Synchronous Carotid Body and Glomus Jugulare Tumors : A Case Report and Review of Literature.

Paragangliomas are rare neuroendocrine tumors that are usually benign in nature. They may be either familial or sporadic in their occurrence. Numerous neuroendocrine tumors are collectively included under the umbrella of paragangliomas. Among them, carotid body tumors and glomus jugulare tumors are extremely rare. Thus, we present a rare case of 29-year-old male who was admitted with hearing difficulties and tinnitus in the left ear, with swelling on the left side of the neck. Based on clinical and radiological findings, a diagnosis of left-sided glomus jugulare with carotid body tumor was made. The patient underwent a twostage surgery with an interval of approximately 2 months. Histopathology revealed a paraganglioma. Herein, we present the clinical features, imaging findings, management, and a brief review of literature on the classification, evaluation, and management of carotid body and glomus jugulare tumors. Paraganglioma is a slow-growing tumor. The synchronous occurrence of carotid body and glomus jugulare tumors is infrequent. Microsurgical resection remains the primary treatment modality. Therefore, our patient underwent two-stage surgery. The rarity of occurrence and the proximity and adherence to vital neurovascular structures have resulted in the treatment of paragangliomas remaining a challenge.

Metachronous Skull Base Paraganglioma Surgical Management: 2-Dimensional Operative Video.

Paragangliomas (PGLs) are benign hypervascular tumors that can develop in head and neck at different locations, primarily in the carotid bifurcation, jugular bulb, tympanic plexus, and vagal ganglia.1 Different gene mutations have been linked to the familial inherited forms, which can represent approximately 30% of all PGLs.1,2 These are classified into 5 different clinical syndromes: PGL 1 to 5.1 These patients have increased risk for synchronous and metachronous lesions requiring an extensive work-up for hormone secretion and other associated neoplasms, as well as attentive follow-up for lifelong management.1,3 Surgical resection is the best treatment option as it can be curative when the resection is total.2-4 Preservation of the lower cranial nerve function is central to the management of head and neck PGLs, given the gravity of bilateral injuries.3 Irradiation therapy should be considered if the risk for bilateral lower cranial nerve injuries is high.5 Surgically, intrabulbar resection with preservation of the medial wall of the jugular bulb protects the lower cranial nerve function.3 Other technical finesses, including maintaining the facial nerve in its bony fallopian canal (facial bridge), avoiding carotid artery sacrifice, preservation of the ear canal, and preoperative embolization, contributed markedly to outcome improvement.2,3 We report a case of a 34-yr-old male with PGL 3 with a left glomus jugulare tumor that recurred and a right carotid body tumor. Patient consented to surgery and photography. Image at 3:44 republished from Al-Mefty and Teixeira,3 with permission from JSNPG.

[Analysis of misdiagnosis glomus jugulare tumor].

Objective:To analyze the causes of misdiagnosis of patients with glomus jugulare tumor. Method:The clinical data of 116 patients with glomus jugulare tumor were retrospectively analyzed. Result:Among the 116 patients, 65 were misdiagnosed, the average duration of misdiagnosis was 4.90 years. The misdiagnosed diseases were chronic otitis media or cholesteatoma in 25 cases, neurological tinnitus or hearing loss in 16 cases, facial paralysis in 6 cases, external auditory canal masses in 4 cases, secretory otitis media in 3 cases, neck mass in 3 cases, intracranial neoplasma in 2 cases, middle ear cancer in 1 case, vocal cord paralysis in 1 case, stomatitis in 1 case, optic nerve head edema in 1 case, middle ear hemangioma in 1 case and vascular tinnitus in 1 case. Conclusion:The misdiagnosis of glomus jugulare tumor can be summarized as follows: the patients' first symptoms were not specific; lack of auxiliary examination, especially imaging examination; physicians have insufficient understanding of imaging and pathological examination of glomus jugulare tumor.

LINAC radiosurgery for glomus jugulare tumors: retrospective - cohort study of 23 patients.

BACKGROUND: Glomus jugulare tumors (GJTs) are uncommon and locally disruptive tumors that usually arise within the jugular foramen of the temporal bone. Surgery was the treatment of choice up until recently. In the last decades, however, radiosurgery has surfaced as a promising alternative treatment by providing excellent tumor control with low risk of cranial nerve injuries. Our aim was to examine the results of radiosurgery specifically, linear accelerator stereotactic radiosurgery (LINAC SRS) for GJT treatment. We hypothesized that radiosurgery will reduce the size of the tumor and improve neurological symptoms. DESIGN AND METHOD: Between January 1, 1994 and December 31, 2013, 30 patients with GJTs were treated in Sheba Medical Center using LINAC SRS treatment. Comprehensive clinical follow-up was available for 23 patients. Sixteen patients were female and seven males with a median age of 64 years, with a range of 18-87 years. In 19 of the patients, LINAC SRS was the primary treatment, whereas in the remaining four cases, surgery or embolization preceded radiosurgery. The median treated dose to tumor margin was 14 Gy (range 12-27 Gy), and the median tumor volume before treatment was 5 ml (range 0.5-15 ml). RESULTS: Following the LINAC SRS treatment, 14 of 23 patients (60%) showed improvement of previous neurological deficits, nine patients (40%) remained unchanged. At the end of a follow-up, tumor reduction was seen in 13 patients and a stable volume in eight (91% tumor control rate). Two cases of tumor progression were noted. Three patients (13%) had post- SRS complications during the follow-up, two of which achieved tumor control, while in one the tumor advanced. CONCLUSIONS: LINAC SRS is a practical treatment option for GJTs, with a high rate of tumor control and satisfactory neurological improvement.

[Diagnosis and treatment of glomus tumors of the skull base and neck]. / Diagnostik und Therapie von Glomustumoren der Schädelbasis und des Halses.

Glomus tumors (paragangliomas) are rare, usually benign, neuroendocrine tumors that arise from nonchromaffin cells and can be multifocal or familial. They grow slowly, often infiltratively and rarely form metastases. A distinction is made between carotid, jugular, tympanicum and vagal tumors. Depending on the location, clinical symptoms include neck swelling, hearing loss with pulse-synchronous ear noise or cranial nerve failures (IX-XI). Diagnosis is often delayed because of nonspecific symptoms. Diagnostically, tumor expansion and perfusion can be well visualized by color Doppler sonography. In the T2-weighted MRI, the tumor is hyperintense, after contrast agent administration a strong enhancement occurs due to the strong vascularization. In DSA (digital subtraction angiography), even small glomus tumors can be detected. Therapeutically, operative tumor resection after embolization with, e.g., polyvinyl alcohol particles or liquid embolization can be performed. If the patient is in poor general condition or if the tumor is inoperable, different treatment techniques (intensity-modulated radiotherapy, gamma or cyber-knife) may be considered as a conservative therapeutic alternative.

Preservation of the facial and lower cranial nerves in glomus jugulare tumor surgery: modifying our surgical technique for improved outcomes.

PURPOSE: To describe the neurological results obtained in six patients with large Fisch C glomus jugulare tumors (GJT) in which a less aggressive, nerve-preserving surgical strategy was used to reduce surgical morbidity. METHODS: Prospective study of six patients with Fisch C GJT who underwent surgery in a tertiary care referral center from February 2015 to August 2017 with an average follow-up of 18 months. The intervention is the surgical technique used and the main outcome measures are recurrence and the functional preservation of the facial and lower cranial nerves. RESULTS: Gross total removal was obtained in the six patients with preservation of the medial wall of the jugular bulb protecting the lower cranial nerves. After follow-up, we obtained a House-Brackmann (H-B) grade II in three patients who were managed with an inferior facial nerve transposition. One patient managed with a facial bridge technique preserved a normal facial function and two patients who presented a H-B III before surgery went to H-B V after surgery and recovered to a H-B III after 4 months. Four patients were presented with dysphagia after surgery and required nasogastric tube placement. The average time for removal with return to normal oral feeding was 4.3 weeks. Three patients with preoperative Xth nerve dysfunction showed an adequate compensation of the opposite vocal fold in the postoperative period without dysphonia or aspiration. CONCLUSION: The surgical techniques used in these patients provided good functional preservation without recurrence after an 8-30-month follow-up.

[Vascularized tumor of the retroauricular skin]. / Gefäßreicher Tumor der retroaurikulären Haut.

An 84-year-old female patient with loss of hearing and otorrhoea went to an otolaryngologist. On examination, a polypoid mass in the auditory canal and a retroauricular skin lesion, possibly seborrheic keratosis, were found. Only the skin was submitted for histological examination and showed a cutaneous highly vascular lesion with an associated "zellballen" of clear cells without atypia. The initial diagnosis was that of a cutaneous glomangioma. Immunohistochemical findings favored diagnosis of a paraganglioma. Additional clinical information revealed a large mastoid tumor mass. Therefore, the findings were compatible with an jugulotympanic paraganglioma with infiltration of the overlying skin. This possibility was initially not considered in differential diagnosis, as cutaneous glomangiomas are relatively common (typically located on the hands). Cutaneous paragangliomas, however, are not reported.

Long-term outcomes after radiosurgery for glomus jugulare tumors.

AIMS AND BACKGROUND: The treatment of glomus jugulare tumors (GJT) remains controversial due to high morbidity. Historically, these tumors have primarily been managed surgically. The purpose of this retrospective review was to assess the tumor and clinical control rates as well as long-term toxicity of GJT treated with radiosurgery. METHODS: Between 1993 and 2014, 30 patients with GJT (31 tumors) were managed with radiosurgery. Twenty-one patients were female and the median age was 59 years. Twenty-eight patients (93%) were treated with radiosurgery, typically at 14 Gy ( n = 26), and 2 patients (7%) with stereotactic radiosurgery. Sixteen cases (52%) had undergone prior surgery. RESULTS: The mean follow-up was 4.6 years (range 1.5-12). Crude overall survival, tumor control, clinical control, and long-term grade 1 toxicity rates were 97%, 97%, 97%, and 13% (4/30), respectively. No statistically significant risk factor was associated with lower tumor control in our series. Univariate analysis showed a statistically significant association between patients having 1 cranial nerve (CN) involvement before radiosurgery and a higher risk of lack of improvement of symptoms (odds ratio 5.24, 95% confidence interval 1.06-25.97, p = .043). CONCLUSIONS: Radiosurgery is an effective and safe treatment modality for GJT. Patients having 1 CN involvement before radiosurgery show a higher risk of lack of improvement of symptoms.

Long-term effectiveness and safety of stereotactic gamma knife surgery as a primary sole treatment in the management of glomus jagulare tumor.

OBJECTIVE: This study aims to report and confirm long-term effectiveness and safety of stereotactic Gamma Knife Surgery as a primary sole treatment in the management of 40 glomus jagulare tumors patients. PATIENTS AND METHODS: Retrospective analysis of clinical and radiological outcomes of 40 GJTs consecutive patients treated with GKS as primary sole treatment at International Medical Center (IMC), Cairo-Egypt from the beginning of 2005 till the end of 2014,with mean follow-up period of 84 months (range 36-156 months), mean tumor volume was 6.5 cc, and mean peripheral radiation dose of 15 Gy, to mean isodose curve of 38%. RESULTS: The most common neurological deficit at initial evaluation was bulbar symptoms in 24 patients, followed by pulsatile tinnitus in 22, deterioration of hearing in 20 patients. The overall clinical control achieved in 92.5% of patients, while actuarial tumor size control rate post- GKS was 97.5% at 3 years, 97% at 5 years and 92% at 10 years of follow-up period. CONCLUSIONS: Gamma knife surgery could be used effectively and safely as a primary sole treatment tool in the management of glomus jugulare tumors.

Acquired Vascular Tumors of the Head and Neck.

Vascular neoplasms of the head and neck present with a wide spectrum of signs and symptoms. Diagnosis requires a high index of suspicion and is usually made after tumors are large enough to be visually apparent or cause symptoms. This article discusses the most common acquired benign and malignant vascular tumors, with an emphasis on their evaluation and treatment.

Robotic radiosurgery of head and neck paragangliomas: a single institution experience.

AIM: CyberKnife® is a robotic stereotactic radiotherapy system. The aim of this study is to evaluate the effectiveness and the safety of CyberKnife® on treating head and neck paragangliomas and to report our results. METHODS: Between March 2009 and June 2014, 12 patients with head and neck paragangliomas have been referred to our clinic: in three cases had jugular paragangliomas, five cases had carotid body paragangliomas and three cases had tympanic paragangliomas. One patient had bilateral neck paragangliomas (right neck; carotid body paraganglioma, left neck; jugular pargangliomas). All of them received fractionated stereotactic radiotherapy with CyberKnife® up to a total median dose of 24 Gy (reference isodose 67-90%). The median tumor volume was 35.5 cc (range, 5.3-113.8 cc). The median follow up was 30 months (range, 0-66 months). Local tumor control was assessed according to RECIST criteria on follow-up imaging studies. RESULTS: There were no acute or late toxicity related with stereotactic radiotherapy after treatment. No local tumor progression was observed on magnetic resonance imaging and none of our patients showed progressive clinical status. Seven tumors shrinked in size (54%). Five tumors (46%) had stable size during follow up. Local control rate was 100%. CONCLUSION: Stereotactic radiotherapy is a good alternative to surgery for the treatment of head and neck paragangliomas coming up with a clear benefit of acute and late side effects. CyberKnife® seems to be a safe and efficient system treating head and neck paragangliomas.

Endovascular Approach to Glomus Jugulare Tumors.

BACKGROUND: Paragangliomas are benign neuroendocrine tumors derived from the glomus cells of the vegetative nervous system. Typically, they are located in the region of the jugular bulb and middle ear. The optimal management is controversial and can include surgical excision, stereotactic radiosurgery and embolization. CASE REPORT: We report the endovascular approach to three patients harboring glomus jugulare paragangliomas. In all cases incomplete occlusion of the lesions was achieved and recanalization in the follow-up period was revealed. Two patients presented no clinical improvement and the remaining one experienced a transient withdrawal of tinnitus. CONCLUSIONS: It is technically difficult to achieve complete obliteration of glomus jugulare tumors with the use of embolization and the subtotal occlusion poses a high risk of revascularization and is not beneficial in terms of alleviating clinical symptoms.

Tumors Presenting as Multiple Cranial Nerve Palsies.

Cranial nerve palsy could be one of the presenting features of underlying benign or malignant tumors of the head and neck. The tumor can involve the cranial nerves by local compression, direct infiltration or by paraneoplastic process. Cranial nerve involvement depends on the anatomical course of the cranial nerve and the site of the tumor. Patients may present with single or multiple cranial nerve palsies. Multiple cranial nerve involvement could be sequential or discrete, unilateral or bilateral, painless or painful. The presentation could be acute, subacute or recurrent. Anatomic localization is the first step in the evaluation of these patients. The lesion could be in the brain stem, meninges, base of skull, extracranial or systemic disease itself. We present 3 cases of underlying neoplasms presenting as cranial nerve palsies: a case of glomus tumor presenting as cochlear, glossopharyngeal, vagus and hypoglossal nerve palsies, clivus tumor presenting as abducens nerve palsy, and diffuse large B-cell lymphoma presenting as oculomotor, trochlear, trigeminal and abducens nerve palsies due to paraneoplastic involvement. History and physical examination, imaging, autoantibodies and biopsy if feasible are useful for the diagnosis. Management outcomes depend on the treatment of the underlying tumor.

Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective.

OBJECTIVE The aim of this study was to analyze the neurological functional outcome and recurrent risks in surgically treated jugular foramen paragangliomas (JFPs) and to propose an individualized therapeutic strategy. METHODS Clinical charts and radiological information were reviewed retrospectively in 51 consecutive cases of JFPs. Less-aggressive surgical interventions were adopted with the goal of preserving neurovascular structures. Scheduled follow-up was performed. RESULTS The mean age of the patients in the cases reviewed was 41.6 years, and the group included 27 females (52.9%). The mean preoperative Karnofsky Performance Scale (KPS) score was 78.4. The mean lesion size was 3.8 cm. Forty-three cases (84.3%) were Fisch Type D, including 37 cases (72.5%) of Type Di1 and Di2. Thirty-seven cases (72.5%) were Glasscock-Jackson Type III-IV. Gross-total resection and subtotal resection were achieved in 26 (51.0%) and 22 (43.1%) cases, respectively. Surgical morbidities occurred in 23 patients (45.1%), without surgery-related mortality after the first operation. The mean postoperative KPS scores at discharge, 3 months, 1 year, and most recent evaluation were 71.8, 77.2, 83.2, and 79.6, respectively. The mean follow-up duration was 85.7 months. The tumor recurrence/regrowth (R/R) rate was 11.8%. Compared with preoperative status, swallowing function improved or stabilized in 96.1% and facial function improved or stabilized in 94.1% of patients. A House-Brackmann scale Grade I/II was achieved in 43 patients (84.3%). Overall neurological status improved or stabilized in 90.0% of patients. Pathological mitosis (HR 10.640, p = 0.009) was the most significant risk for tumor R/R. A 1-year increase in age (OR 1.115, p = 0.037) and preoperative KPS score < 80 (OR 11.071, p = 0.018) indicated a risk for recent poor neurological function (KPS < 80). Overall R/R-free survival, symptom progression-free survival, and overall survival at 15 years were 78.9%, 86.8%, and 80.6%, respectively. CONCLUSIONS Surgical outcomes for JFPs were acceptable using a less-aggressive surgical strategy. Most patients could adapt to surgical morbidities and carry out normal life activities. Preserving neurological function was a priority, and maximal decompression with or without radiotherapy was desirable to preserve a patient's quality of life when radical resection was not warranted. Early surgery plus preoperative devascularization was proposed, and radiotherapy was mandatory for lesions with pathological mitosis.

A Case of En Plaque Meningioma of Jugular Foramen with Image Findings of Differential Diagnosis

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.

Stereotactic radiosurgery of glomus jugulare tumors: current concepts, recent advances and future perspectives.

Stereotactic radiosurgery (SRS), a very highly focused form of therapeutic irradiation, has been widely recognized as a viable treatment option in the management of intracranial pathologies including benign tumors, malign tumors, vascular malformations and functional disorders. The applications of SRS are continuously expanding thanks to the ever-increasing advances and corresponding improvements in neuroimaging, radiation treatment techniques, equipment, treatment planning and delivery systems. In the context of glomus jugulare tumors (GJT), SRS is being more increasingly used both as the upfront management modality or as a complementary or salvage treatment option. As its safety and efficacy is being evident with compiling data from studies with longer follow-up durations, SRS appears to take the lead in the management of most patients with GJT. Herein, we address current concepts, recent advances and future perspectives in SRS of GJT in light of the literature.

Leksell Gamma Knife radiosurgery of the jugulotympanic glomus tumor: long-term results.

OBJECT: Glomus tumors usually display indolent behavior, and the effectiveness of radiation in stopping their growth can be assessed after long-term follow-up. Currently only midterm results of radiosurgery are available, so the authors included patients treated by Gamma Knife at least 10 years ago in this study to obtain a perspective of long-term results. METHODS: During the period from 1992 to 2003, the Gamma Knife was used to treat 46 patients with glomus tumors. The age of the patients ranged from 21 to 79 years (median 56 years). Gamma Knife radiosurgery was the primary treatment in 17 patients (37%). Open surgery preceded radiosurgery in 46% of cases, embolization in 17%, and fractionated radiotherapy in 4%. The volume of the tumor ranged from 0.2 to 24.3 cm(3) (median 3.6 cm(3)). The minimal dose to the tumor margin ranged between 10 and 30 Gy (median 20 Gy). RESULTS: One patient was lost for follow-up after radiosurgery. Clinical follow-up was available in 45 patients and 44 patients were followed with MRI in a follow-up period that ranged from 12 to 217 months (median 118 months). Neurological deficits improved in 19 (42%) of 45 patients and deteriorated in 2 patients (4%). Tumor size decreased in 34 (77%) of 44 patients with imaging follow-up, while an increase in volume was observed in 1 patient (2%) 182 months after radiosurgery and Gamma Knife treatment was repeated. One patient underwent another Gamma Knife treatment for secondary induced meningioma close to the glomus tumor 98 months after initial radiosurgical treatment. Seven patients died 22-96 months after radiosurgery (median 48 months), all for unrelated reasons. CONCLUSIONS: Radiosurgery has proved to be a safe treatment with a low morbidity rate and a reliable long-term antiproliferative effect.

Infratemporal fossa approach type a with transcondylar-transtubercular extension for Fisch type C2 to C4 tympanojugular paragangliomas.

BACKGROUND: The classic infratemporal fossa type A approach (IFTA-A) permits superior and anterior exposure of the jugular foramen. The transcondylar-transtubercular extension facilitates posteroinferior and medial access to the jugular foramen. The purpose of this study was to present the IFTA-A with transcondylar-transtubercular extension and to assess its surgical results. METHODS: A review of patients with tympanojugular paraganglioma who underwent resection through the IFTA-A with transcondylar-transtubercular extension was performed. RESULTS: In all, 39 patients were included in the study. The average follow-up was 23.6 months. Gross total tumor removal was achieved in 87.2% of the cases and there was evidence of recurrence in 5.9% of this group. CONCLUSION: The transcondylar-transtubercular extension of the classic IFTA-A is aimed at making the excision of Fisch type C2 to C4 tympanojugular paragangliomas simpler and safer by drilling out one third of the lateral part of the occipital condyle and removing the jugular process and jugular tubercle.

Successful Onyx embolization of a giant glomus jugulare: case report and review of nonsurgical treatment options.

BACKGROUND: Otorrhagia is an uncommon but severe symptom of patients with large glomus jugulare tumors that erode through the tympanic membrane. In this case report we describe the use of transarterial embolization for long-term palliative management of otorrhagia in a patient with an unresectable glomus jugulare tumor. CASE DESCRIPTION: A 53-year-old woman presented with intermittent otorrhagia 10 years after subtotal resection of a glomus jugulare tumor. Follow-up magnetic resonance imaging showed progressive enlargement of the tumor with significant extension into the posterior fossa. Resection was thought to be impractical; therefore, transarterial embolization was offered as a palliative measure to help reduce the frequency and severity of bleeding episodes. RESULTS: Long-term control of otorrhagia was achieved after three rounds of intra-arterial embolization. In round one, the tumor was embolized from multiple external carotid artery feeding branches by the use of polyvinyl alcohol particles. In round two, the internal carotid artery was sacrificed by embolizing the cavernous and petrous segments with coils. In round three, persistent feeders from the cervical internal carotid artery were embolized with Onyx. In 6 years of clinical follow-up, the patient has had no otorrhagia or new neurological deficits. Serial magnetic resonance imaging revealed there has been no significant interval tumor growth. CONCLUSION: Long-term control of otorrhagia from glomus jugulare tumors can be safely achieved by intravascular embolization with Onyx.

Cough syncope in a 43-year-old woman with glomus jugulare tumor.

We present an unusual case of recurrent cough syncope in a 43-year-old woman, which was initially thought to be seizures. Syncopal episodes were triggered by paroxysms of cough and were characterized by unresponsiveness and myoclonic jerks in her extremities. She had a left-sided glomus jugulare tumor that extended into the posterior cranial fossa with evidence of worsening communicating hydrocephalus on brain imaging. We postulate that bouts of cough produced increased intracranial pressure both by raising intrathoracic and intraabdominal pressures as well as by transient obstruction to cerebrospinal fluid flow secondary to intermittent tonsillar herniation during cough. This resulted in diffuse decrease in cerebral blood flow causing syncope. The patient's syncopal episodes decreased in frequency once an external ventricular drain was placed followed by a ventriculoperitoneal shunt. Search for factors that can increase intracranial pressure seems warranted in patients with recurrent cough syncope.