Paragangliomas of the head and neck.

Paragangliomas are rare neuroendocrine tumors that can be found from the skull base to the pelvis. Head and neck paragangliomas have been historically treated with surgery. However, surgical resection adds risk of injury to vascular structures and cranial nerves that can lead to morbidity such as hoarseness, dysarthria, dysphagia, or aspiration. Recently, improved understanding of the behavior of these tumors and increasing experience in non-surgical treatments, such as observation and radiation therapy, have changed the paradigms of management of this entity. Multiple series now show a trend toward a more conservative management, with a higher percentage of patients being observed or treated with radiotherapy. Several factors should be taken into consideration when deciding the most appropriate treatment for head and neck paragangliomas, starting by differentiating carotid body tumors from non-carotid body tumors. In general, surgical resection is normally recommended for carotid body tumors as the complications from treatment are usually minimal. In contrast, for non-carotid body tumors, surgery is often associated with significant functional impairment due to cranial nerve paralysis. As such, non-surgical treatment is now usually recommended for this subset of head and neck paragangliomas. In young patients with no comorbidities and a small to medium carotid body tumors, surgery should be considered. Moreover, surgery should be offered for secreting tumors, malignant tumors, tumors with rapid growth or increase in symptomatology, and when radiotherapy cannot be performed. Conversely, conservative management with active surveillance or radiotherapy can be offered in the remaining cases in order to avoid unnecessary morbidity while still providing acceptable tumor control.

An aortic body carcinoma with sarcomatoid morphology and chondroid metaplasia in a French Bulldog.

An 11-year-old female French Bulldog was presented with a mass at the base of the heart, detected by X-ray and echocardiography. Clinical abnormality included abdominal retention by ascites. Radiation therapy was performed for 5 weeks. The mass volume didn't change during the radiotherapy. The condition became worse and the dog died 6 months after the initial presentation and necropsy was performed. Grossly, the mass, 12.5 × 6.5 × 6.0 cm in size, was found at the base of the heart. Histopathological examination revealed that cardiac mass was composed of alveolar, bundle and diffuse proliferation of neoplastic cells. Most of the neoplastic cells showed a spindle morphology; in some areas small round or polyhedral neoplastic cells were observed. Occasional cartilage metaplasia was seen multifocal in the mass, and it was surrounded by the sarcomatoid proliferation. Electron microscopy revealed a few neuroendocrine granules in the cytoplasm of spindle and polyhedral neoplastic cells. Metastatic cells in the lungs which had not irradiated demonstrated typical morphology of aortic body tumors. Based on these findings, the case was diagnosed as an aortic body carcinoma with sarcomatoid morphology and chondroid metaplasia.

Radiotherapy for benign head and neck paragangliomas.

PURPOSE: To update our experience treating benign head-and-neck paragangliomas (PGs) with radiotherapy (RT). METHODS: A total of 149 patients with 176 PGs received curative-intent RT; 126 received RT to 1 PG and 23 to 2 or more PGs. The most common dose fractionation schedule was 45 Gy/25 once-daily fractions/5 weeks which was used to treat 147 PGs (83.5%) in 123 patients (82.6%). Patients were followed with physical examination and CT/MRI. The median follow-up for all patients was 10.6 years (range, 0.2-50.4 years); the median follow-up for surviving patients was 11.1 years (range, 0.2-50.4). RESULTS: The 5-year, 10-year, and 15-year outcomes were: local control, 99%, 96%, and 95%; distant metastasis-free survival, 99%, 99%, and 99%; cause-specific survival, 98%, 98%, and 98%; and overall survival, 90%, 75%, and 64%, respectively. No patient developed a moderate or severe complication, or a radiation-induced second tumor or malignant transformation of the benign PG. CONCLUSION: RT is an effective treatment for head-and-neck PGs with a low risk of complications.

Stereotactic body radiation therapy for heart-base tumors in six dogs.

INTRODUCTION: Heart-base tumors are increasingly treated with radiotherapy, yet safety and efficacy are incompletely understood. This case series describes outcomes after stereotactic body radiation therapy (SBRT) for presumed chemodectoma. ANIMALS: Six pet dogs. METHODS: A retrospective study was performed, including dogs with a clinical diagnosis of chemodectoma and treatment with three-fraction SBRT (30 Gy total). RESULTS: Heart-base tumors, presumed or confirmed to be chemodectomas, were diagnosed via histopathology or imaging. Treatment was delivered with intensity modulation and cone-beam computed tomography-based image guidance, using a linear accelerator and robotic couchtop. Intrafraction respiratory motion was managed with either neuromuscular blockade and breath-holding (n = 3) or high-frequency jet ventilation (n = 3); mean total anesthesia times for each technique were 165 and 91 min per fraction, respectively. Four tumors were assessed after SBRT; tumor volume decreased by 30-76%. Possible treatment-related complications included cough, tachyarrhythmias, and congestive heart failure. Two dogs experienced sudden death 150 and 294 days after SBRT. Three dogs are alive 408-751 days after SBRT, and one dog died of unrelated disease 1,228 days after SBRT. CONCLUSIONS: This SBRT protocol resulted in rapid tumor volume reduction, and jet ventilation effectively reduced treatment delivery times. However, cardiac arrhythmias (presumably tumor or treatment associated) and sudden death were common after SBRT. Therefore, SBRT is a potentially useful treatment but may not be appropriate for dogs with incidentally diagnosed, slowly growing tumors, which are not causing cardiovascular disturbances. Longer follow-up and larger case numbers are needed to more completely define safety and impact of treatment on long-term survivability.

Glomuvenous malformations: dual PDL-Nd:YAG laser approach.

Glomuvenous malformations are uncommon simple vascular malformations that might be present at birth or appear during childhood that have been classically classified as a subtype of venous malformations. Sclerotherapy and surgery have been used in the past as treatments for this condition although with disappointing results in large glomangiomas. The treatment of these lesions has still not been standardized. We conducted a retrospective study of 17 patients treated with dual wavelength PDL-Nd:YAG. The majority of the patients experience a reduction of at least a 60% in their glomuvenous malformations. Treatment was well-tolerated, and adverse effects were rare.

Four generations of SDHB-related disease: complexities in management.

SDHB mutations are linked to the familial paraganglioma syndrome type 4 (PGL4), which is associated with predominantly extra-adrenal disease and has high metastatic rates. Despite the lower penetrance rates in carriers of SDHB mutations compared to mutations in other paraganglioma susceptibility genes, the aggressive behavior of SDHB-linked disease warrants intensive surveillance to identify and resect tumors early. Patients with similar SDHB genotypes in whom the PGL syndrome manifests often exhibit very heterogeneous phenotypes. Tumors can arise in various locations, and management can be considerably different, depending on tumor site and pathology. We present a case series of five SDHB mutation carriers over four generations from the same family to illustrate the complexities in management.

Management of multicentric paragangliomas: Review of 24 patients with 60 tumors.

BACKGROUND: The purpose of this study was to reflect our experience in the management of multicentric head and neck paragangliomas, including genetic study and counseling, diagnostic tools, types of treatment, and the need for monitoring. METHODS: A retrospective review of 24 patients with multicentric paraganglioma, including a total of 60 paragangliomas: 37 carotid body tumors, 13 jugulotympanic paragangliomas, and 10 vagal paragangliomas. RESULTS: A total of 26 surgical procedures were performed, including the resection of 36 paragangliomas. Four tumors were pending surgery at the time of this review. Radiotherapy was administered in 7 patients with 9 tumors. A "wait-and-scan" policy with periodic MRI imaging tests was instituted for 9 patients with 12 paragangliomas. CONCLUSION: The knowledge of the different modalities of management is especially relevant in patients with multicentric paragangliomas. In every case, benefits and potential risks of all treatment options may be taken into consideration for every individual patient.

Radiotherapy for benign head and neck paragangliomas: a 45-year experience.

BACKGROUND: Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly involve the carotid body, jugular bulb, vagal ganglia, and temporal bone. Treatment options include surgery, radiotherapy (RT), stereotactic radiosurgery (SRS), and observation. This article briefly reviews our 45-year institutional experience treating this neoplasm with RT. METHODS: From January 1968 through March 2011, 131 patients with 156 benign paragangliomas of the temporal bone, carotid body, jugular bulb, or glomus vagale were treated with RT at a median dose of 45 Gy in 25 fractions. The mean and median follow-up times were 11.5 years and 8.7 years, respectively. RESULTS: Five tumors (3.2%) recurred locally after RT, all within 10 years of treatment. The overall local control rates at 5 and 10 years were 99% and 96%, respectively. The cause-specific survival rates at 5 and 10 years were 98% and 97%, respectively. The distant-metastasis free survival rates at 5 and 10 years were 99% and 99%, respectively. The overall survival rates at 5 and 10 years were 91% and 72%, respectively. There were no severe complications. CONCLUSION: RT for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. Surgery is reserved for patients in good health whose risk of associated morbidity is low. SRS may be suitable for patients with skull base tumors <3 cm where RT is logistically unsuitable. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.

Treatment of head and neck paragangliomas with external beam radiation therapy.

PURPOSE: To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. METHODS AND MATERIALS: From 1990 to 2009, 66 patients with 81 head and neck paragangliomas were treated by conventional external beam radiation therapy in 25 fractions at a median dose of 45 Gy (range, 41.4-68 Gy). One case was malignant. The median gross target volume and planning target volume were 30 cm(3) (range, 0.9-243 cm(3)) and 116 cm(3) (range, 24-731 cm(3)), respectively. Median age was 57.4 years (range, 15-84 years). Eleven patients had multicentric lesions, and 8 had family histories of paraganglioma. Paragangliomas were located in the temporal bone, the carotid body, and the glomus vagal in 51, 18, and 10 patients, respectively. Forty-six patients had exclusive radiation therapy, and 20 had salvage radiation therapy. The median follow-up was 4.1 years (range, 0.1-21.2 years). RESULTS: One patient had a recurrence of temporal bone paraganglioma 8 years after treatment. The actuarial local control rates were 100% at 5 years and 98.7% at 10 years. Patients with multifocal tumors and family histories were significantly younger (42 years vs 58 years [P=.002] and 37 years vs 58 years [P=.0003], respectively). The association between family predisposition and multifocality was significant (P<.001). Two patients had cause-specific death within the 6 months after irradiation. During radiation therapy, 9 patients required hospitalization for weight loss, nausea, mucositis, or ophthalmic zoster. Two late vascular complications occurred (middle cerebral artery and carotid stenosis), and 2 late radiation-related meningiomas appeared 15 and 18 years after treatment. CONCLUSION: Conventional external beam radiation therapy is an effective and safe treatment option that achieves excellent local control; it should be considered as a first-line treatment of choice for head and neck paragangliomas.

Experiences in the treatment of patients with multiple head and neck paragangliomas.

PURPOSE: To analyze treatment results in the multidisciplinary management of patients with multiple head and neck paragangliomas (HNPs). METHODS: Retrospective analysis including all patients with multiple HNPs (VP, vagal paraganglioma; JTP, jugulotympanic paraganglioma; CBT, carotid body tumor) treated between 2000 and 2013 at a tertiary referral center. RESULTS: Ten patients (three men, seven women) had 25 HNPs (two VPs, eight JTPs, and 15 CBTs). The age range at diagnosis was 31-71 years (mean 40.9 years, median 37 years). Nine tumors (four CBTs, three JTPs, two VPs) were treated only with stereotactic radiotherapy (SRT; 50.4-56Gy, mean 55.3Gy) or in one case intensity-modulated radiotherapy (60 Gy). Nine tumors were treated with surgery alone (eight CBTs, one JTP) and three JTPs with subtotal surgery combined with adjuvant SRT. A "wait and scan" strategy was used in three cases (two CBTs, one JTP). The mean follow-up period was 4.3 years (range 0.1-13 years, median 4 years). The rate of tumor control with surgery and/or SRT was 100% (21/21). One patient with a wait-and-scan strategy for CBT had slow asymptomatic progression during a 13-year follow-up. CONCLUSIONS: The treatment results in this series of patients with multiple HNPs show that a very high rate of long-term tumor control with low morbidity can be achieved using tailored and individualized approaches. All of the different treatment strategies available should be discussed with the patient. In particular, the treatment should involve a multidisciplinary team of experts in the fields of nuclear medicine, genetics, pathology, radiology, radio-oncology, and surgery.

Clinical response and tumor control based on long-term follow-up and patient-reported outcomes in patients with chemodectomas of the skull base and head and neck region treated with highly conformal radiation therapy.

BACKGROUND: The purpose of this study was to evaluate long-term outcome after radiation therapy (RT) for skull base or head-and-neck chemodectomas. METHODS: We treated 39 patients with chemodectomas with RT. Patients were treated with either single dose stereotactic radiosurgery, fractionated stereotactic radiotherapy, or intensity modulated radiotherapy (IMRT), depending on the size and anatomy of the lesion. At primary diagnosis, 16 patients were treated with surgical resection (41%), 4 with interventional embolization (10%), and 19 with primary RT (49%). Single doses of 18 Gy/80% isodose were applied. For fractionated treatments delivered as primary RT, a median total dose of 57.6 Gy was delivered. Five patients were treated with re-irradiation, of which 4 were treated with fractionated regimens; total dose was 28.8 Gy, 30 Gy, 40 Gy, and 56 Gy in 1.8 to 2 Gy single fractions. RESULTS: Clinical symptoms improved in 18 patients (46%), remained unchanged in 10 patients (26%), and worsening of sequelae was observed in only 1 patient, which was associated with tumor progression. Actuarial local control was 97% at 10 years. Overall survival was 89% at 5 years and 87% at 10 years. Deaths were not disease-related except in 3 patients with tumor progression. No secondary malignancies were observed. CONCLUSION: RT has been established as a treatment alternative for patients with glomus tumors. Long-term local control is very high, with good clinical response to treatment.

Malignant pheochromocytoma and paraganglioma: a population level analysis of long-term survival over two decades.

BACKGROUND AND OBJECTIVES: Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare tumors. Aims of this study were to describe and to compare demographic, clinical, pathologic, and survival characteristics of malignant PHEO and PGL. METHODS: Patients were identified in SEER, 1988-2009. Analyses included chi-square, ANOVA, Kaplan-Meier, and Cox proportional hazard regression. RESULTS: Gender distribution and mean age were similar for PHEO and PGL. Surgery was performed in 74.3% of PHEO and 78.9% of PGL; external beam radiation was administered in 8.0% of PHEO and 28.1% of PGL (P < 0.001). Compared to PGL, PHEO were larger (mean size 7.7 vs. 4.5 cm PGL, P = 0.001) and more were SEER-staged as localized (17.3% vs. 49.6%, respectively, P < 0.001). PGLs were more often located in the trunk than in the head/neck (53.8% vs. 38.0%, P < 0.001). PHEO had lower overall and disease-specific survival than PGL (54.0% and 73.5% vs. 73.3% and 80.5% for PGL, respectively, P < 0.001 and P = 0.118). Independent factors associated with mortality for PHEO included not undergoing surgery and metastases at diagnosis; for PGL, these were age 61-75 years, size ≥5 cm, and presenting with metastases. CONCLUSIONS: Malignant PHEO has a more aggressive course than malignant PGL; long-term survival has not improved over the last two decades. Multi-institutional efforts should be pursued to seek novel treatments.

Use of three-dimensional conformal radiation therapy for treatment of a heart base chemodectoma in a dog.

CASE DESCRIPTION: A 9-year-old spayed female mixed-breed dog was evaluated because of a progressively worsening, nonproductive cough and gagging of 1 year's duration. CLINICAL FINDINGS: Physical examination results were unremarkable. A cranial mediastinal mass was identified at the heart base with 3-view thoracic radiography. A CT scan of the thorax revealed an invasive mass surrounding major vessels at the heart base that was not considered surgically resectable. Thoracoscopic biopsy specimens of the cranial mediastinal mass were obtained, and histologic evaluation revealed that the tumor was a chemodectoma. TREATMENT AND OUTCOME: On the basis of results of the CT scan, a 3-D conformal radiation therapy plan was generated with computer treatment-planning software. The patient was treated with external beam radiation therapy; a 6-MV linear accelerator was used to deliver a prescribed dose of 57.5 Gy in twenty-three 2.5-Gy fractions. The cough improved following radiation therapy. Prior to treatment, the tumor volume was calculated to be 126.69 cm(3). Twenty-five months following radiation therapy, a follow-up CT scan was performed and there was a >50% reduction in tumor volume at that time. Disease progression causing pericardial, pleural, and peritoneal effusion and syncopal episodes occurred 32 months following radiation therapy, which were treated with pericardectomy and additional radiation therapy. The dog was still alive and doing well 42 months following initial radiation treatment. CLINICAL RELEVANCE: Conformal radiation therapy provided an additional treatment option for a nonresectable heart base chemodectoma in the dog of this report; conformal radiation therapy was reasonably tolerable and safe.

[Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan?]. / Endokrine Chirurgie zervikaler Paragangliome: Operation, Strahlentherapie oder "wait and scan"?

BACKGROUND: Head and neck paraganglioma (HNP) represent rare endocrine tumors. Therapy is decided on genetic findings, tumor characteristics (e.g. tumor size, localization and dignity), age of patient and symptoms. In terms of local control radiation therapy is as equally effective as surgery but surgical morbidity rates secondary to cranial nerve injuries remain high. PATIENTS: Based on 6 patients with 11 solitary (4 patients) and multiple (2 patients) HNP (8 carotid body tumors, 1 vagal, 1 jugular and 1 jugulotympanic paraganglioma) the specific characteristics of the need for surgery as well as correct choice of treatment in cases of sporadic succinate dehydrogenase (SDH) negative and hereditary SDH positive HNP will be exemplarily demonstrated. RESULTS: A total of 6 carotid body tumors (four sporadic, two hereditary) were resected in 4 patients, five as primary surgery and one as a revision procedure. In one case a preoperative embolization was performed 24 h before surgery. Malignancy could not be proven in any patient. The 30-day mortality was zero. In the patient with bilateral hereditary carotid body tumors, unilateral local recurrent disease occurred. After resection of the recurrent tumor permanent unilateral paralysis of the laryngeal nerve, glossopharyngeal nerve and hypoglossal nerve occurred. All patients were followed-up postoperatively for a mean of 64 months (range 23-78 months) with a local tumor control rate of 100%. The overall survival rate after 5 years was 100%. CONCLUSIONS: Given a very strict indication with awareness of surgical risks selective surgery has a key position with low postoperative morbidity in the treatment of HNPs. We prefer surgery for small unilateral paraganglioma, malignant or functioning tumors.

[Treatment results of temporal bone area paragangliomas]. / Wyniki leczenia przyzwojaków kosci skroniowej.

INTRODUCTION: Temporal bone paragangliomas are rare, benign neuroendocrine tumors. Depend on their location at skull base, they can extend intracranially and extracranially. They characterize by slowly growth and late nonspecific clinical manifestation. Location of tumor and anatomical condition require suitable operational technique and cause related complications. AIM: The aim of this study was analysis of surgical procedure and evaluation of treatment results of patients with paragangliomas of the temporal bone hospitalized in Department of Otolaryngology of Warsaw Medical University in years 2000-2010. MATERIAL AND METHODS: 34 cases of patients were retrospectively studied. Clinical data, level of tumor advancement according to Glasscock & Jackson and Fisch classification were analyzed. Intraoperative and imaging studies data were used to evaluate extent of lesions. Suitable operative approaches and outcome of treatment were also analyzed. CONCLUSION: In group of 34 patients 26 of them had monocentric and 8 multicentric neoplasms. Age ranged 15-73-years-old. Most common symptoms were: loss of hearing, tinnitus and headache. Preoperative embolization was carried in 10 cases. Most common surgery technique: tympanotomy and hypotympanotomy posterior and infratemporal fossa of Fisch type A approaches. Radiotherapy was used in 2 cases and surgery with radiation therapy in 7 cases. Most commonly observed complication in surgical treatment was intermittent paresis.

[Nonchromaffin paragangliomas (chemodectoma) of the middle ear in the material of the ENT Department Medical University of Gdansk]. / Przyzwojaki niechromochlonne (chemodectoma) ucha srodkowego w materiale Kliniki Gdanskiej.

INTRODUCTION: Nonchromaffin paraganglioma (chemodectoma) is the most common neoplasm of the middle ear. The diagnosis and treatment is one of the most challenging in otolaryngology. THE AIM OF THE STUDY: was clinical and epidemiological analysis of patients with nonchromaffin paraganglioma of the middle ear. MATERIAL AND METHODS: The material consisted of 13 patients (10 females, 3 males), age 19-62 years, treated for paraganglioma of the middle ear in the ENT Department Medical University of Gdansk between 1968 and 2009. We analyzed the symptoms and results of additional diagnostic tests necessary to establish the diagnosis. RESULTS: We have diagnosed 12 cases of benign chemodectoma (92.3%) and 1 case of chemodectoma malignum (7.7%). The surgical treatment was applied in 6 patients (46.1%), 4 patients (30.8%) received complementary radiotherapy and 3 patients (23.1%) were treated only with radiotherapy. CONCLUSIONS: The diagnosis of paragangliomas of the ear is difficult and should be based on clinical data, audiometric tests, CT, MRI and histological examination. Surgical treatment of paragangliomas is limited to early stages of the disease. The results of the research show that the best therapeutic method in advanced cases is surgical treatment with complementary radiotherapy.

Hereditary paraganglioma syndrome associated with SDHD gene mutations: a patient with multicentric presentation treated with radiotherapy. Case report.

INTRODUCTION: Extra-adrenal paragangliomas are rare tumors arising from neuroendocrine cells. Sporadic and hereditary forms have been recognized. Among the latter, the PGL1 and PGL4 syndromes are associated with a high risk of multiple localizations. The treatment of choice for paragangliomas is surgical resection, but in some cases surgery can be difficult due to particular or multiple tumor sites or may result in severe neurological deficits. In such cases radiotherapy can be an effective alternative. In this paper we describe the case of a patient affected by hereditary paraganglioma syndrome with multicentric presentation who was treated at our center by external radiotherapy. CASE REPORT: A 55-year-old man presented in April 2008 with multiple paragangliomas: one in the left pontocerebellar angle, two in the middle neck, one mediastinal paraaortic mass, and an abdominal paraaortic lesion. The left pontocerebellar and mediastinal tumors were treated with three-dimensional conformal radiotherapy (3D-CRT) at total doses of 50.40 Gy and 55.80 Gy, respectively. The neck lesions were treated with intensity-modulated radiotherapy (IMRT) at a total dose of 55.80 Gy. The abdominal paraaortic lesion was surgically resected. RESULTS: No severe acute or late toxicity as evaluated with the EORTC-RTOG scale was observed. Fourteen months after the end of radiotherapy a whole body CT scan showed that the tumor lesions were stable in size and in their relations to contiguous structures. The arterial pressure was controlled by medical therapy and urine catecholamine levels were within the normal range. CONCLUSIONS: We believe that in patients affected by unresectable paragangliomas radiotherapy is a safe and effective alternative to surgery. The use of high-dose conformity techniques such as 3D-CRT and IMRT will allow higher local control rates with relatively few side effects thanks to the possibility of dose escalation and reduction of the amount of irradiated healthy tissues.

Multidisciplinary approach in the treatment of malignant paraganglioma of the glomus vagale: a case report.

Malignant paraganglioma of the glomus vagale is a rare tumor entity originating from paraganglia or glomus cells. It typically affects middle age. It differs from benign paraganglioma because of its rapid growth and more aggressive clinical behavior. We report the case of a 40-year-old man presenting with a 5 cm lesion in the upper right cervical region detected by computed tomography (CT) and magnetic resonance imaging (MRI), which also showed enlargement of ipsilateral spinal and jugulodigastric lymph nodes with contrast enhancement. Clinical manifestations at diagnosis included a partial neurological deficit involving the right cranial nerves X, XI and XII. Tumor vascularization was assessed by digital angiography. The tumor mass was entirely removed by a right cervical approach with en-bloc resection with the regional lymph nodes. Histopathological examination showed a paraganglioma with cellular pleomorphism, necrotic microfoci, perineural infiltration and angiogenesis. Massive metastases in two of three jugulodigastric and one spinal lymph nodes on the right side were also detected. Postoperative workup included MRI, positron emission tomography (PET)/CT, meta-iodine-benzyl-guanidine (MIBG) scan, liver ultrasound and chest radiography. Subsequently, the patient underwent conformal radiotherapy with concomitant cisplatin administration. At the last clinical and radiological follow-up examination 5 years after completion of treatment, the patient was free of tumor recurrence. The integrated treatment by surgery and chemoradiation was feasible and effective in the management of this rare case of malignant paraganglioma of the glomus vagale. Multicenter studies should be done to increase the knowledge of tumor presentation and natural history and to analyze the possible treatment options.

Excellent local control of paraganglioma in the head and neck with fractionated radiotherapy.

AIMS: Radiotherapy is an important treatment option for paraganglioma in the head and neck region. It seems to be highly effective and avoids important surgical morbidity, which can impair quality of life. The aim of this study was to evaluate the outcomes of radiotherapy for paraganglioma of the head and neck region in order to inform our future practice. MATERIALS AND METHODS: The cohort of patients for the present study comprised 21 patients who received radiotherapy between 1998 and 2008. Follow-up ranged from 6 to 132 months, median 55 months. The mean age was 48.7 years, range 20-78 years. The female:male ratio was 2 : 1. Two patients had confirmed familial tumour syndromes. The gross tumour volume in 20 cases ranged from 1.3 to 74 cm(3), mean 23.2 cm(3), median 14.7 cm(3). Five patients were treated with intensity-modulated radiotherapy. The median dose was 50 Gy in 30 fractions. RESULTS: The crude 5-year local control rate was 95% (20/21), although the 5-year actuarial local control rate was 87%. The one patient who relapsed, at 45 months after radiotherapy, had a comparatively small tumour of 10.8 cm(3). A relationship between tumour volume and local control seems unlikely. It was possible to obtain details of side-effects from electronic records for 11 patients. Grade 3 headache, which resolved, was the most serious acute side-effect. One patient had three teeth extracted due to exacerbation of dental caries, and one had deterioration of hearing thought to be due to a combination of tumour and radiotherapy. There were two serious complications in patients who had embolisation, which we no longer use. CONCLUSIONS: Our results show a high level of efficacy for fractionated external beam radiotherapy, with minimal toxicity, in keeping with other series. This should encourage the use of radiotherapy as primary treatment for paragangliomas of the head and neck region.