Treatment of paragangliomas with radiation therapy

Paragangliomas of the head and neck may be treated successfully with surgery, radiation therapy, or stereotactic radiosurgery. The choice of treatment depends on the location and extent of the tumor, the presence of multiple tumors, the age and health of the patient, and the preference of the patient and attending physician. This article reviews the role of radiation therapy in the treatment of patients with paragangliomas of the head and neck.     

Diagnosis and management of cervical paragangliomas: the Freiburg experience

BACKGROUND: Paragangliomas of the head and neck represent rare tumors of neural crest origin that arise from chemoreceptors. They are highly vascular neoplasms that are benign in the majority of cases. There are controversial discussions concerning the different treatment options for cervical paragangliomas. PATIENTS: Between January 1992 and November 2003 a total of 13 patients with 15 cervical paragangliomas were treated at the Department of Otorhinolaryngology - Head and Neck Surgery, University of Freiburg. Our study includes eight female and five male patients with a mean age of 39.9 years (range: 20 - 79 years). There were 14 carotid body tumors and one vagal paraganglioma. RESULTS: 14 tumors were resected and one underwent primary radiation. In four patients we noted a total of 8 permanent, postoperative nerve deficits. During follow-up no recurrent tumors were seen. CONCLUSION: Complete surgical removal represents the therapy of choice for the majority of cervical paragangliomas. In cases of multiple head and neck paragangliomas or in patients with underlying diseases, primary radiation should be discussed as a treatment option.     

Evaluation of paragangliomas presenting as a cervical mass on color-coded Doppler sonography.

OBJECTIVES: To assess the potential of color Doppler sonography to identify cervical mass lesions as paragangliomas. STUDY DESIGN: Prospective evaluation. METHODS: Fifteen patients with 18 paragangliomas (13 carotid body tumors, 3 vagal and 2 temporal) presenting as a mass in the neck were investigated using color Doppler sonography. RESULTS: All paragangliomas presented in B-mode sonography as solid, well-defined, and hypoechoic tumors. Color Doppler imaging revealed hypervascularity in 15 (82%) tumors. No flow signal was detected in 3 carotid body tumors at standard velocity encoding (30 cm/s). Only carotid body tumors could be assessed in their full extent. Temporal and vagal paragangliomas were only partially visible. Carotid body tumors showed a splaying of the carotid bifurcation with displacement of the external carotid anteriorly and both the internal carotid and the internal jugular vein posteriorly. Anterior displacement of both carotid arteries and posterior displacement of the internal jugular vein was found in the 3 vagal paragangliomas. The caudal tumor extension of the 2 temporal paragangliomas was recognized within the expanded lumen of the internal jugular vein. According to the direction of tumor growth and vascular supply, the intratumoral flow signal was predominantly directed upward in carotid body tumors and downward in vagal and temporal paragangliomas. CONCLUSIONS: Based on the appearance in the B-mode, the hypervascularity, the relationship toward the carotid arteries and the internal jugular vein, and the intratumoral flow direction, color Doppler sonography was able to establish the diagnosis and type of a paraganglioma.     

Treatment decision and estimation of growth of head and neck paragangliomas

PurposeHead and neck paragangliomas are slow growing tumors where observation has become more widely accepted. Tumor growth rate as well as predictors of increased tumor growth were analyzed with the goal to identify factors to better predict disease progression and counsel patients.Materials and methodsMulti-institutional retrospective cohort study from 2011 to 2020.Results130 head and neck paragangliomas in 125 patients were analyzed. 38 were observed (30.4%), 16 radiated (12.8%), and 71 underwent surgery (56.8%). Surgical patients were significantly younger (p = 0.038) and with more genetically mediated paragangliomas (p = 0.026). Significantly more patients were asymptomatic in the observation group (p = 0.005). Of the 39 observed tumors, 43.6% (n = 17) grew with a tumor doubling time of 5.67 years. More than half of the observed paragangliomas had no growth. When examining symptoms postoperatively and at follow-up, the surgical cohort had significantly more worsening symptoms (p = 0.007) and new cranial neuropathies (p = 0.031).ConclusionsHead and neck paragangliomas have slow growth rates if they grow at all. Patients in the surgical cohort had more clinical symptoms at presentation and worsening postoperative symptoms.     

Temporal paragangliomas

Temporal paragangliomas (PGL) are usually limited to the paraganglionar system with a sporadic or familial origin. Familial PGL have recently been shown to be associated with germline alterations in SDH group of genes, and occasionally are associated with a variety of genetic multisystemic disorders (von Hippel–Lindau disease, multiple endocrine neoplasia type 2 and neurofibromatosis type 1). Temporal bone PGL are normally located in the region of the jugular foramen and on the promontory along the Jacobson nerve. Occasionally, vagal PGL may reach the jugular foramen and behave as jugular PGL. Treatment of temporal PGL must be based on the biological behavior of the tumour, age and medical condition of the patient, location and size of the PGL, and potential for treatment induced morbidity. The main treatment modalities for PGL are surgery and radiation therapy. Patients with large temporal PGL whose resection would result in potentially disabling morbidity are often selected for radiation therapy or wait and scan policy. Small tympanic PGL where resection may be carried out with a low morbidity risk can be removed through an endomeatal tympanotomy. Jugular PGL limited to the infralabyrinthine region involving only the vertical segment of the ICA, can benefit of an extended facial recess approach, which allows a postoperative normal hearing and facial nerve function. For more extensive disease in the middle ear or around the ICA, external auditory canal preservation is not possible and some kind of facial nerve mobilization is required. Preservation of lower cranial nerves is facilitated by intrabulbar dissection, previous extradural ligation of the sigmoid sinus. Management of large intracranial involvement is controversial, although most authors advocate resection of the tumour in a single stage. Surgical control of the tumour can be expected in 70–85% of the patients and is clearly dependent on the tumour stage. Tumour size determines success in hearing and lower cranial nerves preservation. Presented at the 77th annual meeting of the German Society for Oto-Rhino-Laryngology, Head and Neck Surgery, 24–28 May 2006, Mannheim, Germany.     

Radiotherapy of paragangliomas

From 1964 to 1980 28 patients with extensive paragangliomas were treated with radiation therapy. Nine patients had a carotid body tumour and 19 had a temporal bone tumour. During the 16 year period the radiation dose was gradually reduced from 60 Gy in 6 weeks to 40 Gy in 4 weeks. Continuous tumour control was achieved in all patients (follow-up 1.5-18 years) with no difference between glomus caroticum tumours and glomus tympanicum tumours, and complications were few, certainly when 40-50 Gy was given in 4-5 weeks.     

Vagal paragangliomas

The management of vagal paragangliomas is extremely challenging. Treatment of these lesions must be tailored individually for each patient. The best treatment modality depends on the patient's age and health and the size and extent of the tumor. This article discusses clinical presentation, multicentric and malignant vagal paragangliomas, evaluation, embolization, surgical management, and special considerations in the management of vagal paragangliomas.     

Malignant head and neck paragangliomas: Treatment efficacy and prognostic indicators

Purpose

Malignant head and neck paragangliomas (MHNPs) are rare and occur in 6%–19% of all HNPs. We sought to identify predictors of survival and compare efficacy of treatment modalities to inform management of this rare disease.

Materials and methods

We performed a retrospective cohort study of MHNP cases in the National Cancer Institute Surveillance Epidemiology and End Results database (SEER) from 1973 to 2009. We identified 86 patients with MHNP who had documented regional or distant tumor spread with a median follow-up of 74 months. We used Cox proportional hazard models to assess the significance of demographic factors and treatment on five-year overall survival.

Results

The most common treatment was surgery alone (36.0 %), followed by surgery with adjuvant radiation (33.7%). Five-year overall survival was 88.1% for surgery alone and 66.5% for adjuvant radiation (p = 0.2251). In univariate analysis, regional (vs. distant) spread (HR 0.23, p < 0.0001), surgery alone (HR 0.29, p < 0.0001) and primary site in the carotid body (HR 0.32, p = 0.006) conferred significant survival advantage whereas age > 50 (HR 4.04, p < 0.0001) worsened survival. Regional (vs. distant) spread (HR 0.42, p = 0.046) and age > 50 (HR 2.98, p = 0.005) remained significant in multivariate analysis. In patients with regional-only disease, five-year overall survival was 95.4% for surgery alone compared to 75.6% for surgery with radiation (p = 0.1055).

Conclusions

This is the largest and most contemporary series of MHNP patients. Age and tumor stage are significant factors in predicting survival. Surgical resection significantly improves survival outcomes. From this analysis, the value of adjuvant radiation is not clear.     

Jugulotympanic paragangliomas.

Jugulotympanic paragangliomas are the most common tumors of the middle ear and temporal bone. Although these larger tumors can prove to be formidable, the advent of microscopic and skull base surgical techniques has greatly enhanced the ability to treat and manage these tumors.     

Immunohistochemical analysis of paragangliomas

Objective. To find a biological marker that can give information about the clinical behaviour of head and neck paragangliomas. Materials and methods. Immunohistochemical analysis was performed on 114 paragangliomas of which 68 were hereditary. A broad panel comprising eight chief cell (type 1) markers (PGP 9.5, NSE, Leu-Enkephalin, Met-Enkephalin, Chromogranin, Serotonin, Somatostatin, HNK-Leu 7) and two sustentacular cell (type 2) markers (S 100, GFAP) were applied to paraffin embedded slides. Results. It was possible to distinguish between chief andsustentacular cells on the basis of marker specificity but no significant correlation between staining pattern and clinical criteria indicative of tumour progression, DNA-ploidy or tumour localization was found. Conclusion. Clinically immunohistochemical reactivity ofparagangliomas cannot serve as a marker for their growth pattern, furthermore one could hypothesize for the used panel of markers that the underlying cause of their clinically different behaviour is not reflected by diminished differentiation as might be reflected by decreased production of tumour associated antigens or hormones.     

Diagnostic evaluation of temporal paragangliomas

Paragangliomas are rare tumours deriving from paraganglia--small bodies of neuroendocrine tissue. Approximately 3% of paragangliomas occur in the head and neck area accounting for about 0,6% of all tumours of this region. Head and neck paragangliomas are divided into two groups: neck tumours (carotid body tumor and vagal paraganglioma) and temporal paragangliomas (jugular bulb tumor and tympanic paraganglioma). Temporal paragangliomas arise from paraganglia located in the petrous part of the temporal bone. Radiological evaluation of these lesions is essential for confirming the diagnosis and surgery planning. Most useful methods are magnetic resonance imaging and computed tomography. These modalities deliver important information about tumour location, extension and the state of the surrounding structures. Carotid angiography is a second step diagnostic method, performed in patients with jugular bulb paraganglioma. It has a diagnostic value and allows palliative or preoperative embolization of the tumor. Ultrasonographic evaluation and scintigraphy also provide advantages in diagnostic management.     

Laryngeal and sinonasal paragangliomas

Laryngeal paragangliomas are classified as supraglottic and infraglottic. This article defines each type of paraganglioma, discusses the clinical features and diagnoses, and covers the surgical management. This article also addresses sinonasal paragangliomas, including their clinical features, diagnosis, and treatment.