Paraganglioma yugular bilateral: reporte de un caso y revisión de la literatura / Bilateral jugular paraganglioma: case report and review of the literature

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.

Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.

A Case of En Plaque Meningioma of Jugular Foramen with Image Findings of Differential Diagnosis

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.

Diagnosis and management of jugulare glomus tumor and carotid body tumor / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate diagnosis method and treatment experience of jugulare glomus tumor (JGT) and carotid body tumor (CBT).@*METHOD@#Retrospective analysis. Clinical materials from 4 patients at one genealogy with JGT and CBT were collected. The clinical features, radiological characteristics, surgical methods and prognosis were assessed. The proband suffered from left JGT and left CBT, her younger sister had right CBT as well as her younger female cousin had bilateral CBT and right JGT, her older male cousin had bilateral CBT and left JGT. These JGT and CBT in four patients were resected under general anesthesia after preoperative ultrasound, CT or CT angiography (CTA), MRI, and digital subtraction angiography (DSA) examination as well as preoperative vascular embolization.@*RESULT@#The surgery was uneventful in four cases, and there was no hemiplegia or deaths. All patients were followed up for 2 years to 29 months without recurrence. Six months after operation, the hoarseness also disappeared in the older male cousins but did not improve significantly in younger female cousin. The younger female cousin get right peripheral facial paralysis 1 year after surgery and her temporal bone CT indicated a right JGT.@*CONCLUSION@#It is very important to assess JGT and CBT by Ultrasound, CT, CTA, MRI and DSA. Surgical resection is the first optional treatment for JGT and CBT. Blood loss can be reduced by preop erative vascular embolization. Serious complications could be avoid by operating under microscope, which can supply a clear surgical field and make the surgeon to protect the large blood vessels and nerve.

Application of far lateral craniocervical approach in the microsurgical treatment of the jugular foramen tumors / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate the administration of far lateral craniocervical approach in the jugular foramen (JF) tumors.</p><p><b>METHODS</b>A retrospective analysis was performed in 14 cases of JF tumors (9 neurilemmomas, 3 meningiomas, 1 glomus jugulare tumor, and 1 adenoid cystic carcinoma) surgically treated between January 2009 and January 2012, with focus on the surgical approach. Six patients (6/14) showed hydrocephalus. The tumor type was composed of 5 intracranial and intraforamen tumors with patent or occluded jugular bulb, 1 intracranial tumor with extension into the upper cervical canal, 4 extracranial and intra foramen tumors, 4 intra- and extracranial dumbbell-shaped communicating tumors involving the parapharyngeal space above C2 or extending caudally below C3. Far lateral postcondylar approach (FLPC) was carried out in 2 cases, far lateral tansjugular process approach (FLTJP) in 3 cases, combined FLPC + C1-2 semi-laminectomy approach in 1 case, combined FLTJP + trans-C1 transverse process approach in 7 cases, and combined FLTJP + neck approach with dissection of carotid sheath to the skull base in 1 case. Endovascular embolotherapy prior to surgical resection was performed in 1 glomus jugulare tumor.</p><p><b>RESULTS</b>Total tumor removal was achieved in 12 patients and subtotal removal in 2 patients, with no cerebrospinal fluid leakage or operative mortality. New cranial nerve paresis occurred after surgery in 1 case of facial nerve and 1 case of lower cranial nerve. Transient worsening of preoperative lower cranial nerve deficits was noted in 3 patients. Long-term follow-up study ranging from 5 to 32 months (average 13.7 months) showed 7 patients with lower cranial nerve deficits (6 preexisting and 1 new), with exception of one preoperative lower cranial nerve dysfunction due to the infiltration of an adenoid cystic carcinoma, experienced favorable improvement with recovery of adequate swallowing function, but voice disturbance remained in 4 cases. One patient with new facial nerve deficit presented with partial improvement and the hydrocephalus in 6 patients all spontaneously regressed. There was no tumor recurrence in patients receiving total removal and no tumor progression in patients undergoing subtotal removal.</p><p><b>CONCLUSIONS</b>FLTJP is a basic approach for JF tumors. The combined cranial and cervical approach should be considered in those tumors extending into the upper cervical canal and parapharyngeal space. The associated hydrocephalus seldom requires additional surgical management.</p>

Anestesia intravenosa total en cirugía de tumor carotídeo / Total intravenous anesthesia in carotid surgery tumor

Mujer de 18 años operada en junio del 2011 por el Servicio de Cabeza y Cuello del Hospital Nacional Arzobispo Loayza, por una tumoración cervical izquierda. La tomografía en fase angiográfica mostró un tumor estadio Shamblin III en bifurcación carotidea. Programada para exeresis de glomus carotídeo y ante los antecedentes de secuelas neurológicas postquirúrgicas de este estadio, se elige la técnica de anestesia intravenosa total con propofol, modo infusión controlada por objetivo, por sus propiedades de protección cerebral, e infusión de remifentanilo. Se monitorizó profundidad anestésica continua con índice biespectral. En el transoperatoio se lacera carótida común, se clampa la arteria durante 5 horas con liberación intermitente al cabo distal para evidenciar circulación cerebral colateral. Se coloca bypass con injerto de safena externa y se realiza exeresis final de tumor. Paciente hemodinámicamente estable, pasa a la unidad de recuperación con Ramsay y luego de 11 horas de anestesia.

Female, 18 years old, operated in June 2011 by Servicio de Cabeza y Cuello del Hospital Nacional Arzobispo Loayza, because of a left cervical tumor. The angiography phase of the CT scan showed a carotid bifurcation tumor Shamblin III stage. Scheduled for exeresis of carotid glomus and aware of the fact of postoperative neurological sequelae of this stage tumor, we choose the total intravenous anesthesia technique with propofol in target controlled infusion, because of its brain protective properties, and infusion of remifentanil. Depth of anesthesia was monitors continuously with bispectral index. Common carotid artery was lacerated during surgery, and it was clamped for 5 hours with intermittent release of the distal stump to asses collateral cerebral circulation. A bypass with saphenous graft was placed and the tumor exeresis was performed. A stabled patient went to the recovery unit with Ramsay 52 after 11 hours of anesthesia.

Microsurgery via modified far-lateral approach for giant dumbbell-shaped jugular foramen tumors / 癌症

<p><b>BACKGROUND AND OBJECTIVE</b>During the resection of jugular foramen tumors via the basic far lateral approach, the jugular foramen tumor area as well as its adjacent structures, especially the intracranial part, can be better exposed, which avoids stripping of the petrous part of temporal bone and displacement of facial nerve, and protects the patient's hearing from damage. However, when applied in tumors developed from ventral to the brain stem and middle fossa, with extracranial tumors, this surgical approach seems to be inadequate and limited. This study was to explore the microsurgical technique and clinical value for treating giant dumbbell-shaped tumors at jugular foramen (JF) via a modified far lateral approach.</p><p><b>METHODS</b>A retrospective analysis was performed in 16 patients with huge dumbbell-shaped tumors at JF which were removed through the modified far lateral approach (suboccipital transjugular-jugular tubercle-jugular process) between January 2001 and December 2008. The process of operation, and pre-and postoperative clinical data were included in the analysis.</p><p><b>RESULTS</b>Gross total tumor removal was achieved in 14 cases, subtotal removal in 1 case, and partial removal in 1 case. Follow-up examinations in most patients demonstrated that the patient with an obvious preoperative deficit had a good recovery. During the follow-up from three months to seven years, 10 (76.9%) cases with lower cranial nerve involvement showed obvious improvement of symptom after operation, 8 (80.0%) cases with facial palsy obtained various degrees of alleviation, and 7 (77.8%) cases with hearing impairment at different levels restored hearing. Two patients developed new lower cranial nerve palsies after operation, and underwent functional rehabilitation in the three-month follow-up.</p><p><b>CONCLUSIONS</b>Modified far lateral approach is helpful for removing the huge tumors at JF, especially for tumors extending to the petroclival region ventral part of pontomedullary junction. It has a higher rate of total resection, preoperative cranial nerve function impairment is expected to restore, and also has the advantage of protecting the facial nerve, labyrinth and vertebral artery structure from unnecessary damage.</p>

Surgical management of jugular foramen tumors / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To report the clinical manifestations, imaging characteristics, surgical approaches, managements, and outcome of jugular foramen tumors. The detailed clinical information of this extremely rare tumor was presented, with special emphasis on certain key issues, e. g, the preoperative estimation, perioperative management, surgical skill and experience, which exerted an influence on the significance of total tumor resection and preventing complications.</p><p><b>METHODS</b>From 1985 to 2007, 42 patients with jugular foramen tumor (30 cases of jugular paragangliomas and 11 cases of tumor with particular pathological types) were enrolled in this study. Prior to surgical procedures, all patients were subjected to systematic imaging examinations on temporal bone, such as CT, HRCT, CTA, and MRI, and some patients were further examined by angiography or embolization according to the individual situations. The infratemporal type A and combined translabrinthin and/or transchecholea approaches were selected for the treatment of 30 cases of jugular paragangliomas; while, the modalities of infratemporal type A, enlarged mastoidectomy, or mastoid-neck approach were employed for the remaining 11 specific cases.</p><p><b>RESULTS</b>Forty-two patients in this report were categorized into beyond C types based on FISCH classification in which all had invaded to posterior fossa. In the 31 cases, the major initial clinical symptoms were tinnitus, hearing loss, and facial palsy; while, in the 11 specific cases, the main symptoms did not possess any unique trait for the diagnosis and 5 of which were found via CT or MRI examination by chance. Facial nerve management included permanent anterior transposition (19 cases), facial nerve bridge technology (16 cases), interposition graft (4 cases), VII-XI jump graft (2 cases), and VII-XII anastomosis (1 case).</p><p><b>CONCLUSIONS</b>The preoperative estimation of tumor in nature was of great importance in the determination of proper surgical approaches and the infratemporal type A could fully meet the requirement for resection of tumors in jugular foramen. Facial nerve anterior rerouting could provide a clear visual field during the procedure, especially for the lesions in anterior tympanic cavity. In most cases, the facial nerve bridge technology could also fulfill the needs for complete tumor resection as well as the better preservation of facial function. In case of considering the sacrifice of internal carotid artery, balloon test occlusion was indispensable for preoperative estimation. The CT or MRI characteristics of tumors with particular pathological types were different from those of jugular paragangliomas. The preoperative management, surgical skills, and experience played a pivotal role in complete tumor resection.</p>

Digital subtraction angiography on glomus jugularis tumors / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>To analysis the effectiveness of preoperative digital subtraction angiography (DSA) on glomus jugularis tumors.</p><p><b>METHODS</b>A retrospective study of 14 cases of glomus jugularis tumors which received preoperative DSA was carried out. Among them, 10 cases were accepted balloon occlusion test and selective embolization of supplied vessels.</p><p><b>RESULTS</b>Hypervascular changes was found in all 14 cases and compression of internal carotid artery was found in 5 cases. Ascending pharyngeal artery was the main supplied vessel. Balloon occlusion test was applied in 10 cases, 2 were intolerable and 8 were passed occlusion test. Ten cases were accepted embolizations and tumor blood supply was conspicuous decreased. Neurovascular complications did not occurred after DSA but postoperatively in 2 cases. Intraoperative bleeding was less in embolization patients than that of without embolization.</p><p><b>CONCLUSIONS</b>Preoperative DSA association with balloon occlusion test and embolizations might be a safe option on surgery of glomus jugularis tumors.</p>

Glomus tumors

Glomus tumours of the temporal bone occur in the region of the jugular bulb middle ear. They are rare, highly vascular, slow growing tumours and most are benign. Tumours that originate from the jugular bulb and extend to involve the middle ear are referred to as glomus jugular tumours. Those that are found in the middle ear around the otic ganglia in the tympanic plexus are known as Glomus tympanicum. Yhese tumours occur predominantly in women in the fifth and sixth decades of life. Because of the insidious onset of symptoms, these tumours often go unnoticed and there is often a significant delay in diagnosis. Morbidity in these cases is determined by their size and position. 1. To study the age/sex incidence of patients suffering from glomus tumours of the temporal bone. 2. To see various clinical presentations with which these tumours present and their variation according to the age and sex. Our study design was non-inter-ventional descriptive. This study was performed at CMH Rawalpindi and Multan from January 2000 to June 2002. We included ten patients of glomus tumours of the temporal bone in our study. 8 out of these were females and two were males. Though most of the patients belonged to middle age group yet few were also from the younger and older groups. All the patients underwent CT scan with and without contrast, MRI of the requisite site and carotid angiography. We found that these tumours were predominantly present in females, mostly in the middle age group. Individual symptoms were studied in detail and their presence was found to be directly proportional to the increasing age of patients. It is concluded that due to the slow growth of this tumour the diagnosis is often delayed until it is extensive. Therefore clinicians should be more vigilant about this rare disease and must keep it in their differentials

jugular foramen: a review of old and new imaging techniques and diagnoses

The imaging anatomy of the jugular foramen [JF] has been described. This is followed by an account of the various historical techniques used for diagnostic visualization. It is obvious that imaging interest has swung almost entirely to newer modalities as a result of dissatisfaction with plain films and conventional tomograms. In many parts of the world, places where no imaging facilities are available, a base view 50-70 degrees short of full extension, and a lateral oblique view are still of value in diagnosing a large foramen or fossa. Demonstration of the exact anatomic details of the bone is not possible without high resolution computed tomography [HRCT]. Conventional pleuridirectional tomography has retreated but remained complementary to computed tomography and a valuable tool in its absence. Computed tomography [CT] and magnetic resonance [MR] provide more information on intracranial or extracranial extensions of tumours as well as on the involvement of adjacent soft tissues. Four-vessel arteriography and jugular venography add important diagnostic characterization. The diagnostic criteria for each of the pathological conditions and the size of the lesion has been described with the justification for the use of a particular techniques. In nontumoral conditions, CT demonstrates smooth intact margin of the JF, except in cerebral venous thrombosis where phase contrast MR venography is the imaging modality of choice. In most tumoral lesions CT shows areas of infiltrative bone involvement, most schwannomas and meningiomas cause smooth enlargement rather than erosion. The multiplanner nature of MR imaging gives better definition and extent of soft tissues lesions, as well as more specific diagnostic criteria. Most of the tumors show low to intermediate signal intensity on T1- weighted images, and intermediate to high intensity on T2-weighted images, and enhance strongly. Each lesion requires a combination of available modalities to reach the specific diagnosis, sometimes the use of three dimentional CT is indicated, particularly in pre- and postsurgical intervention

Pulsatile tinnitus: Audiologic and MR imaging diagnostic lssues

Pulsatile tinnitus often presents a diagnostic and management dilemma to the neurologist. Correct diagnosis is imperative because in the majority of cases, there is a treatable underlying etiology. In addition, failure to make proper diagnosis may be disastrous because in some patients, a life-threatening intracranial disease may be present. This study was carried out aiming to investigating the feasibility of differentiating the possible causes of pulsatile tinnitus, particularly in patients with normal otoscopy, using clinical, audiological and radiological, tools. 29 patients complaining of pulsatile tinnitus were evaluated by history taking, clinical examination, laboratory investigations, otoscopy, basic audiological evaluation, MRI for petrous bone and brain and MRA. Glomus jugulare tumor was diagnosed in 3 patients. Benign intracranial hypertension [BIH] was diagnosed in 4 patients. Intraventricular neoplasm in 2 patients. Internal carotid artery stenosis in one patient. Post-traumatic pseudoaneurysm in one patient. Scalp AVM in one patient. Temporal bone metastasis in one patient. Severe anemia in one patient and no identifiable cause [idiopathic] in 15 patients. A unilateral mild low frequency pseudosensorineural hearing loss was identified in 10 patients with normal otoscopy. Hearing loss was normalized after elimination of tinnitus by applying a light digital pressure over the ipsilateral internal jugular vein

Tratamento radioterápico dos tumores glômicos do osso temporal / Radiation therapy for glomus tumors of the temporal bone

O tratamento dos tumores glômicos tem sido motivo de controvérsia desde sua primeira publicação, podendo ser cirúrgico, radioterápico ou apenas expectante. OBJETIVO: O objetivo do estudo foi avaliar a efetividade e as complicações do tratamento radioterápico para esses tumores. FORMA DE ESTUDO: clínico com coorte transversal. MATERIAL E MÉTODO: Trata-se de uma coorte histórica de pacientes com tumor glômico jugular submetidos à radioterapia. Os critérios de controle da doença foram não haver progressão dos sintomas ou disfunção de nervos cranianos, sem aumento do tamanho da lesão ao exame físico ou controle radiológico. Avaliamos também a presença de seqüelas do tratamento. RESULTADOS: Foram incluídos 12 pacientes, sendo oito mulheres. O tempo de follow-up variou de 3 a 35 anos, com uma media de 11,6 anos. Os principais sintomas foram: hipoacusia, zumbido pulsátil e tontura ou vertigem. Os achados de exame físico mais freqüentes foram massa pulsátil retrotimpanica, paralisia facial e anacusia, sendo os tumores estadiados segundo a classificação proposta por Fisch. A radioterapia foi realizada com acelerador linear com doses variando de 4500-5500 Rads por 4-6 semanas. As seqüelas mais comuns foram a dermatite, estenose do conduto auditivo externo, anacusia e paralisia facial. DISCUSSÃO: Os sintomas e achados de exame físico e o método e dosagem da radioterapia não diferiram daqueles encontrados na literatura. Todos os pacientes tiveram melhora dos sintomas e apenas um não foi considerado como tendo controle da doença. As complicações do tratamento foram de pouca repercussão, com exceção da anacusia e da paralisia facial. CONCLUSÃO: A radioterapia é uma alternativa viável para o tratamento desses tumores pela boa resposta e baixo índice de complicações. Deve ser considerada especialmente em tumores mais avançados onde um procedimento cirúrgico pode trazer grande morbidade.

The treatment of glomic tumors has been controversial since its first description. It can be done with surgery, radiotherapy or just expectation. AIM: The objective of this paper was to evaluate the effectiveness and complications of radiotherapy. STUDY DESIGN: clinical with transversal cohort. MATERIAL AND METHOD: It was made a retrospective review in the charts of the patients with glomus jugulare tumors treated with radiotherapy. Disease control was determined by (1) no progression of symptoms or cranial nerve dysfunction or (2) no progression of the lesion in radiological follow-up. It was also evaluated the follow-up period and the sequelae of the treatment. RESULTS: Twelve patients were included, 8 of then women. The follow-up period was from 3 to 35 years, with a media of 11,6 years. The main symptoms were: hearing loss, pulsate tinnitus, dizziness and vertigo. The signs were pulsate retrotympanic mass, facial palsy and cofosis. The tumors were staged using Fisch's classification. The radiotherapy was performed with linear accelerator with dose ranging from 4500-5500 in 4-6 weeks. In the follow-up period were possible to identify sequelaes like dermatitis, meatal stenosis, cofosis and facial palsy. DISCUSSION: The signs and symptoms were the same found in the medical literature. The type and dosages of the radiotherapy were also the same of others reports. All patients had improvement of the symptoms and only one was not considered as having disease controlled. Complications were, in general, minor complications, with exception of the cofosis and facial palsy. CONCLUSION: Radiotherapy is a viable alternative to treatment of these tumors because their good response and low level of complications. It should be considered specially in advanced tumors where a surgical procedure could bring a high level of morbidity.

Paragangliomas de cabeza y cuello / Parangangliomas of the head and neck

Los parangangliomas son tumores altamente vascularizados que se originan de células provenientes de la cresta neural. El tratamiento de elección es la resección quirúrgica completa, lo cual se ve dificultado por su abundante irrigación y por su estrecha relación anatómica con importantes estructuras vásculo-nerviosas. En el presente estudio se revisa retrospectivamenete la experiencia del Departamento de Otorrinolaringología de Clínica Las Condes en el manejo quirúrgico de estas neoplasias. Entre los años 1998 y 2003 se trataron 5 pacientes portadores de paragangliomas de cabeza y cuello: un glomus yugular (GY), 2 glomus carotídeo (GC) y 2 glomus timpánicos (GT). El estudio imagenológico consistió en tomografía computarizada (TC) para todos los casos, complementada con resonancia nuclear magnética (RNM) y/o angiografía en algunos pacientes. El abordaje quirúrgico fue el indicado para cada lesión: timpanotomía retroauricular (GT), cervicotomía (GC) y abordaje infratemporal tipo A de Fisch (GY). En todos se logró una resección tumoral completa, sin presentarse complicaciones perioperatorias de consideración ni recurrencias hasta la fecha. Contando con un adecuado estudio imagenológico y una cuidadosa planificación quirúrgica, la resección de los paragangliomas de cabeza y cuello puede realizarse con un bajo índice de complicaciones, reservando la radioterapia como alternativa terapéutica únicamente en aquellos casos inoperables.

Intradural jugular foramen tumors

Onze pacientes com lesões expansivas do forame jugular associadas ou não a componente extradural foram submetidos a ressecção cirúrgica no Hospital das Clínicas da Universidade Estadual de Campinas (UNICAMP) entre 1998 e 2001. Foi utilizada cirurgia combinada com dissecção do pescoço, mastoidectomia sem transposição do nervo facial e técnica de reconstrução miofascial da base do crânio desenvolvida por um dos autores. Quatro pacientes foram operados via craniectomia retrosigmoidea. Ressecção total foi feita em 9 pacientes, subtotal em um e parcial em outro. Nenhum dos 11 pacientes teve progressão da doença em 2 anos de acompanhamento. Não houve mortalidade. Cinco pacientes tiveram déficits adicionais de nervos cranianos baixos. Nove pacientes mantiveram ou melhoraram suas pontuações de acordo com a escala de Karnofsky. Para adequada abordagem do paciente com tumor de forame jugular, são necessários bom conhecimento anatômico da região, exposição cirúrgica ampla, e técnica de reconstrução eficiente para obter cura com baixas taxas de complicações pós-operatórias.

Gamma-Knife Radiosurgery of Upper Cervical Spinal Cord Tumor

OBJECTIVE: The lower limit of gamma knife radiosurgery is considered as foramen magnum. The head is long and narrow in western people, however, the head is short and wide in oriental people. In microcephalic oriental person, gamma knife radiosurgery is tried for the upper cervical cord tumor. METHODS: We have treated seven patients of upper cervical cord tumor with Gamma-plan during the last 8 years. The seven patients consisted of one man and six women, and ages ranging from 25 to 67 years. The histologic diagnoses were cervicomedullary hemangioblastoma in two, meningioma in two, brain stem glioma extending to C2 in one, hemangioma in one, glomus jugulare tumor in one. Preradiosurgical surgical intervention was performed in three patients. Mean marginal dose was 10.8Gy. RESULTS: The median follow-up period was 40 months (12-51 months). Four tumor had markedly decreased in size on follow-up magnetic resonance image. The remaining four cases were stationary in size. There was no complication. CONCLUSION: In microcehalic or normocephalic oriental person, the lower limit of gamma knife radiosurgery is level of second cervical spine.

Postauricular tran-supracondylar approach removed jugular foramen and hypoglossal canal tumors / 中华外科杂志

<p><b>OBJECTIVE</b>Discuss the surgical treatment of jugular foramen and hypoglossal canal tumor, and choice of the best surgical approach.</p><p><b>METHODS</b>32 patients with jugular foramen and hypoglossal canal tumor were operated through the postauricular tran-supracondylar approach, 13 neurinoma, 7 glomus jugular tumors, 4 meningioma, 3 chordoma. 23 were total removed, with a subtotal of 9.</p><p><b>RESULTS</b>21 patients were followed up, mean 2.5 years. 18 patients work normally, 7 of them were hoarseness, 2 of other 3 patients lived by themselves, 1 of existed obstruct of limbs.</p><p><b>CONCLUSIONS</b>Postauricular tran-supracondylar approach exposes satisfactory for the dumbbell-shape tumors of intra-or extra cranial. It has high rate of total removal and low rate of complications. It is the best approach of treatment in jugular foramen and hypoglossal canal tumors. The outcome is good.</p>

Chondrosarcoma of the Temporal Bone: A Case Report

Chondrosarcoma of the temporal bone is a rare lesion. Clinically it has been confused with chordoma, glomus jugulare tumor and meningioma, among other conditions, and due to its anatomic location, cranial nerve palsy is frequently observed. We report a case involving a 50-year-old woman with chondrosarcoma of the temporal bone.

Chondrosarcoma of the Temporal Bone / 대한이비인후과학회지

Chondrosarcoma of the temporal bone is a rare neoplasm constituting only 0.16% of intracranial tumors. Type of cranial nerve involvement in chondrosarcoma of the temporal bone is related to the specific anatomic location of the tumor. Clinically it has been confused with multiple sclerosis, glomus jugulare tumors, meningiomas, and chordomas. Ablative surgical treatment seems to be the procedure of choice and the value of irradiation or chemotherapy is still under debate and long term follow up is necessary. The authors experienced a case of chondrosarcoma of the temporal bone in a 27 year-old female with facial palsy. The patient underwent wide excision via transmastoid and suboccipital approach and then postoperative radiotherapy. She is free of local recurrence or metastasis for postoperative 18 months.