Glomus Jugulare Tumors Treatment & Management

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. ^[30]

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. ^[5]

Surgery is the treatment of choice for glomus jugulare tumors. However, radiation therapy, particularly stereotactic radiosurgery (eg, Gamma Knife surgery), has been shown to provide good tumor growth control with a low risk of treatment-related cranial nerve injury. ^{[1, 17, 18, 19, 20, 31, 32, 33, 34]}  Numerous studies of stereotactic radiosurgery have shown reduction or stabilization of tumor size and improvement in overall neurologic deficit. ^{[1, 17, 18, 19, 20]}  

The short- and intermediate-term risk of progression to nonserviceable hearing following stereotactic radiosurgery for jugular paraganglioma is low.  In a series of 85 patients who underwent SRA for the treatment of jugular paraganglioma, the Kaplan-Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. ^[35]

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 growthi and mprovement of tinnitus and overall neurological status, as well as cranial nerve function. ^[36]

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. ^[37]

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. ^[38]

In a study of 28 patients treated with radiosurgery and 2 patients with stereotactic radiosurgery, crude overall survival, tumor control, clinical control, and long-term grade 1 toxicity rates were 97%, 97%, 97%, and 13% (4/30), respectively. No statistically significant risk factor was associated with lower tumor control in the series. Univariate analysis showed a statistically significant association between patients having 1 cranial nerve (CN) involvement before radiosurgery and a higher risk of lack of improvement of symptoms (odds ratio 5.24, 95% confidence interval 1.06–25.97, P=0.043). ^[39]

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. ^[40] 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. ^[40]

Because this tumor is rare and may present with various symptoms, surgery may be contraindicated for various reasons, including age and general physical condition. Surgical resection of the glomus tumor is relatively simple and complication free for type I tumors. Large tumors that affect the lower cranial nerves and extend beyond the petrous apex carry a significant risk of postoperative complications, especially in older patients. In these cases, other modalities of treatment should be considered (eg, embolization, radiation, Gamma Knife rardiosurgery, intratumoral injection of cyanoacrylate glue).

Ehret et al evaluated the effectiveness and safety of image-guided robotic radiosurgery (RRS) and reported overall local control of 99% after a median follow-up of 35 months and stated that 56% of patients experienced symptom improvement or recovered entirely after undergoing RRS. ^[15] Another study reported that quality of life analyses after RRS revealed no significant decline, while bodily pain significantly decreased. ^[41]

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 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 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 need to be treated with a combined otologic and neurosurgical approach. An infratemporal approach with a skull base resection and a posterior fossa exploration is 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 ^[31] 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. ^[5]

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. ^{[9, 10]} However, a study by Prahbu showed that even complex glomus tumors can be managed surgically. ^[42]

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. ^[43]

(See the images below.)

A significant decrease of tumor vascular blush (arrows) following embolization of a norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension.

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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.

Surgical approach depends on the localization and extent of the tumor. 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 is advisable in the attempt to remove the entire tumor. ^[44]

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.

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 after radiation therapy in a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations.

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Corresponding MRI of the tumor depicted in the previous image indicating no evidence of tumor growth over time.

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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, sigmoid sinus thrombosis, secondary tumor development, pituitary-hypothalamic insufficiency, CSF leak, tumor growth, and radiation necrosis of bone, brain, or dura. ^[5]

Toxicities of single-fraction stereotactic radiosurgery (SRS) include vertigo, nausea, and headache along with lower cranial neuropathies. ^[45]