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.     

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.     

Familial multiple cervical paragangliomas: report of a kindred and review of the literature

Heredofamilial paragangliomas account for less than 10% of those arising in the head and neck. Multiplicity, multicentricity, and bilaterality is roughly three times more common than in the spontaneous variety. Not unlike other hereditary neuroendocrine tumor syndromes, familial paragangliomas appear to follow an autosomal dominant transmission, with variable penetrance and expressivity. This article describes a surgical experience with nine bilateral, multicentric cervical paragangliomas (7 carotid body, 1 vagal, and 1 sympathetic) occurring in four siblings less than 35 years of age. The literature on familial paragangliomas of the head and neck is reviewed. The postulated genetic mechanisms accounting for these and other hereditary tumors are discussed. The clinical and surgical aspects of spontaneous and familial paragangliomas are compared.     

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.     

Sinonasal paraganglioma: endoscopic resection with a 4-year follow-up

BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors. In the head and neck, they are most commonly seen at the carotid body, the temporal bone, or along the vagus nerve. Sinonasal paragangliomas are extremely rare. Most cases reported describe management by open surgical resection, with sublabial and/or transfacial approaches. METHODS: We present a case of a large sinonasal paraganglioma successfully managed by endoscopic resection. RESULTS: The patient has no evidence of recurrence after 4 years of follow-up. CONCLUSIONS: This case demonstrates that sinonasal paragangliomas might be amenable to endoscopic resection. However, the most important priority to consider when selecting a surgical technique is whether adequate excision with clear margins can be obtained. Furthermore, because of the aggressive nature of these tumors, regardless of the surgical approach, long-term follow-up is recommended.     

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.     

Trends in neurovascular complications of surgical management for carotid body and cervical paragangliomas: a fifty-year experience with 153 tumors

Almost 75% of carotid body and cervical paragangliomas are adherent to or surround adjacent arteries and cranial nerves. Their resection can result in neurovascular injury, stroke, and excessive blood loss. To assess trends in neurovascular complications, we reviewed 153 carotid body and cervical paragangliomas that were surgically managed between 1935 and 1985. Results of the past 10 years were compared with two previous time periods: period I (1935 to 1965), when carotid artery reconstruction was uncommon at our institution, and period II (1966 to 1975), when methods of intraoperative electroencephalographic monitoring and carotid patch angioplasty were being developed. During the past 10 years (period III), surgical approach to these tumors has included intraoperative monitoring of cerebral blood flow, selective use of shunts, vein patch or graft reconstructions after extensive tumor resections, and mobilization of the parotid gland to facilitate adequate exposure of high tumors. Although tumor resection was attempted in 80% of patients in period I, surgical resection was complete in 98% during periods II and III. Three trends were observed: (1) The perioperative stroke rate has decreased dramatically from 23% in period I to 2.7% in period III (p = 0.007); (2) the perioperative mortality rate has been reduced from 6% in period I to no deaths in the past 10 years, but (3) the rate of postoperative cranial nerve dysfunction remains unchanged over 50 years (period I, 46%; period III, 40%). The median tumor size among patients with postoperative complications was significantly larger than those without complications (median size: 17 vs. 7 cm3, p = 0.004).(ABSTRACT TRUNCATED AT 250 WORDS)     

Sympathetic paraganglioma as an unusual cause of Horner's syndrome

BACKGROUND: Paragangliomas are rare tumors arising from paraganglionic tissue of neural crest origin. They are present in any location where autonomic ganglia are found. The most common location in the head and neck is the carotid body, followed by the jugular bulb and vagus nerve. METHODS: A 30-year-old woman with a slowly growing left neck mass, aniscoria, and left eyelid ptosis was found to have a vascular tumor consistent with a paraganglioma arising near the left carotid bifurcation. After preoperative embolization, the patient underwent resection of the tumor. RESULTS: The tumor was found to be arising from the left sympathetic trunk and did not involve any other surrounding structures. Histopathologic analysis revealed the typical findings of a paraganglioma. CONCLUSIONS: Sympathetic paragangliomas are exceedingly rare tumors in the head and neck and should be considered in the differential diagnosis when clinical and radiographic evidence suggest a paraganglioma. The presentation is typically a slow-growing neck mass with the presence of an ipsilateral Horner's syndrome.     

Malignant carotid paraganglioma. A case report

The most common sites of paragangliomas occurring in the head and neck region are the carotid body and vagal glomus. The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination. Few cases of malignant carotid body paraganglioma have been reported in the literature. We report a case managed in the ENT Department of Grenoble university hospital in 1993. A 53-year-old woman presented a bilateral functional (dopamine secretion) carotid tumor, stage II on the right side and stage I on the left side according to the Shamblin classification. Two successive excisions with complete resection were performed without complication. Two years after the second surgical excision, bone and liver metastases were diagnosed and visualized with a Ostreoscan scintigraphy. Clinical course was rapidly unfavorable and the patient died a few weeks later. Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy. Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential. Histology in needed to establish the diagnosis of metastasis. A histology specimen may not be possible for a functional paraganglioma. In this case scintigraphy can provide the diagnosis. Malignant forms are more frequent in case of functional paraganglioma, more particularly in case of dopamine secretion.     

Dignität von Glomus-caroticum-Tumoren

Introduction

Tumors of the carotid body are rare paragangliomas (incidence 0.012%) originating from sympathetic fibres of the carotid bifurcation. Growth is slow and they frequently become symptomatic through local mechanical compression of neighboring vascular and neural structures. The aim of this study is to present the diagnosis, therapy and course in patients with a carotid body tumor treated at our department of the Düsseldorf University Hospital and to discuss rates of recurrence and also dignity during the long-term follow-up.

Patients and methods

Included in this retrospective study were all patients treated for a carotid body tumor between January 1988 and June 2008. At follow-up examination the current history was recorded and a physical examination, sonography and duplex sonography were carried out. Furthermore each patient completed the questionnaires QLQ-C30 of the European Organisation for Research and Treatment of Cancer (EORTC) and the module for head and neck QLQ-H&N35 to assess quality of life.

Results

In our collective of 36 patients consisting of 13 men (36%) and 23 women (64%) with an average age of 48.33 years (range 17–78 years), 16 patients presented with a local neck swelling and 5 patients each had difficulties swallowing or hoarseness, respectively. Preoperatively Horner’s syndrome was found in one patient. A total of 22 tumors were found on the right side of the neck (52.38%), 20 were found on the left side (47.62%) and 6 patients showed a bilateral carotid body tumor (16.67%), 3 of which were bilaterally excised. The other 3 patients are still under surveillance without surgery. Altogether surgery of 39 carotid body tumors was performed in 36 patients. In all 39 cases (primary surgery n=34, recurrence surgery n=5) the tumors were macroscopically excised in toto. Parts of the vagus nerve had to be resected in 3 patients (7.69% Shamblin type II n=1, Shamblin type III n=1) and resection of blood vessels was necessary during 10 operations. The survival rate after 1 year was 100%, after 2 years 96.3% and after 5 years 92.6%. A local recurrence was diagnosed in 2 patients (5.13%). In one patient a second operation was necessary and in the other patient there was a non-progressive swelling in the carotid bifurcation which had existed for 14 years and which was conservatively left untreated. Peripheral neural lesions could be found in 12% (3/25) at long-term follow-up. None of the patients showed evidence of local or remote metastasization of a carotid body tumor.

Conclusions

Surgical extirpation of carotid body tumors can be regarded as the only curative option with an overall mortality of 0%. Morbidity is low when applying vascular surgical techniques (2.56% for central lesions). The incidence of peripheral nervous lesions is high reflecting the radicality of the resection (64.10%) but is outweighed by the benefits. In the long-term follow-up the rate of permanent peripheral neural lesions decreased to 12%. Due to a potentially infiltrating and disseminating growth, carotid body tumors should be regarded as semi-malignant and should therefore be indicated for surgery at the time of diagnosis. Whether the incidence of carotid body tumors will rise due to increased routine diagnostic examination of the head and neck region using sonography and tomography remains to be seen.     

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.     

Malignant neuroendocrine tumor presenting in the mandible

The carotid body, jugulotympanic, vagal, and laryngeal paragangliomas are the most common paragangliomas respectively within the head and neck, although unusual examples in other sites have been described. Among these are several references in the literature to a mandibular or "alveolar body" paraganglioma, even though such an entity does not appear to have been adequately documented. This report describes a case which presented as an extensive osteoblastic tumor involving the mandible and which radiographically appeared as a typical osteosarcoma. However, the immunohistochemical and electron microscopic features were of a neuroendocrine tumor and the histologic pattern suggested a paraganglioma. The tumor was nonfunctional and an extensive search failed to reveal any further lesions. Eight months after resection of the lesion, metastatic deposits appeared within the left iliac crest and lumbar vertebra. The origin of this tumor is discussed.     

Subglottic paraganglioma

Paragangliomas are uncommon neuroendocrine tumors. In the head and neck region they are most commonly associated with the carotid body, vagus nerve, jugulotympanic paraganglia, and occasionally the superior and inferior laryngeal paraganglia. Laryngeal paragangliomas and subglottic paragangliomas are rare. There have been nine reported cases in the English literature of subglottic paragangliomas. We present a case of this unusual lesion and discuss histologic characteristics and surgical treatment. © 1997 John Wiley & Sons, Inc. Head Neck 19 : 54–56, 1997.     

颈动脉体瘤62例临床治疗体会

目的：探讨颈动脉体瘤合理的临床治疗方案，减少并发症的发生，提高临床治愈率。方法：系统回顾收治的62例颈动脉体瘤临床资料，分析手术方法与并发症的关系，并对存活病例进行随访观察。结果：手术后近期60例存活，2例死亡（均为颈动脉结扎手术引起急性脑缺血所致），2例发生面神经损伤，2例迷走神经损伤，出院时56例获得临床治愈，无任何并发症。60例存活病例均获得6个月至10年随访，平均4年5个月，1例术后5年复发，2例术后3年因其它疾病死亡。结论：肿瘤单纯切除和肿瘤切除，颈动脉重建是安全，有效的治疗方案，手术中脑神经的保护和减少脑缺血时间是预防术后严重并发症的关键。     

Surgical Management of Carotid Body Tumors

Background  Carotid body tumors (CBT) should be considered when evaluating every lateral neck mass. Methods  A retrospective study was conducted of 52 patients with 57 CBT. The surgical approach and complications were reviewed. All patients were operated on without preoperative embolization. Results  Multifocal paraganglioma (PG) were detected in six cases. A succinate dehydrogenase subunit D (SDHD) mutation was discovered in four patients. Vascular peroperative complication occurred in one case. Vascular reconstruction was decided peroperatively in five cases (8.8%). Vascular reconstruction was 0% for Shamblin 1 or 2 tumors, but 28.5% for Shamblin 3. A postoperative nerve paresis was reported in 24 patients (42.1%) and vagal nerve paralysis persisted in four cases (7.01%). The rate of serious complications, e.g., permanent nerve palsy, preoperative and postoperative complications, was 14.03%; it was 2.3% for Shamblin 1 or 2 tumors and 35.7% for Shamblin 3. One patient had malignant PG with node metastasis and was not referred for radiotherapy. No recurrence or metastasis was reported after 6-year follow-up. Conclusion  Early surgical treatment is recommended in almost all patients after preoperative evaluation and detection of multifocal tumors. Surgical excision of small tumors was safe and without complication, but resection of Shamblin 3 tumors can be challenging. Routine preoperative embolization of carotid body paragangliomas is not required.     

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.     

Tumors of the carotid body: Experience with 41 operative cases

Evaluation of a patient with a carotid body tumor requires a complete head and neck examination with special attention to the cranial nerves and careful search for additional paragangliomas in other sites. Angiography is very helpful in making the diagnosis and assessing the ipsilateral and contralateral carotid artery. Surgery should be performed early, since group I tumors are easily excised and are associated with a low surgical morbidity. Surgical excision should be performed by head and neck and vascular surgeons, applying shunting or reconstructive techniques when appropriate. With improved diagnostic and surgical techniques, the morbidity and mortality should continue to approach zero.     

Management of vagal paraganglioma: is operative resection really the best option?

BACKGROUND: Vagal paragangliomas cannot be resected without sacrifice of the vagal nerve. The risk of bilateral vocal cord palsy has been reason to postpone treatment of this benign and slow growing neoplasm in hereditary cases. Postponement could be considered for solitary cases as well. METHODS: An institute-based review of 48 patients with vagal paragangliomas over the past 30 years was performed. RESULTS: Forty-eight patients with 58 vagal paragangliomas were studied. All but 4 patients had multiple paragangliomas and should be considered hereditary cases. The 10 patients that underwent an operation lost the vagal nerve; 60% of them had additional cranial nerve palsy postoperatively. In the group of patients who were followed for an average period of 8.5 years, 3 patients (8%) developed cranial nerve palsy. CONCLUSIONS: Aggressive treatment of vagal paragangliomas leads to unnecessary early loss of vagal nerve function. A period of clinical and radiologic follow-up preceding an operation may lead to prolonged preservation of voice and swallowing functions in these patients, without grave consequences for other lower cranial nerves.     

Cervical sympathetic schwannoma: case report

OBJECTIVE AND IMPORTANCE: Approximately 20 to 27% of neck masses in the parapharyngeal space may be benign nerve sheath tumors. Cervical sympathetic chain schwannomas or vagal nerve schwannomas are most common. We report a patient with a cervical sympathetic chain schwannoma. CLINICAL PRESENTATION: The patient was a 47-year-old Caucasian man with a 7-month history of a painless, enlarging right neck mass. No hoarseness or Horner's syndrome was found preoperatively. Formal head and neck examination in the otolaryngology department revealed no vocal cord dysfunction. INTERVENTION: By use of a standard right carotid incision, a tumor was identified growing from the cervical sympathetic chain posterior to the carotid bifurcation. A gross total resection was performed and a section of the cervical sympathetic chain was sacrificed. CONCLUSION: Cervical sympathetic chain schwannomas are unusual tumors that require microneurosurgical resection for cure. We discuss the differential diagnosis, evaluation, surgical management, and pathological characteristics of such tumors and review the literature.