[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.

Low-grade (polymorphous) adenocarcinoma of the middle ear mimicking a jugulotympanic paraganglioma.

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.

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.

Surgery, radiotherapy or a combined modality for jugulotympanic paraganglioma of Fisch class C and D.

OBJECTIVES: To identify the risks associated with surgery, radiotherapy or a combined treatment approach for Fisch class C and D jugulotympanic paraganglioma, in order to develop an individualised approach for each patient depending on Fisch class, age, mutation presence, tumour size growth rate and presenting symptoms. DESIGN: A retrospective multicenter cohort study with all patient records of patients with a head and neck paraganglioma in the Radboudumc, Nijmegen and the St. Elisabeth Hospital, Tilburg, the Netherlands. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: We found highest local control rates after tumour debulking with postoperative radiotherapy in case of residual tumour growth, referred to as the combined treatment group, (100%; n = 19), which was significantly higher than the surgical group (82%; n = 17; P = 0.00), but did not differ from the radiotherapy group (90%; n = 29). There were significantly less complications in the radiotherapy group, when compared to surgery (63 vs 27%; P = 0.002) and the combined group (44 vs 27%; P = 0.016). Furthermore,: using a logistic regression model, we found that pretreatment tumour growth was a negative predictor for post-treatment cranial nerve function recovery (OR = 50.178, P = 0.001), reducing the chance of symptom recovery (67.3% vs 35.7%) post-treatment. CONCLUSIONS: Radiotherapy should be the treatment of choice for the elderly. For younger patients, tumour debulking should be considered, with potential radiotherapy in case of residual tumour growth.

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.

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.

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.

[New progress in the diagnosis and treatment of jugular paragangliomas].

Jugular paraganglioma is the most common neoplasms in the jugular foramen. It is a slow-growing wiht abundant blood supply, and is intimately associated with critical neurovascular structures at the skull base. In this paper, the latest advance in pathophysiology, surgical treatment, radiotherapy were reviewed based on previous literature, providing reference for clinical diagnosis, treatment and future research.

Glomus jugulare: a rare cause of facial nerve palsy.

PURPOSE: To report a case of an unusual presentation of a glomus jugulare tumour. DESIGN: Case report. RESULTS: Glomus jugulare is a very rare brain tumour that usually presents with tinnitus, hearing loss, dysphagia and hoarseness. We report a case where this extremely rare diagnosis presented quite differently, with ipsilateral proptosis and subsequent facial nerve palsy. Furthermore, the sibling of the presented case had also been diagnosed with the same tumour. CONCLUSIONS: There have only been a few case reports in the literature describing facial nerve palsy in the context of a glomus jugulare tumour. This case highlights that although paragangliomas are exceedingly rare causes of facial palsy, they should be included in the differential diagnosis.

Metastatic Malignant Glomus Jugulare Tumor: A Rare Case Report with Clinical Manifestations and Treatment Approach.

BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.

Jugular foramen paragangliomas: management, outcome and avoidance of complications in a series of 75 cases.

Jugular foramen paragangliomas are rare skull base tumours posing multiple complex diagnostic and management problems. We did a study to evaluate surgical technique, outcome and complications in 75 cases of tumours treated by multidisciplinary approach (i.e. combined neurosurgery, neuroradiology, ear, nose and throat surgery and intensive care unit team). Retrospective study on 75 consecutive patients with jugular foramen paragangliomas treated surgically from 1989 to 2005. Preoperative balloon occlusion test was performed in all patients as well as embolization (100%). A combined limited infratemporal and juxtacondylar approach was used in all patients. Gross total resection was achieved in 59 patients (78.7%). The most common complication was represented by lower cranial nerve deficits in five patients (6.6%), which was only temporary in three. Postoperative facial nerve weakness occurred in five cases (6.6%) and resolved in three of them. The remaining two patients underwent facial nerve reconstruction by hypoglossal/facial nerve anastomosis. Four patients (5.3%) had a postoperative cerebrospinal fluid leak, which was successfully treated by lumbar drainage. Two patients (2.7%) died because of complications related to surgical injury of lower cranial nerves: one patient developed aspiration pneumonia and septicemia and the second one developed a large cervico-bulbar hematoma that led to severe respiratory distress and ultimately global cerebral hypoxia. Paragangliomas are rare and complex skull base lesions that may be managed with low morbidity and mortality if a multidisciplinary approach is considered. Facial and lower cranial nerve postoperative deficits can be limited.

Gamma knife radiosurgery for glomus jugulare and tympanicum.

OBJECTIVE: To establish the role of gamma knife radiosurgery (GKS) as a treatment strategy for glomus jugulare and tympanicum. METHOD: A retrospective review of 14 glomus tumors, including 11 glomus jugulare and 3 glomus tympanicum tumors, which were treated by GKS in Taipei Veterans General Hospital from 1993 to 2009, was conducted. Two of these cases had undergone prior surgery with partial tumor resection, and the other 12 cases received primary treatment with GKS after a thorough neuroimaging and cerebral angiography. The tumor volume ranged from 6.5 to 22.1 ml. The maximum dose at the tumor center ranged from 21.6 to 26.3 Gy. All 14 patients were regularly followed up by clinical and radiological evaluations. The median follow-up time was 40.3 months. RESULTS: All 14 patients had significant tumor regression after radiosurgery. The median tumor volume reduction was 34.0% (range 3-79%). Only 1 patient had temporary tumor volume progression (24% increment 6 months post-treatment), accompanied with unilateral facial palsy (from grade III to grade IV) and hearing impairment (from grade I to grade II). The tumor volume of this patient had regressed by 12 months, but facial palsy persisted. The tumor control rate in the series was 100% (n = 14/14), and the preservation rate of cranial nerve function was 92.8% (13/14). There was no complication of lower cranial nerve damage after radiosurgery. CONCLUSION: GKS appeared to be a good alternative or adjuvant to microsurgical resection in patients who are not amenable to complete surgical eradication, with an excellent tumor control rate and little morbidity after long-term follow-up.

[Imaging and assessment of glomus jugulare in MRI and CT techniques]. / Rola tomografii komputerowej i rezonansu magnetycznego w obrazowaniu i ocenie zaawansowania klinicznego przyzwojaków opuszki zyly szyjnej.

INTRODUCTION: Paragangliomas are rare group of tumors originating from paraganglionic tissue. The most common site of occurrence of the head and neck paragangliomas are: carotid body, jugular foramen (glomus jugulare) and tympanic cavity (glomus tympanicum). Magnetic resonance (MR), computed tomography (CT) and angiography are the modalities of choice in detecting and characterizing paragangliomas. AIM: The aim of this study was to compare different imaging techniques of CT and MR in visualization, detecting and characterizing paragangliomas of the jugular foramen. METHODY: Usefulness of CT and MR techniques was analyzed on the basis of CT and MR examinations performed in the group of three patients with different clinical history. CONCLUSIONS: Multidetector CT scanning is especially useful to show subtle destruction of the temporal bone on early stage of disease. MR is the modality of choice in assessing middle ear, scull base or posterior fossa involvement and monitoring growth of paraganglioma. MR and CT angiography are both useful methods to identify arterial feeders of the paragangliomas, and in some cases due to better availability in clinical practice can be alternative to angiography. Angiography in combination with embolization will mainly be used prior to surgical resection.

Contemporary Management of Jugular Paragangliomas With Neural Preservation.

OBJECTIVES: Management of jugular paragangliomas (PGL) has evolved toward subtotal resection (STR). The purpose of this study is to analyze neural preservation and adjuvant treatment for long-term local control. STUDY DESIGN: Retrospective chart review. SETTINGS: Tertiary neurotology practice. SUBJECTS AND METHODS: Adults undergoing surgical treatment of jugular PGL between 2006 and 2019. Patients, disease, and treatment variables were collected retrospectively. Single predictor logistic regression was used to ascertain predictors of regrowth or need for salvage radiation. RESULTS: A total of 41 patients (median age, 47 years; 76% female) were identified. Most patients presented with advanced-stage disease (Glasscock-Jackson stage III-IV = 76%). Subtotal resection (STR) was performed in 32 (78%) patients. Extended STR (type 1) was the most commonly performed conservative procedure (n = 19, 59%). Postoperative new low cranial neuropathy (LCN) involving CN X and XII was rare (n = 3 and n = 1, respectively). Seventeen patients (41%) underwent postsurgical therapy for tumor regrowth or recurrence, including 15 patients who underwent adjuvant (n = 4) or salvage (n = 11) radiation. Overall tumor control of 94.7% was achieved at a mean follow-up of 35 months. All patients treated with combined modality treatment had local control at last follow-up. Logistic regression identified no single predictor for postsurgical radiation treatment or salvage-free survival. CONCLUSION: Management of jugular PGL with a conservative approach is safe and effective with a low rate of new LCN deficit. Active surveillance of residual tumor with salvage radiation for growth results in excellent long-term tumor control.

A comparative review of multidetector CT angiography and MRI in the diagnosis of jugular foramen lesions.

AIM: To compare the efficiency of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the diagnosis of jugular foramen lesions. MATERIALS AND METHODS: The imaging of 15 patients with tumours predominantly occurring at the jugular foramen was retrospectively reviewed, with postoperative pathology data available for 11 patients. MDCT was performed at arterial phase and MRI with standard sequences and contrast enhancement. All imaging was blindly re-reported by an experienced neuroradiologist. RESULTS: Pathology reported six glomus jugulare tumours and five neuromas, which were all correctly diagnosed using MDCT. A confident diagnosis was also made in the remaining four cases based on the pattern of enhancement. Only glomus tumours enhanced in the arterial phase. Overall, MRI was used to make a confident diagnosis in eight patients. One showed no enhancement and was correctly diagnosed as a neuroma, and seven demonstrated the tumour flow voids characteristic of a glomus tumour. The remaining seven cases all showed a similar enhancement pattern and could not be confidently differentiated between a neuroma or a glomus tumour. MDCT angiography enabled a confident assessment of the jugular vein in all cases, but MRI was inconclusive in a third of cases. Also, in the nine cases of glomus tumour diagnosed using MDCT, an enlarged feeding artery was identified in eight patients. CONCLUSION: MDCT is more accurate than MRI in diagnosing glomus tumours, and in particular, neuromas. It also offers valuable preoperative vascular information to the surgeon.

The safety and efficacy of gamma knife surgery in management of glomus jugulare tumor.

BACKGROUND: Glomus jugulare is a slowly growing, locally destructive tumor located in the skull base with difficult surgical access. The operative approach is, complicated by the fact that lesions may be both intra and extradural with engulfment of critical neurovascular structures. The tumor is frequently highly vascular, thus tumor resection entails a great deal of morbidity and not infrequent mortality. At timeslarge residual tumors are left behind. To decrease the morbidity associated with surgical resection of glomus jugulare, gamma knife surgery (GKS) was performed as an alternative in 13 patients to evaluate its safety and efficacy. METHODS: A retrospective review of 13 residual or unresectable glomus jagulare treated with GKS between 2004 and 2008.. Of these, 11 patients underwent GKS as the primary management and one case each was treated for postoperative residual disease and postembolization. The radiosurgical dose to the tumor margin ranged between 12-15 Gy. RESULTS: Post- gamma knife surgery and during the follow-up period twelve patients demonstrated neurological stability while clinical improvement was achieved in 5 patients. One case developed transient partial 7th nerve palsy that responded to medical treatment. In all patients radiographic MRI follow-up was obtained, the tumor size decreased in two cases and remained stable (local tumor control) in eleven patients. CONCLUSIONS: Gamma knife surgery provids tumor control with a lowering of risk of developing a new cranial nerve injury in early follow-up period. This procedure can be safely used as a primary management tool in patients with glomus jugulare tumors, or in patients with recurrent tumors in this location. If long-term results with GKS are equally effective it will emerge as a good alternative to surgical resection.

Postauricular, transpetrous, presigmoid approach for extensive skull base tumors in the petroclival region: the successes and the travails.

PURPOSE: Postauricular, transpetrous, presigmoid approach combines a supra/infratentorial exposure with partial petrosectomy to access third to the twelfth cranial nerves and extraaxial lesions situated anterolateral to brainstem. It provides a shorter working distance to large petrosal, petroclival, cerebellopontine, and cerebellomedullary cisternal lesions and their extensions to the subtemporal-infratemporal areas. This study reviews the surgical technique, corridors of extension, and complications encountered utilizing this approach for excising extensive lesions in these locations. METHODS: The lesions (n = 14) included petroclival meningiomas [(n = 5), including three recurrent lesions], dumbbell lower cranial nerve schwannomas (n = 2), giant acoustic schwannomas (n = 2), recurrent giant trigeminal nerve schwannoma (n = 1), glomus jugulare (n = 3), and recurrent petrous aneurysmal bone cyst (n = 1). The approach was combined with a retrosigmoid suboccipital craniectomy (n = 3), with an infratemporal approach (n = 2), and with an extreme lateral transcondylar approach and a translabyrinthine approach in one patient each, respectively. External auditory canal was not ligated in nine patients, superior petrosal sinus and tentorial division was performed in all patients, and sigmoid sinus-internal jugular vein was excised in three patients (with a glomus jugulare (n = 1) and petroclival meningioma (n = 2), respectively). Repair was performed with fat-fascia, pedicled pericranium, and temporalis muscle. Lumbar drain was placed for three to five postoperative days. RESULTS: Total excision was performed in nine patients. Small tumor remnants were left attached to the brainstem (n = 3, petroclival meningioma), carotid canal and cavernous sinus (n = 1, glomus jugulare), and sigmoid sinus-jugular bulb (n = 1, recurrent trigeminal schwannoma). A two-staged procedure was performed in three patients. Two patients with recurrent giant petroclival meningiomas died: one with lower cranial nerve paresis due to aspiration pneumonitis and the other with cerebrospinal fluid otorrhoea and secondary meningitis. CONCLUSIONS: The approach facilitates direct tumor decompression and its retraction away from the brainstem without initially encountering the intracisternal cranial nerves and neuraxis. It provides multiple corridors for excising extensive posterior fossa tumors. Preoperative assessment of sigmoid sinus dominance, jugular bulb height, labyrinth, vein of Labbe, and space available through Trautman's triangle considerably helps in complication avoidance.