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.     

Catecholamine-secreting paragangliomas of the base of the skull. Report of two cases

Two cases of catecholamine-secreting paragangliomas of the base of the skull are described. The patients presented with uncontrollable hypertension and, after investigation, tumors were discovered in the regions of the glomus jugulare and pterygopalatine ganglion, respectively. After cardiovascular stabilization and tumor embolization, the tumors were surgically removed, with subsequent resolution of hypertension. The incidence of these tumors is discussed.     

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.     

Malignant paraganglioma of the glomus jugulare: a case report.

Jugular paragangliomas are noted for their tendency to recur, but frank malignant behaviour is rare. We report a case in which a patient harbouring a recurrent tumour of the glomus jugulare re-presented with metastatic spread to mediastinal lymph nodes. Review of the 16 reported cases provides information which can be a basis for management of the malignant form of this tumour.     

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.     

Primary adenocarcinoma of the temporal bone mimicking paragangliomas: radiographic and clinical recognition

Three cases of primary adenocarcinoma of the temporal bone which simulated a glomus jugulare tumor (on the basis of a thorough preoperative evaluation) are detailed. In each case, a vascular mass was seen beneath an intact tympanic membrane and angiography revealed the presence of a highly vascular mass centered on the jugular bulb. Computed tomography revealed erosive changes within the jugular fossa that were characteristic of a glomus jugulare tumor; in two cases, a significant posterior fossa extension was documented. The intraoperative findings failed to suggest a lesion different from paraganglioma in two of the cases; in the remaining case, the erosion of both bone and dura was diffuse and more suggestive of a malignant neoplasm. A discussion of the clinical behavior of adenocarcinomas of the middle ear is included in order to emphasize the importance of differentiating these lesions preoperatively from the more commonly encountered paragangliomas.     

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.     

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.     

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.     

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.     

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.     

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.     

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.     

Studies for Estimating the Biologic Behavior and Prognosis of Paragangliomas in the Head and Neck

Despite a large number of histopathologic and immunohistochemical studies, the biologic behavior and prognosis of paragangliomas (glomus tumors) of the head and neck still remain uncertain. In the present study 36 specimens from 32 patients who underwent surgery for a paraganglioma were examined. The examinations included routine histology, quantitative DNA analysis based on image cytometry, immunohistochemical detection of the proliferating cell nuclear antigen (PCNA) along with visualization of nucleolar organizer regions (AgNOR). According to LeCompte, the paragangliomas were histologically divided into three subcategories: 16 patients had a paragangliomatous tumor. 14 patients had an adenomatous tumor, and 6 patients had an angiomatous tumor. Quantitative DNA analysis revealed three categories of tumors with characteristical DNA pattern; DNA type I tumors were pure diploid, DNA type II tumors had stemlines at 2c and 4c and were therefore recognized as diploid-tetraploid. Aneuploid cells were not apparent in these two groups. DNA type III tumors had stemline ploidies exceeding 2c and 4c. Aneuploid cells were present in all of these tumors. The biologic behavior of these lesions therefore must be recognized as suspicious. DNA type III tumors and adenomatous tumors showed the highest values for the PCNA scores, indicating a higher proliferation rate and a more rapid growth pattern in these lesions. Twenty patients could be followed over a period of up to 110 months. Five of these patients developed a recurrent tumor. All of them had DNA type III tumors. The DNA indices showed significantly higher values in the recurrent tumor group. The 2c deviation index (DI) and the entropy value had the highest prognostic significance. No correlation to clinical follow-up was found for the AgNOR score. Based on these results, prognostic indices for paragangliomas were developed: patients with a tumor having a 2c DI exceeding 2.0, entropy value of more than 4.0. 5c exceeding rate more than 8.0, and a PCNA score more than 20.0% can be recognized as being at high-risk for developing recurrent disease.     

Studies for estimating the biologic behavior and prognosis of paragangliomas in the head and neck

Despite a large number of histopathologic and immunohistochemical studies, the biologic behavior and prognosis of paragangliomas (glomus tumors) of the head and neck still remain uncertain. In the present study 36 specimens from 32 patients who underwent surgery for a paraganglioma were examined. The examinations included routine histology, quantitative DNA analysis based on image cytometry, immunohistochemical detection of the proliferating cell nuclear antigen (PCNA) along with visualization of nucleolar organizer regions (AgNOR). According to LeCompte, the paragangliomas were histologically divided into three subcategories: 16 patients had a paragangliomatous tumor. 14 patients had an adenomatous tumor, and 6 patients had an angiomatous tumor. Quantitative DNA analysis revealed three categories of tumors with characteristical DNA pattern; DNA type I tumors were pure diploid, DNA type II tumors had stemlines at 2c and 4c and were therefore recognized as diploid-tetraploid. Aneuploid cells were not apparent in these two groups. DNA type III tumors had stemline ploidies exceeding 2c and 4c. Aneuploid cells were present in all of these tumors. The biologic behavior of these lesions therefore must be recognized as suspicious. DNA type III tumors and adenomatous tumors showed the highest values for the PCNA scores, indicating a higher proliferation rate and a more rapid growth pattern in these lesions. Twenty patients could be followed over a period of up to 110 months. Five of these patients developed a recurrent tumor. All of them had DNA type III tumors. The DNA indices showed significantly higher values in the recurrent tumor group. The 2c deviation index (DI) and the entropy value had the highest prognostic significance. No correlation to clinical follow-up was found for the AgNOR score. Based on these results, prognostic indices for paragangliomas were developed: patients with a tumor having a 2c DI exceeding 2.0, entropy value of more than 4.0. 5c exceeding rate more than 8.0, and a PCNA score more than 20.0% can be recognized as being at high-risk for developing recurrent disease.     

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.     

Non-chromaffin paraganglioma of the orbit. Case report

The non chromaffin paraganglioma of the orbit is a relatively rare tumor. To our knowledge only 25 cases have been reported in the world literature. We report on the case of a 39-year-old woman who was treated surgically for the removal of an orbital paraganglioma 10 years ago. She had complained of proptosis and her right eyeball was slightly displaced upwards and laterally. A transcranial operation was performed and the tumor, located medically and weighing 9'5 grs., was completely removed; it was encapsulated. 10 years after this total excision there was no evidence of recurrence. From a histological point of view the tumor cells closely resembled those of paragangliomas of the carotid body and glomus jugulare.     

Diagnosis and management of paragangliomas of the skull base

In appropriately selected patients, glomus tumors of the head and neck are best treated surgically. Unresectability is not a factor in therapeutic planning for local disease control. Existing techniques and exposures for tumor removal can be reliably applied to these paragangliomas, with acceptable morbidity and mortality. A team approach to this problem is mandatory.