Cervical paragangliomas-tumor control and long-term functional results after surgery.

Objective: To report long-term functional results of the surgical treatment of cervical paragangliomas. Patients and Methods: A retrospective review of 22 patients with 34 head and neck paragangliomas of which 27 were resected between 1981 and 2004. Of these, 16 were carotid body tumors and 11 were vagal paragangliomas. There were 13 women and 9 men with an average age of 48.6 years (range, 26 to 75 years; median, 49 years) and the mean follow-up period was 82 months (range, 3 to 184 months; median, 61 months). Results: There were 13 solitary tumors of which 5 were carotid body tumors and 8 vagal paragangliomas. Multiple head and neck paragangliomas were seen in 9 patients (41%). The incidence of associated multiple tumors was 64.3% for carotid body tumors and 38.5% for vagal paragangliomas. Complete tumor resection was achieved in all but 1 patient in whom a small intradural residual vagal paraganglioma had to be left. The internal carotid artery was preserved in all carotid body tumor resections. Lower cranial nerve deficits were sustained in 1 carotid body tumor resection only, but in all cases with multiple tumors. All patients with vagal paragangliomas had or developed a vagal nerve paralysis. In 4 cases minor complications developed postoperatively. No recurrent tumors were seen during the follow-up period. Conclusions: Even in large head and neck paragangliomas surgical treatment provides excellent tumor control with low postoperative morbidity. A wait-and-scan policy may be more appropriate for those patients with multiple tumors, advanced age, or high operative risk and for those whose tumors have recurred following radiotherapy.     

The current surgical management of carotid body paragangliomas.

To determine if recent trends in evaluation and therapy have contributed to the successful surgical management of carotid body paragangliomas, we reviewed our experience over the past decade. Nineteen carotid body paragangliomas were identified in 17 patients. Eleven patients underwent complete, preoperative embolization of their afferent arteries with one complication. Calculated carotid body paragangliomas surface areas did not differ between the embolized 64.6 +/- 43.3 cm2 and nonembolized 63.0 +/- 57.9 cm2 lesions. Intraoperative blood loss was lower (p = 0.02) in the patients treated with embolization (372 +/- 213 ml) compared with their cohorts (609 +/- 564 ml). However, the operative times were equivalent 4.1 hours versus 4.5 hours in both groups. Intraoperative electroencephalographic (EEG) monitoring was used in 10 patients; in one patient the EEG indicated intraoperative thrombosis of the carotid artery, which was successfully treated by thrombectomy without complications. Two patients required carotid bifurcation resection and vascular reconstruction to remove the entire tumor; a late stroke manifested by contralateral hand weakness developed in one of these patients. The incidence of cranial nerve injury was low at 16%, with one transient ramus mandibularis paresis and two instances of vocal cord dysfunction. Two additional patients had a postoperative Horner's syndrome. We conclude that by diminishing intraoperative blood loss through complete and careful preoperative embolization and use of intraoperative EEG monitoring along with careful surgical technique, the complications associated with this challenging operation are facilitated and diminished.     

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.     

The perioperative management of the petrous carotid artery in contemporary surgery of the skull base

Optimal techniques for the preoperative assessment and intraoperative management of the petrous carotid artery remain undefined. While purposeful "avoidance" of this structure may result in partial tumor removal, limited exposure of the petrous carotid artery may lead to inadvertent injury with life-threatening neurovascular sequelae. Twenty-five cases are reported in which surgical manipulation of the petrous carotid artery was necessary to accomplish total tumor removal or gain operative exposure to the skull base. A standard diagnostic radiographic assessment consisted of high-resolution computed tomography, magnetic resonance imaging, and a 4-vessel angiography. Preoperative balloon occlusion of the involved internal carotid artery was performed in four patients. Surgical approaches used in this series were broadly classified as: infratemporal-anterolateral (14), pterional-infratemporal (6), or pterional-anterolateral (5). Intraoperative management of the carotid artery consisted of total decompression in 19 cases, decompression with mobilization in four patients, and resection in two instances. Major neurovascular complications included one stroke and death caused by arterial occlusion, one stroke and death caused by arterial spasm, one stroke caused by brain edema, and one death related to a postoperative carotid hemorrhage. Other nonvascular complications included brain swelling, cranial nerve palsies, dysphagia, ataxia, cerebrospinal fluid fistulae, flap necrosis with wound infection, and pneumocephalus. Invasive and noninvasive methods are outlined for the preoperative assessment of the petrous carotid in cases of advanced skull base disease and intraoperative management options are detailed.     

Bilateral carotid body tumor resection in a female patient

IntroductionCarotid body tumors also called carotid paragangliomas are rare neuroendocrine neoplasms derived from neural crest cells, approximately 3% of all paragangliomas occur in the head and neck area (Xiao and She, 2015); although they represent 65% of the head and neck paragangliomas (Georgiadis et al., 2008).Presentation of caseWe present the therapeutic management of a 65-year-old woman with bilateral carotid body tumors. The patient presented to medical clinic for unrelated signs and symptoms of weight loss, dyspepsia, and epigastric pain. Physical examination showed bilateral non-tender neck masses for which imaging studies were ordered resulting in the diagnosis of bilateral carotid tumor. Surgical resection was staged with one week of distance between each tumor resection.DiscussionCarotid Body Tumors can arise from the paraganglia located within the adventitia of the medial aspect of the carotid bifurcation.Resection is the only curative treatment. Carotid body tumors resection represents a special challenge due to potential neurovascular complications.ConclusionsSurgical resection of carotid body tumors represents a special challenge to the surgeon because of the complex anatomical location of the tumor, including close relationship with the cranial nerves, involvement of the carotid vessels and large vascularization of the tumor. With the advance of diagnosis and improvement in surgical techniques as well as the understanding of biological behavior of tumors, surgical treatment has become a safer alternative for treating these tumors.     

Late outcome after surgical management of carotid body tumors from a 20-year single-center experience

Background and aims  Surgical resection is the treatment of choice for carotid body tumors. The aim of this study was to assess not only the perioperative, but also the long-term outcome after surgical treatment. Patients/methods  All patients that were operated on a carotid body tumor at our institution between 1986 and 2006 were reviewed. Data collection included patient profile, intraoperative findings and postoperative outcome. Results  Seventeen patients (11 female, six male) with 17 carotid body tumors (12 right, five left sided) were identified. Mean patient age at treatment was 49 years (range 19 to 76 years). Eight patients (47.1%) had large Shamblin type III tumors. Complete tumor resection was achieved in 16 of 17 cases (94.1%). Malignacy could not be proven in any patient. The 30-day mortality and stroke rates were 0. The incidence of temporary and permanent cranial nerve deficit was 41.2% and 11.8%, respectively. Patients with type III tumors had significantly higher risk of neurologic complications than patients with smaller tumors (p = 0.0152). The median postoperative follow-up was 6.4 years (range 1.5 to 20 years). The overall survival rate was 82.4%; the disease-specific survival rate was 94.1% (16 of 17 patients). One patient (5.6%) died of local tumor recurrence 3 years after a R1 resection. All the other patients showed no signs of local recurrence or metastases. Conclusions  The surgical therapy of carotid body tumors shows low long-term morbidity, mortality, and recurrence rates. Cranial nerve injury is mostly temporary but a relevant procedure-related complication. Surgical resection is indicated also for small, asympomatic tumors, because of the uncomplicated resectability of these tumors. Presented at the Annual Scientific Congress of the German, Swiss and Austrian Societies for Vascular Surgery, Basel Switzerland, September 2007.     

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.     

全程脑保护概念在颈动脉内膜切除术中的应用

目的　探讨全程脑保护在颈动脉内膜切除术(CEA)中的应用价值。方法　2002年1月至2004年8月在全程脑保护概念指导下对20例患者行CEA手术。术前所有患者中仅1例行颈动脉造影,其余均行颈部彩超和CTA检查。给予全身麻醉、选择性颈动脉转流、血管彩色Duplex( TCD)脑血流监测,术后脑保护方法是保留气管插管,脑冰袋、维持血压平稳,在TCD指导下应用脱水药物。结果　20例患者中,7例行颈动脉转流,转流率为35%。15例(85%)用补片做成形术。全部病例手术后恢复良好,无任何神经系统合并症发生,无手术死亡。结论　全程脑保护概念应用于CEA手术,能有效减少神经系统合并症的发生。     

Prosthetic patch infection after carotid endarterectomy.

OBJECTIVES and DESIGN: prospective audit of the management and outcome of prosthetic patch infection after carotid endarterectomy (CEA) at Leicester Royal Infirmary plus review of the literature. RESULTS: 8/936 CEA patients (0.85%) developed a prosthetic patch infection. Responsible organisms included MRSA (n=3), Staphylococcus epidermidis(n=2), haemolytic Streptococcus (n=1), Staphylococcus aureus(n=1) and Pseudomonas (n=1). Early wound complications preceded 4/5 infections presenting within 9 weeks of surgery. In addition to systemic antibiotics and debridement, management included patch removal and: (i) carotid ligation (n=3), vein patch repair (n=1), vein bypass (n=3). One patient had antibiotic irrigation of the in-situpatch. No patient died, one suffered a disabling postoperative stroke and two had temporary cranial nerve injuries. Including this series, a literature review identified 43 prosthetic patch infections, 91% culturing Staphylococci or Streptococci. Cumulative freedom from perioperative stroke/death or re-infection was 65% at 2 years. Patients treated by patch excision and autologous venous reconstruction had the best outcome with a cumulative freedom from perioperative stroke/death or re-infection of 91% at 2 years. CONCLUSION: prosthetic patch infection after CEA is rare. This study emphasises the importance of close surveillance of early wound complications. Surgical decision-making, especially the safety of carotid ligation, was facilitated by access to transcranial Doppler.     

Treatment of recurrent cerebrovascular disease. Review of a 10-year experience.

OBJECTIVE: The authors determined whether carotid endarterectomy in patients with recurrent cerebrovascular disease poses a greater perioperative risk than for those individuals undergoing first-time carotid endarterectomy. SUMMARY BACKGROUND DATA: A percentage of patients undergoing carotid endarterectomy for atherosclerosis experience recurrent cerebrovascular disease. Reoperation may be difficult because of postoperative scarring of the soft tissues of the neck and the carotid artery itself. Such patients were believed to be at greater risk for perioperative morbidity than those undergoing first-time carotid endarterectomy. METHODS: To address this concern, the authors retrospectively reviewed their experience with 69 patients who underwent repeat carotid endarterectomies over a recent 10-year period of time. This subgroup represented 6.4% of 1072 total carotid endarterectomies performed during the same time period. The average extent of stenosis on the operated side was 81% and the time elapsed after previous endarterectomy averaged 83 months. Twelve patients (17.4%) had contralateral internal carotid occlusion, and 30 patients (43.5%) had undergone previous endarterectomies on the contralateral side. RESULTS: Complications within 30 days of operation included two deaths (2.9%) and one stroke (1.4%), for a combined stroke and death rate of 4.3%. Six patients developed cervical hematomas requiring drainage; one of these had rupture of a saphenous vein patch. No patient had a significant cranial nerve injury in the reoperative group, whereas 2.0% of patients undergoing first-time carotid endarterectomy had cranial nerve injuries. Overall, these results compared favorably with a combined stroke and death rate of 4.0% among 1003 patients who underwent first-time carotid endarterectomy during the same period. CONCLUSIONS: This review suggests that repeat carotid endarterectomy can be performed safely in individuals with severe recurrent carotid stenosis, with morbidity and mortality rates similar to those for patients undergoing first-time carotid endarterectomies. For this population, reoperative carotid endarterectomy represents a safe and important mechanism for the prevention of stroke.     

Carotid surgery following previous carotid endarterectomy is safe and effective

With the perceived high risk of repeat carotid surgery, carotid angioplasty and stenting have been advocated recently as the preferred treatment of recurrent carotid disease following carotid endarterectomy. An experience with the operative treatment of recurrent carotid disease to document the risks and benefits of this procedure is presented. A review of a prospectively acquired vascular registry over a 10-year period (Jan. 1990-Jan. 2000) was undertaken to identify patients undergoing repeat carotid surgery following previous carotid endarterectomy. All patients were treated with repeat carotid endarterectomy, carotid interposition graft, or subclavian-carotid bypass. The perioperative stroke and death rate, operative complications, life-table freedom from stroke, and rates of recurrent stenosis were documented. During the study period 56 patients underwent repeat carotid surgery, comprising 6% of all carotid operations during this period. The indication for operation was symptomatic disease recurrence in 41 cases (73%) and asymptomatic recurrent stenosis >/=80% in 15 cases (27%). The average interval from the prior carotid endarterectomy to the repeat operation was 78 months (range 3 weeks-297 months). The operations performed included repeat carotid endarterectomy with patch angioplasty in 31 cases (55%), interposition grafts in 19 cases (34%), and subclavian-carotid bypass in 6 cases (11%). There were three perioperative strokes with one resulting in death for a perioperative stroke and death rate of 5.4%. One minor transient cranial nerve (CN IX) injury occurred. Mean follow-up was 29 months (range, 1-116 months). Life-table freedom from stroke was 95% at 1 year and 90% at 5 years. Recurrent stenosis (>/=80%) developed in three patients (5.4%) during follow-up, including one internal carotid artery occlusion. Two patients (3.6%) underwent repeat surgery. Repeat surgery for recurrent cerebrovascular disease following carotid endarterectomy is safe and provides durable freedom from stroke. Most patients are candidates for repeat endarterectomy with patching, but interposition grafting is often required. These results strongly support the continued role of repeat carotid surgery in the treatment of recurrent carotid disease.     

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.     

颈动脉体瘤诊治的临床分析：附38例报告

目的：总结颈动脉体瘤（CBT）的临床特征与诊治经验。 方法：回顾性分析2008年10月—2019年4月在中南大学湘雅医院血管外科治疗的38例CBT患者资料,其中男14例,女24例;年龄23~76岁;单侧36例,双侧2例;40个瘤体中,Shamblin I型6个、II型12个、III型22个。 结果：所有患者均行颈部CTA或MRA明确诊断。38例患者中,3例单侧患者行保守治疗,其余35例患者共37个瘤体行手术切除治疗,其中1例手术患者术前行DSA检查并行双侧颈外动脉栓塞术。无术中死亡病例,手术平均时间（140±48）min,术中平均出血量（194±148）mL;Shamblin I型病变均行单纯CBT切除,Shamblin II、III型病变行单纯CBT切除或CBT切除+其他手术（颈部动脉离断、重建、结扎）。所有手术患者术后病理检查均证实为颈部良性副神经节瘤。术后发生短暂脑神经损伤8例,永久脑神经损伤2例,死亡1例。单纯CBT切除患者的神经损伤发生率明显低于CBT切除联合其他手术患者（P<0.05）。随访半月至10年,手术患者未出现肿瘤复发及其他并发症。3例保守治疗患者均带瘤生存。 结论：CTA或MRA为诊断CBT的首选方法,手术切除是CBT的首选治疗方法。手术方式的选择还需根据瘤体的大小形态以及分型决定。     

Carotid body tumours and other cervical paragangliomas: diagnosis and management in 25 patients

This report of a series of 25 patients with cervical paragangliomas reviews the experience of one surgeon over a period of 13 years. A total of 26 resections were performed with no mortality and no hemiplegia. Two patients required carotid reconstruction. Eight patients had a permanent cranial nerve palsy but in seven this was due to involvement of the nerve by the tumour. Lymph node involvement occurred in two patients. Only one patient has developed recurrent tumour. These tumours are locally malignant and resection offers a chance of cure.     

颈动脉体瘤的外科治疗：附24例报告

目的:总结颈动脉体瘤(CBT)的诊治经验及其手术并发症的防治。方法:回顾性分析1999年1月—2012年9月收治的24例颈动脉体瘤患者共30侧资料。其中双侧肿瘤6例,单侧18例。结果:24例均手术治疗,其中Shamblin I型17侧行单纯瘤体剥除;Shamblin II型7侧行瘤体剥除及颈外动脉切除;6侧Shamblin III型侧行瘤体剥离、颈内动脉部分切除伴颈内动脉重建术。24例患者肿瘤均完整切除,无手术死亡病例,术后出现短暂性脑神经损伤5侧(16.7%),永久性脑神经损伤1例(3.33%)。随访1~15年,未出现延迟性并发症及肿瘤复发。结论:手术是CBT的最有效方式,根据肿瘤大小及与动脉关系决定手术方式,预后良好。     

Paragangliomas of the neck.

Between 1967 and 1990 inclusive, 28 patients with paragangliomas of the neck were diagnosed at the University of Alabama at Birmingham Affiliated Hospitals. There were 11 men and 17 women, whose ages ranged from 12 to 76 years (mean, 47 years). Tumor locations included the carotid bodies (19 cases), the vagus nerves (three), supraglottic larynx (two), the left lateral pharyngeal wall (one), posterior to the right jugular vein (not otherwise defined) (one), subcutaneous neck tissue (one), and a cervical lymph node with unknown primary (one). Diagnostic workup included angiography (23 cases) with preoperative embolization (three), computed tomography (one), magnetic resonance imaging (two), and urinary catecholamine assay (four). All 28 patients underwent resection of the lesions. Cranial nerve damage occurred in 11 patients (39%). There were no perioperative deaths or cerebrovascular accidents, although one of two saphenous vein grafts became thrombotic after carotid body tumor resection.     

Nonendarterectomy procedures of the carotid artery: a five-year review

Although the efficacy of carotid endarterectomy has been well established, nonendarterectomy procedures of the carotid bifurcation have only sporadically been reported. Of 334 consecutive nontraumatic carotid procedures performed on 321 patients from July 1992 until May 1997, 306 (91.6%) were carotid endarterectomies, 14 (4.2%) were carotid-subclavian bypasses/transpositions, and 14 (4.2%) were nonendarterectomy procedures of the carotid artery. These latter 14 cases (nine females and five males; mean age, 63 years) were all symptomatic (neurological or painful mass) and included carotid kink/coil resection (n = 3; 0.9%), endarterectomy and vertebral transposition (n = 2; 0.6%), carotid aneurysm resection (n = 2; 0.6%), carotid body tumor resection (n = 2; 0.6%), carotid stump ligation/external endarterectomy (n = 1; 0.3%), infected/bleeding carotid patch removal with vein graft replacement (n = 1; 0.3%), saphenous vein graft replacement (n = 1; 0.3%), carotid dilatation for fibromuscular dysplasia (n = 1; 0.3%), and descending aorta to carotid bypass (n = 1; 0.3%). With 30 day follow-up complete for all 334 carotid operations, 10 perioperative strokes (2.9%) and five deaths (1.5%) occurred for a combined stroke/death rate of 3.3 per cent. Of the 14 nonendarterectomy carotid artery operations, there were no strokes or deaths; with mean follow-up of 13 months, 13 patients (92.9%) are asymptomatic, patent, and disease-free. Three severe transient cranial nerve (CN) neuropraxias (21.4%), one myocardial infarction (7.1%), and one late death (mesenteric ischemia at 2 months), however, occurred. Although no statistical differences in stroke, death, and stroke/death occurred between the endarterectomy versus the nonendarterectomy group, transient CN injury was more common in the nonendarterectomy group (21.4% versus 4.1%; P = 0.027). Although nonendarterectomy procedures of the carotid bifurcation are infrequently needed, they seem safe, effective, and indicated in selected patients, despite a higher incidence of transient CN injury.     

Paraganglioma of the neck. Analysis of 32 operated cases]

Between january 1965 and november 1990, 32 operations for neck paraganglioma were performed: 29 chemodectomas (carotid body tumors) and 3 paragangliomas of the vagus nerve. Seven subjects were affected with bilateral chemodectomas and one of them showed concurrent unilateral vagal paraganglioma. Two paragangliomas were malignant, with invasion of the latero-cervical lymphnodes revealed at operation. Four individuals came to observation from two different families, suggesting familiarity. Preoperative diagnosis was correctly made in 12 of 18 asymptomatic chemodectomas (66.6%), ten of whom observed during the last decade: angiography is the gold standard for diagnosis but CT scan, ultrasound and NMR imaging are going to earn the confidence of physicians for precise evaluation of latero-cervical masses. Surgery is to date the treatment of choice, and the results are dependent on the size of the tumor and the involvement of the neighbouring vascular, nervous and visceral structures. According to the majority of the literature, the 29 chemodectomas were classified in the three groups of Shamblin: I: 4 cases; II: 10; III: 15. Twenty out of the 24 transient or permanent postoperative complications took place in the third group: in five instances some procedures of internal carotid artery reconstruction were needed. Fourteen complications for chemodectomas and 2 for vagal paragangliomas affected the cranial nerves; three transient and one permanent ischemic central neurological deficits occurred in the group III chemodectomas. Not any operative mortality was registered in this series.     

Malignant catecholamine-secreting carotid body paraganglioma

The second known case of a malignant catecholamine-secreting (DA)-secreting carotid body paraganglioma is presented. Dopamine synthesis and secretion can be increased in malignant tumors derived from neural crest cells. Whether this is true, in addition, for extra-adrenal paragangliomas is not yet clear. Malignant paragangliomas of the carotid body and larynx, although rare, frequently have been accompanied by increased catecholamine secretion. Malignant catecholamine-secreting carotid body paragangliomas are best treated by composite resection (internal carotid artery and neck dissection), with special attention being given to measures preventing severe hypertension and arrhythmias in the perioperative period.     

Complex tumors of the glomus jugulare: criteria,treatment, and outcome

OBJECT: Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors--multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome--that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. METHODS: In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. CONCLUSIONS: Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intrabulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.