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.     

Combined endovascular and surgical treatment of head and neck paragangliomas--a team approach

BACKGROUND: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. METHODS: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. RESULTS: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. CONCLUSIONS: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen.     

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.     

Treatment of Recurrent and Residual Glomus Jugulare Tumors

Residual and recurrent glomus jugulare tumors are rare but challenging. Treatment options include microsurgical resection, stereotactic radiotherapy, a combination of modalities, and “observation.” Choice of treatment must be made on a case-by-case basis, considering patient age, health status, location and size of tumor, status of the lower cranial nerves, and, of course, patient desire. Surgery is preferred when total resection of the tumor with preservation of function is deemed achievable. When function of the lower cranial nerves has been compromised, total surgical resection may also be possible, provided that the patient's health allows it. Cases where function is still preserved despite presence of a large tumor are more challenging, and a combination modality may be most effective. The goal of treatment is to provide tumor control with low morbidity. Current surgical techniques and the availability of stereotactic radiotherapy make this possible in the majority of cases.     

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.     

Bilateral carotid body tumor resection in a female patient

IntroductionCarotid body tumors also called carotid paragangliomas are rare neuroendocrine neoplasms derived from neural crest cells, approximately 3% of all paragangliomas occur in the head and neck area (Xiao and She, 2015); although they represent 65% of the head and neck paragangliomas (Georgiadis et al., 2008).Presentation of caseWe present the therapeutic management of a 65-year-old woman with bilateral carotid body tumors. The patient presented to medical clinic for unrelated signs and symptoms of weight loss, dyspepsia, and epigastric pain. Physical examination showed bilateral non-tender neck masses for which imaging studies were ordered resulting in the diagnosis of bilateral carotid tumor. Surgical resection was staged with one week of distance between each tumor resection.DiscussionCarotid Body Tumors can arise from the paraganglia located within the adventitia of the medial aspect of the carotid bifurcation.Resection is the only curative treatment. Carotid body tumors resection represents a special challenge due to potential neurovascular complications.ConclusionsSurgical resection of carotid body tumors represents a special challenge to the surgeon because of the complex anatomical location of the tumor, including close relationship with the cranial nerves, involvement of the carotid vessels and large vascularization of the tumor. With the advance of diagnosis and improvement in surgical techniques as well as the understanding of biological behavior of tumors, surgical treatment has become a safer alternative for treating these tumors.     

Jugular foramen paragangliomas: management, outcome and avoidance of complications in a series of 75 cases

Jugular foramen paragangliomas are rare skull base tumours posing multiple complex diagnostic and management problems. We did a study to evaluate surgical technique, outcome and complications in 75 cases of tumours treated by multidisciplinary approach (i.e. combined neurosurgery, neuroradiology, ear, nose and throat surgery and intensive care unit team). Retrospective study on 75 consecutive patients with jugular foramen paragangliomas treated surgically from 1989 to 2005. Preoperative balloon occlusion test was performed in all patients as well as embolization (100%). A combined limited infratemporal and juxtacondylar approach was used in all patients. Gross total resection was achieved in 59 patients (78.7%). The most common complication was represented by lower cranial nerve deficits in five patients (6.6%), which was only temporary in three. Postoperative facial nerve weakness occurred in five cases (6.6%) and resolved in three of them. The remaining two patients underwent facial nerve reconstruction by hypoglossal/facial nerve anastomosis. Four patients (5.3%) had a postoperative cerebrospinal fluid leak, which was successfully treated by lumbar drainage. Two patients (2.7%) died because of complications related to surgical injury of lower cranial nerves: one patient developed aspiration pneumonia and septicemia and the second one developed a large cervico-bulbar hematoma that led to severe respiratory distress and ultimately global cerebral hypoxia. Paragangliomas are rare and complex skull base lesions that may be managed with low morbidity and mortality if a multidisciplinary approach is considered. Facial and lower cranial nerve postoperative deficits can be limited.     

Jugular foramen: microscopic anatomic features and implications for neural preservation with reference to glomus tumors involving the temporal bone

OBJECTIVE: Our goals were to study the normal histological features of the jugular foramen, compare them with the histopathological features of glomus tumors involving the temporal bone, and thus provide insight into the surgical management of these tumors with respect to cranial nerve function. METHODS: Ten jugular foramen blocks were obtained from five human cadavers after removal of the brain. Microscopic studies of these blocks were performed, with particular attention to fibrous or bony compartmentalization of the jugular foramen, the relationships of the caudal cranial nerves to the jugular bulb/jugular vein and internal carotid artery, and the fascicular structures of the nerves. In addition, we studied the histopathological features of 11 glomus tumors involving the temporal bone (10 patients), with respect to nerve invasion, associated fibrosis, and carotid artery adventitial invasion. RESULTS: A dural septum separating the IXth cranial nerve from the fascicles of Cranial Nerves X and XI, at the intracranial opening, was noted. Only two specimens, however, had a septum (one bony and one fibrous) producing internal compartmentalization of the jugular foramen. The cranial nerves remained fasciculated within the foramen, with the vagus nerve containing multiple fascicles and the glossopharyngeal and accessory nerves containing one and two fascicles, respectively. All of these nerve fascicles lay medial to the superior jugular bulb, with the IXth cranial nerve located anteriorly and the XIth cranial nerve posteriorly. All nerve fascicles had separate connective tissue sheaths. A dense connective tissue sheath was always present between the IXth cranial nerve and the internal carotid artery, at the level of the carotid canal. The inferior petrosal sinus was present between the IXth and Xth cranial nerves, as single or multiple venous channels. The glomus tumors infiltrated between the cranial nerve fascicles and inside the perineurium. They also produced reactive fibrosis. In one patient, in whom the internal carotid artery was also excised, the tumor invaded the adventitia. CONCLUSION: Within the jugular foramen, the cranial nerves lie anteromedial to the jugular bulb and maintain a multifascicular histoarchitecture (particularly the Xth cranial nerve). Glomus tumors of the temporal bone can invade the cranial nerve fascicles, and infiltration of these nerves can occur despite normal function. In these situations, total resection may not be possible without sacrifice of these nerves.     

Cervical paragangliomas-tumor control and long-term functional results after surgery.

Objective: To report long-term functional results of the surgical treatment of cervical paragangliomas. Patients and Methods: A retrospective review of 22 patients with 34 head and neck paragangliomas of which 27 were resected between 1981 and 2004. Of these, 16 were carotid body tumors and 11 were vagal paragangliomas. There were 13 women and 9 men with an average age of 48.6 years (range, 26 to 75 years; median, 49 years) and the mean follow-up period was 82 months (range, 3 to 184 months; median, 61 months). Results: There were 13 solitary tumors of which 5 were carotid body tumors and 8 vagal paragangliomas. Multiple head and neck paragangliomas were seen in 9 patients (41%). The incidence of associated multiple tumors was 64.3% for carotid body tumors and 38.5% for vagal paragangliomas. Complete tumor resection was achieved in all but 1 patient in whom a small intradural residual vagal paraganglioma had to be left. The internal carotid artery was preserved in all carotid body tumor resections. Lower cranial nerve deficits were sustained in 1 carotid body tumor resection only, but in all cases with multiple tumors. All patients with vagal paragangliomas had or developed a vagal nerve paralysis. In 4 cases minor complications developed postoperatively. No recurrent tumors were seen during the follow-up period. Conclusions: Even in large head and neck paragangliomas surgical treatment provides excellent tumor control with low postoperative morbidity. A wait-and-scan policy may be more appropriate for those patients with multiple tumors, advanced age, or high operative risk and for those whose tumors have recurred following radiotherapy.     

Operative management of tumors involving the cavernous sinus

In the past, neurosurgeons have been reluctant to operate on tumors involving the cavernous sinus because of the possibility of bleeding from the venous plexus or injury to the internal carotid artery (ICA) or the third, fourth, or sixth cranial nerves. The authors describe techniques for a more aggressive surgical approach to neoplasms in this area that are either benign or locally confined malignant lesions. During the last 2 years, seven tumors involving the cavernous sinus have been resected: six totally and one subtotally. The preoperative evaluation included axial and coronal computerized tomography, cerebral angiography, and a balloon-occlusion test of the ICA. Intraoperative monitoring of the third, fourth, sixth, and seventh cranial nerves was used to assist in locating the nerves and in avoiding injury to them. The first major step in the operative procedure was to obtain proximal control of the ICA at the petrous apex and distal control in the supraclinoid segment. The cavernous sinus was then opened by a lateral, superior, or inferior approach for tumor resection. Temporary clipping and suture of the ICA was necessary in one patient. None of the patients died or suffered a stroke postoperatively. Permanent trigeminal nerve injury occurred in three patients; in two, this was the result of tumor invasion. One patient suffered temporary paralysis of the third, fourth, and sixth cranial nerves, and in another the sixth cranial nerve was temporarily paralyzed. Preoperative cranial nerve deficits were improved postoperatively in three patients. Radiation therapy was administered postoperatively to four patients. These seven patients have been followed for 6 to 18 months to date and none has shown evidence of recurrence of the intracavernous tumor.     

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.     

Management of cervical paragangliomas: review of a 15-year experience

Background and aims Cervical paragangliomas are highly vascular neoplasms and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors.Materials and methods Thirteen patients with 14 paragangliomas were treated in our institution during a period of 15 years. There were eight women (61.5%) and five men (38.5%) with a mean age of 41.3±15 years. A painless lateral neck mass was the main finding in 69.2% of patients. There was no evidence of a functional tumor. Carotid angiography was performed in all patients to define the vascular anatomy of the lesion. The 78.6% of paragangliomas underwent selective embolization of the major feeding arteries. Surgical resection followed within the next 48 h.Results The majority of the lesions were paragangliomas of the carotid bifurcation (85.7%), while one patient was diagnosed with a jugular and one with a vagal paraganglioma. In one patient, bilateral paragangliomas in the carotid bifurcation were detected. There was no evidence of malignancy in any case. Preoperative embolization has proven successful in reducing tumor vascularity. Vascular reconstruction was necessary in one patient. The main postoperative complication was transient cranial nerve deficit in seven (53.8%) patients, and a permanent Horner’s syndrome was documented in one patient. No stroke occurred. The jugular paraganglioma was treated with irradiation due to skull base extension with significant symptomatic relief.Conclusion Combined therapeutic approach with preoperative selective embolization followed by surgical resection by an experienced team offers a safe and effective method for complete excision of the tumors with a reduced morbidity rate.     

A combined infratemporal and posterior fossa approach for the removal of giant glomus tumors and chondrosarcomas

Giant glomus jugulare tumors with a large posterior fossa extension are considered either inoperable or at least requiring of a two-stage operation. Likewise, the surgical approach and treatment for chondrosarcomas of the temporal bone are controversial. We describe a combined approach in which, with the aid of microsurgical and laser techniques, such tumors can be removed in one stage. The surgical approach involves a lateral infratemporal approach combined with a posterior fossa craniectomy. This technique was used in seven cases: five glomus jugulare tumors and two chondrosarcomas. There were no deaths, and surgical morbidity consisted of weakness in the facial nerve in four of these patients and gastrointestinal hemorrhage and respiratory distress syndrome in one patient.     

Management of Carotid Body Paragangliomas and Review of a 30-Year Experience

Carotid body tumors are rare neoplasms and must be considered in the evaluation of all lateral neck masses; early surgical removal has been recommended. In this study, the medical records of 29 patients with 34 carotid body paragangliomas who were treated at our institution between 1971 and 2001 were retrospectively reviewed. An overview is provided of this lesion, including diagnosis, classification, metastatic potential, possible secretory function, operative techniques, and nonsurgical methods of management. Carotid body tumors may be familial and are more often bilateral in these instances; five patients (17%) had bilateral tumors in this series. The criterion for malignancy is demonstrated by metastatic tumor in lymph nodes or distant organs. Three patients (10%) had malignant tumors, one with hepatic metastases. One patient (3%) in our series exhibited abnormal serotonin production. Vascular reconstruction was necessary in eight cases (28%). No stroke occurred, however, two arterial thromboses (7%), five permanent cranial nerve deficits (17%), and one death (3%) from massive pulmonary embolism were seen. Our experience demonstrates that early operative management is warranted to avoid the possibility of eventual metastasis and progressive local invasion to the point of inoperability.     

Outcomes after surgical resection of head and neck paragangliomas: a review of 61 patients

We reviewed the postoperative functional outcome following surgical resection of paragangliomas in patients with and without preoperative cranial nerve dysfunction. Patients who underwent surgical resections of head and neck paragangliomas were reviewed with functional outcomes defined as feeding tube and/or tracheostomy dependence, need for vocal cord medialization, and incidence of cerebral vascular accidents as primary end points. Secondary end points included pre- and postoperative function of lower cranial nerves and the impact of this dysfunction on long-term functional status. Sixty-one patients were identified: 27 with carotid paraganglioma (CP), 21 with jugular paraganglioma (JP), 8 with tympanic paragangliomas, 4 with vagal paragangliomas (VPs), and 1 with aortopulmonary paraganglioma. Following resection, 8 patients were feeding tube dependent, 14 patients required vocal cord medialization, 2 patients suffered strokes, but no patients required tracheostomy tubes. Twenty percent of patients (4/20) with JP and postoperative cranial neuropathies were feeding tube dependent, and 80% of patients (4/5) with CP and postoperative cranial nerve dysfunction were feeding tube dependent. Cranial nerve deficits were more common in patients with JP relative to those with CP. However, when cranial nerve dysfunction was present, our patients with CP had a higher incidence of temporary feeding tube dependence. Overall, 98% of patients were able to resume oral nutrition.     

Vagal body tumors

Six cases of vagal body tumor are reviewed. All first presented as painless neck masses with normal cranial nerve function. Otologic symptoms were infrequent, occurring only with temporal bone involvement. In true vagal paragangliomas, cranial nerve and auditory function is usually preserved until there is extensive disease of the skull base. Tumor progression after radiotherapy was documented in four patients, three of whom were treated with 4500 cGy or more. One patient was found to have regional lymph node metastases. The six patients had a total of 10 head and neck paragangliomas, illustrating the high incidence of synchronous and metachronous lesions. Because of the high incidence of multiple lesions, these tumors threaten lower cranial nerves bilaterally in many instances. Because cranial nerve function is preserved until late, and because vagal and accessory nerve paralysis is usually unavoidable with resection, we advocate conservative treatment in selected cases. It may be reasonable to postpone surgery until cranial nerve impairment becomes evident or other vital structures are threatened.     

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.     

Stereotactic radiosurgery for jugular foramen schwannomas.

BACKGROUND: Jugular foramen schwannomas pose difficult management problems because of the surgical risk of lower cranial neuropathy. The indications and results of stereotactic radiosurgery are not well documented. METHODS: We reviewed our 10-year experience in the management of 17 patients who had jugular foramen schwannomas managed with the gamma knife. Thirteen patients previously had undergone surgery (range, 1-6 resections). Four patients had multiple cranial nerve deficits before microsurgical resection; 12 developed multiple lower cranial nerve palsies after resection. Four patients underwent radiosurgery based on imaging criteria alone. Conformal dose planning (tumor margin dose of 12-18 Gy) successfully encompassed the irregular tumor volumes in all patients. RESULTS: Follow-up varied from 6 to 74 months. Tumor size decreased in eight patients, remained stable in eight, and increased in one patient during the average follow-up interval of 3.5 years. Six patients improved and 10 others retained their preradiosurgery clinical status. One patient had an increase in tumor size and clinical deterioration 6 months after radiosurgery and underwent microsurgical resection. No patient developed new cranial nerve or other neurological deficits after radiosurgery. CONCLUSIONS: We believe that gamma knife radiosurgery is an effective alternative to microsurgical resection for patients who have small tumors and intact lower cranial nerve function. It is also effective for patients who have residual or recurrent tumors after microsurgical resection.     

Paraganglioma of the cranial vault. Apropos of a case]

The authors report the case of a 40 years old woman with a fronto parietal tumefaction and intracranial hypertension caused by a paraganglioma of the cranial vault. The clinical follow up is marked by rapid local recurrence of the tumor after complete excision. Usually paragangliomas arise in any area of the body where paraganglionar structures are situated (glomus jugulare, carotid glomus, vagus nerve). Other rare locations have been reported and remain difficult to be explained. The location at the cranial vault is exceptional. this tumor would probably arise from the paraganglionar cells which migrate from the neural crest following the trigeminal branches particularly the ophtalmic division.