|Year : 2020 | Volume
| Issue : 1 | Page : 6-9
Radiotherapy for glomus jugulare tumors: A single-institution experience
Renuka Masodkar1, GK Jadhav1, Sapna Manocha Verma1, Sudheer Kumar Tyagi2
1 Department of Radiation Oncology, Indraprastha Apollo Hospital, New Delhi, India
2 Department of Neurosurgery, Indraprastha Apollo Hospital, New Delhi, India
|Date of Submission||25-Jan-2020|
|Date of Acceptance||01-Feb-2020|
|Date of Web Publication||17-Mar-2020|
Department of Radiation Oncology, Indraprastha Apollo Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
Introduction: Glomus jugulare tumors are rare and usually benign tumors arising within the jugular foramen of the temporal bone. The management options for these locally aggressive tumors include surgery, radiotherapy, and observation. Here, we report outcomes of treating these tumors with radiotherapy. Materials and Methods: Twelve cases of glomus jugulare were treated with radiotherapy from August 2014 to July 2019. The patients were treated using Novalis Tx linear accelerator. The median follow-up period was 45 months (range: 5–60 months). The patients were evaluated for treatment outcomes, neurological status, and treatment-related side effects. Results: Nine patients (75%) were female. The median age was 47 years (range: 32–60 years). Eight patients (66.6%) had left-sided tumor. The median volume of the tumor was 10.81 cm3 (range: 4.4–207.62 cm3). All the patients had cranial nerve deficits ranging from cranial nerves V and VII–XII. The most common symptom was headache (75%), followed by loss of hearing and tinnitus (66.6%). All patients tolerated radiotherapy well with no Grade — reactions. There were no breaks during radiation due to toxicity. One patient reported improvement in swallowing and speech quality during the course of treatment. On follow-up, 5/12 (41.66%) patients reported improvement in neurological symptoms. The rest of them had stable neurological status. Follow-up magnetic resonance imaging showed regression in three patients and stable disease in the rest of them. Conclusion: Radiotherapy is a safe and effective treatment for glomus jugulare tumors. With newer techniques and image guidance, precise treatment delivery to the tumor is possible with protection of normal tissues. In our series, we report a local control of 100%. Hence, radiotherapy could be considered as modality of choice in these tumors.
Keywords: Glomus jugulare, paraganglioma, radiotherapy
|How to cite this article:|
Masodkar R, Jadhav G K, Verma SM, Tyagi SK. Radiotherapy for glomus jugulare tumors: A single-institution experience. Apollo Med 2020;17:6-9
| Introduction|| |
Paragangliomas or chemodectomas are rare and usually benign tumors arising from the paraganglia, clusters of neuroendocrine cells that are associated with the peripheral nervous system. Glomus bodies are found in anatomic sites such as in the jugular bulb, along the tympanic and auricular branch of the tenth nerve in the middle ear. According to the location, glomus tumors can be classified as tympanic, jugulare, or carotid body or designated as arising from other locations.
The mean age at diagnosis ranges from 40 to 50 years. These tumors occur three to four times more frequently in women than in men, suggesting a possible estrogen influence.
Clinical presentation of head-and-neck paragangliomas differs according to the site they arise from. Glomus tumors of the middle ear present with pain or discomfort in the ear, pulsatile tinnitus, and hearing loss. Carotid body tumors present as a painless, slow-growing mass in the neck that may be pulsatile and may be associated with bruits. Glomus jugulare tumors originate at the skull base at the jugular bulb and may be associated with bone destruction. Patients with these tumors may develop cranial nerve deficits; typically, cranial nerves 9 through 12 are involved. These tumors can also originate and spread along the tympanic canaliculus, invading superiorly into the middle ear and inferiorly toward the jugular fossa. Rarely, these tumors can be malignant.
Management options for these tumors include surgery or radiation therapy. The modality of choice remains controversial. Most of the criticism related to the surgical management of glomus jugulare tumors is based on the morbidity associated with cranial nerve loss and the fact that radical removal is almost impossible to achieve since these tumors are histologically aggressive.
With growing emphasis on preserving the quality of life of the patients, radiotherapy has become modality of choice in many cases. The advances and precision associated with modern radiotherapy techniques have allowed higher doses to the tumor, increased accuracy, and protection to the normal tissues. Here, we present our experience of treating glomus jugulare tumors with radiotherapy.
| Materials and Methods|| |
Twelve diagnosed cases of glomus jugulare were treated with radiotherapy from August 2014 to July 2019 at our hospital. Magnetic resonance imaging (MRI) fusion was done for contouring the target for each patient using iPlan system (BrainLab, Germany). The treatment plans were generated using Eclipse software version 13 (ARIA 13 from Varian Medical Systems Inc., USA). The patients were treated using Novalis Tx linear accelerator. ExacTrac X-ray monitoring from BrainLab imaging was used for image guidance before treatment. The treatment technique and radiotherapy dose of each patient are given in [Table 1]. The median follow-up period was 45 months (range: 5–60 months). Patients were followed up with history and physical examination and MRI 1 year posttreatment. The patients were evaluated for treatment outcomes, neurological status, and treatment-related side effects.
|Table 1: Patient and tumor characteristics and the treatment and dose given to each patient|
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| Results|| |
Nine of twelve patients (75%) were female. The median age was 47 years (range: 32–60 years). Eight patients (66.6%) had a left-sided tumor. All the tumors were arising from the jugular foramen and were classified as glomus jugulare tumors. The median volume of the tumor was 10.81 cm3 (range: 4.4–207.62 cm3). All the patients had cranial nerve deficits ranging from cranial nerves V and VII–XII. The most common symptom was headache (75%), followed by loss of hearing and tinnitus (66.6%) and then facial nerve palsy (33.3%). One of the patients had bleeding from the ear for which embolization had to be done before radiation. One of the patients had received radiotherapy elsewhere; however, she presented with loss of hearing and worsening of facial pain and was planned for reirradiation 1 year later at our center. All patients tolerated radiotherapy well with no Grade — reactions. One of the patients developed external otitis media during treatment and was managed accordingly. There were no breaks during radiation due to the toxicity. One patient reported improvement in swallowing and speech quality during the course of treatment. On follow-up, 5/12 (41.66%) patients reported improvement in neurological symptoms. The rest of them had stable neurological status. Follow-up MRI showed regression in three patients [Figure 1] and stable disease in the rest of them.
|Figure 1: (a) Preradiotherapy magnetic resonance imaging image. (b) Postradiotherapy magnetic resonance imaging showing regression of the tumor volume|
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| Discussion|| |
Glomus jugulare tumors are usually benign, slow-growing, vascularized tumors. They are locally aggressive, causing invasion of the jugular foramen and tympanic cavity, expanding inside the skull, and causing destruction of the neurovascular structures.
The treatment options for glomus jugulare tumors include surgery, radiotherapy, and observation. The choice of treatment depends on tumor size, location, patients' age and performance status, and the anticipated morbidity associated with the treatment modalities.
Both surgery and radiotherapy provide high local control rates. However, high rates of morbidity and chances of incomplete resection of these locally aggressive tumors are the main arguments for advocates of primary radiotherapy. On review of literature, around 88.2% of tumors were totally resected on the first surgery, with a surgical control rate of 92.1%. The mortality rate of surgical options is a little more than 1%. Despite the development of microsurgical techniques, there are high rates of morbidity associated with resection of these tumors. Surgery is associated with a high risk of postoperative morbidity due to lower cranial nerve loss. The rate of new cranial nerve injury postsurgery ranges from 22% to 59%.,
With emergence of precision radiation techniques and to provide a good quality of life with minimal morbidity, radiotherapy has become a modality of choice for many cases. The efficacy of radiotherapy is defined by inhibition of tumor growth, absence of recurrence of symptoms, and radiological progression of the disease.
The current recommended dose of conformal radiotherapy is between 40 and 50 Gy delivered over 25 fractions. This dose achieves an optimal therapeutic ratio with satisfactory local tumor control. Glomus tumors are ideal candidates for radiosurgical treatment as well since they do not invade the brain and are well defined on magnetic resonance images. This helps in target delineation, precise stereotactic tracking of the target as well as a smaller volume of irradiated normal tissue. However, tumor size is a limiting factor for stereotactic radiosurgery (SRS).
The common side effect associated with radiotherapy treatment includes mucositis, external otitis media, nausea, and xerostomia. Rarely, patients can develop neurological symptoms of transient glossopharyngeal, vagus, and accessory nerve paralysis with swallowing disorders, aspiration, or dysphonia.
All published studies of conventional radiotherapy report tumor control rates close to 90%., Stereotactic radiotherapy with Gamma Knife, linear accelerator (LINAC), and/or CyberKnife results in good tumor and symptom control rates, ranging from 71% to 100% and 88% to 100%, respectively, with much lower rates of morbidity than with surgery.,,
In a study by Suárez et al., treatment outcomes of a glomus jugulare tumor treated with surgery or radiotherapy were compared. Tumor control failure, complication rates, and the number of cranial nerve palsies after the treatment were found to be significantly higher in surgical series as compared to radiotherapy. The results of SRS and external beam radiotherapy were compared, and no significant differences were observed in tumor control.
In a series of 156 head-and-neck paragangliomas which were treated with fractionated radiotherapy with a median dose of 45 Gy in 25 fractions, a local control rate at 5 and 10 years of 99% and 96%, respectively, was reported without any severe complications.
In a report of 27 patients treated with LINAC-based SRS with a single median therapeutic dose of 15 Gy (range: 11–20 Gy), 10/27 patients showed a significant improvement of their previous neurological complaints and the rest remained unchanged. MRI showed a partial remission in 12 and a stable disease in 15 patients. The actuarial overall survival rates after 5 and 10 years were 100% and 95.2%, respectively. Fractionated stereotactic radiotherapy is also a treatment option with high efficacy and low toxicity profile, especially in cases of large tumor volume. The outcomes with fractionated stereotactic radiosurgery are comparable with those of single-fraction SRS.
Golanov et al. reported results in 34 patients treated either with SRS or hypofractionation using CyberKnife. The mean prescribed dose was 17 ± 3.1 Gy (range: 13.7–22 Gy). Hypofractionation using 3–7 fractions with a total dose of 18–35 Gy was used for thirty patients. At mean follow-up of 8 months, they had a 100% tumor control rate. The occurrence of neurological deficits after radiotherapy was found to be rare, and in most of the cases, it is transient.
Gamma Knife radiosurgery is also an effective treatment option for glomus jugulare tumors. Published series have reported a high rate of tumor control with low rates of cranial nerve morbidity.
In a meta-analysis to assess the tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors, the patients undergoing SRS had the lowest rates of recurrence and achieved the highest rates of tumor control (P< 0.01) as compared to subtotal resection, gross total resection, and subtotal resection + postoperative SRS. Patients who underwent gross tumor resection sustained worse rates of cranial nerve deficits with regard to cranial nerves IX–XI as compared to those who underwent SRS alone.
In this study of 12 cases, patients had a good local tumor control rate with external beam radiotherapy and fractionated SRS with minimal morbidity. Most of the patients had stable neurological status and did not progress radiologically indicating the efficacy of radiotherapy treatment.
| Conclusion|| |
Radiotherapy is a safe and effective treatment option for glomus jugulare tumors. With newer techniques and image guidance, precise treatment delivery to the tumor is possible with protection of the normal tissues. In this series, we report a local control of 100%. Hence, radiotherapy could be considered as modality of choice in these locally aggressive tumors.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kumar V, Abbas A, Fausto N, Aster J. Robbins and Cotran: Pathological Basis of Disease. 8th
ed. Philadelphia, PA: Saunders; 2010. p. 755.
Perez CA, Thorstad WL. Unusual nonepithelial tumors of the head and neck. In: Halperin EC, editor. Perez and Brady's Principles and Practice of Radiation Oncology. 5th
ed. Philadelphia; 2007. p. 997-1034.
Konefal JB, Pilepich MV, Spector GJ, Perez CA. Radiation therapy in the treatment of chemodectomas. Laryngoscope 1987;97:1331-5.
Gilbo P, Morris CG, Amdur RJ, Werning JW, Dziegielewski PT, Kirwan J, et al
. Radiotherapy for benign head and neck paragangliomas: A 45-year experience. Cancer 2014;120:3738-43.
Pareschi R, Righini S, Destito D, Raucci AF, Colombo S. Surgery of glomus jugulare tumors. Skull Base 2003;13:149-57.
Gottfried ON, Liu JK, Couldwell WT. Comparison of radiosurgery and conventional surgery for the treatment of glomus jugulare tumors. Neurosurg Focus 2004;17:E4.
Cece JA, Lawson W, Biller HF, Eden AR, Parisier SC. Complications in the management of large glomus jugulare tumors. Laryngoscope 1987;97:152-7.
Tran Ba Huy P. Radiotherapy for glomus jugulare paraganglioma. Eur Ann Otorhinolaryngol Head Neck Dis 2014;131:223-6.
de Andrade EM, Brito JR, Mario SD, de Melo SM, Benabou S. Stereotactic radiosurgery for the treatment of glomus jugulare tumors. Surg Neurol Int 2013;4:S429-35.
Larner JM, Hahn SS, Spaulding CA, Constable WC. Glomus jugulare tumors. Long-term control by radiation therapy. Cancer 1992;69:1813-7.
Krych AJ, Foote RL, Brown PD, Garces YI, Link MJ. Long-term results of irradiation for paraganglioma. Int J Radiat Oncol Biol Phys 2006;65:1063-6.
Pollock BE. Stereotactic radiosurgery in patients with glomus jugulare tumors. Neurosurg Focus 2004;17:E10.
Guss ZD, Batra S, Limb CJ, Li G, Sughrue ME, Redmond K, et al
. Radiosurgery of glomus jugulare tumors: A meta-analysis. Int J Radiat Oncol Biol Phys 2011;81:e497-502.
Suárez C, Rodrigo JP, Bödeker CC, Llorente JL, Silver CE, Jansen JC, et al
. Jugular and vagal paragangliomas: Systematic study of management with surgery and radiotherapy. Head Neck 2013;35:1195-204.
El Majdoub F, Hunsche S, Igressa A, Kocher M, Sturm V, Maarouf M. Stereotactic LINAC-radiosurgery for glomus jugulare tumors: A long-term follow-up of 27 patients. PLoS One 2015;10:e0129057.
Henzel M, Hamm K, Gross MW, Surber G, Kleinert G, Failing T, et al
. Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life. Strahlenther Onkol 2007;183:557-62.
Schuster D, Sweeney AD, Stavas MJ, Tawfik KY, Attia A, Cmelak AJ, et al
. Initial radiographic tumor control is similar following single or multi-fractionated stereotactic radiosurgery for jugular paragangliomas. Am J Otolaryngol 2016;37:255-8.
Golanov AV, Kapitanov DN, Pronin IN, Shelesko EV, Zolotova SV, Shchurova IN, et al
. First experience of cyberknife stereotactic radiotherapy for glomus jugulare tumors. Zh Vopr Neirokhir Im N
N Burdenko 2012;76:30-6.
Ibrahim R, Ammori MB, Yianni J, Grainger A, Rowe J, Radatz M. Gamma knife radiosurgery for glomus jugulare tumors: A single-center series of 75 cases. J Neurosurg 2017;126:1488-97.
Ivan ME, Sughrue ME, Clark AJ, Kane AJ, Aranda D, Barani IJ, et al
. A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors. J Neurosurg 2011;114:1299-305.