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.     

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.     

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.     

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.     

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.     

Multicentric chemodectoma in the head and neck

A case of a multicentric chemodectoma, with the unusual combination of glomus vagale and glomus tympanicum, is presented. The patient, though asymptomatic, showed elevated levels of urinary catecholamines, suggesting some biochemical activity of the tumour. Multicentricity was unsuspected prior to selective carotid arteriography. Intra-operative haemostasis was assisted by the utilization of pre-operative selective embolization of the tumour mass. Subsequently, the injected Ivalon was confirmed histologically in the specimen. The asymptomatic small glomus tympanicum tumour was treated using embolization alone but only time will determine the effectiveness of this method of management. The vagus nerve was anatomically spared, but complete function had not returned after 2 years of follow-up.     

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.     

Functional tumors of the organ of Zuckerkandl.

Chromaffin-reacting pheochromocytomas of the adrenal medulla are the most frequently encountered functional paraganglionic neoplasms. However, extra-adrenal pheochromocytomas as well as non-chromaffin paragangliomas, including those of the carotid body and glomus jugulare, may produce symptoms from catecholamine secretion. One of the extra-adrenal sites from which these tumors arise is from a collection of para-aortic, paraganglion cells around the origin of the inferior mesenteric artery. This collection of paraganglia was described in fetuses by Zuckerkandl in 1901 and has subsequently been referred to as the organ of Zuckerkandl. The diagnosis and management of these neoplasms differ somewhat from that of adrenal pheochromocytomas, but excellent results are often obtained by excision of these lesions. Four patients with functional tumors of the organ of Zuckerkandl are reviewed together with the other reported cases in the literature.     

Surgical therapy of glomus vagale tumors

Lying between the carotid bifurcation and the jugular foramen, glomus vagale tumors share characteristics with paragangliomas of those two structures, such as invasion of the carotid artery, destruction of the skull base, and cranial neuropathies. This capability for local invasion provides a therapeutic challenge with regard to the proper assessment of tumor extent and the selection of appropriate treatment. In order to clarify an approach to the management of glomus vagale tumors, we reviewed a 10-year experience with 15 patients treated for this tumor at the University of Zürich Department of Otolaryngology, using a new system of classification. This system highlights the relative position of a vagal paraganglioma to the jugular foramen and is helpful in designing the proper therapy. Pitfalis in surgical technique, recommended preoperative evaluation, and the roles of balloon occlusion and irradiation in the treatment of these tumors, are discussed.     

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.     

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.     

Improvement of vocal cord paresis after thyroidectomy

Iatrogenic vocal cord paralysis is a well-publicized complication of thyroid and parathyroid operations. Less appreciated is the improvement of vocal cord function after resection of a thyroid or parathyroid tumor. Over the last 22 years, 14 patients presented with vocal cord paresis in the presence of thyroid or parathyroid tumors. Of these 14 patients, nine had complete resolution of paresis following resection of the thyroid or parathyroid tumors: three had a thyroid carcinoma impinging upon the nerve, three had large colloid goiters, two had a follicular adenoma and one had a parathyroid adenoma displacing the nerve. In five of the 14 patients the vocal cord paralysis persisted after operation. In three, the pathology accounted for the vocal cord paralysis and was not amenable to operative improvement: one patient had an unresectable anaplastic thyroid carcinoma, one patient had long-standing idiopathic unilateral vocal cord paralysis, and one patient had laryngeal adenoid cystic carcinoma with thyroid invasion. The fourth patient had an extensive thyroid hemangioma. The paralysis persisted after resection. The fifth patient had long-standing idiopathic vocal cord palsy. A preoperative vocal cord paresis in a patient with thyroid or parathyroid disease does not indicate permanent loss of recurrent nerve function, even in the presence of carcinoma. In this series, vocal cord function was restored in 9 of 10 patients with resectable thyroid or parathyroid tumors.     

Contiguous bilateral head and neck paragangliomas in a carrier of the SDHB germline mutation

Paragangliomas are extremely rare neoplasms with multicentric presentation usually linked to familial tumor syndromes. This patient presented with the uncommon combination of concurrent bilateral carotid body tumors and a unilateral glomus jugulare mass that demonstrated vascular continuity. During treatment, the patient was found to be heterozygous for the SDHB germline mutation, an autosomal dominant genotype of the familial paraganglioma syndromes associated with increased malignancy. The unique profile of the SDHB patient as regards primary evaluation, surgical considerations, and extended surveillance was explored and has led to a proposed treatment algorithm for these patients.     

Vocal cord paralysis and reoperative parathyroidectomy. A prospective study.

One hundred sixty-three patients undergoing reoperative parathyroidectomy were evaluated before and after operation to determine the incidence of, risk factors for, and morbidity of vocal cord paralysis. These patients were compared to 77 patients undergoing initial parathyroid operation, only one of whom had vocal cord paralysis on postoperative indirect laryngoscopy (1.3%). Preoperative examination of the reoperative patients revealed vocal cord paralysis from initial exploration in 11 patients who were excluded from this study. After re-exploration, 10 patients (6.6%) had vocal cord paralysis, eight unilateral and two bilateral. Right vocal cords were paralyzed twice as often as left. In 90%, vocal cord paralysis was associated with removal or biopsy of an ipsilateral gland. Vocal cord paralysis occurred despite intraoperative visualization of the recurrent laryngeal nerves. Preoperative localization, parathyroid gland pathology, and concomitant thyroidectomy were not associated with increased risk of vocal cord paralysis. Hoarseness was the major symptom. Tracheostomy was required for two patients, one was permanent. One patient was treated for aspiration with a temporary gastrostomy. Nine of 10 patients had return of normal voice quality in an average of 4 months time. On examination 4 years or more after surgery, two of five patients had normal vocal cord motion. The oblique anatomic course of the right recurrent laryngeal nerve may account for the greater frequency of right vocal cord paralysis.     

Meningiomas of the jugular foramen.

Meningiomas of the jugular foramen manifest the same signs and symptoms as glomus jugulare tumors. They arise from arachnoid cells lining the jugular bulb and grow slowly, infiltrating the temporal bone and posterior fossa. These lesions, however, are more clinically treacherous than glomus tumors. Meningiomas infiltrate surrounding bone and nerve tissue and require wide margins of resection to prevent recurrence. Eight of these lesions have been managed in the past 5 years at the House Ear Clinic using modern imaging and skull base techniques. Two have recurred after "total" microsurgical removal. Presentation, radiologic evaluation, and management guidelines are reviewed.     

Preoperative Ultrasonography Assessment of Vocal Cord Movement During Thyroid and Parathyroid Surgery

Background

Preoperative detection of vocal cord palsy is important in thyroid and parathyroid surgery. However, routine fiberoptic laryngoscopy may bring patients unnecessary discomfort. The aim of this study was to determine the feasibility of using surgeon-performed ultrasonography (US) as a screening tool for preoperative assessment of vocal cord movement.

Methods

In the first phase, patients had both laryngoscopic and US examination before surgery. In the second phase, patients had US evaluation first. Those with abnormal vocal cord movement on US, with invisible cord movement, or presenting with significant vocal symptoms underwent laryngeal examination.

Results

In all, 93 (82?%) of 114 patients had successful US evaluation of vocal cord movement during the first phase. Two of them had vocal cord paralysis. In the second phase, vocal cord movement could be evaluated by US in 349 (84?%) of 415 patients. Four patients with abnormal movement were confirmed to have vocal cord palsy by laryngoscopy. None of 46 symptomatic patients with normal movement on US had vocal cord palsy. One other patient whose cord movement could not be seen by US had vocal cord palsy on laryngoscopic examination.

Conclusions

Surgeon-performed US appears to be a relatively accurate method for assessing vocal cord movement in the preoperative setting. It can be used to select patients to undergo laryngoscopic examination before thyroidectomy and parathyroidectomy.     

Radiotherapy for chemodectoma of the carotid body and ganglion nodosum.

Chemodectomas of the carotid body and of the glomus vagale are uncommon tumors of the head and neck. Between 1981 and 1986, 6 patients with advanced chemodectomas of either the carotid body (5 patients) or the glomus vagale (1 patient) were treated with radiotherapy at the University of Florida. Tumors were bilateral in 2 patients. Five patients have had no evidence of disease progression for 5, 5.5, 7, 7, and 8.5 years following irradiation. One of the 5 patients experienced complete regression, 2 noted partial regression, and 2 remained stable (without progression). One patient, who had received 2 prior operations and a course of irradiation at another institution, died of locally recurrent disease 5 years after re-irradiation at the University of Florida. No patient experienced a significant complication secondary to irradiation.     

Therapeutic management of postoperatively diagnosed bilateral recurrent laryngeal nerve paralysis

Bilateral vocal cord paralysis is a rare but potentially dangerous postoperative complication in thyroid gland surgery. There is a controversial discussion about therapeutic management of postoperative bilateral vocal cord paralysis. METHODS: We analysed the frequency of bilateral nerve palsy in 985 operations. The disease of thyroid gland, the operative procedure, the exposure of laryngeal nerve, the mobility of vocal cord detected by an otolaryngologist, clinical symptoms and therapy of patients with bilateral paralysis were analysed. All patients were examined immediately postoperatively and 5 days, 14 days, 6 and 12 months after resection. RESULTS: The overall transient bilateral palsy rate was 0.7%, the permanent 0.3%. The palsy rate depended on the disease of thyroid gland. After resection of simple goitre we found a 0.2% transient injury rate (0.1% permanent), after operation of thyroid cancer 2.0% transient (1.0% permanent) and in cases of recurrent goitre 5.9% transient (1.9% permanent) palsies. The immediate postoperative symptoms are also very different. There are patients suffering from stridor and dyspnoea, patients with dysphonia without dyspnoea and those without any symptoms. These different clinical symptoms are due to the different grade of laryngeal nerve damage and the resulting position of vocal cords. The bilateral paralysis was completely temporary in 4 cases. 12 months later 4 patients suffered from dysphonia. Only in 3 patients with thyroid cancer and a preoperative unilateral vocal cord paralysis tracheostomy was necessary after operation. The vocal cord mobility did not recover in these 3 cases after 12 months and the patients are not decannulated. DISCUSSION: Bilateral paralysis is only relevant in thyroid cancer and recurrent goitre. The symptoms varies and no patient should leave the hospital without examination of the vocal cords by an otolaryngologist. Because vocal cord paralysis is temporary in most cases an emergency tracheostomy is seldom indicated.