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Radiosurgery for Glomus Tumors

(chemodectomas, paraganglioma, glomus jugulare or glomus tympanicum)

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Chemodectomas of the head and neck (also known as nonchromaffin paraganglioma and glomus tumors) are unusual neoplasms that arise from extra-adrenal paraganglia. These tumors grow slowly, with a median tumor doubling time of 4.2 years.

Treatment options include surgery, endovascular embolization, and radiotherapy (RT) (conventionally fractionated external beam RT [EBRT] and stereotactic radiosurgery). The treatment modalities are difficult to compare because these tumors are rare and no randomized trials have been conducted. In addition, the treatment arms in most of the reported studies are rarely equivalent and are evaluated in a retrospective fashion.

Local tumor control rates after surgery for glomus jugulare and tympanicum tumors vary from 20% to 95% with most authors reporting 80-90% control rates.. The frequency of treatment-related morbidity, mainly new cranial nerve deficits, varies considerably from 0% to 100% . Conventionally fractionated EBRT has been reported to produce local control rates of 80-100% with minimal treatment-related morbidity.. Gamma knife radiosurgery (GKRS) has been reported to control 100% of these tumors with negligible treatment-related morbidity. (IJOBP 2002 Mar 15;52(4):953-6)

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Radiosurgery has been used in the treatment of glomus tumors with good results  some of the doses in the literature as noted below
  • 12 - 18Gy     Mayo
  • 12 - 20Gy (13.5Gy) Graz
  • 10-30Gy (16.5 )Prague
  • 10-12Gy (19.4Gy) Prague
Glomus jugulare tumor. (see article) A rare tumor that grows at the base of the skull. Tumors that arise from collections of tissue (so-called "paraganglionic tissue") that looks and behaves biochemically like that found in the medulla (deep tissue) of the adrenal gland (and that in some tumors produce adrenaline and related molecules produced in the adrenal gland) that occur along the course of the glossopharyngeal and other lower cranial nerves. The tumors grow in proximity to the jugular foramen which is an opening in the base of the skull that allows for communication between the intracranial and extracranial spaces -- below and deep to the ear, deep in the neck deep to the angle of the jaw.
Compression of nerves and other structures in proximity to a glomus tumor leads to symptoms that most frequently include:
1. decreased hearing on the side of middle ear tumor invasion
2. pulsatile (with every heartbeat) ringing of the ear (tinnitus) -- (compression of the auditory nerve)
3. vertigo -- (invasion of the labyrinth)
Findings on physical examination:
4. mass (blue shadow) behind the ear drum in the middle ear seen by shining a light and looking into the ear (otoscope).
5. sound of rushing blood (bruit) heard with a stethoscope over the mastoid bone

location:  Jacobson's nerve (middle ear)                                   name : glomus tympanicum
       inferior tympanic canal, adjacent to jugular bulb :                        glomus jugulare
       along course of Arnold's nerve, descending facial canal :             glomus of facial canal
Large tumors:

Large tumors are liable to compress one or more of the lower cranial nerves causing neurologic deficits like difficulties with swallowing (glossopharyngeal and vagus nerves).Invasion of nearby structures: Posterior fossa extension and growth can lead to brainstem dysfunction due to tumor mass compression.
Blockage of the sigmoid sinus (blood flowing out of the brain back to the heart) can lead to backup of blood with increased intracranial pressure.
Treatment of glomus tumors is with embolization, surgery, and possibly radiation.

 

 

Glomus jugulare tumor: tumor control and complications after stereotactic radiosurgery.

Foote RL, Pollock BE, Gorman DA, Schomberg PJ, Stafford SL, Link MJ, Kline RW, Strome SE, Kasperbauer JL, Olsen KD.   Head Neck 2002 Apr;24(4):332-8

Division of Radiation Oncology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA. foote.robert@mayo.edu

BACKGROUND: We evaluated toxicity and long-term efficacy of stereotactic radiosurgery in patients with symptomatic or progressive glomus jugulare tumors. METHODS: Twenty-five consecutive patients (age, 30-88 years; 17 women, 8 men) who underwent stereotactic radiosurgery with the Leksell Gamma Knife (dose, 12-18 Gy) were prospectively followed. MRI and clinical examinations were performed at 6 months and 1, 2, and 3 years, and then every 2 years. RESULTS: None of the tumors increased in size, 17 were stable, and 8 decreased (median imaging follow-up, 35 months; range, 10-113 months). Symptoms subsided in 15 patients (60%); vertigo occurred in 1, but balance improved with vestibular training (median clinical follow-up, 37 months; range, 11-118 months). No other new or progressive neuropathy of cranial nerves V-XII developed. CONCLUSIONS: Stereotactic radiosurgery can achieve excellent tumor control with low risk of morbidity in the treatment of glomus jugulare tumors. The lower cranial nerves can safely tolerate a radiosurgical dose of 12 to 18 Gy.

Stereotastic radiosurgery for glomus jugulare tumors.

Jordan JA, Roland PS, McManus C, Weiner RL, Giller CA.  Laryngoscope 2000 Jan;110(1):35-8

Department of Otolaryngology, The University of Texas Southwestern Medical Center, Dallas, USA.

OBJECTIVES/HYPOTHESIS: Surgery is considered to be the mainstay of treatment for glomus jugulare tumors. A subset of patients are poor surgical candidates based on age, medical problems, tumor size, or prior treatment failure. The purpose of this study was to review our results with stereotactic radiosurgery (gamma knife treatment) in this group of patients, with particular attention to adverse reactions and symptom relief. STUDY DESIGN: Retrospective review and phone survey. METHODS: Charts were reviewed for size and location of tumor, history of previous treatment, symptoms before and after treatment, amount of radiation received, acute and late complications, and functional level before and after treatment. Pre-treatment and posttreatment magnetic resonance imaging scans were also reviewed. Identified patients were then contacted for a phone interview. RESULTS: Eight patients were identified. Phone interviews were conducted with four patients. Four patients had failed previous treatment. Follow-up ranged from 7 to 104 months. One patient experienced an acute complication: intractable vertigo requiring hospitalization. No patient experienced delayed cranial neuropathies. No patient reported worsening of any of the following symptoms: pulsatile tinnitus, hearing loss, facial weakness, hoarseness, or difficulty swallowing. Three patients reported improvement in their pulsatile tinnitus. Two patients reported improvement in hearing loss, and one patient each reported improvement in vertigo and difficulty swallowing. CONCLUSIONS: Preliminary results suggest that stereotactic radiosurgery is useful to control symptoms and may be delivered safely in patients with primary or recurrent glomus jugulare tumors who are poor surgical candidates.

Gamma knife radiosurgery for glomus jugulare tumours.

Eustacchio S, Leber K, Trummer M, Unger F, Pendl G.  Acta Neurochir (Wien) 1999;141(8):811-8

Department of Neurosurgery, Karl-Franzens Medical School, Graz, Austria.

The aim of this clinical study was to determine the tumour control rate, clinical outcome and complication rate following gamma knife treatment for glomus jugulare tumours. Between May 1992 and May 1998, 13 patients with glomus tumours underwent stereotactic radiosurgical treatment in our department. The age of these patients ranged from 21 to 80 years. The male:female ratio was 2:11. Six patients had primary open surgery for partial removal or recurrent growth and subsequent radiosurgical therapy. Radiosurgery was performed as primary treatment in 7 cases. The median tumour volume was 6.4 cm3 (range: 4.6-13.7 cm3). The median marginal dose applied to an average isodose volume of 50% (30-50%) was 13.5 Gy (12-20 Gy). In 10 patients, a total of 48 MRI and CT follow-up scans were available. The remaining three patients have been excluded from the postradiosurgical evaluation since the observation time (t < 12 months) was too short or patients were lost to follow up. The median interval from Gamma Knife treatment to the last radiological follow-up was 37.6 months (5-68 months). In 4 patients (40%) decreased tumour volumes were observed and in 6 cases (60%) the tumour size remained unchanged. Neurological follow-up examinations revealed improved clinical status in 5 patients (50%), a stable neurological status in 5 patients (50%) and no complications occurred. According to our preliminary experience Gamma Knife radiosurgery represents an effective treatment option for glomus jugulare tumours.

Gamma Knife radiosurgery of the glomus jugulare tumour - early multicentre experience.

Liscak R, Vladyka V, Wowra B, Kemeny A, Forster D, Burzaco JA, Martinez R, Eustacchio S, Pendl G, Regis J, Pellet W.    Acta Neurochir (Wien) 1999;141(11):1141-6

Stereotactic and Radiation Neurosurgery, Hospital Na Homolce, Prague, Czech Republic.

Leksell Gamma Knife was used to treat 66 patients with glomus jugulare tumour at 6 European sites between 1992-1998. The age of the patients ranged between 18-80 years (median 54 years). Gamma Knife radiosurgery was a primary treatment in 30 patients (45. 5%). Open surgery preceded radiosurgery in 24 patients (36.4%), embolisation in 14 patients (21.2%) and fractionated radiotherapy in 5 patients (7.6%). The volume of the tumour ranged 0.5-27 cm(3) (median 5,7 cm(3)). The minimal dose to the tumour margin ranged between 10-30 Gy (median 16.5 Gy). After radiosurgery 52 patients were followed, the follow up period was 3-70 months (median 24 months). Neurological deficit improved in 15 patients (29%) and deteriorated in 3 patients (5,8%), one transient and two persistant. Neuroradiological follow up using MRI or CT was performed in 47 patients 4-70 months (median 24 months) after radiosurgery. Tumour size decreased in 19 patients (40%) while in the remaining 28 patients (60%) no change in the tumour volume was observed. None of the tumours increased in volume during the observation period. Control angiography was performed in 6 patients. Pathological vascularisation completely disappeared in one patient, reduced in two and there was no change in the remaining three. Radiosurgery proves to be a safe treatment for glomus jugulare tumour with no mortality and no acute morbidity. Because of its naturally slow growth rate, up to 10 years of follow up will be necessary to establish a cure rate after radiosurgery for these lesions.

Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors.

Saringer W, Khayal H, Ertl A, Schoeggl A, Kitz K.   Minim Invasive Neurosurg 2001 Sep;44(3):141-6

Department of Neurosurgery, University of Vienna, Vienna, Austria.

This study was performed to assess the impact of gamma knife radiosurgery (RS) in the treatment of glomus jugulare tumors. Between February 1993 and February 1999, thirteen patients (9 women, 4 men; mean age 63.5 years, range 29 to 79 years) underwent stereotactic radiosurgery for glomus jugulare tumors with the Leksell Gamma Knife at the Neurosurgical Department of the University of Vienna. Four patients, mean age 74.5 years, range 67 to 79 years, underwent radiosurgery as the only treatment. Nine patients received radiosurgery as adjuvant therapy after previous treatment had failed: surgical resection in 9 patients and additional fractionated external beam radiation in two of these patients. Pretreatment evaluation included the staging of all tumors according to the Fisch Classification: De1 (7), De2 (1), Di1 (4) and Di2 (1). The mean follow-up period was 4.2 years, range 0.7 to 6.7 years. Ten patients, 77 %, were treated prior to 1997, the mean follow-up period being 5 years. Six patients showed no clinical changes, while six experienced an improvement of their clinical symptoms and neurological deficits. One patient was lost to follow-up. Radiation-induced transient cranial nerve neuropathies were observed in two patients. Serial MRI scans revealed tumor control in all patients, with unaltered tumor size in 10 and shrinkage in three patients. The results indicate that RS is an attractive treatment option for glomus jugulare tumors and will occupy an increasingly important role in the management of these tumors in selected patients.

Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale.

Hinerman RW, Mendenhall WM, Amdur RJ, Stringer SP, Antonelli PJ, Cassisi NJ.    Head Neck 2001 May;23(5):363-71

Department of Radiation Oncology, University of Florida Health Science Center, PO Box 100385, Gainesville, Florida 32610-0385, USA.

PURPOSE: To evaluate the results of treatment for 71 patients with 80 chemodectomas of the temporal bone, carotid body, or glomus vagale who were treated with radiation therapy (RT) alone (72 tumors in 71 patients) or subtotal resection and RT (8 tumors) at the University of Florida between 1968 and 1998. METHODS AND MATERIALS: Sixty-six lesions were previously untreated, whereas 14 had undergone prior treatment (surgery, 11 lesions; RT, 1 lesion; or both, 2 lesions) and were treated for locally recurrent disease. All three patients who received prior RT had been treated at other institutions. Patients had minimum follow-up times as follows: 2 years, 66 patients (93%); 5 years, 53 patients (75%); 10 years, 37 patients (52%); 15 years, 29 patients (41%); 20 years, 18 patients (25%); 25 years, 12 patients (17%); and 30 years, 4 patients (6%). RESULTS: There were five local recurrences at 2.6 years, 4.6 years, 5.3 years, 8.3 years, and 18.8 years, respectively. Four were in glomus jugulare tumors and one was a carotid body tumor. Two of the four patients with glomus jugulare failures were salvaged, one with stereotactic radiosurgery and one with surgery and postoperative RT at another institution. Two of the five recurrences had been treated previously at other institutions with RT and/or surgery. Treatment for a third recurrence was discontinued, against medical advice, before receiving the prescribed dose. There were, therefore, only 2 failures in 65 previously untreated lesions receiving the prescribed course of RT. The overall crude local control rate for all 80 lesions was 94%, with an ultimate local control rate of 96% after salvage treatment. The incidence of treatment-related complications was low. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the temporal bone and neck. There were no severe treatment complications

Recurrent head-and-neck chemodectomas: a comparison of surgical and radiotherapeutic results.

Elshaikh MA, Mahmoud-Ahmed AS, Kinney SE, Wood BG, Lee JH, Barnett GH, Suh JH.    Int J Radiat Oncol Biol Phys 2002 Mar 15;52(4):953-6

Department of Radiation Oncology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.

PURPOSE: To compare the outcome of salvage radiotherapy (RT) and surgery for recurrent head-and-neck chemodectomas. MATERIALS AND METHODS: We retrospectively studied 70 patients with benign chemodectomas of the head and neck treated with surgery at the Cleveland Clinic between July 1969 and August 1999; 29 of these patients were diagnosed with recurrent tumors. Salvage RT was used in 12 patients (gamma knife radiosurgery for 7, conventional external beam RT for 4, and intensity-modulated RT for 1 patient). The median follow-up was 55 months for the entire group of 70 patients. RESULTS: The median time to recurrence was 36 months. Of the recurrences, 16 were glomus jugulare, 7 were carotid body tumors, 5 were glomus tympanicum, and 1 was thyroid paraganglioma. RT was used in 12 patients (9 patients with glomus jugulare, 2 with glomus tympanicum, and 1 with thyroid paraganglioma). Surgery was performed in 17 patients (7 patients with glomus jugulare, 7 with carotid body, and 3 with glomus tympanicum). For patients with glomus jugulare and glomus tympanicum tumors, the 5-year freedom from disease progression was 100% for patients treated with RT vs. 62% for those treated with surgery (p = 0.0124). Seven patients with carotid body tumors and 1 patient with thyroid paraganglioma were treated successfully with surgery and RT, respectively. No significant side effects were associated with RT; however, postoperative complications occurred in 8 of the 17 surgery patients (new cranial nerve palsies, meningitis, infection, and cerebrospinal fluid leak). CONCLUSION: Salvage RT appears superior to surgery and should be considered the treatment of choice for patients with recurrent glomus jugulare and glomus tympanicum tumors. For recurrent carotid body tumors, surgery produced excellent local control, most likely because of the easier resectability in this location.

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