Surgical Management of Giant Transdural Glomus Jugulare Tumors with Cerebellar and Brainstem Compression

Objective The objective of this study is to discuss the management of advanced glomus jugulare tumors (GJTs) presenting with intradural disease and concurrent brainstem compression.Study Design This is a retrospective case series.Results Over the last decade, four patients presented to our institution with large (Fisch D₂; Glasscock-Jackson 4) primary or recurrent GJTs resulting in brainstem compression of varying severities. All patients underwent surgical resection through a transtemporal, transcervical approach resulting in adequate brainstem decompression; the average operative time was 12.75 hours and the estimated blood loss was 2.7 L. All four patients received postoperative adjuvant radiotherapy in the form of intensity-modulated radiation therapy or stereotactic radiosurgery. Combined modality treatment permitted tumor control in all patients (range of follow-up 5 to 9 years).Conclusion A small subset of GJTs may present with intracranial transdural extension with aggressive brainstem compression mandating surgical intervention. Surgical resection is extremely challenging; the surgical team must be prepared for extensive operating time and the patient for prolonged aggressive rehabilitation. Newly diagnosed and recurrent large GJTs involving the brainstem may be controlled with a combination of aggressive surgical resection and postoperative radiation.     

Glomus tumours of the hand: Review of literature

Glomus tumours are rare benign vascular neoplasms commonly found in the hand particularly in subungual region. Though, its aetiology remains largely unknown, several hypotheses have been made to explain the etiopathogenesis and cause of pain. These tumours usually present as a bluish or pinkish red discolouration of the nail plate with classical triad of localised tenderness, severe pain, and cold sensitivity. Nevertheless, differential diagnosis of other painful tumours, such as leiomyoma, eccrine spiradenoma, haemangioma, neuroma, osteochondroma, or mucous cyst should always be kept in mind while evaluating a patient with severe pain in the tip of the finger. In addition to the different clinical tests including Love's pin test, Hildreth's test, and trans-illumination test, imaging studies such as magnetic resonance imaging (MRI), ultrasonography, and radiography are often helpful in the diagnosis. Complete surgical excision is a must to get complete relief from the symptoms and to avoid recurrence. Several approaches have been described in the literature. Different surgeons may have different choices and may prefer one approach over the other depending on the anatomical location of the tumours. The purpose of this article is to review the important aspects of glomus tumours in hand concerning their aetiology, clinical presentation, diagnosis, management, and recurrence.     

Treatment of Recurrent and Residual Glomus Jugulare Tumors

Residual and recurrent glomus jugulare tumors are rare but challenging. Treatment options include microsurgical resection, stereotactic radiotherapy, a combination of modalities, and “observation.” Choice of treatment must be made on a case-by-case basis, considering patient age, health status, location and size of tumor, status of the lower cranial nerves, and, of course, patient desire. Surgery is preferred when total resection of the tumor with preservation of function is deemed achievable. When function of the lower cranial nerves has been compromised, total surgical resection may also be possible, provided that the patient's health allows it. Cases where function is still preserved despite presence of a large tumor are more challenging, and a combination modality may be most effective. The goal of treatment is to provide tumor control with low morbidity. Current surgical techniques and the availability of stereotactic radiotherapy make this possible in the majority of cases.     

Fibrin Sealant Injection: An Aid to Reduce Venous Bleeding during Jugular Bulb and Sigmoid Sinus Dissection in Glomus Jugulare (Jugulotympanic Paraganglioma) Surgery

Glomus jugulare (jugulotympanic paraganglioma) surgery requires tumor dissection in the region of the jugular bulb, upper internal jugular vein, and sigmoid sinus. Despite ligation or external compression of the sigmoid sinus proximally and ligation of the internal jugular vein distally, troublesome venous bleeding can arise from the inferior petrosal sinus or condylar veins at the medial wall of the jugular bulb. Excessive packing in this area can place the integrity of the lower cranial nerves at risk. We report a technique in which Tisseel^® fibrin sealant is injected into the ligated sigmoid sinus and internal jugular vein. This forms an internal cast around the tumor in the sigmoid-jugular complex and helps seal the inferior petrosal sinus and condylar veins. This allows for safer dissection with reduced venous bleeding. Our experience in five cases has shown this technique to be effective.     

Glomus jugulare tumor: tumor control and complications after stereotactic radiosurgery

BACKGROUND: We evaluated toxicity and long-term efficacy of stereotactic radiosurgery in patients with symptomatic or progressive glomus jugulare tumors. METHODS: Twenty-five consecutive patients (age, 30-88 years; 17 women, 8 men) who underwent stereotactic radiosurgery with the Leksell Gamma Knife (dose, 12-18 Gy) were prospectively followed. MRI and clinical examinations were performed at 6 months and 1, 2, and 3 years, and then every 2 years. RESULTS: None of the tumors increased in size, 17 were stable, and 8 decreased (median imaging follow-up, 35 months; range, 10-113 months). Symptoms subsided in 15 patients (60%); vertigo occurred in 1, but balance improved with vestibular training (median clinical follow-up, 37 months; range, 11-118 months). No other new or progressive neuropathy of cranial nerves V-XII developed. CONCLUSIONS: Stereotactic radiosurgery can achieve excellent tumor control with low risk of morbidity in the treatment of glomus jugulare tumors. The lower cranial nerves can safely tolerate a radiosurgical dose of 12 to 18 Gy.     

Craniofacial resection for anterior skull base tumours

Summary This study presents a series of 10 patients with anterior skull base tumours, treated by a team of neurosurgeons and head- and neck surgeons. The series included 7 malignant tumours of the nose and paranasal sinuses and 1 retinoblastoma, all with intracranial extension through the lamina cribrosa. There were also 2 patients with an anterior base meningioma, growing into the ethmoid sinus and the nasal cavity.8 tumours were resected by a combined bifrontal craniotomy and uni- or bilateral rhinotomy. In 2 cases a bifrontal craniotomy alone without facial incision sufficed. The skull base was closed with a pediculated pericranial flap and a split-thickness free skin graft underneath.There were no postoperative problems of wound infection, CSF-leakage or meningitis. Recurrent tumour growth or systemic metastasis occurred in 5 out of 7 patients with malignant tumours, 6 months to 2 years postoperatively.The related literature and especially questions of operative indications and technique, including different possibilities of closure and reconstruction of the skull base, are discussed.     

Mucocele of neurosurgical interest: Clinical considerations on five cases

Summary The authors present five patients with mucocele, submitted to neurosurgery. Two had mucoceles spreading from the frontal and/ or the anterior ethmoidal sinuses and had only compressive mass symptoms, either on the ocular globe or on the frontal lobe or on both. Three patients had mucoceles growing from the sphenoid and/ or posterior ethmoidal sinuses. In these latter, the mass symptoms were less evident. All the patients suffered excruciating retro-ocular pain and two presented cranial nerve damage.The correct diagnosis in these cases is crucial to avoid a too aggressive treatment since these patients are generally sent to a neurosurgeon for a suspected cranial base malignancy or an invasive pituitary adenoma. The principles of a correct differential diagnosis and of operative treatment are outlined based on an analysis of the literature and the authors experience.     

Management of a glomus jugulare tumour with internal carotid artery involvement

A previously healthy 40-year-old female presented for surgical resection of a large glomus jugulare tumour with extensive involvement of the carotid siphon and intracranial extension. Conduct of anaesthesia with specific reference to cerebral protection is discussed. A combination of induced hypothermia, barbiturate therapy, normotension, normocarbia and prior clamping of the distal internal carotid artery was chosen. The role of barbiturates as a therapeutic intervention is debated.     

Gamma Knife Radiosurgery for Glomus Jugulare Tumours

Summary  The aim of this clinical study was to determine the tumour control rate, clinical outcome and complication rate following gamma knife treatment for glomus jugulare tumours. Between May 1992 and May 1998, 13 patients with glomus tumours underwent stereotactic radiosurgical treatment in our department. The age of these patients ranged from 21 to 80 years. The male : female ratio was 2 : 11. Six patients had primary open surgery for partial removal or recurrent growth and subsequent radiosurgical therapy. Radiosurgery was performed as primary treatment in 7 cases. The median tumour volume was 6,4 cm³ (range: 4,6–13,7 cm³). The median marginal dose applied to an average isodose volume of 50% (30–50%) was 13,5 Gy (12–20 Gy). In 10 patients, a total of 48 MRI and CT follow-up scans were available. The remaining three patients have been excluded from the postradiosurgical evaluation since the observation time (t<12 months) was too short or patients were lost to follow up. The median interval from Gamma Knife treatment to the last radiological follow-up was 37,6 months (5–68 months). In 4 patients (40%) decreased tumour volumes were observed and in 6 cases (60%) the tumour size remained unchanged. Neurological follow-up examinations revealed improved clinical status in 5 patients (50%), a stable neurological status in 5 patients (50%) and no complications occurred. According to our preliminary experience Gamma Knife radiosurgery represents an effective treatment option for glomus jugulare tumours.     

The Use of the Temporoparietal Fascial Flap in Preventing CSF Leak after Lateral Skull Base Surgery

Objective Determine the effectiveness of the temporoparietal fascia flap (TPFF) with adipose tissue in preventing cerebrospinal fluid (CSF) leaks for lateral skull base tumor reconstruction.Design A retrospective chart review from 2005 to 2010 was conducted of patients undergoing skull base tumor resection. Patients with TPFF reconstruction were compared with those with adipose packing alone based on lumbar drain placement, tumor size, extent of dissection, and incidence of CSF leak. Data was analyzed with a Fisher exact test at p < 0.05.Setting Tertiary care institution.Main Outcome Measures Incidence of CSF leak.Results A total of 16 patients had a TPFF reconstruction; 20 had adipose only. Four TPFF patients had lumbar drain placement, as did six in the adipose-only group. Six patients had a CSF leak, all in the adipose-only group (p = 0.02). Patients with a lumbar drain were more likely to have larger tumors (p = 0.01) and to have a CSF leak if they had adipose-only reconstruction (p = 0.07).Conclusions Lateral skull base reconstruction using TPFF with adipose tissue is easily performed and has a low operative morbidity. Early results show a significant decrease in the rate of CSF leak using TPFF, particularly in high-risk patients.     

Glomus tumours in the long finger and in the thumb of a young patient with neurofibromatosis-1 (Nf-1)

Abstract

Glomus tumours are rare in adults, and such tumours may be associated with neurofibromatosis-1 (Nf-1). Here we describe successful treatment of two glomus tumours initially in the long finger and, one year later, in the thumb in a 17-year young girl with Nf-1.     

Surgical treatment of anterior skull base tumours

Summary Skull base tumours represent a special challenge to surgeons due to the complex anatomy of the area. While small tumours are easy to remove, large lesions can pose complex situations. The most difficult aspects are not only the approach and removal, but specially the repair of the defects created by the resection of the tumour.We present here our experience with the surgical removal of tumours on the anterior skull base. To achieve a good approach, we resort to a bifrontal craniotomy including the cilliar arches. To obtain a skull base bone flap that can be used for repair at the end of the procedure, we remove the roof of the nose and a part of the medial wall and roof of both orbits. While the tumour is removed, the skull base bone flap is autoclaved to kill all tumoural cells. At the end of the procedure this bone flap is replaced, wrapped with a flap of pericranium. Provided no orbit needs to be emptied, no other flap is needed to reconstruct the area. One advantage is that the surgical cavity is not occluded with tissues, thus facilitating early identification of any recurrence. The area can be explored with the aid of an endoscope introduced into the nasal cavities through the nostrils, and in case of doubt, biopsies taken from all suspicious areas.Our technique facilitates the repair of the surgical defect, and while not compromising the healing process it has a very low incidence of CSF leaks and infections.     

Surgical management of carotid body tumours: a 24-year surgical experience

BACKGROUND: Carotid body tumours (CBT) are rare tumours, best treated by complete surgical resection. However, there is no uniform agreement on the method of resection. The aim of this study was to review our 24 years' experience of meticulous subadventitial excision of CBT. METHODS: A retrospective study, from March 1980 to September 2004 of patients with CBT was undertaken, detailing presentation, diagnosis and treatment and postoperative complications. RESULTS: Twenty-five patients (six men and 19 women) with an age range of 23-72 years had been operated on, and all were treated by surgical excision. All patients had neck mass. No patient had a positive family history. Angiography was the main method of diagnosis. All of the patients had unilateral tumours. There was no postoperative permanent neurological deficit. Temporary neurological problems developed in only four patients. External carotid artery was ligated in three patients to facilitate excision of the tumour. Surgical care limited blood loss to an average of 480 mL. CONCLUSIONS: Subadventitial excision, carried out meticulously, allowed complete resection to be achieved in all of the patients with minimal morbidity and no surgical mortalities. This method is therefore recommended. Facilities for shunting and arterial repair should always be available.     

Hyperostotic paraganglioma of occipitotemporal bone

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.     

Tumours of the glomus jugulare

Two cases are reported using a two stage procedure for the excision of glomus jugulare tumours. The anaesthetic problems which this may produce are discussed, and recommendations are made regarding the anaesthetic conduct for this procedure. A two stage, combined procedure is suggested to reduce anaesthetic morbidity.