Skull Base Paragangliomas Research Articles

Paraganglioma With Intracranial Invasion Demonstrated on 68Ga-DOTATATE PET/CT.

68Ga-DOTATATE PET/CT demonstrates superior sensitivity compared with other cross-sectional and functional imaging techniques in detecting head and neck paragangliomas. It is the primary recommended imaging modality for the evaluation of head and neck paragangliomas, especially in patients suspected of having locally aggressive, multifocal, or metastatic disease, as well as those being considered for targeted peptide-receptor therapy. Herein, we demonstrate the 68Ga-DOTATATE PET/CT findings of a skull base paraganglioma with extensive local invasion and intracranial extension.

The Role of Internal Carotid Artery Stent in the Management of Skull Base Paragangliomas.

Background: After two decades from its introduction in the lateral skull base paraganglioma surgery, the indications and results of preoperative internal carotid artery stenting should be critically assessed. Materials and Methods: Monocentric retrospective study on 26 patients affected by head and neck paragangliomas (19 tympanojugular paragangliomas, 4 carotid body paragangliomas, 3 vagal paragangliomas) preoperatively treated with internal carotid artery stents between 2008 and 2023. The preoperative findings, the intraoperative complications and the final surgical results were analyzed. Results: The stent complication rate was less than 3.1%. Self-expanding highly flexible intracranial nitinol stents were applied. In all cases, it was possible to completely mobilize the internal carotid artery and perform a vascular dissection of the tumor. Gross total tumor resection was possible in 85% of cases. The median follow up was 7.83 y (SD +/- 3.93 y). No local recurrence was observed. Conclusions: The preoperative vascular stent facilitates tumor dissection from the internal carotid artery without risk of vascular damage, helping the surgeon to achieve surgical radicality. The vascular stent is indicated in the case of revision surgeries, circumferential involvement of the vessel and in cases with non-insufficient intracerebral crossflow. Procedural complications, temporary antiplatelet therapy and delay of surgery are the limitations of the procedure.

The Radiosurgery Society Case-Based Discussion of the Management of Head and Neck or Skull Base Paragangliomas with Stereotactic Radiosurgery and Radiotherapy

Stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (FSRT) have been used for the treatment of head and neck or skull base paraganglioma for a considerable time, demonstrating promising local control rates and a favorable safety profile compared with surgical approaches. Nevertheless, the choice of treatment must be carefully tailored to each patient's preferences, tumor location, and size, as well as anticipated treatment-related morbidity. This case-based review serves as a practical and concise guide for the use of SRS and FSRT in the management of head and neck or skull base paragangliomas, providing information on the diagnosis, treatment, follow-up considerations, and potential pitfalls.

Multi-Disciplinary Approach to Skull Base Paragangliomas.

The treatment of skull base paragangliomas has moved towards the use of cranial nerve preservation strategies, using radiation therapy and subtotal resection in instances when aiming for gross total resection would be expected to cause increased morbidity compared to the natural history of the tumor itself. The goal of this study was to analyze the role of surgery in patients with skull base paragangliomas treated with CyberKnife stereotactic radiosurgery (SRS) for definitive tumor control. A retrospective review identified 22 patients (median age 65.5 years, 50% female) treated with SRS from 2010-2022. Fourteen patients (63.6%) underwent microsurgical resection. Gross total resection was performed in four patients for tympanic paraganglioma (n = 2), contralateral paraganglioma (n = 1), and intracranial tumor with multiple cranial neuropathies (n = 1). Partial/subtotal resection was performed for the treatment of pulsatile tinnitus and conductive hearing loss (n = 6), chronic otitis and otorrhea (n = 2), intracranial extension (n = 1), or episodic vertigo due to perilymphatic fistula (n = 1). Eighteen patients had clinical and imaging follow-up for a mean (SD) of 4.5 (3.4) years after SRS, with all patients having clinical and radiological tumor control and no mortalities. Surgery remains an important component in the multidisciplinary treatment of skull base paraganglioma when considering other outcomes besides local tumor control.

THU100 Case Of A Large Suprasellar-clival Mass: Prolactinoma Vs Paraganglioma

Abstract Disclosure: L.N. Madera Marin: None. Y. Rivera-Nieves: None. M.A. Ortiz-Rivera: None. A. Rosado-Burgos: None. L. El Musa Penna: None. W. Medina-Torres: None. L. Sepulveda-Garcia: None. M. Ramirez: None. L.A. Gonzalez-Rodriguez: None. M. Alvarado: None. N. Canales: None. Introduction Prolactin secreting adenomas comprise 30-40% of all pituitary adenomas. Generally, serum prolactin levels correlate with tumor size. Giant prolactinomas measure >4 cm, most have mass related clinical effects. Even large masses respond to cabergoline. Some may require surgical intervention. We present a case of a large pituitary mass associated with elevated prolactin levels, but with an interesting histopathologic analysis. Patient Description 30 y/o male patient with new onset decreased peripheral vision. Retinologist evaluation showed no abnormalities. Laboratories done by PCP found with altered thyroid function tests, but no treatment offered at the time. Two months later, the patient complained of worsening peripheral vision and was found with a left dilated pupil. A head CT scan was performed that showed a large brain mass. Admitted for urgent neurosurgery evaluation and high dose dexamethasone was initiated. ROS was remarkable for weight gain over the past year, and decreased morning erections, although denied decreased libido or erectile dysfunction. MRI showed an infiltrative skull base tumor extending from the clivus to the parasellar area encasing the left cavernous segment of the internal carotid artery with expansion of the sella obliterating the pituitary gland, and extension into the left optic canal. Pre-operative laboratories: Prolactin >877 ng/ml (2-17ng/ml), Cortisol 1.80 mcg/dL (4-22 mcg/dL), TSH 1.45 uIU/ml (0.4-4.0 uIU/ml), Sodium 142mMol/L (135-145mMol/L). Patient underwent left pterional craniotomy. Initial intra-operative pathology: “Positive for Neoplasia”. Post-operative laboratories: Prolactin >877 ng/ml, TSH 0.229 uIU/ml, FSH <0.30 mIU/ml (1.2-7.8 mIU/ml), and LH 0.16 mIU/ml (1.5-9.3 mIU/ml). Case Outcomes/Discussion Central hypothyroidism and hypogonadism confirmed, secondary hypocortisolism could not be assessed due to emergent corticosteroid infusion upon admission. A presumptive diagnosis of a giant prolactinoma was made for which cabergoline therapy was initiated. Final pathologic diagnosis revealed a paraganglioma. Urine fractionated metanephrines and catecholamines were normal. Immunostaining for prolactin requested. Paragangliomas arise from neuroendocrine cells, from either sympathetic or parasympathetic ganglia and can be solitary or multiple. Skull base and neck paragangliomas are usually benign while ∼20% of abdominal paragangliomas (sympathetic) are malignant. Anterior skull base paragangliomas are predominantly located in the sella and are rare but have been described in the literature. Very rare cases of pheochromocytomas or paragangliomas associated to pituitary adenomas have been reported. It has been named as the three P association, thought to be related to succinate dehydrogenase B and D mutations. Moreover, there are no cases in the literature of paragangliomas secreting prolactin. Presentation: Thursday, June 15, 2023

Multidisciplinary Management of Lateral Skull Base Paragangliomas: A 20-Year Experience.

Objectives Paragangliomas of the lateral skull base are rare, largely benign, neuroendocrine tumors. Little evidence exists to support clinicians in the management of these tumors. The present study evaluates considerations in the multidisciplinary workup and management of paragangliomas affecting the lateral skull base. Methods A STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) checklist compliant retrospective review of adult patients with lateral skull base paragangliomas over 20 years (2002-2021) was performed. Patient and tumor data were collected from patient health care records. Results Seventy patients were identified including 21 (30.0%) males and 49 (70.0%) females. The mean length of follow-up was 87.3 months (range: 12-239 months). Twenty-nine (41.4%) patients had Fisch A or B tumors. Overall, 57 patients (81.4%) were offered treatment upfront, with 13 patients (18.6%) initially undergoing active surveillance. Younger age and reduced American Society of Anesthesiologists (ASA) grade was significantly associated with a decision to offer treatment at presentation (both p = 0.03), while all patients with succinate dehydrogenase (SDH) mutations ( n = 10) and tumor secretion ( n = 4) were offered treatment. Patients with Fisch A/B tumors ( p = 0.01), cranial neuropathies ( p = 0.01), and smaller tumors ( p = 0.01) were more likely to undergo surgical resection. At the time of the last follow-up, the proportion of patients with a cranial neuropathy was notably lower in the observation (3/12, 25.0%) and radiotherapy (1/6, 16.7%) groups. Conclusion In our series, younger, fitter patients with SDH mutations, cranial neuropathies, or tumor secretion were more likely to be offered upfront treatment. There was a low incidence of new cranial neuropathy in the patients selected for observation during long-term follow-up.

Multiple myeloma mimicking metastatic skull base paraganglioma in [68Ga]Ga-DOTATOC-PET/CT and 2-[18F]FDG-PET/CT imaging.

Multiple myeloma mimicking metastatic skull base paraganglioma in [68Ga]Ga-DOTATOC-PET/CT and 2-[18F]FDG-PET/CT imaging.

Surgical Candidacy in Skull Base Paragangliomas: An Institutional Experience.

Objective The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. Materials and Methods This was a retrospective observational study conducted in patients who presented with skull base paragangliomas between January 2017 and December 2019. Primary data, including indication for surgery, the approach used, the extent of resection, complications, and postoperative lower cranial nerve status were studied. Results A total of 21 cases of skull base paragangliomas were analyzed, including seven cases of tympanic paraganglioma, 10 cases of jugular foramen paraganglioma, three cases of multiple paragangliomas, and one case of vagal paraganglioma. Indications for surgery were young age, bleeding from ear, neck mass with upper aerodigestive pressure symptoms, lower cranial nerve paralysis, and patients with intracranial pressure symptoms. Total excision was done in 11 patients, near-total excision in five patients, subtotal in three patients, and surgery was not done in two patients. Facial nerve paralysis was the most common complication observed, followed by bleeding and flap necrosis. Radiotherapy was considered as adjuvant treatment wherever indicated. Conclusion A thorough knowledge and understanding of the pathophysiology of the skull base paragangliomas and its management strategies can help to achieve excellent results in terms of tumor clearance and reduction in complications. A multidisciplinary team approach and meticulous skull base surgical techniques have a significant role to play in the management of paragangliomas, especially in developing countries where availability of radiosurgery is still a challenge.

Onyx embolization of skull base paragangliomas: a single-center experience.

Skull base paragangliomas are highly vascular tumors that are often embolized before surgical resection; however, the safety and efficacy of preoperative embolization using an ethylene vinyl alcohol copolymer (Onyx; Medtronic, Dublin, Republic of Ireland) in these tumors is unknown. This retrospective cohort study evaluated patient outcomes after preoperative embolization of skull base paragangliomas using Onyx. We retrospectively analyzed data from all patients with skull base paragangliomas who underwent preoperative Onyx embolization at our institution (January 01, 2005-December 31, 2017). Patient, tumor, embolization, and outcomes data were extracted by reviewing inpatient and outpatient clinical and imaging records. Seven patients were studied (5/7 [71%] female), 6 with glomus jugulares and 1 with a glomus vagale. The median age was 52years, and the most common presenting symptom was cranial neuropathy (6/7 [86%]). The tumor vascular supply was from the ascending pharyngeal artery in all 7 cases (100%) with additional feeders including the occipital artery in 5 (71%); internal carotid artery in 3 (43%); middle meningeal, vertebral, and internal maxillary artery each in 2 (29%); and posterior auricular artery in 1 (14%). The median postembolization tumor devascularization was 80% (range, 64-95%). The only postembolization complication was a facial palsy in 1 patient. Preoperative embolization with Onyx affords excellent devascularization for the majority of skull base paragangliomas, and it may facilitate resection of these hypervascular lesions. The advantages provided by Onyx with respect to penetration of intratumoral vessels must be weighed against the risk of cranial neuropathy.

18F-Choline PET/CT Detected Skull Base Paraganglioma

We present a case of a right skull base paraganglioma detected using F-choline PET/CT in a 63-year-old man. The F-choline PET/CT scan was performed to assess a known prostate cancer. In addition, the scan demonstrated a mildly choline-avid (SUVmax, 3.8) tumor within the carotid sheath of the right skull base, extending through the jugular foramen to the cerebellomedullary and cerebellopontine angles. F-choline may provide a superior alternative to FDG in imaging paragangliomas of the skull base because, unlike FDG, there is no significant F-choline uptake in the adjacent brain.

18F-FDOPA PET/CT Combined with MRI for Gross Tumor Volume Delineation in Patients with Skull Base Paraganglioma.

In this simulation study, we assessed differences in gross tumor volume (GTV) in a series of skull base paragangliomas (SBPGLs) using magnetic resonance imaging (MRI), 18F-dihydroxyphenylalanine (18F-FDOPA) combined positron emission tomography/computed tomography (PET/CT), and 18F-FDOPA PET/MRI images obtained by rigid alignment of PET and MRI. GTV was delineated in 16 patients with SBPGLs on MRI (GTVMRI), 18F-FDOPA PET/CT (GTVPET), and combined PET/MRI (GTVPET/MRI). GTVPET/MRI was the union of GTVMRI and GTVPET after visual adjustment. Three observers delineated GTVMRI and GTVPET/MRI independently. Excellent interobserver reproducibility was found for both GTVMRI and GTVPET/MRI. GTVPET and GTVMRI were not significantly different. However, there was some spatial difference between the locations of GTVMRI, GTVPET, and GTVPET/MRI. The Dice similarity coefficient median value was 0.4 between PET/CT and MRI, and 0.8 between MRI and PET/MRI. The combined use of PET/MRI produced a larger GTV than MRI alone. Nevertheless, both the target-delivered dose and organs-at-risk conservancy were respected when treatment was planned on the PET/MRI-matched data set. Future integration of 18F-FDOPA PET/CT into clinical practice will be necessary to evaluate the influence of this diagnostic modality on SBPGL therapeutic management. If the clinical utility of 18F-FDOPA PET/CT and/or PET/MRI is confirmed, GTVPET/MRI should be considered for tailored radiotherapy planning in patients with SBPGL.

Advances in the Management of Skull Base and Carotid Paragangliomas

Advances in the Management of Skull Base and Carotid Paragangliomas

Protontherapy of head and neck paragangliomas: A monocentric study

PurposeThe aim of this study was to assess efficacy and safety of proton beam therapy of paragangliomas of the head and neck, rare benign tumours developed close to crucial structures such as cranial nerves and vascular tissues. Patients and methodsTen patients with a paraganglioma of the head and neck were treated from 2001 to 2014 with image-guided proton therapy. Neurological and ear nose throat symptoms were collected in addition to audiometric testing, before and after the treatment. Acute and late toxicities were assessed according to the Common Terminology Criteria for Adverse Events (CTCAE) v4. ResultsMedian age at diagnosis was 52.6years (range: 18.2–65.8years). Proton therapy was the exclusive treatment in six patients and four patients had a postoperative radiotherapy. Median dose was 50.4Gy relative biological effectiveness (RBE; range: 45.0–67.0Gy). With a median follow-up of 24.6months (range: 6.7–46.2 months), local tumour control rate was 100% (stable, n=10). No upper grade 2 acute toxicity was reported. To the latest news, seven patients had controlled symptoms (improved, n=1, stabilized, n=6). One patient out of seven with initial tinnitus had a decrease in his symptoms, while the six other patients had a sustained stabilization. ConclusionProton beam therapy is an effective and well-tolerated treatment modality of skull base paragangliomas, with documented functional benefit. A longer follow-up is planned in order to assess local control and long-term toxicities.

Specific features of surgical treatment of lateral skull base paragangliomas

The objective of this study is to increase the efficacy of lateral skull base paragangliomas surgical treatment. In this article the authors present the result of 20 cases of surgery in patients with lateral skull base paragangliomas. Five patients with C2–С3 tumors (according to the classification suggested by U. Fisch and D. Mattox) underwent surgery using type A infratemporal fossa approach. Good functional results have been obtained, the function of the lower cranial nerves has been saved. In 5 patients the facial nerve palsy restored to the level 3 according to House-Brackmann classification system. No cases of tumor recurrence in the postoperative period (the maximum follow-up period was 16 month) have been observed. Using the infratemporal fossa approach during lateral skull base paragangliomas excision provides complete removal of the neoplasm, visualization and avoidance of the VII, IX–XII cranial nerves injure and prevention of tumors intracranial extension and the internal carotid artery damage.

Skull Base Paraganglioma: Diagnosis, Treatment of New Cases, and an Overview of the Literature

Objective: The purpose of this presentation is to report on new cases of paragangliomas treated in the University ENT and Neurosurgery Department in Utrecht. Moreover to compare the existing evidence concerning the efficacy and safety of surgery, radiotherapy or wait-and-scan policy.

Initial experience with dual-lumen balloon catheter injection for preoperative Onyx embolization of skull base paragangliomas.

OBJECT Paragangliomas are highly vascular head and neck tumors for which preoperative embolization is often considered to facilitate resection. The authors evaluated their initial experience using a dual-lumen balloon to facilitate preoperative embolization in 5 consecutive patients who underwent preoperative transarterial Onyx embolization assisted by the Scepter dual-lumen balloon catheter between 2012 and 2014. OBJECT The authors reviewed the demographic and clinical records of 5 patients who underwent Scepter-assisted Onyx embolization of a paraganglioma followed by resection between 2012 and 2014. Descriptive statistics of clinical outcomes were assessed. RESULTS Five patients (4 with a jugular and 1 with a vagal paraganglioma) were identified. Three paragangliomas were embolized in a single session, and each of the other 2 were completed in 3 staged sessions. The mean volume of Onyx used was 14.3 ml (range 6-30 ml). Twenty-seven vessels were selectively catheterized for embolization. All patients required selective embolization via multiple vessels. Two patients required sacrifice of parent vessels (1 petrocavernous internal carotid artery and 1 vertebral artery) after successful balloon test occlusion. One patient underwent embolization with Onyx-18 alone, 2 with Onyx-34 alone, and 1 with Onyx-18 and -34. In each case, migration of Onyx was achieved within the tumor parenchyma. The mean time between embolization and resection was 3.8 days (range 1-8 days). Gross-total resection was achieved in 3 (60%) patients, and the other 2 patients had minimal residual tumor. The mean estimated blood loss during the resections was 556 ml (range 200-850 ml). The mean postoperative hematocrit level change was -17.3%. Two patients required blood transfusions. One patient, who underwent extensive tumor penetration with Onyx, developed a temporary partial cranial nerve VII palsy that resolved to House-Brackmann Grade I (out of VI) at the 6-month follow-up. One patient experienced improvement in existing facial nerve weakness after embolization. CONCLUSIONS Scepter catheter-based Onyx embolization seems to be safe and effective. It was associated with excellent distal tumor vasculature penetration and holds promise as an adjunct to conventional transarterial Onyx embolization of paragangliomas. However, the ease of tumor penetration should encourage caution in practitioners who may be able to effect comparable improvement in blood loss with more conservative proximal Onyx penetration.

A Retrospective Analysis of Tumor Volumetric Responses to Five-Fraction Stereotactic Radiotherapy for Paragangliomas of the Head and Neck (Glomus Tumors)

Background: Skull base paragangliomas (SBP) are locally expansile tumors that can be treated with stereotactic radiotherapy with favorable results. This report describes the results of 31 patients with SBP treated with CyberKnife radiotherapy delivering a total dose of 25 Gray in five fractions. Methods: All patients treated with five-fraction CyberKnife radiotherapy at a single institution were identified between 2007 and 2013. Tumor volumetric analyses were performed to assess responses to radiotherapy. Results: Median follow-up was 24 months with a range of 4-78 months. Local control and overall survival were 100%. Of the 20 patients who presented with tinnitus, 12 reported improvement (60%), of whom 6 reported complete resolution. There was a 37.3% reduction in tumor volume among all patients (p = 0.16). On subset analysis of patients with ≥24 months of follow-up, tumor volume decreased 49% (p = 0.01). The rate of grade 1-2 toxicity was 19%, with no grade 3 or worse toxicity. Conclusion: A five-fraction CyberKnife-based stereotactic radiotherapy approach is safe and efficacious for the management for patients with SBP. Our findings suggest the potential use of this strategy as a definitive or salvage treatment option for SBP.

Microsurgical treatment of large and giant tympanojugular paragangliomas.

Background:Tympanojugular paragangliomas (TJPs) are benign, highly vascularized lesions located in the jugular foramen with frequent invasion to the temporal bone, the upper neck, and the posterior fossa cavity. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there is no consensus regarding the optimal management while minimizing treatment-related morbidity. In this study, we assessed the interdisciplinary microsurgical treatment and outcome of large TJP collected at a single center.Methods:Out of 54 patients with skull base paraganglioma, 14 (25%) presented with large TJP (Fisch grade C and D). Posterior fossa involvement was present in 10 patients (Fisch D). Eleven patients presented with hearing loss, two patients with mild facial nerve palsy, and two patients with lower cranial nerve deficits. Two other patients with previous surgery presented with tumor regrowth.Results:Preoperative embolization was performed in 13 cases. Radical tumor removal was possible in 10 patients. Hearing was preserved in four patients with normal preoperative audiogram. The facial nerve was preserved in all patients. Temporary facial nerve palsy occurred in two patients and resolved in long-term follow-up. In three patients, preexisting facial nerve palsy remained unchanged. Persistent vocal cord palsy was present in three patients and was treated with laryngoplasty. The global recovery based on the Karnofsky performance scale was 100% in 10 patients and 90% in 4 patients.Conclusion:Preoperative embolization and interdisciplinary microsurgical resection are the preferred treatment for selected patients due to high tumor control rates and good long-term results.

Microsurgery of Skull Base Paraganglioma

Microsurgery of Skull Base Paraganglioma

Book Review: Microsurgery of Skull Base Paragangliomas

Book Review: Microsurgery of Skull Base Paragangliomas