Paragangliomas of the head and neck

doi:10.1016/j.oraloncology.2003.09.004

Oral Oncology

Volume 40, Issue 6, July 2004, Pages 563-575

https://doi.org/10.1016/j.oraloncology.2003.09.004 Get rights and content

Abstract

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently neighboring vascular structures. Physiologic activity is rare in these neoplasms and they may exhibit patterns of inheritance which predispose their occurrence in families, often with multicentricity. These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties. Other rare sites where this tumor may occur include; the larynx, sinonasal chambers and orbit. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Magnetic resonance represents the most important imaging modality for the evaluation and characterization of suspected head and neck paraganglioma. Definitive management for these lesions should be carefully considered in relation to both tumor and patient-oriented factors, especially in regard to the potential morbidity of treatment. Surgery and radiation therapy represent the main treatment modalities for paraganglioma. The selection of treatment depends on the size, location, and biologic activity of the tumor as well as the overall fitness of the patient. Although radiotherapy may be effective in arresting growth of these tumors, rarely is the neoplasm eliminated without surgical resection. Surgery may be associated with significant morbidity, primarily as a consequence of incurring major cranial nerve injury. Patient selection (relative to age and medical condition) should be carefully considered prior to recommending aggressive surgery for paragangliomas of head and neck, especially in those patients at risk for disabling surgical morbidity.

Introduction

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. They comprise a unique subset of lesions of the head and neck and can be subdivided in two groups: sympathetic and parasympathetic derived tumors. Virtually all head and neck paragangliomas arise from the parasympathetic nervous system. These tumors have been referred to by various terms, but generally the site of origin defines the name given to these tumors (e.g., carotid body tumor (CBT) or carotid paraganglioma, jugular paraganglioma, vagal paraganglioma or glomus intravagale, etc.). Previously, they were designated as chemodectomas or glomus tumors, but at present the term paraganglioma is preferred.

Although all paragangliomas contain cytoplasmic neurosecretory granules (and, consequently, possess in theory a potential for exhibiting some signs of physiologic activity), only a minority of paragangliomas (on the order of 1–3%) demonstrate actual clinical evidence of hyperfunctioning.¹ Patients with clinical signs or symptoms suggestive of excessive catecholamine release should be evaluated for the presence of a functional paraganglioma or multiple tumors (as, for example, in case of patients with paragangliomas associated with other neural crest derived neoplasms in the multiple endocrine neoplasia syndromes). Multiple paragangliomas may occur in up to 40% of familial paragangliomas and in up to 10% of sporadic tumors.²

Within the head and neck, branchiomeric paraganglionic tissue may be found associated with vascular structures, with high concentrations nearby the carotid artery and jugular bulb. This tissue constitutes the paraganglion system, a term used to characterize an organization of neural crest derived chromaffin cells located outside of the adrenal glands.³ The majority (90%) of tumors arising from the system originate in the adrenal gland and are designated as pheochromocytoma. The remaining 10% of neoplasms arise from the abdomen (85%), some in the thorax (12%), and some less commonly in the head and neck area (3%).⁴ The majority of these neoplasms occur as solitary tumors; the notable exceptions are represented by familial syndromes such as the multiple endocrine neoplasia syndromes (MEN), types II A and II B, and Carney's complex, which includes the triad of gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal functional paraganglioma.⁵ Paragangliomas may exhibit multicentricity.

Paragangliomas of the head and neck generally present in mid-adult life as asymptomatic, nonfunctional, space occupying lesions. Head and neck paragangliomas have a higher occurrence in females, whereas tumors of extra-adrenal origin arising outside the head and neck occur more commonly in males.⁴

Normal paraganglionic distribution in the head and neck may be found to parallel the course of the autonomic nervous system, i.e., jugulotympanic and vagal plexi and carotid body. Carotid body neoplasms account for about 60% of head and neck paragangliomas and are situated at the bifurcation of the common carotid artery within the adventitia. The middle ear is the next most common site of head and neck paragangliomas. Jugular tumors located in the region of the jugular foramen commonly traverse the skull base to be located both intra- and extra-cranially and within the jugular vein. Vagal paragangliomas account for less than 5% of all head and neck paragangliomas, and only approximately 200 cases have been reported in the medical literature.⁶ Neoplasms associated with the vagus nerve originate from the nodose ganglion and are usually intimately associated with the nerve. Other sites of paraganglionic tissue within the head and neck where tumors may rarely arise include: the nasopharynx, nasal cavities, paranasal sinuses, larynx, thyroid gland and orbit.

The overwhelming majority of head and neck paragangliomas are benign. Malignant behavior has been noted in approximately 4% of jugulotympanic tumors and 6% and 16% of carotid body and vagal paragangliomas, respectively.⁷ Histologic criteria are not accepted as a definite sign of malignancy. All but one case of “metastasizing paraganglioma” of the larynx have been reclassified as atypical carcinoids.[8], [9], [10], [11], [12], [13], [14] The only acceptable criterion of malignancy is the presence of metastasis to the cervical lymph nodes or distant sites, such as lung, liver and skin. The aggressive behavior of a paraganglioma does not imply malignancy.⁶ One recent review¹⁵ included 59 cases of malignant paragangliomas of the head and neck extracted from the National Cancer Data Base and diagnosed between 1985 and 1996. Of the 51 cases of malignant paraganglioma in which location of metastasis was recorded, 68.6% were confined to regional lymph nodes and 31.4% of patients had distant disease.¹⁵ Malignancy in paragangliomas is more common in the sporadic, nonfamilial patients than the hereditary form.¹⁶

Section snippets

Familial paraganglioma

10–50% of paraganglioma cases are familial. They are frequently multiple and bilateral and have an earlier age of detection. The gene for familial paragangliomas (PGL1) has recently been identified at the 11q23 locus.¹⁷ At this time, at least two other genetic loci (PGL2 and PGL3) have been established. This gene is transmitted in an autosomal dominant pattern with genomic imprinting. This implies that affected men have a 50% chance of having an affected child, whereas affected women will not

Classification

Neuroendocrine neoplasms constitute a morphologically heterogeneous group of lesions. This spectrum is categorized by the World Health Organization according to germ cell line of origin, i.e., neural or epithelial.¹⁸ Paragangliomas arise from the former whereas the latter give rise to the family of carcinoid tumors and small neuroendocrine carcinomas. This categorization has been revised by Wick¹⁹ to include two groups of neuroendocrine tumors based on the cell of origin. Group I neoplasms

Gross pathology

Paragangliomas typically appear to the head and neck surgeon as sharply circumscribed polypoid masses; they have a firm to rubbery consistency. As they are highly vascular tumors, paragangliomas often bleed readily when incised. Jugulotympanic paragangliomas may have a deeper red coloring when viewed from behind the overlying tympanic membrane; carotid body paragangliomas may range from a deep red to brown-grey appearance.

While CBTs are usually clearly delineated from the surrounding

Clinical characterization

Paragangliomas, in general, exhibit a slow rate of growth with the potential to remain stable and thus clinically silent over years. Reports have suggested that tumors which have been followed radiographically show an increase in size of less than 5 mm per year.²⁰

The carotid paraganglioma or CBT is the most common head and neck paraganglioma, although the incidence is low. A review of nearly 1000 tumors noted a 31% incidence of bilaterality among familial cases and a 4% incidence of bilateral

Clinical and radiographic evaluation

The evaluation for suspected paraganglioma should begin with a careful history, especially in regard to family history of surgery for head and neck tumors. A thorough cranial nerve and neurotologic evaluation is performed. The biochemical activity of these tumors is reported at 1–3%. Most authors agree that biochemical activity should be sought only if the patient is symptomatic.²⁷ Magnetic resonance imaging (MRI) represents the most important imaging technique for evaluation and

Treatment

Definitive treatment for head and neck paraganglioma must be considered in relation to the biologic activity of the tumor, patient age and medical condition, tumor site, and potential for treatment related morbidity. Observation with interval follow-up utilizing radiographic studies as a measurement tool represents an initial management strategy in many patients with head and neck paragangliomas. Unless functional catecholamine secretion is noticed, urgent therapy is not necessary.

The main

Carotid body tumor (carotid body paraganglioma)

Traditionally, surgical resection has been the mainstay of treatment for CBT. Surgical morbidity has been reduced as a consequence of improvements in anesthesia and vascular surgical technique.³³ The risks of arterial injury during resection have been shown to increase with increasing tumor size, but without significant increase in stroke rate because of vascular reconstruction.³⁴ Injury to the surrounding cranial nerves (esp IX, X, XI) represent a more significant concern during resection of

Jugulotympanic paraganglioma (glomus jugulare tumor)

Glomus jugulare tumors represent the most common tumor of the middle ear. Jugulotympanic paragangliomas are the second most common tumor of the temporal bone. There is no clear racial predilection, but females are 4–6 times more commonly affected than males. Glomus tumors exhibit a familial tendency and, in this form, males are more commonly involved. An autosomal dominant inheritance pattern has been suggested.

A slow, inexorable growth bereft of symptoms is characteristic. When far advanced,

Vagal paraganglioma (glomus vagale)

Despite the rarity of the vagal paraganglioma, several series have been reported.[34], [43], [44], [45], [46], [47], [48] As in CBT, surgical resection has traditionally been advocated for cervical glomus vagale tumors. However, surgical morbidity related to cranial nerve dysfunction is more common to vagal tumors then for CBT. As vagal tumors are situated more cephalad than CBT, the approach for these neoplasms is similar to that used for jugulotympanic tumors, and may result in multiple

Laryngeal paraganglioma

True laryngeal paragangliomas are rare lesions and more than 70 cases have been reported.[12], [50], [51], [52], [53], [54], [55], [56], [57], [58], [59] This is a rare presentation, and most of the cases reported in the literature are isolated case series. The classic presentation of the patient is a sore throat, hoarseness, and rarely airway issues due to the large size of laryngeal paraganglioma. The fiberoptic evaluation invariably reveals a submucosal mass in the supraglottic larynx, the

Sinonasal paraganglioma

Relative to all head and neck paraganglioma, sinonasal paraganglioma are rare and only approximately 30 cases have been reported since 1957.[61], [63], [64], [65] They occur most commonly in middle-aged women. No familial cases have been reported. A multifocal paraganglioma has been also reported involving the paranasal sinuses.⁶⁵

Pathologic studies have demonstrated paraganglionic tissue at the terminal part of the pterygopalatine fossa, and there is also a close anatomic association with the

Thyroid paraganglioma

The very existence of true primary thyroidal paragangliomas is a matter of considerable debate; while some authors are willing to accept a very few cases of paraganglioma of the thyroid as real entities,[74], [75], [76] other authors have asserted that such tumors simply do not exist.⁷⁷ One problem with invoking the notion of a primary thyroidal paraganglioma is the absence of documented normal paraganglia within the thyroid parenchyma. One counter argument to this might be to speculate, as

Conclusion

Paragangliomas of the head and neck are uncommon, slow-growing tumors of neuroendocrine origin arising from the parasympathetic nervous system. Although they contain neurosecretory granules, they rarely are physiologically active (1–3%). The vast majority are benign. There is a low malignancy rate which is determined by metastasis, not local invasion.

The most common paraganglioma in the head and neck is the carotid body tumor followed by tympanic paragangliomas. They are slightly more common in

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We report a case of vagal paraganglioma occurring in a 13-year-old male which is an extremely rare presentation in this age group. The patient presented with a large solitary painless progressively growing mass in posterior triangle of neck. External jugular vein was stretched and trachea was deviated medially. The mass was arising via a twig from vagus nerve and was surgically excised. Diagnosis was established post-operatively through histopathological analysis.
Vagal paraganglioma is a rare occurrence in male teenagers and may mimic schwannoma, neuroma, jugular meningioma, or other gangliomas. Surgical excision is mainstay of treatment but resultant vagal complications and neurological consequences are usually unavoidable. Nonetheless, the prognosis may be easily improved with sound surgical judgement, skill, and routine follow-up.
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Paragangliomas (PGLs) are a group of neuroendocrine tumors that arise in the parasympathetic or sympathetic ganglia. Head and neck PGLs (HNPGLs) comprise 65% to 70% of all PGLs and account for 0.6% of all head and neck cancers. The majority of HNPGLs are benign, and 6% to 19% of all HNPGLs develop metastasis outside the tumor site and significantly compromise survival. PGLs can have a familial etiology with germline sequence variations in different susceptibility genes, with the gene encoding succinate dehydrogenase being the most common sequence variation, or they can arise from somatic sequence variations or fusion genes. Workup includes biochemical testing to rule out secretory components, although it is rare in HNPGLs. In addition, imaging modalities, such as computed tomography and magnetic resonance imaging, help in monitoring in surgical planning. Functional imaging with DOTATATE-positron emission tomography, 18F-fluorodeoxyglucose, or 18F-fluorohydroxyphenylalanine may be necessary to rule out sites of metastases. The management of HNPGLs is complex depending on pathology, location, and aggressiveness of the tumor. Treatment ranges from observation to resection to systemic treatment. Similarly, the prognosis ranges from a normal life expectancy to a 5-year survival of 11.8% in patients with distant metastasis.
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ReviewParagangliomas of the head and neck

Abstract

Introduction

Section snippets

Familial paraganglioma

Classification

Gross pathology

Clinical characterization

Clinical and radiographic evaluation

Treatment

Carotid body tumor (carotid body paraganglioma)

Jugulotympanic paraganglioma (glomus jugulare tumor)

Vagal paraganglioma (glomus vagale)

Laryngeal paraganglioma

Sinonasal paraganglioma

Thyroid paraganglioma

Conclusion

Otolaryngol. Clin. North Am.

Am. J. Otolaryngol.

Otolaryngol. Clin. North Am.

Otolaryngol. Head Neck Surg.

J. Vasc. Surg.

Am. J. Otolaryngol.

Otolaryngol. Head Neck Surg.

Otolaryngol. Head Neck Surg.

Otolaryngol. Clin. North Am.

Otolaryngol. Head Neck Surg.

Am. J. Med. Sci.

Otolaryngol. Clin. North Am.

Otolaryngol. Head Neck Surg.

Auris. Nasus. Larynx.

Malignant glomus tumors

Laryngoscope

Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management

Am. J. Otol.

Multicentric chemodectoma in the head and neck

Aust. N. Z. J. Surg.

The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma

N. Engl. J. Med.

Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association. Ultrastructural study and review

Cancer

Neuroendocrine carcinomas of the larynx. A study of two types, one of which mimics thyroid medullary carcinoma

Am. J. Surg. Pathol.

Paraganglioma of the larynx. A critical review of the literature

ORL. J. Otorhinolaryngol. Relat. Spec.

Neuroendocrine neoplasms

Identification, classification, treatment, and prognosis of laryngeal paraganglioma. Review of the literature and eight new cases

Ann. Otol. Rhinol. Laryngol.

Laryngeal paraganglioma versus atypical carcinoid tumor

Ann. Otol. Rhinol. Laryngol.

Neuroendocrine tumors of larynx

Ann. Otol. Rhinol. Laryngol.

Familial carotid body tumors: case report and epidemiologic review

Cancer

Repositioning the hereditary paraganglioma critical region on chromosome band 11q23

Hum. Genet.

World Health Organization. International histological classification of tumours. Histological typing of tumours of the upper respiratory tract and ear

Neuroendocrine neoplasia. Current concepts

Am. J. Clin. Pathol.

Does intervention improve the natural course of glomus tumors? A series of 108 patients seen in a 32-year period

Ann. Otol. Rhinol. Laryngol.

Tumors of the head and neck. Clinical and pathological considerations

Pulsatile tinnitus and the vascular tympanic membrane: CT, MR, and angiographic findings

Radiology

Pigmented vagal paraganglioma

J. Laryngol. Otol.

Paraganglioma of the cervical sympathetic trunk

Ann. Otol. Rhinol. Laryngol.

Sympathetic paraganglioma as an unusual cause of Horner's syndrome

Head Neck

Review
Paragangliomas of the head and neck