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.

Fortificación del arroz para corregir la deficiencia de micronutrientes en niños de 6 a 59 meses de edad / Rice fortification to correct micronutrient deficiency in children 6-59 months old

Objetivo. Realizar una revisión sistemática de la literatura sobre la eficacia del consumo de arroz fortificado en el incremento de los niveles de hierro y otros micronutrientes en niños de 6 a 59 meses de edad, con la finalidad de evaluar su utilidad como intervención de salud pública. Métodos. La búsqueda se realizó en las bases de datos Medline, Embase, The Cochrane Library y LILACS. Se incluyeron ensayos clínicos aleatorizados (ECA) que evaluaron el consumo de arroz fortificado comparado con placebo u otras formas de intervención sobre la mejora de los niveles de hierro y otros micronutrientes. Para evaluar la calidad metodológica, se utilizó la lista de verificación CONSORT®. El riesgo de sesgo de los estudios se evaluó según la metodología de la Colaboración Cochrane®. Resultados. Se incluyeron siete ECA en la revisión. Todos los estudios mostraron mejoras significativas en indicadores del estado nutricional de hierro en los grupos intervenidos, sin reportar efectos adversos. No se evidenció mejoría en los niveles de vitamina A, ni en los indicadores antropométricos de peso y talla como resultado secundario de la intervención. Los estudios incluidos mostraron calidad metodológica moderada. Conclusiones. La fortificación del arroz representó una estrategia de intervención eficaz para corregir la deficiencia de hierro en la población infantil menor de cinco años. Su implementación como medida de salud pública requiere estudios locales que evalúen su efectividad en intervenciones a largo plazo y en mayor escala.

Objective. To carry out a systematic review of the literature on the effectiveness of fortified rice consumption in terms of increasing levels of iron and other micro­nutrients in children aged 6-59 months, with a view to evaluating its usefulness as a public health intervention. Methods. A search was conducted in MEDLINE, Embase, Cochrane Library, and LILACS databases. The review included randomized clinical trials (RCTs) that assessed the consumption of fortified rice, compared with a placebo or other forms of intervention, in terms of enhanced levels of iron and other micronutrients. The CONSORT® checklist was used to assess methodological quality. The risk of bias in the studies was assessed using the Cochrane® Collaboration methodology. Results. Seven RCTs were included in the review. All the studies showed significant improvements in indicators of nutritional iron status in the intervention groups, without reporting adverse effects. There was no evidence of improvement in vitamin A levels or in anthropometric indicators of weight and height as a secondary result of the intervention. The included studies showed moderate methodological quality. Conclusions. Rice fortification was an effective intervention strategy to correct iron deficiency in children under age 5. For implementation as a public health measure, local studies are needed to assess its effectiveness in long-term and large-scale interventions.

Hyperostotic Esthesioneuroblastoma: Rare Variant and Fibrous Dysplasia Mimicker

A 65-year-old male presented with a 3-year history of orbital symptoms. An imaging-based diagnosis of fibrous dysplasia involving the skull base was made at another institution. CT showed a diffuse sinonasal mass and ground-glass appearance of the bones of the anterior skull base with bony defects and mucocele formation. MRI demonstrated an accompanying intracranial and orbital rind of soft tissue mass along the hyperostotic bones. FDG-PET showed corresponding intense hypermetabolism. Small cysts were observed at the tumor-brain interface. Biopsy revealed esthesioneuroblastoma with bone infiltration that is compatible with the hyperostotic variant of esthesioneuroblastoma. There are a few cases of hyperostotic esthesioneuroblastoma reported in the literature.

Advances in Computed Tomography Evaluation of Skull Base Diseases

Introduction: Computed tomography (CT) is a key component in the evaluation of skull base diseases. With its ability to clearly delineate the osseous anatomy, CT can provide not only important tips to diagnosis but also key information for surgical planning. Objectives: The purpose of this article is to describe some of the main CT imaging features that contribute to the diagnosis of skull base tumors, review recent knowledge related to bony manifestations of these conditions, and summarize recent technological advances in CT that contribute to image quality and improved diagnosis. Data Synthesis Recent advances in CT technology allow fine-detailed evaluation of the bony anatomy using submillimetric sections. Dual-energy CT material decomposition capabilities allow clear separation between contrast material, bone, and soft tissues with many clinical applications in the skull base. Dual-energy technology has also the ability to decrease image degradation from metallic hardwares using some techniques that can result in similar or even decreased radiation to patients. Conclusions: CT is very useful in the evaluation of skull base diseases, and recent technological advances can increase disease conspicuity resulting in improved diagnostic capabilities and enhanced surgical planning...

Advances in Magnetic Resonance Imaging of the Skull Base

Introduction: Over the past 20 years, magnetic resonance imaging (MRI) has advanced due to new techniques involving increased magnetic field strength and developments in coils and pulse sequences. These advances allow increased opportunity to delineate the complex skull base anatomy and may guide the diagnosis and treatment of the myriad of pathologies that can affect the skull base. Objectives: The objective of this article is to provide a brief background of the development of MRI and illustrate advances in skull base imaging, including techniques that allow improved conspicuity, characterization, and correlative physiologic assessment of skull base pathologies. Data Synthesis Specific radiographic illustrations of increased skull base conspicuity including the lower cranial nerves, vessels, foramina, cerebrospinal fluid (CSF) leaks, and effacement of endolymph are provided. In addition, MRIs demonstrating characterization of skull base lesions, such as recurrent cholesteatoma versus granulation tissue or abscess versus tumor, are also provided as well as correlative clinical findings in CSF flow studies in a patient pre- and post-suboccipital decompression for a Chiari I malformation. Conclusions: This article illustrates MRI radiographic advances over the past 20 years, which have improved clinicians' ability to diagnose, define, and hopefully improve the treatment and outcomes of patients with underlying skull base pathologies...

Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.

Vestibular schwannoma: 825 cases from a 25-year experience

Introduction: Acoustic nerve tumors have been recognized as a clinico-pathologic entity for at least 200 years, and they represent 90% of cerebellopontine angle diseases. Histologically, the tumors are derived from Schwann cells of the myelin sheath, with smaller tumors consisting of elongated palisade cells, while in large tumors, cystic degeneration can be found in the central areas, possibly due to deficient vascularization. We retrospectively reviewed 825 cases of vestibular schwannomas, reported between January 1984 and August 2006, in which the patients underwent surgery to remove the tumor. Objective: To evaluate signs, symptoms, aspects of clinical diagnosis, including the results of audiological and imaging studies, and surgical techniques and complications. Methods: A retrospective chart review. The medical records of all patients undergoing surgical treatment for schwannoma during the period indicated were reviewed. Results and Conclusion: Hearing loss was the first symptom reported in almost all cases, and tumor size was not proportional to the impairment of the auditory threshold. The surgical techniques allowed safe preservation of facial function. In particular, the retrolabyrinthine route proved useful in small tumors, with 50% preservation of hearing...

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...

Metastatic hepatocellular carcinoma presenting as facial nerve palsy and facial pain / 대한간학회지

Facial nerve palsy due to temporal bone metastasis of hepatocellular carcinoma (HCC) has rarely been reported. We experienced a rare case of temporal bone metastasis of HCC that initially presented as facial nerve palsy and was diagnosed by surgical biopsy. This patient also discovered for the first time that he had chronic hepatitis B and C infections due to this facial nerve palsy. Radiation therapy greatly relieved the facial pain and facial nerve palsy. This report suggests that hepatologists should consider metastatic HCC as a rare but possible cause of new-onset cranial neuropathy in patients with chronic viral hepatitis.

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.

Osteoma mastoideo: considerações sobre dois casos e revisão da literatura / Mastoid osteoma: consideration on two cases and literature review

Introdução/Objetivo: Osteoma da mastoide é um tumor osteogênico benigno, considerado raro nesta localização. Até o ano de 2006 havia cerca de 150 casos relatados na literatura médica. Na sua etiopatogenia incluem fatores relacionados com a embriogênese, metaplasia, trauma e inflamação. Geralmente são assintomáticos e manifestam-se através de tumoração protruindo da região retroauricular. Tomografia computadorizada tem sido útil no seu diagnóstico e planejamento cirúrgico. Seu resultado cirúrgico é bom do ponto de vista estético e curativo. Método: Foram estudados dois casos de osteoma mastoideo e analisados quanto ao seu quadro clínico, exames de imagens, tratamento e resultados. Resultados: Ambos localizados na região mastoidea direita. Ausência de história de trauma prévio nesta região. Exame neurológico normal. Tomografia computadorizada craniana revelou lesão hiperdensa envolvendo a córtex do osso temporal na região mastoidea, compatível radiologicamente com osteoma. Foram submetidos à exérese total da lesão. Exame anatomo-patológico: Osteoma. Apresentaram resultados estéticos e curativos excelente. Não houve recidiva ou transformação maligna. Conclusões: O osteoma localizado na região mastoidea é um tumor benigno raro. Provoca deformidade estética local. Geralmente é assintomático. O exame de eleição é a tomografia computadorizada. Apresenta diagnóstico diferencial com hemangioma, displasia fibrosa, osteosarcoma e metástase osteoblástica. Não apresenta transformação maligna. Seu tratamento é cirúrgico e com resultados estéticos e curativos excelente.

Introduction/Objective: The mastoid osteoma is a benign osteogenic tumor, considered to be uncommon in such location. By 2006, there were about 150 cases reported in the medical literature. Its etiopathogenesis includes factors relating to embryogenesis, metaplasia, trauma and inflammation. They are generally asymptomatic and appear through tumoration protrusion from the retroauricular region. Computed tomography has been useful for its diagnosis and surgical planning. Its surgical results are good from a aesthetic and curative point of view. Method: We studied two cases of mastoid osteoma and analyzed them as for their clinical state, imaging exams, treatment and results. Results: Both locating in the right mastoid region. Absence of a previous trauma in this region. Normal neurological exam. The cranial computed tomography revealed a hyperdense lesion involving the cortex of the temporal bone in the mastoid region, radiologically compatible with osteoma. They were submitted to complete exeresis of the lesion. Anatomopathological exam: osteoma. They had excellent aesthetic and curative results. There was no recurrence or malignant transformation. Conclusions: The osteoma located in the mastoid region is an uncommon benign tumor. It provokes local aesthetic deformity and is generally asymptomatic. The choice exam is computed tomography. It has a differential diagnosis with hemangioma, fibrous dysplasia, osteosarcoma and osteoblastic metastasis. The exam does not present malignant transformation. The treatment is surgical and with excellent aesthetic and curative results.

Schwannoma de forame jugular: relato de caso / Jugular foramen schwannoma: case report

Introdução: As lesões do forame jugular são raras, sendo os paragangliomas os tumores mais comuns desta região seguidos pelos schwannomas. Os schwannomas são tumores benignos de crescimento lento e origem principalmente nos nervos cranianos sensitivos. Quando localizados no forame jugular, apresentam-se clinicamente com perda auditiva, ataxia e cefaleia, sendo comuns as alterações dos nervos cranianos IX, X e XI. A excisão cirúrgica é a opção terapêutica de escolha. Relato do Caso: Relatamos o caso de um paciente adulto jovem que procurou o serviço de Otorrinolaringologia do Hospital Universitário de Brasília com quadro de otalgia e otorreia piossanguinolenta no ouvido esquerdo, associada a zumbido, hipoacusia e tumor no conduto auditivo externo. Iniciada investigação diagnóstica com tomografia computadorizada e biópsia, ambas com resultados inconclusivos. Realizadas então nova biópsia e ressonância nuclear magnética, com diagnóstico definitivo de schwannoma do foram jugular. Na ocasião a lesão foi definida como tipo D pela classificação de Kaye-Pellet, por envolver o forame jugular, apresentar formato de halter e componentes intra e extracraniano. Realizada cirurgia por craniotomia com excisão parcial do tumor. Não houve sequelas do procedimento, apesar da persistência dos sintomas otológicos. Comentários Finais: O caso é um exemplo de uma doença rara, com pouco mais de duzentos casos relatados na literatura, e apresentação clínica incomum. O desafio diagnóstico e terapêutico é enfrentado pelas equipes de Otorrinolaringologia e Neurocirurgia, que programam para breve nova abordagem cirúrgica combinada para extirpação completa do tumor.

Introduction: Lesions of the jugular foramen are uncommon, and the paragangliomas are the most common tumors in this region, followed by schwannomas. Schwannomas are benign tumors, of slow growth and origin mainly in the sensitive cranial nerves. When located in the jugular foramen, the patients present with clinically auditory hearing loss, ataxia and headache, and cranial nerves IX, X and XI alterations are also common. Surgical excision is the therapeutic choice. Case Report: We reported the case of a young adult patient who sought the Otorhinolaryngology service at the School Hospital of Brasília for investigation of otalgia and chronic infection in the left ear, associated with tinnitus, hypacusis and tumor in the external ear canal. Initial diagnostic investigation by computed tomography and biopsy was inconclusive. New biopsy and magnetic resonance imaging were performed and definitive diagnosis of jugular foramen schwannoma was given. The lesion was then defined as type D by the classification of Kaye-Pellet, for it involved the jugular foramen, presented format of dumbbell and intra and extracranial components. The patient underwent a craniotomy and partial excision of the tumor. He did not have sequels of the procedure, although the otologic symptoms persisted. Final Comments: This case is an example of a rare disease. No more than two hundred cases have been reported in the literature and the clinical presentation is uncommon. The diagnostic and therapeutic challenge is faced by Otorhinolaryngology and Neurosurgery teams who are scheduling a new combined surgical procedure for a complete excision of the tumor.

Multilobular tumour of the caudal cranium causing severe cerebral and cerebellar compression in a dog

Multilobular tumour of bone (MTB) is an uncommon tumour and is usually located in the skull. A 13-year-old mixed breed dog was presented with a two-week history of progressively worsening vestibular dysfunction and cognitive abnormalities; it appeared demented and showed asymmetric ataxia and hypermetria of all limbs. The owner opted to have the animal euthanised. Necropsy revealed a large mass occupying the right occipital, parietal and temporal bones, severely compressing the cerebellum and the right occipital lobe. Histologically, it was characterised by the presence of multiple lobules containing osteoid or cartilage and separated by fibrous septae, features typical of MTB. Lung metastases were evident. To our knowledge, this is the first report of an MTB causing both severe cerebral and cerebellar compression and the second detailed report of an MTB of the occipital bone. MTB should be included in the differential diagnosis of bone tumours as well as in cases with central vestibular disease.

Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

Although intracranial dural metastasis of Ewing's sarcoma is a very rare finding, its imaging characteristics are similar to those of its primary form in the central nervous system. Thus, this tumor must be considered in the differential diagnosis of extra-axial dural masses.