Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

Glomus jugulare in a pediatric patient: case report and literature review.

PURPOSE: The jugular and tympanic glomus are rare neoplasms in the general population, being even more uncommon in the pediatric population. There is considerable morbidity associated with both disease and treatment. Treatment is essentially surgical, carried out in recent years in a multidisciplinary manner using preoperative embolization associated with microsurgery and eventually adjuvant radiotherapy. The outcome depends on the location of the lesion and its proximity to noble structures in addition to multidisciplinary monitoring in the postoperative period. METHODS: In this article, a literature review was carried out in the PubMed database, finding reports from 17 patients diagnosed with the disease. Only articles in English were considered. RESULTS: Moreover, we reported a case of a 14-year-old patient diagnosed with jugulotympanic glomus who underwent radical surgical treatment of the lesion. CONCLUSION: This is a rare case of jugulotympanic glomus in a pediatric patient, who underwent surgical treatment associated with multidisciplinary therapy, with a favorable postoperative outcome.

Three-Corridors Procedure to Operate in the Infralabyrinthine Cervico-Jugulo-Carotico-Tympanic Area.

INTRODUCTION: The aim of the study was to present the results of our experience in three-corridors procedures applied for the tumors and inflammatory lesions of the infralabyrinthine cervico-jugulo-carotico-tympanic area. METHODS: The lesions located in the infralabyrinthine cervico-jugulo-carotico-tympanic area were operated in 13 patients using the 3-corridors technique. The anatomical and functional integrity of the external and middle ears and the facial nerve (FN) could be preserved. RESULTS: The diagnoses were glomus jugulare, infralabyrinthine petrous bone cholesteatoma, jugular foramen schwannoma, and giant-cell tumor. The follow-up duration ranged from 2 to 24 months. No tumor recurrence or growth was encountered in the follow-up. The operations were uneventful. Total surgical excision could be achieved in 10 patients. A second-stage retrosigmoid approach was performed for the total removal of the intracranial tumor remnant in two patients. A wait-and-scan policy has been considered in one patient who had partial resection for a glomus jugulare tumor. CONCLUSION: Three-corridors procedure seems to be a useful technique to operate in the infralabyrinthine, cervico-jugulo-carotico-tympanic area as it takes the advantage of hearing preservation, preservation of the anatomic and functional integrity of the external and middle ear structures as well as the FN.

[Low-grade (polymorphous) adenocarcinoma of the middle ear mimicking a jugulotympanic paraganglioma. German version]. / (Polymorphes) Low-Grade-Adenokarzinom des Mittelohrs als Imitator eines jugulotympanalen Paraganglioms.

A 64-year-old female patient presented with otalgia and hearing loss in the right ear. On otoscopy, the right tympanic membrane was highly vascularized and bulged into the anteroinferior quadrant. High-resolution computed tomography revealed an osteolytic lesion with occupation of the hypotympanum extending into the petrous apex and right parapharyngeal space as well as infiltration of the wall of the right internal carotid artery. MRI strengthened the suspicion of a jugulotympanic paraganglioma. The biopsy material obtained through exploratory tympanotomy was assessed as a low-grade polymorphic adenocarcinoma. The tumor was treated with definitive chemoradiotherapy. Posttherapeutic imaging after 4 months did not show any evidence of tumor progression.

Treatment of patients with glomus jugulare tumours (GJT) and its subjective effect on quality of life (QoL) measures.

Cerebellopontine angle (CPA) tumours account for 6-10% of intracranial tumours. The most common CPA tumours are vestibular schwannomas (VS), also known as acoustic neuromas, benign tumours of the vestibulocochlear nerve. Less common but symptomatic skull base lesions are glomus jugulare tumours (GJT), of which approximately 40% are identified as CPA tumours. Initial symptoms for GJT may include hearing loss and tinnitus and progress to various cranial nerve dysfunctions. Three well-accepted treatment modalities for such tumours include surgical resection, radiotherapy and/or conservative management employing serial MR or CT imaging. Patients' quality of life may be impacted by different treatment methods, so treatment decisions should be client centered.

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.

[A glomus tumour of the bulb of the jugular vein: complications during the postoperative period]. / Glomusnaia opukhol' lukovitsy iaremnoi veny: oslozhneniia posleoperatsionnogo perioda.

The article presents a clinical case that reflects the difficulties of postoperative management of patients after the removal of the jugular glomus tumor.

Jugulotympanic paragangliomas in southern Finland: a 40-year experience suggests individualized surgical management.

Treatment of jugulotympanic paragangliomas (JTPGLs) remains challenging with no clear guidelines for management or follow-up. The aim of this retrospective case-note study was to assess long-term results of operatively and conservatively managed JTPGLs between years 1974-2013. A total of 36 patients with JTPGLs were identified. Clinical characteristics and management outcomes of patients were reviewed. Data were extracted on demographics, symptoms, timing of diagnosis, tumor location and size, embolization, and management, including pre- and post-operative imaging, analysis of operative techniques, and follow-up. Pulsatile tinnitus and hearing loss were the most common presenting symptoms. Thirty-four (94 %) patients were treated with primary surgical therapy and two (6 %) with radiotherapy. The surgical approaches included endaural approach for Fisch Class A tumors and a variety of approaches for Fisch Class B-D tumors with an increasing predilection for function-preserving surgery. Eight (24 %) patients received subtotal resection. Five (15 %) patients had a local recurrence within 10 years after primary surgery. Two (6 %) patients suffered a permanent cranial nerve (CN) deficit after primary surgery. We advocate radical surgery when tumor resection is possible without compromising CNs. Function-preserving surgery with at least a 10-year follow-up for Fisch Class B-D tumors should be considered if CNs are in danger.

[Endovascular treatment of complex vascular pathology in neck and brain region. Case report]. / Komplex nyaki és agyi érpatológia endovascularis kezelése.

Authors present complex multistage endovascular treatment of female patient with an intradural infiltrative, inoperable tumor of the glomus jugulare, a stenosis of the internal carotid artery and an aneurysm of middle cerebri artery. In the literature our case report is the first announcement in which three pathology coexist ipsilateral in one patient and the patient has been treated step by step by endovascular procedures. Beside the endovascular treatment steps they display glomus jugulare tumor's symptoms, staging, treatment options and also the current treatment guidelines of internal carotid artery stenosis and intracranial aneurysms. Orv Hetil. 2017; 158(18): 706-710.

Microsurgical management of primary jugular foramen meningiomas: a series of 22 cases and review of the literature.

This study summarized clinical manifestations, surgical management, histological grading, and long-term outcome of jugular foramen meningiomas (JFMs). Retrospective study was performed in 22 consecutive patients with primary JFMs from January 2004 to October 2010, enrolling 10 men and 12 women with average age of 39.4 (14-57 years). The most common initial symptom is hearing disorder, followed by lower cranial nerve dysfunctions. The tumor was classified into type I (intracranial) in 1, type II (intracranial) in 15, type IV (intracranial-extracranial) in 6, and none type III (extracranial). Surgical approaches mainly included retrosigmoid in 7, far lateral in 10, and juxtacondylar in 5, with some variations. The gross total resection was achieved in 15 cases and subtotal resection in 7. Fourteen patients (63.6 %) developed new or worse neurological deficits immediately after operation, of whom 11 (78.6 %) got alleviation. Postoperatively, keeping airway patency and prevention from aspiration pneumonia is very important. Nasotracheal intubation is much more tolerated than orotracheal intubation for postoperative patient management. WHO grade 2 was found in four cases (18.2 %) and grade 3 in one. During the average time of follow-up in 83.2 months, only one (grade 3) died of tumor regrowth 20 months after surgery and radiosurgery. Five of 17 patients of grade 1 developed tumor regrowth. Radiosurgery provides a good tumor control for tumor regrowth in grade 1, or postoperative grade 2 tumor. In conclusion, JFMs has a favorable long-term overall survival; however, neurological preservation is still challenging, especially low cranial nerves.

An intermediate term benefits and complications of gamma knife surgery in management of glomus jugulare tumor.

Glomus tumors are rare skull base slow-growing, hypervascular neoplasms that frequently involve critical neurovascular structures, and delay in diagnosis is frequent. Surgical removal is rarely radical and is usually associated with morbidity or mortality. Gamma knife surgery (GKS) has gained an increasing dependable role in the management of glomus jugulare tumors, with high rate of tumor growth control, preserving or improving clinical status and with limited complications. This study aims to evaluate intermediate term benefits and complications of gamma knife surgery in management of twenty-two patients bearing growing glomus jugulare tumors at the International Medical Center (IMC), Cairo, Egypt, between 2005 and 2011. The mean follow-up period was 56 months (range 36-108 months); there were 3 males, 19 females; mean age was 43.6 years; 15 patients had GKS as the primary treatment; 2 patients had surgical residuals; 2 had previous radiation therapy; and 3 previously underwent endovascular embolization. The average tumor volume was 7.26 cm3, and the mean marginal dose was 14.7 Gy. Post gamma knife surgery through the follow-up period neurological status was improved in 12 patients, 7 showed stable clinical condition and 3 patients developed new moderate deficits. Tumor volume post GKS was unchanged in 13 patients, decreased in 8, and showed tumor regrowth in 1 patient. Tumor progression-free survival in our studied patients was 95.5% at 5 and 7 years of the follow-up period post GKS. Gamma knife surgery could be used safely and effectively with limited complications as a primary management tool in the treatment of glomus jugulare tumors controlling tumor growth with preserving or improving clinical status especially those who do not have significant cranial or cervical extension, elderly, and surgically unfit patients; moreover, it is safe and highly effective as adjuvant therapy as well.

Observation and partial targeted surgery in the management of tympano-jugular paraganglioma: a contribution to the multioptional treatment.

The objective of this study was to assess the value of a limited tumor excision in tympano-jugular paragangliomas treated with observation with the goal of preventing, or repairing, a neural (VII cranial nerve) or brain damage and continue an otherwise correct observation. This is a retrospective case review. Each patient was submitted to a complete neuro-radiological work-up for diagnosis including CT, MRI, and angiography if needed of tympano-jugular paraganglioma class C. All the cases were submitted to observation and in 16 cases partial targeted surgery was performed. In 10 cases, the procedure involved a targeted removal of the tumor growing in contact with, or invading, the Fallopius. In four cases, the procedure was a petro-occipital trans-sigmoid approach to remove the intradural portion of tumor producing the picture of brain stem compression. In two cases, there was the excision of the bleeding tumor surfaces in the outer ear canal. Follow-up ranged from 4 to 20 years. Success was considered the maintenance of observation without the change of class of tumor extension. In 13 over 16 cases, the observation could be continued, as it still was the more favorable option between natural and surgical morbidity. In the other three cases, a newly installed paralysis of the 9-10 cranial nerves due to tumor growth involved a correction of the strategy to a radical procedure. Partial targeted surgery was directed to cases submitted to observation. It allowed to prevent, or repair, an impending, or actual damage to the facial nerve or the brain stem and to continue the abstentional treatment by keeping the balance between natural and therapy morbidity in favor of observation.

Diagnosis and management of multiple paragangliomas of the head and neck.

Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983-2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4-21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2-18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.

Current perspectives in the management of glomus jugulare tumors.

Glomus jugulare tumors are benign but locally aggressive tumors. Their location, spread to adjacent areas and biological behavior have been extensively studied and reported. However, controversy exists regarding the role of surgery and stereotactic radiosurgery. The optimal plan of management of the tumor in close proximity to the facial and lower cranial nerves, the internal carotid and vertebral arteries, the venous sinuses, and the neuraxis is still nebulous. This review will discuss the differing viewpoints and attempts to propose a rational strategy in dealing with these tumors.

Comparative results of infratemporal fossa approach with or without facial nerve rerouting in jugular fossa tumors.

Jugular fossa tumors are uncommon diseases. During the surgery and due to the interposition of the facial nerve in the tumor approach, the facial nerve must be elevated from the fallopian canal and placed permanently into an anterior position. Although this maneuver provides a wide exposure, most of the patients suffer a long-term total or partial facial palsy. The purpose of this article is to check whether the infratemporal fossa approach without transposition of the facial nerve is equivalent to the approach with rerouting of the facial nerve regarding postsurgical morbidity. The clinical records of 52 patients who underwent an infratemporal fossa approach were reviewed in which 34 patients were segregated into two comparable groups regarding the presence or absence of transposition of the facial nerve. There were 19 women and 15 males. The majority of the patients (73%) had jugular paragangliomas. The mean follow-up of the full series was 66 months. It was statistically significant that the worst facial nerve function at hospital discharge was in the patients who underwent facial nerve transposition (p = 0.001). Equally the facial nerve function in the no-rerouting group 1 year after the surgery was significantly much better than in the rerouting group (p = 0.003). Regarding to survival, recurrence or complications no significant differences were observed between both groups. Our study suggests that most of cases avoiding facial nerve transposition allow significant better functional results thereof without affecting other parameters such as recurrence, complications or survival.

Temporal bone malignancies involving the jugular foramen: diagnosis and management.

PURPOSE: To describe the diagnosis, management and outcomes of temporal bone malignancies involving the jugular foramen. MATERIALS AND METHODS: The charts of 12 patients with temporal bone malignancies involving the jugular foramen were reviewed and analyzed. RESULTS: The most frequent symptoms of temporal bone malignancies involving the jugular foramen were hearing loss (75%), otorrhea (66.67%) and otalgia (66.67%). In addition to the jugular foramen, the most invaded structure was the external auditory canal (91.67%), followed by the middle ear (75%) and facial nerve (58.33%). Four cases (33.33%) were misdiagnosed either with glomus jugulare tumor, Bell's palsy or chronic otitis media. Two patients underwent lateral temporal bone resection, whereas 10 patients underwent subtotal temporal bone resection. The mean follow-up time was 23.83 months (range, 2-85 months). The 2-year disease-free survival rate was 42.3%. CONCLUSIONS: Temporal bone malignancies involving the jugular foramen had a poor prognosis, especially in patients with squamous cell carcinoma. Misdiagnoses including glomus jugulare tumor, Bell's palsy and chronic otitis media were frequently encountered. Temporal bone malignancies involving the jugular foramen can be resected, and the choice of the surgical approach should be individualized.

Preoperative protective stenting of the internal carotid artery in the management of complex head and neck paragangliomas: long-term results.

OBJECTIVES: To investigate the long-term results of preoperative stenting of the internal carotid artery (ICA) in complex head and neck paragangliomas (HNP) as well as to report on indications and technical details of the procedure. METHOD: A comprehensive retrospective review of patients affected by HNP, consecutively operated on and preoperatively treated with stenting of the ICA in a quaternary referral skull base center, was performed. RESULTS: Nineteen patients affected by complex HNP were identified, on whom 21 preoperative stenting procedures were performed. The mean follow-up period after stent insertion was 53.8 months; the patients' age ranged from 33 to 56 years. Fourteen patients were affected by tympanojugular paragangliomas, 4 by vagal paragangliomas and 1 by bilateral carotid body tumors. Five patients presented with recurrent tumors, while 7 presented with multiple or bilateral HNP. There were no complications associated with endovascular procedures. Total tumor removal was accomplished in 52.4% of the cases with 1 recurrence. An advanced stage was the main factor conditioning total removal. Clinical control was obtained in 80% of the patients with residual disease. Total tumor removal from and around the ICA was obtained in 95.2% of the cases. Long-term stent patency was evident in 20 of 21 cases. CONCLUSIONS: Preoperative stenting of the ICA represents a safe and effective procedure in selected cases, obviating the need for balloon occlusion or bypass procedures and reducing the risk of intraoperative vascular injury.

Less invasive transjugular approach with Fallopian bridge technique for facial nerve protection and hearing preservation in surgery of glomus jugulare tumors.

For the past three decades, surgery of glomus jugulare tumors (GJTs) has been characterized by extensive combined head and neck, neuro-otologic, and neurosurgical approaches. In recent years, the authors have modified the operative technique to a less invasive approach for preservation of cranial nerves while achieving satisfactory tumor resection. We evaluated and compared the clinical outcomes of our current less invasive approach with our previous more extensive procedures. The clinical records of 39 cases of GJT surgically treated between 1992 and 2011 were retrospectively reviewed. The less invasive transjugular approach with Fallopian bridge technique (LI-TJ) was used for the most recent five cases. The combined transmastoid-transjugular and high cervical (TM-HC) approach was performed in 30 cases, while four cases were treated with a transmastoid-transsigmoid approach with facial nerve translocation. Operative technique, extent of tumor resection, operating time, hospital stay, and morbidity were examined through the operative records, and a comparison was made between the LI-TJ cases and the more invasive cases. No facial nerve palsy was seen in the LI-TJ group while the TM-HC group demonstrated six cases (17.6%) of facial palsy (House-Brackmann facial nerve function grading scale grade II and III). The complication rate was 0 % in the LI-TJ group and 16.7% in the more invasive group. The mean operative time and hospital stay were shorter in the LI-TJ group (6.4 h and 4.3 days, respectively) compared with the more invasive group (10.7 h and 8.0 days, respectively). The LI-TJ approach with Fallopian bridge technique provided adequate tumor resection with cranial preservation and definitive advantage over the more extensive approach.