Stereotactic radiosurgery for jugular foramen schwannomas: an international multicenter study.

OBJECTIVE: For some jugular foramen schwannomas (JFSs), complete resection is possible but may be associated with significant morbidity. Stereotactic radiosurgery (SRS) is a minimally invasive alternative or adjunct to microsurgery for JFSs. The authors reviewed clinical and imaging outcomes of SRS for patients with these tumors. METHODS: Nine participating centers of the International Gamma Knife Research Foundation identified 92 patients who underwent SRS between 1990 and 2013. Forty-one patients had prior subtotal microsurgical resection. The median interval between previous surgery and SRS was 15 months (range 0.5-144 months). Eighty-four patients had preexisting cranial nerve (CN) symptoms and signs. The median tumor volume was 4.1 cm3 (range 0.8-22.6 cm3), and the median margin dose was 12.5 Gy (range 10-18 Gy). Patients with neurofibromatosis were excluded from this study. RESULTS: The median follow-up was 51 months (range 6-266 months). Tumors regressed in 47 patients, remained stable in 33, and progressed in 12. The progression-free survival (PFS) was 93% at 3 years, 87% at 5 years, and 82% at 10 years. In the entire series, only a dumbbell shape (extension extracranially via the jugular foramen) was significantly associated with worse PFS. In the group of patients without prior microsurgery (n = 51), factors associated with better PFS included tumor volume < 6 cm3 (p = 0.037) and non-dumbbell-shaped tumors (p = 0.015). Preexisting cranial neuropathies improved in 27 patients, remained stable in 51, and worsened in 14. The CN function improved after SRS in 12% of patients at 1 year, 24% at 2 years, 27% at 3 years, and 32% at 5 years. Symptomatic adverse radiation effects occurred in 7 patients at a median of 7 months after SRS (range 5-38 months). Six patients underwent repeat SRS at a median of 64 months (range 44-134 months). Four patients underwent resection at a median of 14 months after SRS (range 8-30 months). CONCLUSIONS: Stereotactic radiosurgery proved to be a safe and effective primary or adjuvant management approach for JFSs. Long-term tumor control rates and stability or improvement in CN function were confirmed.

Gamma Knife radiosurgery for glomus jugulare tumors: a single-center series of 75 cases.

OBJECTIVE Glomus jugulare tumors are rare indolent tumors that frequently involve the lower cranial nerves (CNs). Complete resection can be difficult and associated with lower CN injury. Gamma Knife radiosurgery (GKRS) has established its role as a noninvasive alternative treatment option for these often formidable lesions. The authors aimed to review their experience at the National Centre for Stereotactic Radiosurgery, Sheffield, United Kingdom, specifically the long-term tumor control rate and complications of GKRS for these lesions. METHODS Clinical and radiological data were retrospectively reviewed for patients treated between March 1994 and December 2010. Data were available for 75 patients harboring 76 tumors. The tumors in 3 patients were treated in 2 stages. Familial and/or hereditary history was noted in 12 patients, 2 of whom had catecholamine-secreting and/or active tumors. Gamma Knife radiosurgery was the primary treatment modality in 47 patients (63%). The median age at the time of treatment was 55 years. The median tumor volume was 7 cm3, and the median radiosurgical dose to the tumor margin was 18 Gy (range 12-25 Gy). The median duration of radiological follow-up was 51.5 months (range 12-230 months), and the median clinical follow-up was 38.5 months (range 6-223 months). RESULTS The overall tumor control rate was 93.4% with low CN morbidity. Improvement of preexisting deficits was noted in 15 patients (20%). A stationary clinical course and no progression of symptoms were noted in 48 patients (64%). Twelve patients (16%) had new symptoms or progression of their preexisting symptoms. The Kaplan-Meier actuarial tumor control rate was 92.2% at 5 years and 86.3% at 10 years. CONCLUSIONS Gamma Knife radiosurgery offers a risk-versus-benefit treatment option with very low CN morbidity and stable long-term results.

Stereotactic LINAC-Radiosurgery for Glomus Jugulare Tumors: A Long-Term Follow-Up of 27 Patients.

BACKGROUND: The optimal treatment of glomus jugulare tumors (GJTs) remains controversial. Due to the critical location, microsurgery still provides high treatment-related morbidity and a decreased quality of life. Thus, we performed stereotactical radiosurgery (SRS) for the treatment of GJTs and evaluated the long-term outcome. METHODS: Between 1991 and 2011, 32 patients with GJTs underwent SRS using a linear accelerator (LINAC) either as primary or salvage therapy. Twenty-seven patients (median age 59.9 years, range 28.7-79.9 years) with a follow-up greater than five years (median 11 years, range 5.3-22.1 years) were selected for retrospective analysis. The median therapeutic single dose applied to the tumor surface was 15 Gy (range 11-20 Gy) and the median tumor volume was 9.5 ml (range 2.8-51 ml). RESULTS: Following LINAC-SRS, 10 of 27 patients showed a significant improvement of their previous neurological complaints, whereas 12 patients remained unchanged. Five patients died during follow-up due to old age or other, not treatment-related reasons. MR-imaging showed a partial remission in 12 and a stable disease in 15 patients. No tumor progression was observed. The actuarial overall survival rates after five, ten and 20 years were 100%, 95.2% and 79.4%, respectively. CONCLUSIONS: Stereotactic LINAC-Radiosurgery can achieve an excellent long-term tumor control beside a low rate of morbidity in the treatment of GJTs. It should be considered as an alternative therapy regime to surgical resection or fractionated external beam radiation either as primary, adjuvant or salvage therapy.

Leksell Gamma Knife radiosurgery of the jugulotympanic glomus tumor: long-term results.

OBJECT: Glomus tumors usually display indolent behavior, and the effectiveness of radiation in stopping their growth can be assessed after long-term follow-up. Currently only midterm results of radiosurgery are available, so the authors included patients treated by Gamma Knife at least 10 years ago in this study to obtain a perspective of long-term results. METHODS: During the period from 1992 to 2003, the Gamma Knife was used to treat 46 patients with glomus tumors. The age of the patients ranged from 21 to 79 years (median 56 years). Gamma Knife radiosurgery was the primary treatment in 17 patients (37%). Open surgery preceded radiosurgery in 46% of cases, embolization in 17%, and fractionated radiotherapy in 4%. The volume of the tumor ranged from 0.2 to 24.3 cm(3) (median 3.6 cm(3)). The minimal dose to the tumor margin ranged between 10 and 30 Gy (median 20 Gy). RESULTS: One patient was lost for follow-up after radiosurgery. Clinical follow-up was available in 45 patients and 44 patients were followed with MRI in a follow-up period that ranged from 12 to 217 months (median 118 months). Neurological deficits improved in 19 (42%) of 45 patients and deteriorated in 2 patients (4%). Tumor size decreased in 34 (77%) of 44 patients with imaging follow-up, while an increase in volume was observed in 1 patient (2%) 182 months after radiosurgery and Gamma Knife treatment was repeated. One patient underwent another Gamma Knife treatment for secondary induced meningioma close to the glomus tumor 98 months after initial radiosurgical treatment. Seven patients died 22-96 months after radiosurgery (median 48 months), all for unrelated reasons. CONCLUSIONS: Radiosurgery has proved to be a safe treatment with a low morbidity rate and a reliable long-term antiproliferative effect.

Subtotal resection for management of large jugular paragangliomas with functional lower cranial nerves.

OBJECTIVES: To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease. STUDY DESIGN: Case series with planned chart review. SETTING: Tertiary academic referral center. SUBJECTS AND METHODS: Patients who presented with advanced jugular paragangliomas and functional lower cranial nerves were analyzed. Primary outcome measures included extent of resection, long-term tumor control, need for additional treatment, and postoperative lower cranial nerve function. RESULTS: Twelve patients (mean age, 46.2 years; 7 women, 58.3%) who met inclusion criteria were evaluated between 1999 and 2013. The mean postoperative residual tumor volume was 27.7% (range, 3.5%-75.0%) of the preoperative volume. When the residual tumor volume was less than 20% of the preoperative volume, no tumor growth occurred over an average of 44.6 months of follow-up (P < .01). Four tumors (33.3%) demonstrated serial growth at a mean of 23.5 months following resection, 2 of which were treated with salvage stereotactic radiotherapy providing control through the last recorded follow-up. No patient experienced permanent postoperative lower cranial neuropathy as a result of surgery. CONCLUSION: Subtotal resection of jugular paragangliomas with preservation of the lower cranial nerves is a viable management strategy. If more than 80% of the preoperative tumor volume is resected, the residual tumor seems less likely to grow.

Glomus jugulare tumours: a 15 year radiotherapy experience in South Australia.

Glomus jugulare tumours (GJT) are one of a family of benign hypervascular neoplasms that arise from chief cells of the paraganglionic tissue from the dome of the jugular bulb. Historically, these tumours have primarily been managed surgically but radiation is an alternative treatment modality. The purpose of this retrospective review was to determine the patient survival, tumour control, clinical control rates and long term toxicity of GJT treated with radiation in South Australia. Between 1996 and 30 June 2012, 15 patients with GJT (16 tumours) were managed with radiotherapy. Twelve patients were female and the median age was 62 years. Thirteen patients (87%) were treated with conventional external beam radiotherapy and two patients (13%) with stereotactic radiosurgery. The mean duration of follow-up was 4 years (range 1 month-15 years 4 months). The close-out date for survival analyses was 31 October 2012. Crude overall survival, tumour control, clinical control and long term grade >2 toxicity rates were 100%, 94% (15/16), 94% and 7% (1/16), respectively. The Kaplan-Meier 5 and 10 year clinical and tumour control rates were both 93% (95% confidence interval: 61-99%). Radiation is an effective treatment modality for GJT. With comparable patient survival, arguably improved tumour and clinical control rates relative to surgery, minimal toxicity and ongoing advances in radiotherapy technologies, radiation should be strongly considered for the primary management of GJT.

Infratemporal fossa approach type a with transcondylar-transtubercular extension for Fisch type C2 to C4 tympanojugular paragangliomas.

BACKGROUND: The classic infratemporal fossa type A approach (IFTA-A) permits superior and anterior exposure of the jugular foramen. The transcondylar-transtubercular extension facilitates posteroinferior and medial access to the jugular foramen. The purpose of this study was to present the IFTA-A with transcondylar-transtubercular extension and to assess its surgical results. METHODS: A review of patients with tympanojugular paraganglioma who underwent resection through the IFTA-A with transcondylar-transtubercular extension was performed. RESULTS: In all, 39 patients were included in the study. The average follow-up was 23.6 months. Gross total tumor removal was achieved in 87.2% of the cases and there was evidence of recurrence in 5.9% of this group. CONCLUSION: The transcondylar-transtubercular extension of the classic IFTA-A is aimed at making the excision of Fisch type C2 to C4 tympanojugular paragangliomas simpler and safer by drilling out one third of the lateral part of the occipital condyle and removing the jugular process and jugular tubercle.

Robotic stereotactic radiosurgery in patients with unresectable glomus jugulare tumors.

We evaluated the treatment results of robotic stereotactic radiosurgery (SRS) in our patients with unresectable glomus jugulare tumors (GJTs). The medical charts of fourteen patients with GJT, who were treated with robotic SRS, were retrospectively evaluated. The gross tumor volume was described as the clinical target volume. The median dose to the tumor was 25 Gy in median 5 fractions. The dose was normalized to 80% isodose line. All patients were evaluated for tumor growth and clinical outcome every 6 months in the first 2 years and then annually. Median follow-up was 39 months (range, 7-60 months). Lesions were stable in 8 patients, and tumor regression was observed in 6 patients. We did not observe any treatment related toxicity in our patients. In conclusion, according to our early experience, robotic SRS seems to be successful treatment option in the management of unresectable GJTs.

Radiosurgery for glomus jugulare tumors.

Glomus jugulare tumors arise from adventitial chemoreceptor tissue in the jugular bulb. Although histologically benign, these tumors can be locally aggressive because of their proximity to the lower cranial nerves and major vascular structures. Traditional treatment involves microsurgical removal with or without endovascular embolization, but morbidity following total resection can result in injury to the facial and lower cranial nerves. Radiosurgery has recently emerged as a promising alternative to older therapeutic strategies for treatment of glomus jugulare tumors. This article reviews the latest benefits of radiosurgery and demonstrates how this modality represents an effective treatment option for glomus jugulare tumors with excellent tumor control and low risk for morbidity. In addition, this article will detail the role of minimally invasive sub-total resection of glomus jugulare tumors as a surgical complement to gamma knife therapy.

Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life.

BACKGROUND AND PURPOSE: For glomus jugulare tumors, the goal of treatment is microsurgical excision. To minimize postoperative neurologic deficits, stereotactic radiosurgery (SRS) was performed as an alternative treatment option. Stereotactic fractionated radiotherapy (SRT) could be a further alternative. This study aims at the assessment of local control, side effects, and quality of life (QoL). PATIENTS AND METHODS: Between 1999-2005, 17 patients were treated with SRT. 11/17 underwent previous operations. 6/17 received primary SRT. Treatment was delivered by a linear accelerator with 6-MV photons. Median cumulative dose was 57.0 Gy. Local control, radiologic regression, toxicity, and symptomatology were evaluated half-yearly by clinical examination and MRI scans. QoL was assessed by Short Form-36 (SF-36). RESULTS: Median follow-up was 40 months. Freedom from progression and overall survival for 5 years were 100% and 93.8%. Radiologic regression was seen in 5/16 cases, 11/16 patients were stable. Median tumor shrinkage was 17.9% (p=0.14). Severe acute toxicity (grade 3-4) or any late toxicity was never seen. Main symptoms improved in 9/16 patients, 7/16 were stable. QoL was not affected in patients receiving primary SRT. CONCLUSION: SRT offers an additional treatment option of high efficacy with less side effects, especially in cases of large tumors, morbidity, or recurrences after incomplete resections.

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

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.

Image cytometric nuclear DNA quantitation of paragangliomas in tissue sections. Prognostic significance.

OBJECTIVE: Image cytometric quantitation of nuclear DNA of paragangliomas may provide prognostic information that cannot be obtained from histopathologic study. Flow cytometry has demonstrated DNA aneuploid tumors to have a higher risk of progression than diploid neoplasms. STUDY DESIGN: DNA ploidy of 56 paragangliomas was assessed by image cytometry of 5-micron, Feulgen-stained, formalin-fixed, paraffin-embedded tissue sections. RESULTS: Thirty-three (59%) paragangliomas were diploid and 23 (41%) aneuploid. Of the 30 adrenal pheochromocytomas, 15 (50%) were diploid. Thirteen (93%) of the 14 carotid body tumors were diploid. Five of seven (71%) glomus jugulare tumors and two of five (40%) extraadrenal paragangliomas were aneuploid. During a mean follow-up of 57 months (range, 1 month to 36 years) of 44 patients with 47 paragangliomas, 33 (75%) were alive and without disease; 7 (16%), including 1 glomus jugulare, 2 carotid body and 4 pheochromocytoma patients, developed recurrences/metastases. By multivariate analysis, image cytometric DNA ploidy was predictive of disease-free survival for adrenal pheochromocytomas. No significant differences in overall survival, disease-free survival or recurrence/metastasis rate were noted between other diploid and aneuploid tumors. CONCLUSION: Aneuploidy suggests a risk of early recurrence for adrenal pheochromocytomas.

Glomus jugulare tumours: a review of 61 cases.

A retrospective study of 61 patients with glomus jugulare tumours treated at the National Hospital for Neurology and Neurosurgery, and at the Royal National Throat, Nose and Ear Hospital, London. The average age at presentation was 41.7 years. The patients were mainly treated by a posterolateral combined otoneurosurgical approach. 42/61 of the patients had total or subtotal excision of their tumours, 7/61 had partial removal and the remaining 11/61 had no operation. Only one case required a 2-staged procedure. There were two deaths in the postoperative period, one from intracerebral haemorrhage and the other from the left hemisphere infarction. Postoperative radiotherapy was given to 5/7 of the patients who had partial removal. 3/40 of the patients with total removal had postoperative radiotherapy, and a further 3/40 had received radiotherapy pre-operatively. Of the 11 patients who did not undergo surgery, 7/11 were treated with radiotherapy and 4/11 had embolisation only.

[Morphology and clinical aspects of paragangliomas in the area of the head-neck]. / Morphologie und Klinik der Paragangliome im Kopf-Hals-Bereich.

Clinical data and tumor specimens from 63 patients were investigated, and included 22 glomus caroticum tumors and 41 tumors of the tympano-jugulare region. All specimens could be differentiated by their histological growth patterns into four categories. The clinical data revealed that 45% of the female patients with paragangliomas suffered from goitre, one of whom died in thyrotoxic crisis after surgery. Two patients died from sequelae of other serious diseases. In general, postoperative morbidity was solely caused by cranial nerve palsies depending on tumor extent. From the data presented, a conclusion is drawn that serious concurrent diseases are the limiting factor for surgery. Early detection is the best way to reduce postoperative morbidity if total removal of the tumor is required.

Radiation therapy for paragangliomas of the temporal bone.

Treatment of paragangliomas of the temporal bone (glomus jugulare and glomus tympanicum tumors) is controversial, with both surgery and radiation therapy having their advocates. This paper discusses the experience at the University of Arizona Health Sciences Center in treating 10 cases of this uncommon tumor between 1971 and 1988. Seven of 10 cases were initially treated using irradiation and achieved complete tumor control for a mean of 67 months (range = 23-107 months). Two patients, one treated surgically and the other by embolization, had recurrences and were salvaged by radiation, and neither has recurred. The final patient is disease-free 9 months after embolization and surgery. There have been no serious sequelae of treatment. We conclude that moderate-dose irradiation can safely control most temporal bone paragangliomas.

Treatment of glomus tumours. A retrospective survey.

A retrospective survey of symptomatology, treatment and course of disease in 45 patients with glomus tumours treated between 1959 and 1986 at the Radium Centre and ENT Department of the Aarhus University Hospital is presented. Nine patients were treated surgically, 7 had surgery and irradiation combined, and 25 patients were treated solely with radiation therapy. Six patients developed recurrence of tumours. Two patients died of tumour, one of them with pulmonary metastases.

The management of glomus jugulare tumours.

The treatment details of 58 patients treated for glomus jugulare tumours in Newcastle upon Tyne are examined in the light of other studies reported in the literature. For the group of 55 patients treated by radiotherapy, the 20 year survival is 94% (determined actuarially). The 20 year disease-free survival (determined actuarially) is 77%. This is comparable with other series reported. As no glomus tympanicum tumour has recurred following surgery and there has been no morbidity due to these tumours they have not been included in the series. It is recommended that patients who are fit and have tumours confined to the tympanum should have primary surgical treatment. All other patients should be treated by accurately planned radiotherapy, using a dose of 50Gy in 5 weeks to the tumour volume. The morbidity of this treatment policy will be low.

Radiotherapy for jugulo-tympanic paragangliomas (glomus jugulare tumours).

Sixty patients with glomus jugulare tumour, seen at St. Bartholomew's Hospital, London, over forty years, have been reviewed. The term 'jugulo-tympanic paraganglioma' is suggested as a more accurate name for these tumours. The following simple classification is recommended: Group I tumours (tympanic paragangliomas) are those with or without VIIth nerve palsy, but with no other cranial nerve involvement. Group II tumours (jugular paragangliomas) are those with involvement of any cranial nerve other than the VIIth. The method and results of treatment by radiotherapy are discussed. It is concluded that surgery and radiotherapy give comparable results in Group I tumours, but radiotherapy gives superior results in Group II tumours.