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.

Preservation of Cranial Nerves Function in Glomus Jugulare Surgery: 2-Dimensional Operative Video.

Owing to their invasive character, extreme vascularity, and critical location, glomus jugulare tumors present a formidable challenge. Techniques have been developed for safe and successful removal of even giant glomus paragangliomas.1-3 Preoperative evaluation including genetic, hormonal, and multiplicity workup4 has enhanced the safety of surgical management, as did modern preoperative embolization by eliminating excessive blood loss.5 Despite these advancements, surgical outcomes of glomus jugulare remain haunted by cranial nerve dysfunction such as facial nerve palsies and hearing loss, with lower cranial nerves dysfunction being the most morbid. These can be avoided by technical maneuvers to preserve the cranial nerves. The external ear canal is not closed to maintain conductive hearing. The facial nerve is not transpositioned and kept inside a bony protective canal. Cranial nerves IX, X, and XI are the most vulnerable because they pass through the jugular foramen ventral to the venous bulb wall. They are preserved by intrabulbar dissection that maintains a protective segment of the venous wall over the nerves. By mastering the anatomy of the upper neck, meticulous dissection is performed to preserve the course of IX, X, XI, and XII.6,7 Ligation of the jugular vein is delayed until the tumor is totally isolated to avoid diffuse bleeding.7 We present the case of a 60-yr-old woman with a glomus jugulare tumor with intradural, extradural, and cervical extension. The technical nuances of cranial nerves preservation are demonstrated. The patient consented to the procedure and publication of her images. Images at 2:12, 2:50, and 3:09 from Al-Mefty and Teixeira,6 with permission from JNSPG.

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.

Preoperative embolization of jugular paraganglioma tumors using particles is safe and effective.

BACKGROUND: Jugular paragangliomas represent a surgical challenge due to their vascularity and proximity to vital neurovascular structures. Preoperative embolization aids in reducing intraoperative blood loss, transfusion requirements, and improves surgical visualization. Several embolization agents have been used. OBJECTIVE: The aim of this study is to evaluate the safety and efficacy of PVA in pre-operative embolization of jugular paragangliomas. METHODS: A retrospective review of all patients who underwent jugular paraganglioma resection with pre-operative embolization between 2000 and 2020 was performed. Pre-operative data including baseline patient and tumor characteristics were documented. Outcomes of preoperative embolization including extent of devascularization and post-embolization complications were recorded. Early and long-term postoperative outcomes were reported. RESULTS: Twenty-nine patients met study criteria with a median age of 38 years. Average tumor size was 3.4±1.8 cm. The most commonly encountered arterial feeder was the ascending pharyngeal artery followed by the posterior auricular artery. More than 50% reduction in tumor blush was achieved in 25 patients (86.2%). None of the patients experienced new or worsening cranial neuropathy following embolization. Gross total or Near total resection was achieved in 13 patients (44.8%). A STR or NTR was chosen in these patients to preserve cranial nerve function or large vessel integrity. Average intraoperative estimated blood loss was 888 ml, 9 patients (31%) required intra-operative transfusion of blood products. Extent of resection and post-operative complications did not correlate with extent of devascularization. CONCLUSION: Pre-operative embolization of jugular paraganglioma tumors with PVA particles is an effective strategy with a high safety profile.

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.

Diameter-Based Volumetric Models May Inadequately Calculate Jugular Paraganglioma Volume Following Sub-Total Resection.

BACKGROUND: As gross total resection of jugular paragangliomas (JPs) may result in cranial nerve deficits, JPs are increasingly managed with subtotal resection (STR) with postoperative radiological monitoring. However, the validity of commonly used diameter-based models that calculate postoperative volume to determine residual tumor growth is dubious. The purpose of this study was to assess the accuracy of these models compared to manual volumetric slice-by-slice segmentation. METHODS: A senior neuroradiologist measured volumes via slice-by-slice segmentation of JPs pre- and postoperatively from patients who underwent STR from 2007 to 2019. Volumes from three linear-based models were calculated. Models with absolute percent error (APE) > 20% were considered unsatisfactory based on a common volumetric definition for residual growth. Bland-Altman plots were used to evaluate reproducibility, and Wilcoxon matched-pairs signed rank test evaluated model bias. RESULTS: Twenty-one patients were included. Median postoperative APE exceeded the established 20% threshold for each of the volumetric models as cuboidal, ellipsoidal, and spherical model APE were 63%, 28%, and 27%, respectively. The postoperative cuboidal model had significant systematic bias overestimating volume (p = 0.002) whereas the postoperative ellipsoidal and spherical models lacked systematic bias (p = 0.11 and p = 0.82). CONCLUSION: Cuboidal, ellipsoidal, and spherical models do not provide accurate assessments of postoperative JP tumor volume and may result in salvage therapies that are unnecessary or inappropriately withheld due to inaccurate assessment of residual tumor growth. While more time-consuming, slice-by-slice segmentation by an experienced neuroradiologist provides a substantially more accurate and precise measurement of tumor volume that may optimize clinical management.

Long term outcomes with linear accelerator stereotactic radiosurgery for treatment of jugulotympanic paragangliomas.

BACKGROUND: Data supporting linear accelerator (linac) stereotactic radiosurgery (SRS) for jugulotympanic paragangliomas (JTPs) come from small series with minimal follow-up. Herein, we report a large series of JTPs with extended follow-up after frameless linac-based SRS. METHODS: JTPs treated with linac-based SRS from 2002 to 2019 with 1+ follow-up image were reviewed for treatment failure (radiographic or clinical progression, or persistent symptoms after SRS requiring intervention) and late toxicities (CTCAE v5.0). RESULTS: Forty JTPs were identified; 30 were treated with a multifraction regimen. Median clinical and radiographic follow-up was 79.7 (interquartile range [IQR] 31.7-156.9) and 54.4 months (IQR 17.9-105.1), respectively, with a median 4.5 follow-up scans (IQR 2-9). Seven-year progression-free survival (PFS) was 97.0% (95% confidence interval 91.1%-100.0%). PFS was similar between single- and multifraction regimens (log rank P = .99). Toxicity was seen in 7.7% (no grade III). CONCLUSIONS: With extended clinical and radiographic follow-up, frameless linac-based SRS provides excellent local control with mild toxicity <8%.

Glomus Vagale Tumor Resection: 2-Dimensional Operative Video.

A 37-yr-old female with prior transient left facial paralysis presented with hearing loss, headaches, and resolved transient right facial paralysis. The neurological examination demonstrated normal facial movement, left hearing loss, and left vocal cord weakness. Magnetic resonance imaging demonstrated a >3 cm left paraganglioma traversing the jugular foramen. After obtaining informed consent from the patient, the tumor was embolized and then resected via a combined left postauricular infratemporal fossa and transcervical approach with cranial nerve monitoring. The ossicles were removed and the vertical segment of the facial nerve was skeletonized. The jugular bulb was identified in the hypotympanum and the petrous carotid artery was exposed. The digastric muscle was reflected inferiorly and the extratemporal facial nerve was identified. The stylomandibular ligament was transected to unlock the exposure to the infratemporal fossa. The external carotid branches were ligated. The vagus nerve and cervical sympathetic chain were infiltrated with tumor, requiring resection. The presigmoid dura and occluded jugular bulb were opened to complete the tumor resection, while preserving the medial wall. Despite anatomic preservation, the glossopharyngeal, accessory, and hypoglossal nerves were postoperatively weak and a facial paralysis recovered after 1 wk. Magnetic resonance imaging at 1 yr demonstrated a clean jugular foramen, although a thin rim of tumor remained around the petrous carotid.

Paraganglioma yugular bilateral: reporte de un caso y revisión de la literatura / Bilateral jugular paraganglioma: case report and review of the literature

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.

Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.

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.

Single-session image-guided robotic radiosurgery and quality of life for glomus jugulare tumors.

BACKGROUND: Limited data are available on the efficacy and impact on the quality of life (Qol) of single-session image-guided robotic radiosurgery (RRS) for glomus jugulare tumors (GJTs). This study investigates the role of RRS in the management of GJTs and reviews the RRS literature. METHODS: We analyzed 53 GJT patients treated with RRS to evaluate the safety, local control, clinical outcome, and Qol assessed by the SF12v2. RESULTS: The local control was 98% at a median follow-up of 38 months. The median tumor volume was 4.3 cc and tumors were treated with a median dose of 16.5 Gy. At the last follow-up, 35 patients had recovered from their symptoms or experienced symptom improvement. Qol analyses showed no significant decline while bodily pain significantly decreased. CONCLUSIONS: RRS is a safe and efficient tool for the treatment of GJTs. Qol of patients after treatment is stable and tends to improve over time.

Right hypoglossal nerve palsy due to jugular paraganglioma / Parálisis del nervio hipogloso derecho debido a paraganglioma yugular

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Jugular foramen tumour resulting in hypoglossal denervation pseudohypertrophy: a rare and significant cause for tongue asymmetry.

Paragangliomas of the jugular foramen are rare. They may present with symptoms of compression of the glossopharyngeal or vagus nerves, or due to secretion of catecholamines from chromaffin cells within the tumour. This case describes a rare presentation of glomus tumour. A 67-year-old patient presented with a 2-month history of right-sided tongue swelling. She was found to have an obvious swelling on the right side of the tongue but no obvious weakness or fasciculation on initial examination. Ultrasound confirmed diffuse muscle swelling, but no lesion within the tongue. Magnetic resonance imaging of the neck revealed an ipsilateral glomus jugulare tumour that extended to the hypoglossal canal, and had resulted in ipsilateral denervation pseudohypertrophy of the lingual muscles. This paper reviews presentation of glomus jugulare tumours and contributes a novel presentation of a rare entity.

Dural arteriovenous fistula associated with a glomus jugulare tumour presenting with only pulsatile tinnitus.

We present the second known case of a dural arteriovenous fistula (DAVF) associated with a glomus jugulare tumour in a 66-year-old man and the first with a presenting symptom of pulsatile tinnitus. The tumour occluded the left internal jugular vein at the bulb. Our patient opted for monitoring, but the tinnitus progressed and became debilitating, prompting him to proceed with embolisation of the tumour. Angiography revealed a DAVF of the left transverse sinus with retrograde flow. Embolisation of 80% of the tumour did not relieve symptoms. The patient returned for embolisation of the DAVF. Occlusion of the DAVF achieved symptomatic relief. A quandary develops during a procedure when the surgeon discovers that another intervention could satisfy the patient, while the patient is under anaesthesia. The higher flow in the DAVF likely causes the tinnitus in those with a patent sigmoid sinus, and embolisation of the DAVF alone could achieve relief.

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.

Case report of a rare glomus jugulare tumor extended within the extracranial internal jugular vein: emphasis on initial ultrasound findings and literature review.

Glomus jugulare tumor (GJT) is a rare benign neoplasm that is located in the skull base; it is impossible for ultrasonography to detect it in most cases. In this article, we present the case of a rare giant GJT, which extended into the internal jugular vein in the neck, initially detected by ultrasonography and confirmed by MRI and pathology. We highlight the initial ultrasonography findings and review the literature to summarize the ultrasonographic features of GJT.

Stereotactic radiosurgery for jugular foramen schwannomas: an international multicenter study.

OBJECTIVE: For some jugular foramen schwannomas (JFSs), complete resection is possible but may be associated with significant morbidity. Stereotactic radiosurgery (SRS) is a minimally invasive alternative or adjunct to microsurgery for JFSs. The authors reviewed clinical and imaging outcomes of SRS for patients with these tumors. METHODS: Nine participating centers of the International Gamma Knife Research Foundation identified 92 patients who underwent SRS between 1990 and 2013. Forty-one patients had prior subtotal microsurgical resection. The median interval between previous surgery and SRS was 15 months (range 0.5-144 months). Eighty-four patients had preexisting cranial nerve (CN) symptoms and signs. The median tumor volume was 4.1 cm3 (range 0.8-22.6 cm3), and the median margin dose was 12.5 Gy (range 10-18 Gy). Patients with neurofibromatosis were excluded from this study. RESULTS: The median follow-up was 51 months (range 6-266 months). Tumors regressed in 47 patients, remained stable in 33, and progressed in 12. The progression-free survival (PFS) was 93% at 3 years, 87% at 5 years, and 82% at 10 years. In the entire series, only a dumbbell shape (extension extracranially via the jugular foramen) was significantly associated with worse PFS. In the group of patients without prior microsurgery (n = 51), factors associated with better PFS included tumor volume < 6 cm3 (p = 0.037) and non-dumbbell-shaped tumors (p = 0.015). Preexisting cranial neuropathies improved in 27 patients, remained stable in 51, and worsened in 14. The CN function improved after SRS in 12% of patients at 1 year, 24% at 2 years, 27% at 3 years, and 32% at 5 years. Symptomatic adverse radiation effects occurred in 7 patients at a median of 7 months after SRS (range 5-38 months). Six patients underwent repeat SRS at a median of 64 months (range 44-134 months). Four patients underwent resection at a median of 14 months after SRS (range 8-30 months). CONCLUSIONS: Stereotactic radiosurgery proved to be a safe and effective primary or adjuvant management approach for JFSs. Long-term tumor control rates and stability or improvement in CN function were confirmed.