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.

[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.

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.

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.

Acquired Vascular Tumors of the Head and Neck.

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

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.

Jugulotympanic paraganglioma: a rare cause of vertigo.

BACKGROUND: Jugulotympanic paraganglioma generally presents in the 5th or 6th decades of life with tinnitus and hearing loss. In this manuscript, we present a rare case of jugulotympanic paraganglioma presenting in the 9th decade with vertigo as the most bothersome symptom. CASE REPORT: An 83-year-old woman presented with worsening episodes of dizziness of a few months duration. She also complained of tinnitus and hearing loss, more severe on the left side. Examination revealed a red bulging left-sided tympanic membrane, conductive hearing loss, and a bruit at the base of the skull. Dix-Hallpike test was negative. CT head and MRI brain revealed findings consistent with a large left-sided jugulotympanic paraganglioma, which was found to be hormonally inactive on laboratory tests. The patient underwent treatment with radiotherapy, which resulted in partial improvement of symptoms. CONCLUSIONS: Jugulotympanic paraganglioma may manifest in the elderly with the chief complaint of intermittent vertigo, as in our case. A red bulging mass on otoscopy raises the suspicion, necessitating further investigations, including CT and MRI.

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.

Contemporary management of jugular paragangliomas.

Jugular paragangliomas are generally benign slow-growing tumors that can cause pulsatile tinnitus, hearing loss, and cranial nerves neuropathy. Progressive growth can also lead to intracranial extension. Historically, the treatment of choice for these lesions has been gross total resection. However, over the last 15 years, many groups have adopted less invasive management strategies including stereotactic radiation therapy, subtotal resection, and primary observation in order to reduce treatment-associated morbidity. The focus of this article is to review the modern management of jugular paraganglioma, highlighting the evolving treatment paradigm at the Otology Group of Vanderbilt.

Nonparaganglioma jugular foramen tumors.

This article discusses the epidemiology, presentation, and diagnostic work-up of nonparaganglioma jugular foramen tumors, and the management options and predicted outcomes. Paragangliomas are the most common jugular foramen tumors, but other nonparagangliomas are important to consider in a differential for jugular foramen tumors. This article specifically focuses on jugular foramen schwannomas, meningiomas, metastatic disease, and regional pathologies that may extend to the jugular foramen, such as endolymphatic sac tumors, chordomas, and chondrosarcomas. Operative approaches to these tumors are also reviewed.