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Glomus Tumors Treatment & Management

  • Author: Ryszard M Pluta, MD, PhD; Chief Editor: Brian H Kopell, MD  more...
 
Updated: May 12, 2016
 

Medical Therapy

Some cases require no treatment. Often, glomus jugulare tumors are diagnosed within the sixth or seventh decade of life and can be followed by imaging only and may not need surgical intervention.

A study from Vanderbilt University found that in the absence of brainstem compression or concern for malignancy, observation of  glomus jugulare tumors can be a viable initial management approach for elderly patients. Of 15 patients studied (80% female; median age, 69.6 yr), radiologic growth occurred in 5 patients. The median growth rate of the 5 enlarging tumors was 0.8 mm/yr (range, 0.6-1.6 mm/yr) using maximum linear dimension, or 0.4 cm3/yr (0.1-0.9 cm3/yr) with volumetric analysis. No deaths were attributable to tumor progression or treatment.[14]

Medical therapy may be indicated in some cases. Alpha-blockers and beta-blockers are useful for tumors secreting catecholamines. They are usually administered for 2-3 weeks before embolization and/or surgery to avoid potentially lethal blood pressure lability and arrhythmias. Successful treatment of pulmonary metastases with etoposide (VP-16) and cisplatin has been described. In a preliminary report, a somatostatin analogue (octreotide) has been successfully used for growth control of somatostatin receptor–positive tumors.

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Surgical Therapy

Surgery is the treatment of choice for glomus jugulare tumors. However, more recently, radiation therapy, particularly a gamma knife radiosurgery, has been shown to provide good tumor growth control with a low risk of treatment-related cranial nerve injury.[15, 16]

A large retrospective, multicenter, international study analyzed the long-term outcome in 132 patients with primary radiation treatment or radiation after partial resection of a glomus tumor. The study found long-term successful control of the tumor growth, improvement of tinnitus and overall neurological status, as well as cranial nerve function. These results strongly suggest that gamma knife radiosurgery is becoming the treatment of choice for glomus tumors.[17]

Of 22 patients with glomus jugulare tumors who underwent gamma knife surgery, neurologic status improved in 12 patients, 7 showed stable clinical condition, and 3 patients developed new moderate deficits. The average tumor volume was 7.26 cm3. Tumor volume following surgery was unchanged in 13 patients and was decreased in 8; tumor regrowth occurred in 1 patient. Tumor progression-free survival was 95.5% at 5 and 7 years.[18]

A German study of 32 patients who underwent stereotactic radiosurgery for glomus jugulare tumors showed that stereotactic linear accelerator (LINAC) radiosurgery achieved excellent long-term tumor control, along with a low rate of morbidity. According to the study, following LINAC stereotactic radiosurgery, 10 of 27 patients showed a significant improvement of their previous neurologic complaints, whereas 12 patients remained unchanged. No tumor progression was observed. Five patients died due to unrelated causes. Overall survival rates after 5, 10, and 20 years were 100%, 95.2% and 79.4%, respectively.[19]

Because resection of glomus jugulare tumors can be challenging due to their inherent vascularity, preoperative embolization of these tumors with ethylene vinyl alcohol (Onyx) has been proposed.[20] A study by Gaynor et al showed a dramatic reduction of blood loss and facilitation of surgical resection, but these results came at the price of a higher incidence of cranial nerve neuropathy.[20]

The surgical approach depends on the localization and extension of the tumor. Intraoperative monitoring including EEGs and somatosensory-evoked potentials (SSEPs) are routinely used.

Fisch type A tumors (see Pathophysiology) can be excised by a transmeatal or perimeatal approach.

Type B tumors (see Pathophysiology) require an extended posterior tympanotomy.

Type C tumors (see Pathophysiology) require radical resection via a standard combined transmastoid-infratemporal or transtemporal-infratemporal approach with or without internal carotid artery (ICA) trapping, preceded by external carotid artery (ECA) embolization or superselective embolization. Intraoperatively, temporarily occlude the transverse or sigmoid sinus with EEG monitoring to determine whether vein bypass should be performed for total resection. Surgery leads to therapeutic success in about 90% of patients. Intratumoral injection of cyanoacrylate glue has been proposed to control bleeding.

Large type D tumors (see Pathophysiology) need to be treated with a combined otologic and neurosurgical approach. An infratemporal approach with a skull base resection and a posterior fossa exploration are the most advisable in attempting to remove the entire tumor. Partial resection of the tumor needs to be followed by radiation and follow-up MRI/CT scanning.

Radiation therapy and radiosurgery may be indicated. Both classic fractionated radiation therapy (40-50 Gy) and stereotactic radiosurgery (eg, gamma knife surgery) are successful in long-term control of tumor growth[15] and in decrease of catecholamine excretion in functional tumors; however, the short duration of observation after stereotactic radiosurgery does not allow for definite conclusions. Radiation treatment is advised as the sole treatment modality for elderly or infirm patients who are symptomatic, especially those with extensive or growing tumors.

Gross total resection of some extensive tumors may be extremely difficult and may carry unwarranted risk. In such cases, radiotherapy may be indicated to treat residual tumor following subtotal resection.[3, 4] However, a study by Prahbu showed that even complex glomus tumors can be managed surgically.[21]

In a study of 51 patients with jugular foramen tumors who underwent less-aggressive surgical interventions to preserve neurovascular structures, overall tumor recurrence-regrowth-free survival, symptom-progression-free survival, and overall survival at 15 years were 78.9%, 86.8%, and 80.6%, respectively. The tumor recurrence-regrowth rate was 11.8%, swallowing function improved or stabilized in 96.1%, and facial function improved or stabilized in 94.1%. Overall neurologic status improved or stabilized in 90% of patients. In the study patients, the mean lesion size was 3.8 cm, and 43 cases (84.3%) were Fisch Type D, including 37 cases (72.5%) of Type Di1 and Di2. Thirty-seven cases (72.5%) were Glasscock-Jackson Type III-IV. Gross-total resection and subtotal resection were achieved in 26 (51.0%) and 22 (43.1%) cases, respectively.[22]

See the images below.

A significant decrease of tumor vascular blush (ar A significant decrease of tumor vascular blush (arrows) following embolization of a norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension.
CT imaging demonstrates the extent of bony destruc CT imaging demonstrates the extent of bony destruction (white and black arrows) by the tumor. The normal jugular foramen on the left (arrow head) is shown for comparison. The patient subsequently underwent surgical resection of the extracranial portion of this extensive tumor. The remaining intracranial portion was treated with radiation therapy (54 Gy). Follow-up evaluations, including imaging and laboratory investigations, demonstrated long-term control of both tumor growth and catecholamine production.
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Preoperative Details

If routine screening for catecholamine is positive (3 times the reference range), alpha-blockers and beta-blockers are administered for 2-3 weeks before surgery and embolization. This helps to avoid blood pressure lability and arrhythmias. In emergent cases, 3 days of treatment is adequate.

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Intraoperative Details

Surgical approach depends on the localization and extent of the tumor (see Pathophysiology). Fisch type A tumors can be excised by a transmeatal or perimeatal approach. Type B tumors require an extended posterior tympanotomy. Type C tumors require radical resection via a standard combined transmastoid-infratemporal or transtemporal-infratemporal approach with or without ICA trapping, preceded by external carotid artery embolization or superselective embolization. Surgery leads to therapeutic success in about 90% of patients. Treat large type D tumors with a combined otologic and neurosurgical approach. An infratemporal approach with a skull base resection and a posterior fossa exploration are advisable in the attempt to remove the entire tumor.[23]

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Postoperative Details

Patients are usually in the sixth decade of life; therefore, careful monitoring of cardiac function is advisable, especially if a catecholamine secreting tumor was only partially resected.

Postoperative lower cranial nerve deficits need to be carefully diagnosed, and, when present, early rehabilitation is advocated.

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Follow-up

Radiologic and, when indicated, endocrinologic monitoring for tumor growth or regrowth is indicated every 6 months to 1 year for 2 years and then, depending on the dynamics of the tumor behavior, every 2 years. See the images below.

Lateral carotid arteriogram obtained 22 years afte Lateral carotid arteriogram obtained 22 years after radiation therapy in a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations.
Corresponding MRI of the tumor depicted in the pre Corresponding MRI of the tumor depicted in the previous image indicating no evidence of tumor growth over time.
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Complications

Complications of surgery include death, cranial nerve palsies, bleeding, cerebrospinal fluid (CSF) leak, meningitis, uncontrollable hypotension/hypertension, and tumor regrowth.

Complications of radiation include ICA thrombosis, secondary tumor development, pituitary-hypothalamic insufficiency, CSF leak, tumor growth, and radiation necrosis of bone, brain, or dura.

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Outcome and Prognosis

Glomus jugulare tumors may grow slowly and produce cranial nerve palsies that, to a certain point, are benign and mostly cosmetic. However, despite this optimistic assessment, a recent study showed a long-term reduced quality of life in patients with glomus tumors.[2]

The mortality rate is 6.2% among patients treated with radiation and 2.5% among those treated surgically. The overall mortality rate is 8.7%.

Twenty years after treatment, the survival rate is 94%, and 77% of patients remain symptom free. In 1945, Rosenwasser described the first patient diagnosed with glomus jugulare tumor. The patient survived until 1987.[8]

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Future and Controversies

Surgery is the treatment of choice for glomus tumors, and its effectiveness will improve with intraoperative guiding and imaging systems.

The cooperative work of neurosurgeons and neuro-otologists to surgically resect Fisch type A, B, and C tumors has proven to be of value. However, definitive optimal treatment of type D glomus jugulare tumor is still controversial.

Because of its long-term effects on the bone and brain, radiation that is not stereotactically targeted is outdated. Radiosurgery with its influence on neuro-oncology must be proven useful in treatment of these slowly growing tumors.[24]

Continued tumor growth and postsurgical damage to the lower cranial nerves are issues that still need to be successfully addressed.

Recent genetic research on familial glomus jugulare tumors suggests future directions of treatment towards gene manipulation.

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Contributor Information and Disclosures
Author

Ryszard M Pluta, MD, PhD Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Polish Society of Neurosurgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Brian A Iuliano, MD Attending Neurosurgeon, Central Maryland Neurosurgical Associates

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

Additional Contributors

Duc Hoang Duong, MD Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles Drew University of Medicine and Science

Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, North American Skull Base Society

Disclosure: Nothing to disclose.

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Lateral view of the initial carotid arteriogram of a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations. Urine catecholamine levels were elevated, and a pheochromocytoma was suspected. She underwent a negative exploratory laparotomy. She subsequently developed palsies of the IX, X, XI, and XII cranial nerves on the right side. A norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension was identified on radiologic and arteriographic imaging. Arrows delineate the tumor blush. The arrowhead demonstrates a branch of the middle meningeal artery providing blood supply to the tumor. This branch was embolized.
A significant decrease of tumor vascular blush (arrows) following embolization of a norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension.
CT imaging demonstrates the extent of bony destruction (white and black arrows) by the tumor. The normal jugular foramen on the left (arrow head) is shown for comparison. The patient subsequently underwent surgical resection of the extracranial portion of this extensive tumor. The remaining intracranial portion was treated with radiation therapy (54 Gy). Follow-up evaluations, including imaging and laboratory investigations, demonstrated long-term control of both tumor growth and catecholamine production.
Lateral carotid arteriogram obtained 22 years after radiation therapy in a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations.
Corresponding MRI of the tumor depicted in the previous image indicating no evidence of tumor growth over time.
 
 
 
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