Tumor de glomus yugulotimpánico, a propósito de un caso / Jugulotympanic glomus tumor, case presentation

Resumen Los tumores de glomus yugulo-timpánico o paragangliomas son originados de células de la cresta neural y representan un número bajo de los tumores de cabeza y cuello (0,5 %). Su crecimiento lento y síntomas progresivos provocan que se retarde el diagnóstico. Conocer la clínica que frecuentemente cursa con pérdida auditiva unilateral, tinnitus pulsátil y afectación de algún par craneal, puede ayudar a sospechar el diagnóstico de glomus. Se mencionan diferentes tipos de métodos diagnósticos, así como nuevas opciones de tratamiento. Dada la localización y su importante vascularización, hay casos en los que la cirugía abierta es muy riesgosa y se buscan procedimientos terapéuticos que disminuyan las complicaciones y morbilidades en estos pacientes, tanto como procedimientos endovasculares. En el artículo se discute el caso de una femenina de 31 años con clínica de inicio insidioso con hipoacusia izquierda progresiva, tinnitus pulsátil izquierdo y disfonía. En 2013, se realiza resonancia magnética nuclear donde se observa proceso infiltrativo tumoral sugestivo de paraganglioma. Se hace embolización endovascular con onyx de glomus yugulotimpánico en cuatro ocasiones, con seguimiento cada 6 meses por no ser candidata a otro tipo de tratamiento, debido al tamaño y localización de la masa y su condición no resecable.

Abstract Jugulo-tympanic glomus tumors or paragangliomas originate from neural crest cells and represent a low number of head and neck tumors (0.5%). Its slow growth and progressive symptoms cause the diagnosis to be delayed. The knowledge of the clinic that frequently involves unilateral hearing loss, pulsatile tinnitus and cranial nerve involvement can help to suspect the diagnosis. Different types of diagnostic methods are helpfun, as well as new treatment options have been proposed. Given the location and its important vascularization, there are cases in which open surgery is highly risky and alternative therapeutic procedures trying to reduce complications and morbidities in these patients need to be considered, as well as endovascular procedures. This article discusses the case of a 31-year-old female with an insidious onset of progressive left hearing loss, left pulsatile tinnitus and dysphonia. In 2013, nuclear magnetic resonance imaging was performed and a tumor infiltrative process suggesting paraganglioma was observed. Endovascular embolization was performed with onyx of jugulotympanic glomus on four occasions, with follow-up every 6 months, due to the size and location of the mass and its unresectable condition the tumor was considered non surgically resectable.

Cirurgia híbrida na exérese de tumor glômico Shamblin II / Hybrid surgery in excision of a Shamblin II glomus tumor

O tumor glômico é uma neoplasia benigna rara originada de células paraganglionares da crista neural que se desenvolve na camada adventícia do vaso. São tumores não encapsulados e altamente vascularizados. Paciente feminina, 64 anos, foi diagnosticada com tumor glômico hipervascularizado com 5 cm posteriormente à bifurcação carotídea esquerda e oclusão de carótida contralateral. Optou-se por realizar embolização através de acesso endovascular seguida de punção percutânea direta, guiada por angiografia, para preenchimento da área remanescente. Após embolização, realizou-se a exérese cirúrgica do tumor com menor sangramento e maior facilidade para encontrar o plano de clivagem das estruturas adjacentes. Em acompanhamento tardio, a paciente apresenta-se sem recidiva tumoral. O tumor foi classificado como pertencente ao grupo Shamblin II, o qual inclui tumores apresentando de 4 a 6 cm com inserção arterial moderada. Através dessa dupla abordagem, foi possível notar uma redução relativa do sangramento intraoperatório e facilitação de identificação do plano de clivagem, colaborando para sua exérese e evitando o pinçamento cirúrgico

Glomus tumors are rare benign neoplasms originating from paraganglionic cells of the neural crest developing in the adventitious layer of the vessel. They are nonencapsulated and highly vascularized. A 64-year-old female patient was identified with a hypervascularized glomus tumor measuring 5 cm, posterior to the left carotid bifurcation and contralateral carotid occlusion. We performed preoperative embolization via endovascular access followed by direct percutaneous puncture, guided by angiography, to fill the remaining area. After embolization, surgical excision of the tumor was performed with reduced bleeding and it was easier to find the cleavage planes to adjacent structures. At late follow-up, the patient is free from tumor recurrence. The tumor was classified as Shamblin II, measuring 4 to 6 cm with moderate arterial insertion. Through this double approach we observed a relative reduction in intraoperative bleeding and improved identification of the cleavage plane, facilitating excision and avoiding surgical clamping

Application of jugular bulb oxygen saturation in the treatment of severe traumatic brain injury / 中国基层医药

Objective To investigate the application of jugular bulb oxygen saturation (SjvO2) in the treat-ment of patients with severe traumatic brain injury (sTBI).Methods From August 2016 to August 2017,fifty-three patients with sTBI admitted to intensive care unit ( ICU) of Zhoupu Hospital Affiliated to Shanghai University of Medicine & Health Sciences were randomly divided into the intracranial pressure - guided treatment group ( ICP group) and the SjvO2 -guided treatment group (SjvO2 group) by draw lots method.The patients in ICP group ( n=25) were treated according to the routine regimen,and the SjvO2 group (n=28) was dynamically detected SjvO2,and the SjvO2 was maintained at the target range of 55% -75% by adjusting the treatment scheme in addition to the routine treatment regimen.The early GCS score,GOS score and the mortality rate were compared between the two groups three months after injury.Results A total of 36% (9/25) patients had SjvO2 <55% events within 3 days after injury.On the fourth day after injury,the GCS score in the SjvO2 group was better than that in the ICP group [(7.17 ± 1.41)points vs.(6.91 ± 1.10)points,t=1.78,P=0.03],and there was no statistically significant difference in mortality between the two groups at 90 days after injury ( P=0.80).The SjvO2 group had a better prognosis rate compared with ICP group (24.00% vs.10.70% ,χ2 =2.31,P=0.05).Conclusion The treatment strategy of sTBI patients guided by SjvO2 can improve early consciousness level and improve the overall prognosis of sTBI patients.

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.

Spinal Accessory Neuropathy Associated With the Tumor Located on the Jugular Foramen

Spinal accessory neuropathy is commonly caused by iatrogenic injury or secondary to trauma or infection. Nevertheless, the tumor related palsy is rare. We present a case of an 18-year-old male patient suffering from paralysis of his right trapezius and sternocleidomastoid muscle. An electrophysiologic diagnostic study confirmed the spinal accessory neuropathy of the proximal segment. In addition, magnetic resonance imaging showed the location of tumor on the jugular foramen. However, the type of the tumor was not confirmed through biopsy because the patient refused surgical procedure. Based on the study, it is hypothesized that the tumor located on the jugular foramen should be considered as a cause of the spinal accessory nerve of the proximal segment.

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.

Comparison of accuracy of jugular venous oxygen saturation, somatosensory evoked potentials and motor evoked potentials in estimation of occurrence of intraoperative cerebral ischemia in patients undergoing clipping of intracranial aneurysm / 中华麻醉学杂志

Objective To compare the accuracy of jugular venous oxygen saturation (SjvO2),somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) in estimation of the occurrence of intraoperative cerebral ischemia in patients undergoing clipping of intracranial aneurysm.Methods Forty-three ASA Ⅰ or Ⅱ patients of both sexes,aged 18-64 yr,with a body mass index of 20-25 kg/m2,undergoing clipping of intracranial aneurysm,were studied.Anesthesia was induced with sufentanil,rocuronium and propofol.The patients were tracheal intubated and mechanically ventilated.Anesthesia was maintained with remifentanil and propofol.Blood samples were taken from the jugular bulb for detection of SjvO2 before aneurysm clipping or temporary occlusion of parent artery and at 1,3,10,20 and 30 min after clipping aneurysm or temporary occlusion of parent artery.The amplitude and latency of SSEPs and MEPs were recorded simultaneously.The occurrence of cerebral ischemia estimated by SjvO2,SSEPs and MEPs was recorded.The condition of nerve defect was recorded within 3 days after operation and the gold standard of cerebral ischemia was defined as the occurrence of nerve defect.Results Among 43 patients,14 cases were diagnosed as having brain ischemia.The sensitivity and specificity of SjvO2 in estimation of the occurrence of intraoperative brain ischemia were 71％ and 93％,respectively (P ＜ 0.01).The sensitivity and specificity of SSEPs in estimation of the occurrence of intraoperative brain ischemia were 71％ and 62％,respectively (P ＜ 0.05).When the diagnostic criterion of cerebral ischemia was defincd as a decrease in the amplitude of MEPs or prolongation of the latency MEPs,the sensitivity and specificity of MEPs in estimation of the occurrence of intraoperative brain ischemia were 79 ％ and 52 ％,respectively (P ＞ 0.05).When the diagnostic criterion of cerebral ischemia was defined as a loss of the amplitude of MEPs,the sensitivity and specificity of MEPs in estimation of the occurrence of intraoperative brain ischemia were 57％ and 93％,respectively (P ＜0.05).Conclusion The sensitivity of SjvO2 and SSEPs in estimation of the occurrence of intraoperative brain ischemia is higher,however,the specificity of SjvO2 and MEPs is higher,indicating that SjvO2 is a reliable criteria for estimation of the occurrence of intraoperative brain ischemia in patients undergoing clipping of intracranial aneurysm.

Jugulotympanic Paraganglioma, Mimicking a Vascular Tumor: A Brief Case Report

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.

Jugulotympanic Paraganglioma, Mimicking a Vascular Tumor: A Brief Case Report

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.

Temporal bone paraganglioma

OBJECTIVE: The aim of the study was to describe the diagnosissurgical management and outcomes of eight patients with bone paraganglioma.PATIENTS: A series of 8 patients with temporal bone paraganglioma were seen over a 10-year period and operated n by the senior author.RESULTS: The majority of patients presented with hearing loss and pulsatile tinnitus. Other symptoms were facial numbness, facial asymmetry, dizziness, hoarseness, and dysphagia. All patients had pre-operative high resolution computed temography (HRCT) scan and/or magnetic resonance imaging. Pre-operative angiography and embolization facilitated the excision of these tumors in all but one case. There was reversal of pre-existent cranial nerve neuropathy in 3 of 8 cases. Unique to this series were the management of a patient who previously underwent gamma knife radiosurgery, one case in whom the glomus tumor grew from the better hearing ear thus posing the surgical management challenges and a complicated case of postoperative necrosis that required latissimus dorsi flap reconstruction. Other complications were described including post-operative facial paresis which eventually resolved in all patients.CONCLUSION: In our setting, it is possible to achieve good result with acceptable morbidity with surgery with or without postoperative radiotherapy.

Clinical and imaging features of jugular foramen tumors / 中国耳鼻咽喉头颈外科

OBJECTIVE To study the clinical and radiological characteristics of jugular foramen tumors.METHODS The clinical data of 32 patients with tumors of jugular foramen region treated from May 2006 to December 2009 were retrospectively reviewed.All patients were referred to systematic imaging examination of the temporal bone,such as CT(X-ray computed tomography) and MRI(magnetic resonance).Twenty two patients were further examined by angiography and embo1ized 24 hours before operation.Thirty one patients underwent different operations via different approaches, while one patient with glomus jugular tumor didn't receive operation because of fever caused by sigmoid sinus thrombophlebitis.According to pathologic examination, 21 cases were glomus jugular tumors, 10 cranial nerve neurilemmomas.RESULTS In the 32 cases, the major initial clinical symptoms of jugular foramen tumors were tinnitus,hearing loss and facial palsy.Bone erosion and the "salt and pepper" sign were the characteristic appearance of glomus jugular tumor.Neurilemmoma showed compressive bone change, cystic area and moderate enhancement on CT.CONCLUSION The combination of CT and MRI examination is benefit for both diagnosis and the choice of surgical plan.

MR Diagnosis of Glomus Jugulare Tumor / 实用放射学杂志

Objective To evaluate the value of MR in the diagnosis of glomus jugulare tumors.Methods 15 cases (8 in the right and 7 in the left-side) of glomus jugulare tumors were retrospectively analysed, with the emphasis on the locations, MR signal intensities andthe invading appearances to the surrounding structures of tumors. Results The tumors demonstrated as oval or irregular shape. Thesurrounding bones around the jugular fossa were destroyed in 12 masses. The signal intensity of the tumors was heterogeneous and with marked “salt and pepper”appearance in 12 masses(12/15) on plain MR imaging. The masses(13/13)were all markedly enhanced after the injection of Gd-DTPA, in which 10 of them presented with “salt and pepper”appearance. MR angiography was performed in 9 cases, theinternal carotid artery and the internal jugular vein were all displaced and/or encompassed.Conclusion Glomus jugulare tumor can becorrectly diagnosed according to the combination of MR imaging features with the typical localization,“salt and pepper” appearance and bone erosion in the skull base.

Surgical Resection of Glomus Jugulare Tumor via Infratemporal Approach after Preoperative Embolization

Glomus jugulare tumors are usually slow growing, but highly vascularized tumor. The authors report the case of huge glomus jugulare tumor treated by surgical resection with preoperative embolization. A 32-year old male patient presented with a history of right-sided facial palsy and hearing disturbance. Magnetic resonance imaging demonstrated an irregular large mass with multiple signal voids in the right temporal area. Prior to surgical excision of tumor, the patient underwent superselective embolization. Embolization of the tumor and its dominant feeding arteries were achieved. Six days later, the patient underwent an infratemporal fossa approach to remove the tumor. The patient did well postoperatively except CSF leakage that was treated successfully by dura repair. We discuss the usefulness of preoperative embolization and infratemporal approach for the huge glomus jugulare tumor.

Management of operative complications in glomus jugulare tumor surgery / 解放军医学杂志

Objective To investigate the common complications related to glomus jugulare tumors surgery,their influencing factors,and to search for the effective treatments of these complications.Methods A retrospective study was undertaken on the complications observed in 47 patients with glomus jugulare tumors who underwent 51 times of operation via trans external auditory canal approach,the retroauricular approach,and modified infratemporal fossa approach type A.All cases were followed up over 1 month after surgery.Results Of 51 cases,the most frequent complication was total conductive hearing loss which occurred in 21 cases(41.2%),facial paralysis was found in 15 cases(29.4%) and lower cranial nerve palsy was found in 14 cases(27.5%) after one month.Other complications were cerebrospinal fluid(CSF) leak,hearing disability and vertigo,each in 3 cases(5.9%);cerebral infarction,partial auricle necrosis and parotid gland fistula,each occurred in one case(2.0%).Pre-operative selective embolization of feeding vessels can reduce intraoperative blood loss significantly in most cases.Effective hemostasis and hypotensive anesthesia during operation were the important ways to prevent complications and hematoma within operating field.CSF leak in this series was an uncommon complication.Two cases with CSF leak from the wound had been successfully controlled with conservative treatment.One case with CSF leak required surgical management.None of these patients developed meningitis.Conclusions There is a low level of serious post-operative complications in operation on glomus jugulare tumor.Factors found to be relevant to post-operative complications are tumor type and tumor size.The key factors to avoid the complications include surgical experience,surgical skill and preoperative evaluation of patients′ imaging information.