Estudo retrospectivo de prontuários de 59 indivíduos operados de câncer, submetidos a 164 implantes para retenção de prótese facial. RESULTADOS: Dos 59 indivíduos, 14 foram previamente irradiados e receberam 42 implantes. Quarenta e cinco indivíduos não foram irradiados e receberam 122 implantes. Do total de 164 implantes, oito não osseointegraram, dos quais dois foram instalados em osso previamente irradiados. O resultado representou o sucesso de 116 (95,1%) implantes osseointegrados nos indivíduos não irradiados. E sucesso de 40 (95,3%) implantes osseointegrados em ossos irradiados. CONCLUSÃO: Concluiu-se que o uso de implantes extraorais é uma técnica segura e eficaz como suporte e retenção para próteses faciais em indivíduos com deformidades nessa região. A radioterapia não impede a osseointegração.

The aim of this study is to analyze the success of extraoral osseointegrated implants used to support designed to rehabilitate craniofacial deformities. METHOD: This study was based on the retrospective assessment of charts from 59 patients submitted to cancer surgery and who received 164 extraoral implants to contain facial prosthesis. RESULTS: Among 164 implants, 42 were fixed in previously irradiated regions. Eight of the implants did not have osseointegration; and from these, two were fixed in irradiated bone. The result show 116 (95.1%) successfully osseointegrated implants in non-irradiated sites. The success rate among 42 implants fixed in previously irradiated bones was 40 (95.3%) osseointegrated implants. CONCLUSION: The use of extraoral craniofacial implants represents a safe and effective approach to treat facial deformities as a support for the rehabilitation prosthesis. Radiotherapy treatment does not prevent osseointegration.

Tumor gigante en un paciente con melanoma / Giant tumor in a patient with melanoma

Se estudió un paciente masculino, negro, de 17 años de edad, caribeño, aparentemente sano hasta el año 2007 cuando aparece un tumor témporo-parieto-frontal izquierdo de crecimiento rápido, que compromete la cara. Al examen físico se constató, además, piel y mucosas pálidas con desnutrición severa. El diagnóstico histopatológico fue de melanoma maligno de células epiteloides...

The case of a black Caribbean patient aged 17 apparently healthy until 2007 when to appear a left temporal-parietal-frontal tumor of fast growth involving the face. At physical examination authors also confirmed a pale skin and mucosae with a severe malnutrition. The histopathological diagnosis was a malignant melanoma of epithelioid cells...

Abordaje minimamente invasivo supraorbitario lateral Key - hole: experiencia de 64 casos / Minimally invasive lateral supraorbital Key - hole: experience of 64 cases

Este trabajo presenta la experiencia de 64 pacientes tratados con abordaje supraorbitario lateral (key hole): 33 pacientes con hidrocefalia en los que se realizó fenestración de la lamina terminalis, 11 pacientes con extirpación de tumores de base de cráneo, 20 pacientes con aneurisma cerebral de circulación anterior. Se describen algunas modificaciones en la técnica quirúrgica, complicaciones y seguimiento.

This works presents the experience of 64 patients treated with supraorbital approach (key hole): 33 with hydrocephalus in which was performed the fenestration of the lamina terminalis, 11 with cranial basal tumors, 20 with anterior circulation aneurysm. Some modification of the technique, complications and medical following are described.

The evidence-based management of bilateral ethmoid osteomas: diagnosis, endoscopic resection and review of the literature / Tratamiento basado en la evidencia para el osteoma etmoidal bilateral: diagnóstico, resección endoscópica y revisión de la literatura

A 56-year old woman presented with headaches and nasal congestion secondary to bilateral ethmoid osteomas and she made a full recovery following endoscopic resection. Paranasal osteomas are common, benign, encapsulated tumours that are often asymptomatic, but may present similarly to rhinosinusitis and occasionally cause complications depending on their anatomical location. Bilateral ethmoidal osteomas, however, are a rare occurrence and have not been previously reported in the English literature as far as the authors are aware. Ethmoidal and fronto-ethmoidal osteomas, like other paranasal osteomas, are easily diagnosed using computed tomography and are increasingly amenable to endoscopic resection. An evidence-based management plan is presented.

Una mujer de 56 años se presentó con los dolores de cabeza y congestión nasal secundaria a un osteoma etmoidal bilateral, tras su recuperación total a partir de una resección endoscópica. Los osteomas paranasales son tumores comunes, benignos, y encapsulados, a menudo asintomáticos, pero que pueden presentarse de modo similar a la rinosinusitis, y ocasionalmente causar complicaciones, en dependencia de su localización anatómica. Los osteomas etmoidales bilaterales, sin embargo, son una ocurrencia rara y hasta donde sabemos, no ha sido reportado con anterioridad en la literatura en inglés. Los osteomas etmoidales y fronto-etmoidales - al igual que otros osteomas paranasales - se diagnostican fácilmente usando CT, y son cada vez más tratables mediante resección endoscópica. Se presenta un plan de tratamiento basado en la evidencia.

Dermoid cyst of the anterior fontanelle in adults: case report

Head and neck dermoid cysts are lesions relatively rare, which usually occur during childhood as solitary lesions. They are often identified and surgically removed at birth, being uncommon in adults. A 23-year-old male presented with a congenital tumor of the anterior fontanelle, which histopathological examination revealed a dermoid cyst. Surgical intervention is the treatment of choice to remove this lesion. The objective of this study is to report the case, once this type of lesion is rare in adults.

Cistos dermóides de cabeça e pescoço são relativamente raros e, usualmente, ocorrem na infância como lesões solitárias. Eles são diagnosticados e operados ao nascer, na maioria dos casos; portanto, essa é uma lesão incomum no adulto. Um homem de 23 anos apresentava tumoração congênita na fontanela anterior, cujo exame histopatológico revelou ser cisto dermóide. Foi submetido a tratamento cirúrgico. O objetivo desse estudo é relatar o caso, uma vez tratar-se de condição rara no adulto.

Teratoma maduro cráneo facial derecho: presentación de un caso clínico inusual / Mature teratoma cranio facial right: clinical presentation of a case unusual

El teratoma maduro es una patología que se presenta con poca frecuencia en región cráneo facial ya que en la literatura se describen con mayor frecuencia los teratomas en región sacro coccigea. En niños aparece a muy corta edad y en niñas cerca de la pubertad de localización ovárica. Estos tumores deben ser sometidos a resección quirúrgica, trantando de extirparlo en su totalidad para evitar su recidiva. Presentamos el caso de un neonato de 4 días de edad que ingresa al Hospital del Niño de la ciudad de La Paz por presentar una tumoración en región cráneo facial lateral derecha, de consistencia mixta, base no movible, con una dimensión de 10 cm x 10 cm. A objeto de llegar a un diagnóstico que permita la exéresis del tumor, se realizaron varios exámenes complementarios los cuales finalmente posibilitaron su retiro quirúrgico, correspondiendo el tumor a un teratoma maduro cráneo facial derecho. Se publica el presente caso por su localización y dimensiones inusuales.

The ripe teratoma is a pathology that appears seldom in the craneofacial region, since in the literature the teratomas in the sacrococcigeal area are described more often. In boys it appears at a veryyoung age, and in girls close to puberty being localized in the ovaries.These tumours must be surgically excised, trying to extirpate themtotally in order to avoid reappearance of the growth.We present the case of a 4 days old newborn who was admitted tothe Hospital del Niño in La Paz, due to a tumour in the right lateralcraneo facial region, of mixed consistence, base not movable, with adimension of 10 cm x 10 cm.With the purpose to arrive at a diagnose that permitted the excisionof the tumour, various complementary examinations were carried outwhich finally made the surgical extirpation possible being the tumoura ripe right craneo facial teratoma. We publish the present casebecause of its unusual localization and dimensions.

Accesos quirúrgicos a los meningeomas petroclivales: indicaciones, complicaciones y resultados / Surgical approaches for petroclival meningiomas: indications, complications and results

Introducción: Los meningeomas de la región petroclival son responsables de cerca del 13 por ciento de los meningeomas de la base del cráneo. El promedio de edad de los pacientes que los presentan es aproximadamente 45 años En la mayoría de los casos existe extensión a la región paraselar. Material y Métodos: Los autores realizan una revisión crítica de la literatura, presentando los posibles accesos quirúrgicos e sus indicaciones, efectividad e posibles complicaciones. Resultados: Los accesos quirúrgicos para la región petroclival pueden ser: petroso, vía petrosa combinada anterior y posterior, vía presigmoidea combinada supra e infratemporal, órbito-zigomático infratemporal, retrosigmoideo intradural, suprameatal, pterional, retromastoideo, subtemporal y vía combinada retromastoidea-subtemporal. Discusión y Conclusiones: Una valoración neuro-radiológica precisa que permita escoger un abordaje quirúrgico apropiado juntamente con avances en la técnica microquirúrgica y propuestas de nuevos accesos, son factores que han contribuido al progreso significativo en el tratamiento quirúrgico de los meningeomas petroclivales.