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.

Current assessment and management of glomus tumors.

PURPOSE OF REVIEW: To provide an overview on the recent advances in the diagnosis and treatment of glomus tumors (paragangliomas). RECENT FINDINGS: The review focuses on the genetics of paragangliomas and discusses the role of newer therapeutic modalities in the management of jugulotympanic paragangliomas. SUMMARY: Recent advances in the field of genetics allowed the identification of three main loci involved in hereditary paragangliomas. Genetic screening of patients with family history identifies affected individuals. The management of glomus tumors continues to be a challenging task. Surgery remains the mainstay of treatment for young healthy patients with functional cranial nerve deficits. Gamma knife radiosurgery is offered as an adjunctive to limited surgery and as the primary treatment modality in poor surgical candidates or patients with bilateral disease.

Brain Tumour Foundation Award 2007. Glomus jugulare tumours: are they really so benign?

Glomus jugulare tumours are rare, hypervascular and usually benign tumours involving the skull base. Diagnosis can be significantly delayed due to the slow and insidious clinical presentation. The primary manifestations involve auditory and lower cranial nerve deficits. Treatment options may include surgery, radiation and embolization. Surgery is the optimal treatment modality, but is not without serious potential complications. These complications are linked to the location and vascular nature of the tumour. Glomus jugulare tumours present a significant diagnostic and management challenge to all members of the health care team.

Simultaneous contralateral vestibular schwannoma and glomus jugulare tumor: a case report.

To the best of our knowledge, only 3 cases of a simultaneous vestibular schwannoma and a glomus jugulare tumor have been previously reported in the literature. In all 3 cases, the lesions were located on the same side. We report a new case of simultaneous vestibular schwannoma and glomus jugulare tumor that is unique in that the two lesions arose on opposite sides. The glomus tumor was treated with embolization followed by radiotherapy, while the schwannoma was managed via radiologic observation.

Management of jugular paragangliomas: the Gruppo Otologico experience.

OBJECTIVE: The objective of this study was to review the outcome of surgical management in patients of jugular paragangliomas. STUDY DESIGN: We conducted a retrospective case review. SETTING: Tertiary care otology and skull base center. MATERIALS AND METHODS: Fifty-five patients with the diagnosis of a jugular paraganglioma (Fisch Class C and D Glomus Jugulare) were managed over a period of 15 years. All patients with adequate follow up and complete records (53 cases) were reviewed with emphasis on the results of surgical management and the factors influencing them. INTERVENTION: All 53 patients were managed with a view to surgically extirpate the tumor. The primary approach was the infratemporal fossa approach-Type A used in the majority of the patients. In eight cases, the procedure was staged owing to the presence of large intracranial extension. Three patients required additional procedures to ameliorate the after-effects of lower cranial nerve resection. RESULTS: Gross total tumor removal was achieved in 49 patients. There were five cases of recurrence. Coupled with the residual tumors in five patients, the surgical control achieved was 83%. There was no perioperative mortality. There were two cases of postoperative cerebrospinal fluid leak, both of which required surgical exploration and closure. The facial nerve was resected in seven patients. The overall preservation rate of clinically uninvolved lower cranial nerves was 75%. CONCLUSIONS: The low level of complications along with a high surgical control achieved makes surgery the primary mode of treatment in the vast majority of these tumors, regardless of the size and location.

The basic science of glomus jugulare tumors.

Glomus tumors are a fascinating group of lesions. It is a challenge for neurosurgeons and otolaryngologists to resect them completely with minimal morbidity. Laboratory researchers have discovered extremely interesting genetic and molecular biology factors involved in the development and growth of glomus tumors. In this article the author reviews the genetics, protein mutations, angiogenesis and apoptosis associated with tumor formation, and the secretion of vasoactive substances is discussed as well. It is hoped that with further research less invasive measures may be developed to treat these tumors.

Chemodectoma of the head and neck: results of treatment in 84 patients.

Eighty-four patients with chemodectoma of the head and neck presented to the Royal Marsden Hospital between 1949 and 1985. For tumors arising at the skull base (glomus jugulare and glomus tympanicum) 46 were treated with radiotherapy alone resulting in an actuarial local control rate of 73% at 25 years; 13 were treated with surgery plus radiotherapy with no recurrences during a median follow-up of 9 years; 4 had surgery alone but all recurred by 7 years. For tumors of the soft tissues of the neck (carotid body and glomus vagale) 13 were treated with surgery alone with an actuarial control rate of 54% at 15 years; 4 were treated with radiotherapy which resulted in local control at 1, 2, 8 and 11 years; and one patient who received both surgery and radiotherapy remained controlled at 1 year. Although comparison between radiotherapy and surgery in terms of tumor control is not simple, the case is argued for more frequent use of radiotherapy at all sites. This case is strengthened by minimal morbidity from radiotherapy in doses which appear effective: in the range of 45-50 Gy in 25 daily fractions over 5 weeks.

IV. Multiple glomus tumors in the head and neck.

Eight of 76 glomus tumors in the head and neck region are multiple. The most common association is with carotid body tumors; the most common pattern is an ipsilateral glomus tympanicum and carotid body tumor. The approach to clinical evaluation is based on a complete otolaryngologic, neurologic, and neuroradiologic evaluation of all patients with glomus tumors. The rationale for priorities in management is the resection of the most dangerous lesions first. This study confirms the previous observations that glomus tumors have a definite proclivity for multicentricity (10 percent) and a high incidence for other associated malignancies (8 percent).