Temporal Bone Paragangliomas Research Articles

Utility of an Ultrasonic Aspirator in Transcanal Endoscopic Resection of Temporal Bone Paraganglioma

The objective of this study was to evaluate the role of removing middle ear paragangliomas using an ultrasonic aspirator (UA) through a transcanal, exclusively endoscopic approach. Three consecutive patients undergoing transcanal endoscopic resection of middle ear paragangliomas using an UA were retrospectively studied. The primary outcome measure was achieving gross total tumor resection. Secondary outcomes included postoperative hearing and early convalescence. Two glomus tympanicum tumors and one small glomus jugulare were included, and complete tumor resection was achieved endoscopically in all cases. All patients demonstrated intact tympanic membranes and normal facial nerve function at the initial postoperative visit. There were no cases of bone-conduction pure tone threshold increases of more than 15 dB. The UA was helpful when performing exclusively endoscopic transcanal resection of middle ear paraganglioma given its simultaneous suction-aspiration capability.

AN AGGRESSIVE TEMPORAL BONE SDHC PARAGANGLIOMA ASSOCIATED WITH INCREASED HIF-2α SIGNALING

To describe a patient with a germline succinate dehydrogenase (SDHC) gene mutation presenting with primary hyperparathyroidism and a large catecholamine-producing temporal bone paraganglioma (PGL). Evaluation of a SDHC mutation-positive PGL tumor biology using staining for tyrosine hydroxylase (TH), hypoxia-inducible factors 1α (HIF-1α) and 2α (HIF-2α). A 66-year-old man was noted to have a lytic skull base mass during work-up for his primary hyperparathyroidism. Biochemical evaluation with 24-hour urine catecholamines and metanephrines revealed marked elevation of norepinephrine and normetanephrine. Genetic testing revealed a germline SDHC mutation. A partial excision of skull base tumor was performed, which upon further examination revealed PGL. Immunohistochemistry of skull base PGL demonstrated heavy expression of TH and HIF-2α but reduced expression of HIF-1α. The remaining skull base PGL was treated with adjuvant radiation therapy. The patient's normetanephrine levels significantly decreased after surgery and radiation. Here, we report an unusual case of a patient presenting with a germline SDHC mutation-related functional PGL along with concomitant primary hyperparathyroidism. The present case illustrates that overexpression of HIF-2α but not of HIF-1α is linked to the pathogenesis of SDHC mutation-related PGL, and it may be responsible for the aggressive clinical behavior of a usually indolent course of SDHC-related PGLs.

Canal wall reconstruction and conductive hearing preservation for temporal bone paraganglioma.

Canal wall reconstruction and conductive hearing preservation for temporal bone paraganglioma.

The role of wait-and-scan and the efficacy of radiotherapy in the treatment of temporal bone paragangliomas.

To define the role of wait-and-scan and radiotherapy (RT) in the treatment of temporal bone paragangliomas and to review the literature on the outcomes of RT as a treatment modality. This was a retrospective and literature review. The records of 381 patients with 382 tumors between 1988 and 2012 were analyzed. Patients who were subjected to initial wait-and-scan or primary RT were analyzed for tumor response, facial, and lower cranial nerve status on follow-up (FU). None of the 145 patients (38%) with Class A and B tumors were subjected to wait-and-scan or RT and all were operated on. Of the 237 patients (62%) with Class C and D tumors, 47 (19.8%) were subjected to an initial wait-and-scan policy and 1 (0.4%) underwent primary RT. In 24 patients subjected to wait-and-scan with a FU of less than 3 years, tumor remained stable in 22 patients (92%). In 12 patients with a FU between 3 and 5 years, the tumor remained stable or regressed in 10 patients (83%). In 11 patients with a FU of more than 5 years, the tumor remained stable or regressed in 5 patients (45%). A literature review of the results of RT did not conclusively prove that it was effective as a primary modality of treatment for temporal bone paragangliomas. In elderly patients with advanced tumors, a wait-and-scan approach is recommended, and RT is initiated only when fast-growing tumors are detected. There is insufficient evidence in literature to suggest that RT could be an effective alternative to surgery in Class C and D tumors.

Treatment of Head and Neck Paragangliomas With External Beam Radiation Therapy

To retrospectively assess the outcomes of radiation therapy in patients with head and neck paragangliomas. 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). 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. 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.

Wyniki leczenia przyzwojaków kości skroniowej

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. 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. 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. 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.

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.

Long-Term Results of Surgery for Temporal Bone Paragangliomas

Objectives: Assessment of the long-term results of surgery for temporal bone paragangliomas with special consideration of the patient's ability to cope with the functional deficits. Study Design: Retrospective review of 36 patients who had undergone resection of a temporal bone paraganglioma 10 to 15 years previously. Assessment of the patients' subjective view of the functional outcome and quality of life by a questionnaire. Methods: Clinical records were reviewed regarding size of tumor, technique of surgery, supportive therapy, and tumor recurrence. Patients were sent a 50-item questionnaire evaluating their quality of life and the preoperative and postoperative function of cranial nerves VII through XII. Results: A complete tumor removal was achieved in 30 patients (83%). There was only one tumor recurrence. The major negative effects of surgery involved hearing and dysphagia, which deterioriated in 14 and 12 patients, respectively. Thirty-five of the 36 patients (97%) reported that, despite deterioration, the cranial nerve deficits were still acceptable. Seventy-five percent of the patients regained their preoperative quality of life and 97% returned to their previous occupation in 1 to 2 years. Conclusions: The otologic extradural approach allowed complete tumor removal in 83%, with minimal perioperative morbidity. No surgically induced central nervous system lesions occured. Tracheostomy was avoided and all patients resumed oral feeding. Full rehabilitation after removal of class C and CD paragangliomas may take 1 to 2 years. However, the fact that 97% of the patients finally resumed normal social life showed the ability of most patients to cope with the sequelae of surgery even in class C and CD paragangliomas.

An Update on the Surgical Treatment of Temporal Bone Paraganglioma

In 1982, Fisch described his results for the surgical treatment of 74 paragangliomas of the temporal bone, 5 years after his description of the infratemporal fossa approaches (types A and B). This study reviews the subsequent experience of the Department of Otolaryngology—Head and Neck Surgery of the University of Zürich with more than 136 surgically treated cases of paraganglioma of the temporal bone and discusses our current therapy 20 years after the initial description. One hundred nineteen (90%) of the patients had advanced tumors (Fisch class C or C+D), and 81 (68%) had intracranial extension. Total tumor excision was possible in 109 (82%) patients. Subtotal excision was performed in 22 (17%) patients, 21 of whom had intradural tumor invasion. In these cases, the resection was limited not by actual tumor size but by the degree of intracranial intradural tumor extension. Partial tumor excision was undertaken in only 1 patient with a C4De2Di2 tumor. The success rate in preservation of function of the lower cranial nerves was encouraging. Of the 69 patients whose facial nerve status was followed postoperatively, 81% maintained Fisch grade 76 to 100% (House-Brackman grades I and II). Analysis of follow-up data ranging from 2 to 11 years demonstrated 98% disease-free survival when total tumor extirpation was possible. In the patients who underwent subtotal or partial surgical resection there has been no subsequent tumor growth detected by either clinical or neuroradiological evaluation. We have confirmed after more than 20 years of experience that the infratemporal fossa approaches are a safe, highly effective means of surgical management of paragangliomas of the temporal bone, allowing eradication or arrest of disease with minimal morbidity. Limited intradural surgical resection in cases of very extensive tumors can greatly benefit patients for whom complete excision is not an option.

Long-term results of surgery for temporal bone paragangliomas.

Assessment of the long-term results of surgery for temporal bone paragangliomas with special consideration of the patient's ability to cope with the functional deficits. Retrospective review of 36 patients who had undergone resection of a temporal bone paraganglioma 10 to 15 years previously. Assessment of the patients' subjective view of the functional outcome and quality of life by a questionnaire. Clinical records were reviewed regarding size of tumor, technique of surgery, supportive therapy, and tumor recurrence. Patients were sent a 50-item questionnaire evaluating their quality of life and the preoperative and postoperative function of cranial nerves VII through XII. A complete tumor removal was achieved in 30 patients (83%). There was only one tumor recurrence. The major negative effects of surgery involved hearing and dysphagia, which deterioriated in 14 and 12 patients, respectively. Thirty-five of the 36 patients (97%) reported that, despite deterioration, the cranial nerve deficits were still acceptable. Seventy-five percent of the patients regained their preoperative quality of life and 97% returned to their previous occupation in 1 to 2 years. The otologic extradural approach allowed complete tumor removal in 83%, with minimal perioperative morbidity. No surgically induced central nervous system lesions occured. Tracheostomy was avoided and all patients resumed oral feeding. Full rehabilitation after removal of class C and CD paragangliomas may take 1 to 2 years. However, the fact that 97% of the patients finally resumed normal social life showed the ability of most patients to cope with the sequelae of surgery even in class C and CD paragangliomas.

An Update on the Surgical Treatment of Temporal Bone Paraganglioma

In 1982, Fisch described his results for the surgical treatment of 74 paragangliomas of the temporal bone, 5 years after his description of the infratemporal fossa approaches (types A and B). This study reviews the subsequent experience of the Department of Otolaryngology-Head and Neck Surgery of the University of Zürich with more than 136 surgically treated cases of paraganglioma of the temporal bone and discusses our current therapy 20 years after the initial description. One hundred nineteen (90%) of the patients had advanced tumors (Fisch class C or C+D), and 81 (68%) had intracranial extension. Total tumor excision was possible in 109 (82%) patients. Subtotal excision was performed in 22 (17%) patients, 21 of whom had intradural tumor invasion. In these cases, the resection was limited not by actual tumor size but by the degree of intracranial intradural tumor extension. Partial tumor excision was undertaken in only 1 patient with a C4De2Di2 tumor. The success rate in preservation of function of the lower cranial nerves was encouraging. Of the 69 patients whose facial nerve status was followed post-operatively, 81% maintained Fisch grade 76 to 100% (House-Brackman grades I and II). Analysis of follow-up data ranging from 2 to 11 years demonstrated 98% disease-free survival when total tumor extirpation was possible. In the patients who underwent subtotal or partial surgical resection there has been no subsequent tumor growth detected by either clinical or neuroradiological evaluation. We have confirmed after more than 20 years of experience that the infratemporal fossa approaches are a safe, highly effective means of surgical management of paragangliomas of the temporal bone, allowing eradication or arrest of disease with minimal morbidity. Limited intradural surgical resection in cases of very extensive tumors can greatly benefit patients for whom complete excision is not an option.

Prognostic Factors Including Proliferation Markers Ki-67, bax, and bcl-2 in Temporal Bone Paraganglioma

Valuable criteria with which to predict the clinical behavior of the temporal bone paraganglioma or the response to treatment are lacking. The analysis of markers of cell proliferation is a possibility to estimate the prognosis. Extensive patient data on 40 temporal bone paragangliomas were gathered over the years and correlated with the data obtained by staining histologic sections with bcl-2, bax, and MIB I markers of cellular proliferation. The immunohistochemistry was in all cases negative for bcl-2, positive for bax, and for Ki-67 positive in 20% of tumors. The scores for Ki-67 did not correlate with the majority of clinical parameters, except for treatment modality, preoperative hearing loss, and cranial nerve involvement. The tendency toward poorer hearing and a higher incidence of preoperative lower cranial nerve palsies was demonstrated in patients with higher Ki-67 scores. Furthermore, the higher rate of subtotal tumor removals in these patients reveals technical difficulties in accomplishing a radical removal, although the incidence of residual tumors was thus not affected. In view of the present information obtained with proliferation markers, the site of tumor origin still remains the most predictive variable for the course of the disease.

Long-Term Results of Surgery for Temporal Bone Paraganglioma

The only way to resolve the dispute about the effectiveness of surgery versus radiation therapy for glomus tympanicum and jugulare tumors is adequate long-term studies. In a retrospective study with an average follow-up period of 15 years (range 11 to 23 years) we reassessed 11 patients with glomus tympanicum tumors and 11 patients with glomus jugulare tumors. Ten of 11 patients with glomus tympanicum tumor were tumor-free after surgery. A temporary facial palsy and an external meatal wall defect were the only surgical complications. The air-bone gap postoperatively closed to within 10 dB in three patients, to within 20 dB in six patients, and to more than 30 dB in one patient. Nine of 10 patients with glomus jugulare tumor receiving complete resection were tumor-free. Less than half the patients experienced new-onset cranial nerve function loss, and all made satisfactory recovery, eliminating the need for tracheostomy or gastrostomy. In two patients, the hearing could be preserved on the preoperative level, but the majority already presented with deafness. In the long-term, surgery remains a treatment of choice for glomus tympanicum tumors. It is also an extremely effective treatment with low morbidity for glomus jugulare tumors, including those with intracranial extension.

Catecholamine-Secreting Paragangliomas at the Skull Base

Paragangliomas (glomus tumors) comprise 15% of all neoplasms at the skull base. Despite extensive growth, these tumors usually do not secrete active biogenic substances into the circulation in sufficient quantities to produce symptoms. When they do secrete large amounts of catecholamines, they will cause symptoms that mimic a pheochromocytoma. The still confusing nomenclature of paragangliomas is reviewed, and the clinical work-up, surgical treatment, and follow-up of five patients with catecholamine-secreting paragangliomas of temporal bone (3), infratemporal fossa (1), and nasopharynx (1) are presented and discussed.

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.