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.     

Successful Management of a Catecholamine-Secreting Glomus Jugulare Tumor with Radiosurgery Alone

Secretory glomus jugulare tumors are often resected surgically to control the systemic effects of the catecholamines they produce. This involves complex skull base surgery, which carries significant risks and frequent morbidity. Stereotactic radiosurgery (SRS) is a recently recognized treatment for glomus jugulare tumors, though little is known about its use in secretory tumors. Case Report A young fit patient with a catecholamine-secreting glomus jugulare tumor was treated with SRS alone and over the following 37 months her urinary catecholamine excretion fell to near normal levels, and serial magnetic resonance imaging (MRI) confirmed a reduction in tumor volume. Discussion Radiosurgery is an accepted treatment for glomus jugulare tumors and is now readily available to skull base surgeons. In this case a catecholamine-secreting tumor was successfully controlled with radiosurgery alone. Further research and long-term follow-up will determine the role of this treatment in the nonsurgical management of secreting glomus jugulare tumors.     

Lateral Skull Base Surgery: The Otology Group Experience

Lateral skull base surgery has remained the surgical frontier of new developments in the treatment of lesions heretofore difficult to access. Examination of surgical results stimulates technical innovation and provides an intervention risk-benefit ratio assessment for particular lesions useful in management planning. With this in mind, we report the updated collective experience with lateral skull base surgery at the Otology Group over the past 20 years. Two hundred ninety-eight patients underwent surgical intervention for lateral skull base lesions. In 81 patients these lesions were malignant; in 217, benign. Of the benign lesions, 165 were glomus tumors: 139 glomus jugulare, 19 glomus vagale, and 7 glomus tympanicum. The remainder comprised 21 menigniomas, 14 neuromas, two neurofibromas, and a small group of much rarer entities. The philosophy of surgical approach, results, and follow-up are discussed.     

Lateral skull base surgery: the otology group experience

Lateral skull base surgery has remained the surgical frontier of new developments in the treatment of lesions heretofore difficult to access. Examination of surgical results stimulates technical innovation and provides an intervention risk-benefit ratio assessment for particular lesions useful in management planning. With this in mind, we report the updated collective experience with lateral skull base surgery at the Otology Group over the past 20 years. Two hundred ninety-eight patients underwent surgical intervention for lateral skull base lesions. In 81 patients these lesions were malignant; in 217, benign. Of the benign lesions, 165 were glomus tumors: 139 glomus jugulare, 19 glomus vagale, and 7 glomus tympanicum. The remainder comprised 21 menigniomas, 14 neuromas, two neurofibromas, and a small group of much rarer entities. The philosophy of surgical approach, results, and follow-up are discussed.     

Glomus vagale tumor: the significance of early vocal cord paralysis

Glomus body tumors most frequently originate in the middle ear (tympanicum) or on the jugular bulb (jugulare). Tumors that arise from the vagal body account for less than 2.5% of these unique paraganglionic neoplasms. Otologic manifestations of tympanicum and jugulare tumors usually precede or accompany neurologic findings. In reviewing five cases of glomus vagale tumors, the initial symptom of voice change, caused by vocal cord paralysis, preceded the presenting symptoms of hearing loss and tinnitus by an average of 2.5 years. The concept of early cranial nerve involvement by glomus vagale tumors is supported in a literature review. The evaluation of "idiopathic" vocal cord paralysis must include radiographic inspection of the skull base, whereas an accurate, temporal account of presenting symptoms may help distinguish vagal body tumors from other vascular neoplasms of this region.     

Surgical inferences from study of temporal bones with glomus jugulare tumor

Over the past few years, considerable advances have been made in the surgical treatment of glomus jugulare tumors. The long-term results of such surgery for cure of these tumors has yet to be established, however. We have reviewed the temporal bones of two patients who had glomus jugulare tumors. Such study provides a special opportunity to evaluate the major critical areas of involvement of these tumors, which bears on the long-term results of surgery, and is important in developing treatment to cure these benign, though aggressive, tumors.     

Surgery for glomus tumors at the skull base

Glomus tumors of the temporal bone frequently involve the skull base. In the present study, 34% of the tumors occurred with cranial nerve palsies and 15% invaded the CNS. The procedure for surgical resection of glomus tumors of the skull base is described. Results of the procedure included successful surgical resection of 80% of the glomus jugulare tumors and of 94% of the glomus tympanicum tumors. Tumor recurrence and persistence occurred only in the patients with CNS invasion.     

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.     

Radiation or surgery for chemodectoma of the temporal bone: a review of local control and complications

For a presentation to the American College of Surgeons in November 1988, we reviewed the literature concerning primary management of glomus tympanicum and jugulare tumors. From the published series, we examined the local control and treatment-related morbidity for three therapeutic approaches: surgery alone, radiation before or after surgery, and radiation alone. The local control rates were similar, 86%, 90%, and 93%, respectively. Among the surgical series reporting complications, new cranial nerve deficits were common, especially with advanced lesions. Serious sequelae from radiation therapy were rare (2% to 3%). We feel radiation therapy should be considered as primary treatment for glomus jugulare.     

Complex tumors of the glomus jugulare: criteria,treatment, and outcome

OBJECT: Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors--multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome--that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. METHODS: In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. CONCLUSIONS: Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intrabulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.     

Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors.

This study was performed to assess the impact of gamma knife radiosurgery (RS) in the treatment of glomus jugulare tumors. Between February 1993 and February 1999, thirteen patients (9 women, 4 men; mean age 63.5 years, range 29 to 79 years) underwent stereotactic radiosurgery for glomus jugulare tumors with the Leksell Gamma Knife at the Neurosurgical Department of the University of Vienna. Four patients, mean age 74.5 years, range 67 to 79 years, underwent radiosurgery as the only treatment. Nine patients received radiosurgery as adjuvant therapy after previous treatment had failed: surgical resection in 9 patients and additional fractionated external beam radiation in two of these patients. Pretreatment evaluation included the staging of all tumors according to the Fisch Classification: De1 (7), De2 (1), Di1 (4) and Di2 (1). The mean follow-up period was 4.2 years, range 0.7 to 6.7 years. Ten patients, 77 %, were treated prior to 1997, the mean follow-up period being 5 years. Six patients showed no clinical changes, while six experienced an improvement of their clinical symptoms and neurological deficits. One patient was lost to follow-up. Radiation-induced transient cranial nerve neuropathies were observed in two patients. Serial MRI scans revealed tumor control in all patients, with unaltered tumor size in 10 and shrinkage in three patients. The results indicate that RS is an attractive treatment option for glomus jugulare tumors and will occupy an increasingly important role in the management of these tumors in selected patients.     

Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale

PURPOSE: To evaluate the results of treatment for 71 patients with 80 chemodectomas of the temporal bone, carotid body, or glomus vagale who were treated with radiation therapy (RT) alone (72 tumors in 71 patients) or subtotal resection and RT (8 tumors) at the University of Florida between 1968 and 1998. METHODS AND MATERIALS: Sixty-six lesions were previously untreated, whereas 14 had undergone prior treatment (surgery, 11 lesions; RT, 1 lesion; or both, 2 lesions) and were treated for locally recurrent disease. All three patients who received prior RT had been treated at other institutions. Patients had minimum follow-up times as follows: 2 years, 66 patients (93%); 5 years, 53 patients (75%); 10 years, 37 patients (52%); 15 years, 29 patients (41%); 20 years, 18 patients (25%); 25 years, 12 patients (17%); and 30 years, 4 patients (6%). RESULTS: There were five local recurrences at 2.6 years, 4.6 years, 5.3 years, 8.3 years, and 18.8 years, respectively. Four were in glomus jugulare tumors and one was a carotid body tumor. Two of the four patients with glomus jugulare failures were salvaged, one with stereotactic radiosurgery and one with surgery and postoperative RT at another institution. Two of the five recurrences had been treated previously at other institutions with RT and/or surgery. Treatment for a third recurrence was discontinued, against medical advice, before receiving the prescribed dose. There were, therefore, only 2 failures in 65 previously untreated lesions receiving the prescribed course of RT. The overall crude local control rate for all 80 lesions was 94%, with an ultimate local control rate of 96% after salvage treatment. The incidence of treatment-related complications was low. CONCLUSIONS: Irradiation offers a high probability of tumor control with relatively minimal risks for patients with chemodectomas of the temporal bone and neck. There were no severe treatment complications.     

The role of radiation therapy in the management of catecholamine-secreting glomus tumors

The major source of controversy that surrounds the use of radiation for glomus tumors is the finding of persistent chief cells years after completion of the treatment. Questions have been raised as to the viability of the irradiated chief cell and its capacity to proliferate. The radiotherapists consider a stable glomus tumor a radiation "cure," whereas skull base surgeons are fearful that these lesions will continue to slowly grow and cause problems 20 to 30 years later. We have recently managed a patient who was not a candidate for surgery, with a catecholamine-secreting glomus jugulare tumor. After 4750 rad of radiation therapy, no changes in tumor size or in catecholamine secretion have been observed (at 20 months of followup). The implications of the case are discussed.     

Primary fallopian canal glomus tumors

Primary fallopian canal glomus tumor has been reported only once previously, although the occurrence of glomus body tissue in the fallopian canal was documented many years ago. Facial paresis as a presenting symptom of glomus tumors is well known, as is facial nerve invasion by glomus tumors. However, a primary fallopian canal glomus tumor that extends extratemporally to the pes anserinus is unusual. Although facial nerve grafting may be necessary for removal of some glomus jugulare tumors, the need for facial nerve grafting appears to be uniform in the patients with primary fallopian canal glomus tumors. The primary fallopian canal glomus tumors that we report did not involve the jugular fossa or the Jacobson's branch of the glossopharyngeal nerve. Both tumors did extend to the middle ear and mastoid and followed the facial nerve extratemporally. The latter features appear to typify primary fallopian canal glomus tumors.     

Pediatric glomus tumors

Glomus tumors of the middle ear are unusual in adults, but exceedingly rare in children. While a dull, red bulging tympanic membrane in the adult may suggest a glomus tumor, it generally signifies infection in the child. This report details our management of a 10-year-old girl afflicted with bilateral chronic middle ear cleft infection that obscured bilateral glomus tumors. Review of the current English language literature reveals seven additional case reports of otologic glomus tumors in children less than fourteen years of age. Two additional cases are presented that were given to the senior author by personal communication, producing a total of 10 cases for review. Glomus tumors in children may be hidden by otitis media and appear more likely to be endocrine active. Failure to cure the lesion is apparent in five of six case reports of children with glomus jugulare tumors; three of these children are reported to have expired. Complete surgical extirpation is advocated for childhood glomus tumors.     

The long-term side effects of radiation therapy for benign brain tumors in adults

Radiation therapy plays an integral part in managing intracranial tumors. While the risk:benefit ratio is considered acceptable for treating malignant tumors, risks of long-term complications of radiotherapy need thorough assessment in adults treated for benign tumors. Many previously reported delayed complications of radiotherapy can be attributed to inappropriate treatment or to the sensitivity of a developing child's brain to radiation. Medical records, radiological studies, autopsy findings, and follow-up information were reviewed for 58 adult patients (31 men and 27 women) treated between 1958 and 1987 with radiotherapy for benign intracranial tumors. Patient ages at the time of irradiation ranged from 21 to 87 years (mean 47.7 years). The pathology included 46 pituitary adenomas, five meningiomas, four glomus jugulare tumors, two pineal area tumors, and one craniopharyngioma. Average radiation dosage was 4984 cGy (range 3100 to 7012 cGy), given in an average of 27.2 fractions (range 15 to 45 fractions), over a period averaging 46.6 days. The follow-up period ranged from 3 to 31 years (mean 8.1 years). Findings related to tumor recurrence or surgery were excluded. Twenty-two patients had complications considered to be delayed side effects of radiotherapy. Two patients had visual deterioration developing 3 and 6 years after treatment; six had pituitary dysfunction; and 17 had varying degrees of parenchymal changes of the brain, occurring mostly in the temporal lobes and relating to the frequent presentation of pituitary tumors (two of these also had pituitary dysfunction). One clival tumor with the radiographic appearance of a meningioma, developed 30 years post-irradiation for acromegaly. This study unveils considerable delayed sequelae of radiotherapy in a series of adult patients receiving what is considered "safe" treatment for benign brain tumors.     

Gamma Knife Radiosurgery for Glomus Jugulare Tumours

Summary  The aim of this clinical study was to determine the tumour control rate, clinical outcome and complication rate following gamma knife treatment for glomus jugulare tumours. Between May 1992 and May 1998, 13 patients with glomus tumours underwent stereotactic radiosurgical treatment in our department. The age of these patients ranged from 21 to 80 years. The male : female ratio was 2 : 11. Six patients had primary open surgery for partial removal or recurrent growth and subsequent radiosurgical therapy. Radiosurgery was performed as primary treatment in 7 cases. The median tumour volume was 6,4 cm³ (range: 4,6–13,7 cm³). The median marginal dose applied to an average isodose volume of 50% (30–50%) was 13,5 Gy (12–20 Gy). In 10 patients, a total of 48 MRI and CT follow-up scans were available. The remaining three patients have been excluded from the postradiosurgical evaluation since the observation time (t<12 months) was too short or patients were lost to follow up. The median interval from Gamma Knife treatment to the last radiological follow-up was 37,6 months (5–68 months). In 4 patients (40%) decreased tumour volumes were observed and in 6 cases (60%) the tumour size remained unchanged. Neurological follow-up examinations revealed improved clinical status in 5 patients (50%), a stable neurological status in 5 patients (50%) and no complications occurred. According to our preliminary experience Gamma Knife radiosurgery represents an effective treatment option for glomus jugulare tumours.     

Management of Metastatic Glomus Jugulare Tumors

Metastatic glomus jugulare tumors (jugular paragangliomas) are very uncommon: consequently, no single report has been able to accurately describe their biologic or clinical behavior, and there are no established guidelines on caring for patients with these tumors. Identification of metastatic paraganglioma can be difficult because these tumors are known to occur synchronously, many other tumor types appear histologically similar to paragangliomas, and histologically the metastasis may not resemble the primary tumor. Immunohistochemistry, using neuroendocrine markers, and electron microscopy are the two most useful techniques available to identify definitely paragangliomas. A few studies have shown differences in the immunohistochemical staining patterns between benign and metastatic paragangliomas. We reviewed the literature and include two additional cases of metastatic glomus jugulare tumors in this article. We discuss the clinical management of these patients, the importance and usefulness of immunohistochemistry in characterizing these tumors, and their clinical outcomes.     

Tumors of the glomus caroticum

From 1952 to 1982 15 patients (6 male, 9 female) with 20 carotid body tumors have been treated in the surgical departments of the universities of Berlin and Heidelberg. A recurrent tumor occurred in 3 patients, in one case appeared a further recurrence. Two patients presented with a tumor of the glomus jugulare as well as the tumors of the carotid body and with a familial accumulation. While still in the 60's the surgical procedure was in doubt due to high complications and lethality rates today, because of the progress in carotid surgery with the insertion of an intraluminal shunt, the operation is recommended without risk. An important part plays a certain preoperative diagnosis through the B-mode sonography for the operative strategy.     

Glomus jugulare tumors. Possibilities of radiotherapy

From 1964 to 1981, twelve patients with glomus jugulare tumors involving bony lesions of base of skull were treated by high energy radiation. Ten patients presented cranial nerve pairs paralysis. Conventionally fractionated doses of 45 to 60 Gy were applied, and 11 of the 12 patients were alive and clinically stable after follow up for between 3 and 20 years. One patient died of an intercurrent infection after 16 years. Strict irradiation technique is essential to avoid principally neurologic complications. Favorable results after radiotherapy were obtained in extensive tympano-jugular forms.