Gamma knife radiosurgery of the glomus jugulare tumour. The most common tumor to develop in the jugular foramen is a paraganglioma glomus jugulare. Treatment of head and neck paragangliomas moffitt cancer center. Associated with tiny nerves which exit the skull adjacent to the carotid artery and internal jugular vein are tiny groups of cells called paraganglia. Glomus jugulare tumours are classically described as benign tumours with a long time course often measured in decades. The relative carorideo of glomus jugulare with respect to other head and neck paraganglioma varies from publication to publication and depending on definition of the glomus carotideo jugulare, tympanicum and jugulotympanicum. Surgical excision is the preferred method of treatment for benign glomus tumors. Hi i have recently been diagnosed with a glomus jugulare tumour, i am a 43yrs old female.
The jugular foramen is also where the jugular vein and several important nerves exit the skull. A patient with a glomus jugulare often hears their own heart beat in the affected ear pulsatile tinnitus. If the jugular foramen is found to be by and large free of tumor or anatomical distortion, it is most likely to be a tympanomastoid paraganglioma. Among the mentioned choices, glomus jugulare is the only one that can be that extensive. The radiosurgery is a safe and effective therapy for patients with glomus jugulare tumor.
The relative prevalence of glomus jugulare with respect to other head and neck paraganglioma varies from publication to publication and depending on the definition of the terms jugulare, tympanicum, and jugulotympanicum. All glomus jugulare tumors pose therapeutic and surgical challenges with. Your team may recommend a combination of treatments. In 1945, rosenwasser 2 described a tumor which he felt was a carotid body tumor of the middle ear and mastoid, which histologically was described as a glomus jugulare tumor. Jan 28, 20 glomus jugulare tumors are included in a group of tumors called paragangliomas.
Glomus jugulare and glomus tympanicum tumors are now grouped into a single category, jugulotympanic tumors. Jul 28, 2019 glomus yugular pdf glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma. We report a case of secretory glomus jugulare tumor in a 65 year old female who presented with hearing impairment, pulsatile tinnitus, from 3 years and difficulty in swallowing, hoarseness of voice and palpitation from last six months. Glomus jugulare paraganglioma is a paraganglioma of the head and neck that is confined to the jugular fossa. Glomus tumors of the fingers consist of a convoluted arteriovenous anastomosis. If you have problems viewing pdf files, download the latest version of adobe reader. Jeffrey bruce discusses cutting edge brain tumor treatment at aans 20 dr. Unlike other types of cancer, there is no test that determines benign from malignant tumors. Hearing impairment, vocal cord paralysis, tongue deviation, and facial pain suggest involvement of the 8th, 10th, 12th, and 5th cranial nerves.
Glomus tumors paraganglioma glomus tumor are typically benign but highly vascular tumors which arise in association with nerves which exit a very difficult anatomic area beneath the ear. In neurofibromatosis patients with glomus tumors, the sex distribution, tumor location, and tumor burden appear similar to those in patients with isolated glomus tumors. Glomus jugulare and carotid body tumours sciencedirect. Because of the anatomic location of glomus jugulare, this tumor typically involves the seventh, ninth, tenth, and eleventh cranial nerves. To analyze the efficacy of gamma knife radiosurgery gks as a primary or an adjunctive form of therapy. Gamma knife radiosurgery for glomus jugulare tumors. Glomus jugulare tumor is a rare, benign paraganglioma of the skull base that forms in the jugular fossa of the temporal bone and is made up of glomus cells which are neural crest cell derivatives.
Paragangliomas in the skull base are ubiquitous in their distribution and arise from paraganglia or glomus cells situated at the following sites. A rare case of glomus jugulare tumor presenting as. Also called glomus tumors, they arise from paraganglion cells of neuroectodermal origin frequently located near nerves and vessels. Paragangliomas account for % of all neoplasms in the head and neck region, and. On april 18, 1942, at the mount sinai hospital, in new york city, i operated upon a patient with a bleeding mass which arose in the middle ear and protruded from his left ear canal. A glomus jugulare tumor develops when cells in nerves near the temporal bone of the skull begin to rapidly increase in number, forming a mass this tumor gets its name from the nerves, called glomus bodies, and their location in a small opening in the temporal bone, known as the jugulare foramen.
Pdf the goal of this paper is to analyze the extension and relationships of glomus. We aimed to present a classic case of a glomus jugulare tumor and a practical approach to identifying and managing this condition. Glomus jugulare tumors gjt are considered benign, slowgrowing and highly vascularized, and are also known as paragangliomas or chemodectomas. Pdf prospective study of glomus jugulare and tympanicum. Introduction it is the commonest benign tumour of middle ear. Glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma a glomus tumor also known as a solitary glomus tumor, solid glomus tumor, is a rare neoplasm arising from the glomus body and mainly found under the nail, on the fingertip or in the foot 670 they account for less than 2% of all soft tissue tumors. While glomus tympanicum tumors originate in the middle ear space, glomus jugulare tumors originate from the paraganglia tissue around the jugular bulb, particularly the tympanic branch of cn ix jacobsons nerve or auricular branch of. Therapeutic embolization of feeders from these major vessels may be performed in freeflow conditions or with a temporary. Leave the tumour and be monitored yearly, surgery, or radiotherapy. Glomus jugulare tumors are included in a group of tumors called paragangliomas. In recent years, the otologic world has been fascinated by the appearance of reports describing various aspects of the glomus jugulare tumor.
Guild, 1 in 1941, first described small glomus like structures in the adventitia of the jugular bulb. They are included in a group of tumors referred to as paragangliomas, which occur at various sites and include carotid body, glomus vagale, and glomus tympanicum tumors. Two patients are living symptom free following orthovoltage treatment. A case report benzel is one of the founding members of the lumbar spine research society, formed in clinical research has encompassed such issues as hydrocephalus, neonatal hemorrhage, cerebrovascular disorders, cranial trauma, critical care, brain death, microelectromechanical systems memsand of course, spinal. Isaacson gets his third top doctor award of the year dr. It also contains paraganglionic cells derived from neural crest. Surgery withwithout embolization and conventional radiotherapy has been the traditional management option. Glomus tumour dr vineeth g junior resident department of ent government medical college kottayam 2. Most agree however that they are more common than glomus vagale 3. Surgery of glomus jugulare tumors pubmed central pmc. They originate from paraganglionic tissues that can be found in the adventitia of the dome of the jugular bulb. Jan 15, 2019 glomus yugular timpanico pdf glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma. Glomus jugulare tumor gjt is a slowgrowing, benign neural crest tumor arising in the jugular foramen of the temporal bone. Glomus jugulare jugular foramen paraganglioma, temporal bone paraganglioma, glomus tumor is.
This is an openaccess article distributed under the terms of the creative commons attribution license, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. I have seen a consultant in my local hospital and he has given me three options. Glomus jugulare jugular foramen paraganglioma, temporal bone paraganglioma, glomus tumor is a slow growing vascular tumor located at the skull base. Underwent right tympanoplasty with tumor resection, with pathology demonstrating paraganglioma. Glomus yugular pdf glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma. Radiotherapy demonstrated a 65% tumor response rate and a 25% cure rate histologically and clinically. Nonparaganglioma jugular foramen lesions masquerading as glomus jugulare tumors. Stereotactic radiosurgery for the treatment of glomus. A glomus jugulare tumor is a tumor of the part of the temporal bone in the skull that involves the middle and inner ear structures. Current perspectives in the management of glomus jugulare tumors narayan jayashankar 1, suresh sankhla 2 1 department of otorhinolaryngology and skull base surgery, nanavati superspeciality hospital, vile parle, mumbai, maharashtra, india 2 department of neurosurgery, nanavati superspeciality hospital, vile parle, mumbai, maharashtra, india.
Glomus bodies are thermoregulatory shunts concentrated in the dermis of the fingertips and other peripheral sites subject to excessive cold and should be distinguished from unrelated adrenal and extraadrenal paragangliomas, also commonly called glomus tumors. Normally, these nerves respond to changes in body temperature or blood pressure. Diagnosis, management, and complications of glomus tumors. Paragangliomas of the head and neck originate most commonly from the paraganglia within the carotid body, vagal nerve, middle ear, and jugular foramen. A patient with a glomus jugulare often hears their own heart beat in the affected ear pulsa. The lesion on the right enlarges and occludes the jugular foramen with extension towards the carotid canal. Glomus jugulare tumors are rare, slowgrowing, hypervascular tumors that arise within the jugular foramen of the temporal bone. In 1945, rosenwasser 2 described a tumor which he felt was a carotid body tumor of the middle ear and mastoid, which histologically was described as a. Both commonly present as a middle ear mass resulting in tinnitus in 80% and hearing loss in 60%. A rare case of glomus jugulare tumor presenting as papillede. Malignant glomus tumors, or glomangiosarcomasare extremely rare and usually represent a locally infiltrative malignancy. Familial glomangiomas have been associated with a variety of deletions in the glmn glomulin gene, and are inherited in an autosomal dominant manner, with incomplete penetrance. Surgery of glomus jugulare tumors article pdf available in skull base 3.
The excised tumor tissue was identical in every characteristic with proven benign carotid body. A glomus tumor is a rare neoplasm arising from the. Glomus jugulare glomus jugulare tumours glomus jugulare tumor glomus jugulare tumors glomus jugulare paragangliomas. Jeffrey bruce esteemed panelist at another free cancercare teleconference dr. Microsurgical resection of glomus jugulare tumors with facial nerve reconstruction. Pdf abstract a glomus tumor is the most common primary tumor of the middle ear. The associated cranial nerve palsies tend to be more cosmetic than debilitating. Jan 07, 2016 glomus jugulare tumours tend to grow slowly with only a small proportion metastasising. A strong association between type i neurofibromatosis and glomus tumors has been identified. Glomus jugulare tumors are benign but locally aggressive tumors. Glomus tumor treatment symptoms, and treatments aurora. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free.
Pdf gamma knife radiosurgery for glomus jugulare tumors. Glomus jugulare tumor gjt is uncommon and has been shown to develop primarily between the fourth and seventh decades of life 1 2. A glomus jugulare tumor grows in the temporal bone of the skull, in an area called the jugular foramen. Symptoms of these tumors usually include pulsatile tinnitus, hearing loss, facial nerve paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore, episodic hypertension with headaches, and tachycardia. For language access assistance, contact the ncats public information officer. Treatment of glomus jugulare tumors in patients with advanced age. Objective glomus jugulare tumors are rare indolent tumors that frequently involve the lower cranial nerves cns. Jun 14, 2017 glomus jugulare tumors are rare, slowgrowing, hypervascular tumors that arise within the jugular foramen of the temporal bone.
The effectiveness of surgery compared with radiotherapy as the primary treatment for glomus jugulare tumors remains controversial. The cranial nerves of the jugular foramen may be compressed, resulting swallowing difficulty, or ipsilateral weakness of the upper trapezius and sternocleiodomastoid muscles from compression of the spinal. Radical removal of glomus jugulare tumor can be achieved without anterior transposition of the facial nerve. Although these tumours may be locally invasive, most cases are histologically. Current perspectives in the management of glomus jugulare.
The therapeutic results in 72 patients with glomus jugulare and nine with glomus vagale have indicated a surgical cure rate of 80% in the former and 100% in the latter. Pdf surgery of glomus jugulare tumors researchgate. The overall mortality rate is worse for patients treated with radiotherapy although radiotherapy patients are higherrisk patients at the outset. If you have a glomus tumor, your aurora neurosurgical team, including the experts at the aurora neuroscience innovation institute anii, will work with you to determine the best treatment for your needs. We recommend surgical excision as primary treatment for glomus tympanicum tumors and high voltage radiotherapy as primary treatment for glomus jugulare tumors. Jun 07, 2019 a glomus jugulare tumor is a tumor of the part of the temporal bone in the skull that involves the middle and inner ear structures. Sinonasal tract, glomangiopericytoma hemangiopericytoma. Complete resection can be difficult and associated with lower cn injury.
This area contains nerve fibers, called glomus bodies. Glomus jugulare tumors gj are chemodectomas found in the region of the jugular foramen and extending into the temporal bone, middle ear cavity and the infratemporal fossa with frequent invasion of the adjacent jugular bulb, internal carotid artery and the lower cranial nerves. Most agree however that they are more common than glomus vagale 3 these tumors are seen in adults, typically between 40 and 60 years of age, with a moderate. Despite the short followup period and the limited number of patients analyzed, we can infer that radiosurgery produce a tumor growth control with low morbidity, and may be used as a good option to surgical resection in selected cases. Current perspectives in the management of glomus jugulare tumors. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Glomus jugulare paraganglioma radiology reference article. Metastases from glomus jugulare tumours request pdf. In addition to a number of anatomic variants and imaging artifacts, it may be affected by a variety of primary and secondary lesions. A definitive consensus on the best treatment modality for this disease cannot be reached because there are few studies and most of them lack adequate information. Glomus tumors, or paragangliomas, are slowgrowing, usually benign tumors in the carotid arteries major blood vessels in your neck, the middle ear or the area below the middle ear jugular bulb. Diagnosis, management, and complications of glomus tumors of. Simultaneous contralateral vestibular schwannoma and glomus jugulare tumor. May 12, 2019 glomus yugular timpanico pdf glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma.
A retrospective analysis of patients who received gks at. Glomus tumors arose in a nonsubungual location in 38% of patients. Imaging of jugular foramen antibodies or varicella zoster dna in the cerebrospinal fluid. Guild, 1 in 1941, first described small glomuslike structures in the adventitia of the jugular bulb. While it is a rare tumor, it is the most common of the jugular fossa tumors. They are rare tumors, with an overall estimated incidence of 0 000. Microsurgical resection of glomus jugulare tumors with. Glomus jugulare tumors are rare lesions with an annual incidence of approximately 1 in 1. Glomus yugular timpanico pdf glomus tumor was also the name formerly and incorrectly used for a tumor now called a paraganglioma. Epidemiology the relative prevalence of glomus jugulare wi.
Gamma knife radiosurgery gkrs has established its role as a noninvasive alternative treatment option for these often formidable lesions. This is a case of bilateral gloms jugulare paraganglioma. Glomus jugulare tumor information mount sinai new york. A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites including the head, neck, thorax and abdomen. Symptoms of a glomus jugulare tumor include dizziness, difficulty swallowing, hearing issues, pain, or weakness in the muscles of the face. This was the first of these tumors to be reported as such in the. They are usually benign, meaning cells in the tumor are unlikely to spread to other parts of the body. Glomus jugulare gj tumors are paragangliomas found in the region of the jugular foramen.