Classification and management of cervical paragangliomas

INTRODUCTION

Cervical paragangliomas are slow-growing tumours that eventually cause lower cranial nerve palsies and infiltrate the skull base. Surgical treatment may cause the same deficits and, in some, risks more serious neurological deficits. We describe a classification used to guide investigation, consent and management of cervical paragangliomas based on extensive experience.

METHODS

The case notes of patients managed by the senior author at a tertiary referral skull base unit between 1987 and 2010 were reviewed retrospectively. A total of 87 cervical paragangliomas were identified in 70 patients (mean age: 46 years, range: 13–77 years). Of these, 35 patients had 36 vagal paragangliomas, 43 patients had 50 carotid body paragangliomas and 8 had both. One cervical paraganglioma arose from neither the carotid body nor the nodose ganglion. The main outcome measures were death, stroke, gastrostomy and tracheotomy.

RESULTS

All tumours were classified pre-operatively based on their relationship to the carotid artery, skull base and lower cranial nerves. Type 1 tumours were excised with a transcervical approach, type 2 with a transcervical-parotid approach and type 3 with a combined transcervical-parotid and infratemporal fossa approach. Type 4 patients underwent careful assessment and genetic counselling before any treatment was undertaken. There were no peri-operative deaths; two patients had strokes, one required a long-term feeding gastrostomy and none required a tracheotomy.

CONCLUSIONS

The use of a pre-operative classification system guides management and surgical approach, improves accuracy of consent, facilitates audit and clarifies which patients should be referred to specialised centres.     

Intraoperative monitoring of motor cranial nerves in skull base surgery

Intraoperative monitoring of cranial nerves is performed to minimize postoperative cranial nerve dysfunction. We performed electrophysiologic monitoring of motor cranial nerves with a NIM 2 unit from Xomed Treace and a patient multiplexer developed in our clinic. This multiplexer allows simultaneous monitoring of four cranial nerves and is additionally equipped with a bipolar stimulation mode. This intraoperative monitoring was used during 102 skull base operations. Of these, 44 operations were acoustic neuroma removals by translabyrinthine approach and 36 by a middle fossa approach. Various operations, including removal of tumors of the jugular foramen and the infratemporal fossa, were performed in the remaining 22 patients. The facial nerve, being the most frequently monitored nerve, was evaluated both preoperatively and intraoperatively. Electrophysiologic data were evaluated with respect to their predictive value for postoperative facial nerve function. The relative percent decrease in amplitude of the electromyogram after resection compared to that observed before resection seems to be of some predictive value for the postoperative facial nerve function. A 50 to 60% decrease or more is associated with an increase in the House classification. Intraoperative monitoring is a useful tool in skull base surgery, allowing for safer and faster identification of motor nerves in pathologic-anatomic conditions. It allows the surgeon a degree of comfort by providing immediate information regarding the status of the nerve. It may also improve postoperative nerve function and shorten operating time. Additionally, neuromonitoring provides some information about expected postoperative facial nerve function.     

Improved preservation of facial nerve function in the infratemporal approach to the skull base

Although the infratemporal approach described by Fisch provides excellent exposure of the jugular foramen, intrapetrous carotid artery, and lateral skull base, the anterior displacement of the seventh cranial nerve often results in temporary facial paralysis. The use of a modified technique for facial nerve mobilization resulted in significant improvement of both early and final facial function. Since that earlier report, continuous intraoperative electrical facial nerve monitoring has been used during the infratemporal approach in 20 additional cases. Immediate postoperative facial function was normal in 93% of the monitored cases and in 70% of the cases in the unmonitored group. More importantly, no patients in the monitored group developed grade V or VI weakness after surgery, whereas 48% of the unmonitored patients had grade V or VI weakness during the early postoperative period. This article will describe how intraoperative facial nerve monitoring is used during infratemporal surgery and will compare early facial function in 31 unmonitored patients with early facial function in 20 monitored procedures.     

Jugular Foramen Schwannoma: Surgical Experience in Six Cases

Purpose: Schwannomas of the lower cranial nerves are rare tumours. We have surgical experience in six patients.

Material and methods: We have retrospectively reviewed the files of the six patients with jugular foramen schwannomas that were treated in multidisciplinary collaboration in the departments of neurosurgery and ENT. Results: From a clinical point of view, the most important finding was that these tumours usually mimic a vestibular schwannoma. The imaging findings however always allowed the differentiation. The surgical technique included in all cases a suboccipital approach for the intracranial part. When large, the extracranial extension had to be removed through a lateral cervical or transfacial approach. There was no mortality and minor morbidity, with good functional outcome. In subtotally removed tumours, recurrence occurred.

Conclusion: A combined neurosurgical and ENT approach allows for safe removal of these often large skull base tumours.     

Surgical outcomes of lateral approach for jugular foramen schwannoma: postoperative facial nerve and lower cranial nerve functions

The lateral surgical approach to jugular foramen schwannomas (JFS) may result in complications such as temporary facial nerve palsy (FNP) and hearing loss due to the complicated anatomical location. Ten patients with JFS surgically treated by variable methods of lateral approach were retrospectively reviewed with emphasis on surgical methods, postoperative FNP, and lower cranial nerve status. Gross total removal of the tumors was achieved in eight patients. Facial nerves were rerouted at the first genu (1G) in six patients and at the second genu in four patients. FNP of House–Brackmann (HB) grade III or worse developed immediately postoperatively in six patients regardless of the extent of rerouting. The FNP of HB grade III persisted for more than a year in one patient managed with rerouting at 1G. Among the lower cranial nerves, the vagus nerve was most frequently paralyzed preoperatively and lower cranial nerve palsies were newly developed in two patients. The methods of the surgical approach to JFS can be modified depending on the size and location of tumors to reduce injury of the facial nerve and loss of hearing. Careful manipulation and caution are also required for short facial nerve rerouting as well as for long rerouting to avoid immediately postoperative FNP.     

Skull Base Approach to Carotid Artery Lesions: Technique,Indications, and Outcomes

Operative approaches to lesions of the carotid artery at the skull base are challenging and place multiple cranial nerves at risk. Herein, we describe a preauricular approach utilizing anterior dislocation of the temporomandibular joint with microscopic drill-out of the medial glenoid and Eustachian tube to identify and skeletonize the carotid artery in the foramen lacerum. The facial nerve remains undissected during this approach. Nine of 10 patients presented with aneurysm, six spontaneous and three following blunt trauma, and one patient presented with carotid artery rupture after penetrating trauma. Three of the patients presented with cranial nerve (CN) deficits that persisted. One patient was unevaluable preoperatively due to trauma but awoke with multiple CN deficits. Only one of nine evaluable patients suffered a new long-term CN deficit (XI). One patient had persistent temporomandibular joint dysfunction. All patients had long-term patency of the graft or anastamosis and no new neurologic symptoms were reported with a mean follow-up of 55 months. Open approaches to the carotid artery at the skull base are feasible and with careful anatomic dissection can be performed with minimal morbidity in most cases. We present full details and images of the operative approach.     

Dumbbell-shaped jugular foramen schwannomas: surgical management, outcome and complications on a series of 16 patients

Schwannomas of the lower cranial nerves are very rare and the dumbbell-shaped ones are even rarer. The authors report their experience in managing such lesions, usually presenting either with intra- and/or extra-cranial extension through an enlarged jugular foramen. The juxtacondylar approach without sacrificing the labyrinth was used; clinical, radiological and complication features are discussed and analysed. This is a case series study on 16 consecutive patients with lower cranial nerves schwannomas surgically managed during a 14-year period using the juxtacondylar approach. In 13 cases, a complete resection has been achieved whereas in three, the excision was near total. The tumour nerve origin has been identified only in ten cases (62.5%). No death or additional post-operative cranial nerve deficits occurred. Aspiration pneumonia developed in one patient and cerebrospinal fluid leak in another. Pre-operative lower cranial nerve deficits improved in all patients. At a mean follow-up of 6.6 years (range 2–14 years), no radiological tumour recurrence was recorded amongst the patients having complete resection as well as no tumour progression in the group of near total removal. Jugular foramen schwannomas can be radically and safely resected with no additional neurological deficit if a careful pre-operative evaluation and the appropriate surgical approach is implemented. Finally, full cranial nerve functional recovery may be expected after complete resection.     

颈静脉孔及其周围区域肿瘤的显微手术治疗

目的 探讨颈静脉孔及其周围区域肿瘤的显微手术方法 和手术效果. 方法 对11例颈静脉孔区肿瘤行显微手术治疗,采用枕下乙状窦后进路2例,经颈静脉孔进路2例,颞下窝进路4例,乳突-颈联合进路3例. 结果 11例中全切除9例,次全切除2例;病理为神经鞘膜瘤4例,副神经节瘤4例,脑膜瘤1例,黏液软骨肉瘤1例,低分化鳞癌1例.术后并发脑脊液漏1例,经保守治疗痊愈,后组脑神经障碍加重2例,无手术死亡病例,术后随访8个月以上,术后听力较术前改善1例,不变6例,下降4例;9例术前无面瘫的患者,术后3例出现Ⅱ～Ⅲ级(House-Braekmann分级)面瘫,半年后恢复,2例术前有面瘫患者,术后1例改善,1例不变. 结论 根据肿瘤的性质、位置、大小、面神经功能、听力情况和后组脑神经功能,采用合适的手术进路和显微外科技术,能够充分显露肿瘤,较好地切除肿瘤,减少并发症.     

颈静脉孔区巨大哑铃型肿瘤的显微外科治疗

目的 探讨颈静脉孔区巨大哑铃型肿瘤的治疗方法.方法 对10例颈静脉孔区巨大哑铃型肿瘤采用经颞下窝入路或改良枕下远外侧手术入路进行显微外科手术切除,并观察病死率、切除程度、术前术后的颅神经功能和脑脊液漏等主要并发症.结果 全切7例,次全切2例,部分切除1例.术后无一例死亡,脑脊液漏l例,术后出现新的神经功能损害1例,原有损害加重2例.术后9个月,80%的患者颅神经功能恢复,面神经功能1～2级(H-B分级)者7例,6例有残余听力者术后听力保存有3例,术后全切者复发1例.结论 采用颞下窝入路或改良枕下远外侧入路对颈静脉孔区巨大哑铃型肿瘤进行显微外科手术,术野暴露好,全切率高,并发症少,能较好的保护颅神经,术前受损颅神经功能有望恢复.     

Outcomes after surgical resection of head and neck paragangliomas: a review of 61 patients

We reviewed the postoperative functional outcome following surgical resection of paragangliomas in patients with and without preoperative cranial nerve dysfunction. Patients who underwent surgical resections of head and neck paragangliomas were reviewed with functional outcomes defined as feeding tube and/or tracheostomy dependence, need for vocal cord medialization, and incidence of cerebral vascular accidents as primary end points. Secondary end points included pre- and postoperative function of lower cranial nerves and the impact of this dysfunction on long-term functional status. Sixty-one patients were identified: 27 with carotid paraganglioma (CP), 21 with jugular paraganglioma (JP), 8 with tympanic paragangliomas, 4 with vagal paragangliomas (VPs), and 1 with aortopulmonary paraganglioma. Following resection, 8 patients were feeding tube dependent, 14 patients required vocal cord medialization, 2 patients suffered strokes, but no patients required tracheostomy tubes. Twenty percent of patients (4/20) with JP and postoperative cranial neuropathies were feeding tube dependent, and 80% of patients (4/5) with CP and postoperative cranial nerve dysfunction were feeding tube dependent. Cranial nerve deficits were more common in patients with JP relative to those with CP. However, when cranial nerve dysfunction was present, our patients with CP had a higher incidence of temporary feeding tube dependence. Overall, 98% of patients were able to resume oral nutrition.     

颅底脑膜瘤的显微外科治疗

目的　用显微外科技术切除颅底脑膜瘤 ,以提高全切率 ,降低死亡率和致残率。　方法　报道 10 3例经显微外科治疗颅底脑膜瘤 ,根据不同部位的肿瘤采用相应的颅底手术入路切除肿瘤 ,并分析手术方法、结果和并发症。　结果　肿瘤Simpson′sⅠ、Ⅱ级全切除 6 3例 ,次全切除 31例 ,大部切除 9例。术后恢复良好 75例 ,遗留颅神经麻痹 2 4例 ,手术死亡 4例。　结论　颅底脑膜瘤采用颅底外科技术 ,明显缩短了肿瘤显露距离 ,最大限度地减少了脑牵拉 ,同时配合显微外科技术 ,可取得良好效果。     

A multidisciplinary approach to carotid paragangliomas

The surgical management of carotid paragangliomas can be problematic. A multidisciplinary approach was used to include vascular surgery, otolaryngology, and neuroradiology to treat these patients over 9 years. From January 1992 to July 2001, a multidisciplinary team evaluated patients with carotid paragangliomas. Analyzed patient data included age, gender, diagnostic evaluation, tumor size, preoperative tumor embolization, operative exposure, need for extracranial arterial sacrifice/reconstruction, postoperative morbidity including cranial nerve dysfunction, and long-term follow-up. Twenty-five carotid paragangliomas in 20 patients underwent multidisciplinary evaluation and management. Average age was 51 years (range, 28-83 years), and 52% were male. Diagnostic evaluation included computed tomography in 76%, magnetic resonance imaging/magnetic resonance angiography in 52%, catheter angiography in 60%, and duplex ultrasonography in 16%. An extended neck exposure was required in 11 cases (44%), mandibulotomy was used once (4%), and mandibular subluxation was never required. The external carotid artery (ECA) was sacrificed in 8 cases (32%). The carotid bifurcation was resected in 1 patient (4%) requiring interposition reconstruction of the internal carotid artery. Preoperative tumor embolization was performed for 13 tumors (52%). Operative blood loss for patients undergoing preoperative embolization (Group I) was comparable to the nonembolized group (group II): group I lost 365 +/-180 mL versus 360 +/- 101 mL for group II (P = .48). This occurred despite larger tumors (group I - 4.2 cm versus group II - 2.1 cm, P = .03) and a higher mean Shamblin class (group I - 2.5 versus group II - 1.45, P = .001) for group I. There were no perioperative mortalities. Transient cranial nerve dysfunction occurred in 13 CBTs (52%), 2 (8%) of which remained present after 4 months. Patients with carotid paragangliomas benefit from a multidisciplinary team approach. Neuroradiology has been used for selective preoperative embolization, which has decreased estimated blood loss during excision of larger complex tumors. A combined surgical team of otolaryngology and vascular surgery provides for exposure of the distal internal carotid artery as high as the skull base, limited permanent cranial nerve dysfunction, and selective early division and excision of the external carotid artery for complete tumor resection.     

Cranial nerve preservation in lesions of the jugular fossa.

The most significant objection to the proposal of lateral transtemporal cranial base surgery for the treatment of jugular foramen tumors is the perceived lasting morbidity attendant to aggregate cranial nerve loss. As techniques become more standardized and earlier diagnosis generates smaller tumors for treatment, outcome has become more predictable. Surgery has become the recognized management preference for these cranial base lesions. The purpose of this article is to assess the role of surgery in the treatment of jugular foramen lesions, as well as to review some of the technical highlights of conservation surgery, its clinical prerequisites, and reasonable expectations. We review 100 lateral skull base surgical cases of lesions involving the jugular foramen. The majority of these lesions--77 of 100--were paragangliomas. For these glomus tumors, cranial nerve preservation correlated well to tumor size and location. The diversity of the remaining 23 cases prevented any substantive conclusions.     

Multidisciplinary management of giant functional petrous bone paraganglioma

Giant and functional paragangliomas of the skull base are rare. Their endocrinological and surgical management is challenging. We report the case of an aggressive giant noradrenalin-secreting paraganglioma of the right temporal bone. Three procedures of embolisation were performed. The second one was complicated by a hypertensive crisis due to catecholamine release. The tumour was resected via a widened transcochlear approach. Tumour residue was treated by gamma knife radiosurgery, without additional growth at the last follow-up. This case illustrates the interest of multidisciplinary management of giant skull base paragangliomas.     

Jugular foramen schwannomas

Thirteen patients with schwannomas of the jugular foramen were operated on at the Cleveland Clinic between 1974 and 1983. The authors' experience in managing these rare tumors is presented. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen. The more distal lesions will expand inferiorly out of the base of the skull, and the more proximal lesions will enlarge into the posterior fossa. Tumors in the mid region will tend to expand primarily into bone. The schwannoma was primarily intracranial in six patients. In five patients the tumor expanded the bone at the base of the skull, with only a small intracranial component, and in two patients the tumor was primarily extracranial, with a small extension into the bone or posterior fossa. The presentation of the patients varied according to the tumor growth pattern. Deafness, vertigo, and ataxia were present in all patients with a major intracranial component, and in most of these there were only minimal deficits of the jugular foramen nerves. By contrast, lower cranial nerve involvement, including hoarseness and weakness of the trapezius and sternocleidomastoid muscles, occurred in patients in whom the tumor was primarily, within the bone or extracranial. Three of the five patients with the major component of the schwannoma within the bone also had deafness. Symptomatic history was longest in those with tumor mainly involving the bone at the base of the skull, and shortest in patients with entirely extracranial tumor. Surgical resection was accomplished with a joint neurosurgical-otological approach, usually combining a posterior fossa exploration with either a translabyrinthine transcochlear or infralabyrinthine procedure. The exact nature of the operation depended upon the presence of intracranial tumor and on the extent of bone or extracranial involvement. Total excision was performed in all cases. There was no operative mortality, and surgery resulted in loss of function of the ninth, 10th, and 11th cranial nerves in most patients. The major postoperative morbidity consisted of swallowing difficulties and sputum aspiration.     

Vagal body tumors

Six cases of vagal body tumor are reviewed. All first presented as painless neck masses with normal cranial nerve function. Otologic symptoms were infrequent, occurring only with temporal bone involvement. In true vagal paragangliomas, cranial nerve and auditory function is usually preserved until there is extensive disease of the skull base. Tumor progression after radiotherapy was documented in four patients, three of whom were treated with 4500 cGy or more. One patient was found to have regional lymph node metastases. The six patients had a total of 10 head and neck paragangliomas, illustrating the high incidence of synchronous and metachronous lesions. Because of the high incidence of multiple lesions, these tumors threaten lower cranial nerves bilaterally in many instances. Because cranial nerve function is preserved until late, and because vagal and accessory nerve paralysis is usually unavoidable with resection, we advocate conservative treatment in selected cases. It may be reasonable to postpone surgery until cranial nerve impairment becomes evident or other vital structures are threatened.     

Paragangliomas of the parasellar region

Parasellar paragangliomas are rare tumors. As far as we know, only ten cases are described in the literature. Their clinical, pathological, and radiological features and possible origin are discussed in this article and a review of the literature is given. Additionally, we report a new case of a 51-year-old woman with paraganglioma growing in the anterior, middle, and posterior cranial fossa with extended destruction of the skull base. The patient had been suffering from long-standing headaches and facial nerve paresis. Preoperatively, this tumor was suspected to be a meningioma.     

Combined endovascular and surgical treatment of head and neck paragangliomas--a team approach

BACKGROUND: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. METHODS: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. RESULTS: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. CONCLUSIONS: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen.     

Craniofacial and skull base trauma

BACKGROUND: Traumatic craniofacial and skull base injuries require a multidisciplinary team approach. Trauma physicians must evaluate carefully, triage properly, and maintain a high index of suspicion to improve survival and enhance functional recovery. Frequently, craniofacial and skull base injuries are overlooked while treating more life-threatening injuries. Unnoticed complex craniofacial and skull base fractures, cerebrospinal fluid fistulae, and cranial nerve injuries can result in blindness, diplopia, deafness, facial paralysis, or meningitis. Early recognition of specific craniofacial and skull base injury patterns can lead to identification of associated injuries and allow for more rapid and appropriate management. CONCLUSION: Early detection and treatment of craniofacial and skull base traumatic injuries should lead to decreased morbidity and mortality. This review discusses the most common of these injuries, their possible complications, and treatment.     

Management of cervical paragangliomas: review of a 15-year experience

Background and aims Cervical paragangliomas are highly vascular neoplasms and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors.Materials and methods Thirteen patients with 14 paragangliomas were treated in our institution during a period of 15 years. There were eight women (61.5%) and five men (38.5%) with a mean age of 41.3±15 years. A painless lateral neck mass was the main finding in 69.2% of patients. There was no evidence of a functional tumor. Carotid angiography was performed in all patients to define the vascular anatomy of the lesion. The 78.6% of paragangliomas underwent selective embolization of the major feeding arteries. Surgical resection followed within the next 48 h.Results The majority of the lesions were paragangliomas of the carotid bifurcation (85.7%), while one patient was diagnosed with a jugular and one with a vagal paraganglioma. In one patient, bilateral paragangliomas in the carotid bifurcation were detected. There was no evidence of malignancy in any case. Preoperative embolization has proven successful in reducing tumor vascularity. Vascular reconstruction was necessary in one patient. The main postoperative complication was transient cranial nerve deficit in seven (53.8%) patients, and a permanent Horner’s syndrome was documented in one patient. No stroke occurred. The jugular paraganglioma was treated with irradiation due to skull base extension with significant symptomatic relief.Conclusion Combined therapeutic approach with preoperative selective embolization followed by surgical resection by an experienced team offers a safe and effective method for complete excision of the tumors with a reduced morbidity rate.