Am J Otolaryngol t2:278-287,

1991

Vagal Paragangliomas: A Report of Nine Cases CHRISTOPHER ERIKSEN, MD, HARSHA GIRDHAR-GOPAL, MD, AND LOUIS D. LOWRY, MD

Vagal paragangliomas are rare tumors of neural crest origin: fewer than 175 cases have been reported in the English literature. This slow-growing tumor occurs most often at the base of the skull in the parapharyngeal space, but may arise anywhere along the course of the vagus nerve and its branches. Nine clinical presentations and the surgical outcome in seven patients with vagal paragangliomas treated at our institution are reported. A review of the literature, emphasizing evaluation and treatment of these potentially fatal neoplasm& is discussed. Surgical removal is the treatment of choice with vagal tumors. Complications related to the ablation or injury of cranial nerves IX, X, XI, and XII are commonly seen. Surgical techniques that may prevent injury to these vital neural structures are outlined. The postoperative treatment of patients with impaired deglutition secondary to multiple cranial neuropathies is discussed. AM J OTOLARYNGOL 12:278-287. Copyright 0 1991 by W.B. Saunders Company

in the jugular foramen, vagal nerve ganglia, and ciliary ganglia of the eye.7 The function of the intravagal paraganglion is unknown. The etiology of neoplasia is also unknown. The suggestion that paragangliomas are related to high elevation and anoxia was made by Saldana et aL8 The nomenclature of the paraganglionic system and tumors is confusing at best. The chromaffin histochemical reaction, allegedly for catecholamines, is not sensitive; hence, the term “nonchromaffin” paragangliomas is archaic. The term “chemodectoma” is used to imply tumors of chemoreceptor cells. Although the aortic and carotid bodies have a chemoreceptor function, no tumor has been shown to act as a chemoreceptor. The term “glomus tumor” is commonly used to describe these paraganglionic neoplasms. However, a glomus is actually a subcutaneous collection of modified smooth muscle elements unrelated to neuroendocrine cells of paraganglionic tissue.g-11 Therefore, the term “paraganglioma,” proposed by Glenner and Grumley,l’ is the preferred designation, based on the function and anatomic site of origin of these tumors. Head and neck paragangliomas are rare. The pathology department of the Sloan-Kettering Cancer Center (New York, NY) reported only 69 instances in 600,000 cases examined over 38 years.5 They calculated a 5% incidence of vagal paragangliomas. Fewer than 175 cases have been reported in the English literature. 4~12*13Women are reportedly affected more often, with a female to male ratio of 2.7:1.13*‘4 The treatment of choice of vagal body tumors is complete surgical excision. The glomus vagale can undergo malignant transformation and metastasize,15 or infiltrate local tissues at the base

Vagal paragangliomas are slow-growing neoplasms that arise from paraganglia along the course of the vagus nerve. They rank third in frequency among glomus tumors, representing approximately 5% of head and neck paragangliomas. They most commonly present as a neck mass behind, and deep to, the angle of the mandible. There may be associated pulsatile tinnitus and hearing loss. I-3 Vagal nerve paralysis with hoarseness and dysphagia occurs in approximately 30% the mass may have a of cases.4-6 Occasionally, pulsation or bruit. Other cranial nerves may be involved due to invasion or compression of the jugular foramem7 Glomus tumors originate from paraganglionic tissue. The paraganglion cells arise from the neural crest and migrate to their final destinations of the dorsal root ganglions of either the spinal cord or the cranial nerve ganglia. Paraganglions are analogous to the adrenal medulla; however, they lack affinity for chromium salts and therefore have been formerly termed “nonchromaffin” paragangliomas. (Tumors of the adrenal medulla are termed “pheochromocytomas” or “chromaffin” paragangliomas.) Paraganglionic cells are found around the aortic and carotid bodies, lung, middle ear, jugular vein

Received July 12, 1991, from the Department of Otolaryngology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA. Accepted for publication August 5, 1991. Address correspondence and reprint requests to Louis D. Lowry, MD, Clinical Office Bldg, 909 Walnut St, 3rd Floor, Philadelphia, PA 19107. Copyright 0 1991 by W.B. Saunders Company 0196-0709/9111205-0003$5.00/O 278

279

ERIKSEN ET AL of skull and intracranially. Multiple cranial neuropathies can result from continued growth.‘*16 “Paraganglia of the head, neck and superior mediastinum closely resemble the carotid bodies (intercarotid paraganglia). These paraganglia are typically related to arterial vasculature and cranial nerves of the ontogenetic gill arches and are designated as branchiomeric paraganglia. The branchiomeric paraganglia include jugulotympanic, intercarotid, subclavian, laryngeal, coronary, aorticopulmonary and pulmonary paraganglia. Paraganglia of the orbit and the intravagal paraganglia cannot be distinguished from the branchiomeric class on any microscopic basis, but are not intimately associated with arteries and, therefore, should likely be tentatively considered as a separate group.“7 Paragangliomas exhibit a “Zellballen (cell ball) pattern” and ultrastructural neurosecretory granules. There are two cell types, the chief cells (type I epithelial cells) with catecholamine granules and the sustentacular cells or Schwann-like satellite cells (type II supporting cells). They are surrounded by reticulin fibers and capillaries. The light microscopic findings of intravagal paragangliomas are similar to the findings in other branchiomeric paragangliomas. The vascular structures are less prominent and hyalinization of the fibrous stroma is more marked than those of glomus jugulare tumors. Using electron microscopy, the ultrastructure of the intravagal paragangliomas is indistinguishable from other branchiomeric paragangliomas. Electron microscopy demonstrates neurosecretory granules (that may contain catecholamines) similar to those seen in neuroblastomas. Criteria for malignancy are based on clinical, rather than histopathologic, grounds.17 Features such as vascular invasion, necrosis of Zellballen, nuclear pleomorphism, and mitotic rates have not been associated with malignancy.‘0,‘lJ8 Immunohistochemical procedures have been proposed recently as indicators of tumor aggressiveness.‘g*20 Batsakis7 states that the vagal paraganglioma arises from the ganglion nodosum (inferior ganglion) of the vagus nerve. This ganglion derived from nests of paraganglionic tissue within the perineurium of the nerve. Intravagal tumors may erupt at various sites along the nerve.“” Paraganglioma juxtavagale arises from the middle vagal ganglion, often eroding the jugular foramen and possibly the atlas. The superior vagal paraganglioma usually presents as a dumbbell-shaped tumor with intracranial extension. In this report, we review nine cases of vagal paragangliomas with attention to presentation, clinical outcome, and complications of surgery. Two paragangliomas of the laryngeal nerves have been previously reported.gr20

The major morbidity of vagal paraganglioma resection results from neurologic damage. The vagus nerve is sacrificed in most instances, with glossopharyngeal and hypoglossal injury being quite common. Ensuing problems with phonation and swallowing are particularly distressing. CASE REPORTS

Case No. 7 (Inferior Laryngeal Ganglion). A 26-year-old white man (J.M.) was healthy prior to April 1983. He had one episode of hemoptysis that abated after a course of antibiotics, Hemoptysis recurred while jogging 1 year later. Endoscopic examination showed an intraluminal mass of the trachea. A biopsy resulted in profuse bleeding and the patient was packed and underwent tracheostomy. He was transferred to our institution. A 3-cm rubbery mass in the right thyroid lobe was noted. The patient underwent partial laryngectomy in April 1984 and reconstruction with a sternocleidomastoid muscle flap and Montgomery T-tube. Three units of blood were transfused. The preoperative vanillylmandelic acid (VMA) was 12.7 mg/dL [normal, 2 to 10 mg/dL). Postoperatively, the right vocal cord was paretic. The patient was decannulated at 6 months and has a socially acceptable voice. The patient has had no recurrence. This case was previously reported as a paraganglioma of the recurrent laryngeal nerve.*’ There was no family history of paragangliomas. Case No. 2. A 26-year-old white woman (M.K.) presented with a mass in the right neck and hoarseness of 2 years’ duration. Normal VMA levels were obtained preoperatively. There was no family history of paragangliomata. The mass was firm, nontender, mobile, without a bruit, and measured 4 x 6 cm. Laryngeal examination was normal. An angiogram showed a vascular blush above the carotid bifurcation. The patient underwent surgical resection via a lateral cervical approach in February 1986. The specimen is shown in Fig 1. The vagus nerve was sacrificed. Glossopharyngeal nerve paralysis was noted postoperatively. Blood loss was 400 mL; the patient did not receive blood. Pathology revealed atypia and local invasion but no neck node metastasis. Teflon injection of the right vocal cord was performed in June 1986, resulting in voice improvement. The patient swallows well and has had no recurrence. She has had resolution of a hypoglossal nerve paresis.

Case NO. 3. A 76year-old white man (T.L.) was seen in the emergency room April 1987 with an B-month history of vertigo and unsteadiness while walking. He had a right neck mass for more than 1 year. Examination revealed that the 8 x 5 x 5 cm mass nearly filled his oropharynx. A laryngeal examination was normal except for the mass effect. There was no family history of paragangliomata. No symptoms of catecholamine excess were elicited. He was admitted on May 6, 1987, and underwent neuroangiography, which showed a large vascular mass consistent with a vagal paraganglioma. The major blood supply was from the ascending pharyngeal branch of the external carotid artery. The patient underwent resection via a right lateral cervical approach. Dissection from the carotid bifurcation to the base of the skull allowed complete tumor removal. The vagus nerve was sacrificed, but all other

280

VAGAL PARAGANGLIOMA

Figure 1. Gross specimen of a vagal paraganglioma from case no. 2.

1

2

3

4

nerves were left intact. Blood loss was 500 mL; an equal quantity of autologous blood was replaced in the operating room. Postoperatively, the patient did well, with a notable hypoglossal defect. Four hours after surgery the patient became agitated and disoriented. A left visual field cut was noted. Appropriate consultation with a neurologist and a timely computed tomography (CT) scan secured a diagnosis of a right parieto-occipital infarct, likely secondary to an embolic phenomenon. The patient also suffered a memory and cognitive defect that completely resolved over the ensuing 6 months. The patient swallowed well postoperatively with no aspiration. A barium swallow at 8 weeks demonstrated cricopharyngeal spasm. He manifests a persistent right Horner’s syndrome. No recurrence at 4 years is noted. &Se NO. 4. A i’O-year-old white woman (L.N.) presented with symptoms of voice change over 6 years. She was most concerned with a recent parapharyngeal mass causing airway obstruction in the supine position. She also had nasal obstruction for 6 months. There was no family history of paragangliomas. The laryngeal examination was normal. A CT scan demonstrated a large parapharyngeal enhancing lesion (Fig 2). An angiogram showed a large vascular mass with its major blood supply from the external carotid artery (Fig 3). In April 1988, the patient underwent resection by median mandibulotomy. Carotid reanastamosis was required. A tracheotomy was performed. Postoperatively, her swallowing function was dismal and she had persistent paresis of the ipsilateral cranial nerves IX, X, and XII. She had a Teflon augmentation of the ipsilatera1 vocal cord and, later, a cricopharyngeal myotomy. She is unable to eat or drink at present due to aspiration. The patient has no recurrence at 3 years. Computed tomography scans show no evidence of multicentric lesions.

5

cm.

&Se NO. 5. A 86-year-old white woman (S.B.) presented with a left neck mass (Fig 4) that had progressively increased in size over 2 years. There was no family history of paragangliomas. An angiogram showed a vagal paraganglioma with the major blood supply from the external carotid system. Urine catecholamines were normal. She underwent resection via a lateral cervical approach in July 1989. Resection of the tumor encircling the internal carotid was performed, with sacrifice of the vagus nerve. The entire tumor was removed at the jugular foramen. Although the hypoglossal nerve was not sacrificed, it was paretic postoperatively. Her palate on the same side is flaccid, although the gag reflex is intact. The patient received 2 U of autologous blood intraoperatively for a ~,OCKJ-mL blood loss. Postoperatively, the patient was unable to eat. She required a gastrostomy tube, a cricopharyngeal myotomy, and a Teflon vocal cord injection within a few weeks. This patient was able to begin to eat by mouth at approximately the 18th month and had her gastrostomy tube removed. She is without clinical evidence of recurrence from multicentric disease at 1.5 years.

Case NO. 6. A 57-year-old white woman (MC.) had a carotid body tumor resected on April 1990 without transfusion or sequelae. On review of the magnetic resonance image (MRI) and the angiogram, a contralatera1 Z-cm vagal paraganglioma was noted. Resection is anticipated. No neurologic or endocrine abnormalities are noted. Case No. 7. (Superior Laryngeal Nerve Ganglion). An 86-year-old white man was evaluated for dysphagia and hoarseness. A paraganglioma of the superior laryngeal nerve was treated endoscopically, with no sequelae or recurrence, in 1979. This case was previously reported.’

ERIKSENET AL

281

Figure 2. Angiogramfrom case no. 4 demonstrating anterior displacement of internal and external carotid well above the carotid bifurcation at the skull base.

Case NO. 8. A 56-year-old white man (F.B.) presented with a 5-year history of a progressively enlarging neck mass that he was told was benign. He came to our office after a fine needle aspiration was read as malignant. There was no family history of paragangliomas. He underwent resection via a lateral cervical approach with sacrifice of the vagus nerve and sympathetic chain. Concurrent tracheostomy, cricopharyngeal myotomy, and Teflon injection were performed with a 1,2oo-mL blood loss in August 1990. Decannulation was not a problem and the patient is taking most foods by mouth. The ipsilateral hypoglossal is paretic, but the gag reflex is intact. No blood was transfused. There is no clinical or radiographic evidence of multicentricity. &Se NO. 9. A 56-year-old man (W.B.) with a history of alcohol abuse presented with difficulty swallowing. A 10 x 10 cm Iesion of the pharynx and left neck was revealed by MRI (Fig 5). The patient refused surgical intervention. RESULTS Nine patients

were reviewed in this study of All patients were treated by one of us &D-L.). Table 1 reviews the presenting complaints, most commonly that of a neck mass. The patients’ ages ranged between 26 and 86 years. There were four women and five men. One patient had multicentric lesions. No family his-

vagal paragangliomas.

tory of paraganglioma tumors could be elicited in the affected patients. One patient had a slight elevation of urinary catecholamines without symptoms. Seven cases underwent surgery and all were completely resected. No local or distant metastases were noted; therefore, none of the tumors were malignant. None were irradiated. No recurrences have been detected by CT or MRI. No patients underwent embolization because of the risk of embolism in the internal carotid artery. Consent for embolization was obtained in the last three patients. The average blood loss was 813 mL

(Table 2). Cranial neuropathies are outlined in Table 3. Patients no. 1, 2, 3, 5, and 8 had transient problems with swallowing. Patient no. 4 has not been able to resume swallowing and has persistent neuropathies of the glossopharyngeal, vagal, and hypoglossal nerves on the side of tumor resection. Cricopharyngeal myotomy was performed on the last three patients in an attempt to improve pharyngeal emptying. Teflon augmentation was performed on four patients (nos. 2, 4, 5, and 8). The surgical excision of high vagal paragangliomas was via the lateral cervical approach in most patients. A median mandibulotomy with plating was performed on patient no. 4. Protection of the pulmonary tree with permanent tracheostomy has

282

VAGAL PARAGANGLIOMA

Figure 3. Computed tomograph from case no. 4 showing a large parapharyngeal mass.

been necessary from 6 months

in patient no. 4. Follow-up to 11 years.

ranges

DISCUSSION Vagal body tumors are situated more cephalad than the more common carotid body tumor. They arise from the nodose ganglion and commonly extend to the base of the skull at the jugular foramen. ‘l Intracranial extension has been described. Vagus nerve fibers are often “splayed out” over the tumor so that nerve function may be preserved arising until quite late.” Vagal paragangliomas from the inferior ganglion (nodose ganglion) typically present as a painless mass behind the angle

of the mandible and parapharyngeal space.7 This was the most common presenting symptom in our patient population (Table 1). Frequently, the mass bulges into the pharyngeal wall in the peritonsillar area, producing dysphagia. The mass may have palpable vascular pulsations or an audible bruit. Fifty percent of patients will have one or more cranial nerve palsies, most commonly of the vagus nerve-l4 Approximately 50% have had signs or symptoms for 3 years prior to presentation.” Other symptoms and signs may include pharyngeal pain, glossopharyngeal nerve paralysis, pulsatile tinnitus, Horner’s syndrome, jugular foramen syndrome, deafness, and syncope.23 It is our belief that the functional disability from

ERIKSEN

ET AL

283

tion over years may be the outcome. Similar difficulties were noted by Green et al” and Biller et a1.4 Consideration of early vocal augmentation with Teflon has been proposed by Biller et a1.4 Gelfoamz4 or autologous fatz5 may be injected at the time of resection. In our series, late injection of Teflon under local anesthesia was performed on patients no. 2, 4, and 5. Biller et al4 suggest concomitant cricopharyngeal myotomy, as performed on patient no. 8. This procedure was performed late on patients no. 4 and 5 because of persistent dysphagia and pooling of secretions, We have considered suspension or removal of the patulous pyriform sinus as a therapeutic step, but have yet to attempt it.

Figure 4. Computed tomograph from case no. 5 depicting an enhancing lesion in the parapharyngeal space.

glomus vagale is related to the site of the neoplasm rather than to its size. Sacrifice of the vagus nerve was necessary in all cases. High vagal sacrifice produces more morbidity than low sacrifice. The surgeon can plan for the expected neurologic deficits. Hypoglossal paralysis from intraoperative manipulation seems to contribute greatly to the morbidity of the procedure. The risk of aspiration and poor deglutition is highest when ipsilateral glossopharyngeal, vagal, and hypoglossal neuropathies result from surgery. The immobile hemitongue with hypesthesia and pyriform sinus atony contribute to difficulty in the pharyngeal phase of deglutition, pooling of secretions, and delayed aspiration. In our series, the hypoglossal neuropathies were apparently the result of surgical traction rather than sacrifice. The nerve must be treated gently and its function carefully evaluated postoperatively. Return of hypoglossal nerve function has been noted in only one of our patients who suffered paresis. The triple defect of ipsilateral IX, X, and XII nerve ablation seems detrimental to restoration of effective deglutition. One patient (no. 4) is dependent on a gastrostomy tube for nutrition. Electromyographic studies and crossed nerve anastomoses have not been evaluated to our knowledge (Table 3). Slow compensa-

Multicentric Origin. The incidence of multicentricity in head and neck paragangliomas is lo%.6 The incidence of multicentricity in nonfamilial glomus vagale is as high as 200$,.~ In the familial variety of glomus vagale, the incidence rises to 35%.14 Biller et al’s series showed 38% multicentricity.4 The inheritance pattern is autosomal dominant with variable penetrance. In more than 90% of cases, at least one of the neoplasms is a carotid body tumor.14 In our series, the one case in nine represents an 11% rate of multicentric disease, with no familial patterns detected. Malignant. Differentiation between benign and malignant vagal paragangliomas must be made on clinical grounds.‘” For a glomus vagale to be considered malignant, metastases must be documented. Benign and malignant tumors cannot be distinguished histologically. Metastases must be found in sites where chromaffin tissue does not normally occur. The prognosis for metastatic glomus vagale tumors appears to be worse than for the nonmetastatic type; the former exhibit more aggressive neoplastic behavior.13 The frequency of metastases is 10 to 19%.14r’5,23r27 Cervical metastases were noted in 10 (67%) of 15 cases reviewed by Heinrich et a1.13 Distant metastases were noted in 27% of these patients. Twentyseven percent of all patients had lung metastases and 13% had metastases to bone. One case had multiple metastases to the liver, bone, and brain. Therefore, hematogenous as well as lymphatic spread is possible. Glomus vagale metastases may appear sooner than those of carotid body tumors (an average of 3 years v 9.8 years).13 Preoperative Planning. Preoperative planning for the patient with a vagal paraganglioma should include serum catecholamine screening

VAGAL PARAGANGLIOMA

Figure 5. Magnetic resonance image from case no. 9 exhibiting the typical “salt and pepper” appearance on a T, image.

with N-hour metanephrine and VMA levels monitoring to detect the 2% to 5% of secreting glomus tumors.28~2gIf necessary, venous sampling for catecholamines can be performed during angiography.30.3* Barium swallow should be recorded on video to evaluate deglutition and for postoperative comparison. Imaging of glomus vagale tumors should initially include thin-section CT scanning with contrast enhancement. Evaluation of the jugular foramen for neoplastic involvement is necessary. Magnetic resonance imaging is a useful, noninvasive modality for asymptomatic and synchronous tumors, and for postoperative screening for recurrent paragangliomas. Olsen et a13’ demonstrated MRI results on 15 paragangliomas in 10 TABLE

PATIENT No.

AGE (YR)/SEX 26/M 26/F 7oiM 71/F 3oiF 57/F 86iM 56/M 56/M

2.

SYMPTOMS SIGNS Hemoptysis, neck mass Neck mass Vertigo, oropharynx mass Hoarseness, airway obstruction Neck mass Neck mass Dysphagia, hoarseness Neck mass, pain Neck mass

patients. Computed tomography better demonstrated subtle osseous changes of the skull base and middle ear structures. Magnetic resonance imaging showed the characteristic “salt and pepper” pattern in T,-weighted images for all lesions larger than 2 cm. The relationship to local carotid sheath vessels and intracranial structures was better demonstrated by MRI. Smaller lesions are more conspicuous. This modality is a safe, noninvasive method of evaluating patients and family members for subclinical paragangliomas.32s33 Magnetic resonance imaging will likely become the best screening tool. Radionuclide angiography has been used as an initial study in high-risk individuals. It is a highly sensitive test using “Tc

Demographic Data CATECHOLAMINES Slight elevation No No No No No No No No

FAMILY HISTORY No No No No No No

MULTICENTRIC No

MALIGNANT No No No

No

No No No No Yes No

No No

No

No

No

No

No No No No

285

ERIKSEN ET AL TABLE 2.

Blood Loss/Replacement

ESTIMATED BLOODLoss

PATIENTNo. * 1 2 3 4 5

1000 400 500 780 1000

8

1200 mL

REPLACEMENT 750 mL PRBCs None 500 mL autologous blood None 500 mL donor-specific whole blood None

mL mL mL mL mL

292 mL

813 mL

Average

Abbreviation: PRBCs, packed red blood cells. * Patients no. 6 and 9 refused surgery. Patient no. 7 underwent endoscopic removal with negligible blood loss.

gluconate

with

has a lower

a peripheral

complication

bolus

technique

and

contrast

neu-

rate than

roangiography.34 Arteriography gram

is the

tumor

size,

filling

from

ceed

the

modality

when

tervention.

contralateral

occlusion

must

decreased

tumor

risk

prior

angio-

artery with

must embo-

necessary,

should surgical

of cerebral

artery

against

vascularity,

of

Cross-over

to planned

be weighed

operative

carotid

arteriography,

considered

The

cerebral

for evaluation

and vascularity.

Selective

24 to 48 hours

shorter

a four-vessel

extension,

be assessed. lization

with

standard

proin-

spasm

the benefits

less blood

loss,

or of and

time.l’*

Surgical Considerations. The glomus vagale is generally excised through a lateral cervical approach. Sectioning of the mandible at the angle or a median mandibulotomy may be required for adTABLE 3. ADJUNCTIVE PATIENT

Postoperative

Complications

POSTOPERATIVE SWALLOWING

PREOPERATIVE CRANIAL NERVEDEFICIT

equate exposure of the skull base.‘,* A combined skull base-neck approach, as well as an intracranial skull base approach, may be used for dumbbell-shaped tumors with intracranial extenWide exposure is necessary for visusion. 1*23,26,2g alization of the entire tumor mass and adjacent neurovascular structures, and for vascular control. Clearing of the tumor from the common and internal carotid arteries with subadventitial dissection is preferable. A vascular surgeon must be available during the removal of glomus intravagale should carotid injury occur or carotid sacrifice be necessary. Interposition of a reversed venous homograft or a Dacron (DuPont, Wilmington, DE) graft may be required. An intraoperative electroencephalogram is imperative when working on the carotid artery. Performance of a concurrent cricopharyngeal myotomy should be a consideration in preoperative planning.* Jackson et al stress the importance of nasogastric intubation and airway support by tracheostomy in the patient undergoing glomus resection.’ Unilateral vagus nerve interruption will predispose a patient to aspiration pneumonitis, a potentially fatal complication. Bilateral vagal nerve loss at the jugular foramen may result in rapid death following complete laryngeal paralysis, aphonia, vomiting, dilation of stomach, and cardiac arrythmia. Adjunctive techniques such as hypotensive anesthesia, hypothermia, embolization, intravascular balloons, and improved neuroradiographic techniques have improved surgical results in recent years. Blood transfusion can be minimized in a numand Adjunctive

Procedures

COMPLICATIONS AND CRANIAL NERVEDEFICIT

VOICE

1 2 3 4

None None Mild X paresis None

Excellent Excellent Excellent Poor

Fair Fair Fair Poor

X X, XI (XII, resolved) X, XII CVA IX, x XII

5

None

Delayed to 18 mo; fair

Fair

IX, x, XII

6 7 8

None None None

*

*

*

Excellent Fair

Fair Fair

X x, XII

9

None

*

l

*

Abbreviations: WA, cerebral vascular accident; EEG, electroencephalogram. * No surgery has been done at the time of this publication.

PROCEDURES T tube, tracheostomv Teflon augmentation Intraoperative EEG Intraoperative EEG, carotid reanastamosis, tracheotomy, G tube, Teflon augmentation cricopharyngeal myotomy Intraoperative EEG, Teflon augmentation, cricopharyngeal myotomy, G-tube * Endoscopy Teflon augmentation cricopharyngeal myotomy *

VAGAL PARAGANGLIOMA

286

ber of ways. Autologous blood transfusions may be stockpiled a few weeks prior to surgery. Donordirected transfusions are controversial. The use of autotransfusion devices is acceptable as the procedure is rarely contaminated. The preoperative use of transarterial embolization has been discussed previously (Table 2). Other intraoperative considerations include the use of low-wattage bipolar cautery during surgical extirpation, which is to be encouraged to avoid neural injury. As this is an extremely vascular tumor, hemostatic techniques are essential. Bipolar cautery is safer to use in proximity to major neurovascular structures. In addition, the use of dynamic pneumatic compression boots during any prolonged procedure is recommended to decrease the risk of thromboembolism.24

useful technique for improving the timeliness of triggering the pharyngeal swallow.3g During swallowing, techniques such as bearing down, coughing, or clearing the throat may improve laryngeal closure. The throat is cleared, then the patient holds his or her breath while swallowing. After the swallow, the patient should cough prior to inhalation to clear residue from the pharynx. A gastrostomy or percutaneous endoscopic gastrostomy tube may be required if swallowing is inefficient or hazardous. The patient should be made aware of this possibility prior to surgery.

Radiation Therapy. The role of radiotherapy is limited in the treatment of glomus tumors. Marruyana35 notes that tumor irradication is not the end result of radiotherapy management of glomus jugulare. A positive clinical response consists of cessation of tumor growth and involution. Reduced tumor vascularity but little change in tumor cell morphology is observed.35 There is a radiation-induced endarteritis with infarction and fibrosis of the parenchyma.36-38 The superiority of combined therapy or surgery alone over radiotherapy has been demonstrated by a number of studies. 3,28,36,38 The following have been suggested as indications for radiotherapy: (1) refusal of surgery, (2) incomplete excision with skull base or intracranial extension, (3) histologic evidence of metastatic disease, (4) debilitated or medically compromised patients, (5) unresectable tumors, and (6) contralateral neoplasms (to avoid bilateral vagal or hypoglossal pareses). In addition, it may be applied preoperatively to decrease tumor vascularity.35‘38

a%.4,10,14

Postoperative follow-up. The patients should be evaluated postoperatively for the possibility of glossopharyngeal, spinal accessory, hypoglossal, and cervical sympathetic neuropathies. There is a risk of aspiration postoperatively due to the expected ipsilateral vocal cord paresis and the pharyngeal dysfunction associated with high vagal denervation. Speech pathology consultation is recommended to evaluate and treat postoperative complications of deglutition. The patient should be monitored for aspiration by direct visualization or barium study. Thick liquids, puddings, and purees are often better tolerated than thin liquids. The patient should turn toward the affected side when swallowing in order to empty the patulous pyriform sinus. Thermal stimulation may be a

follow-up. Lifelong follow-up should include observation for multicentric paragangliomas (13%) and malignant transformation (10% to 19%). Screening of family members is recommended, as familial incidence is approximately The most challenging long-term goal is the restoration of efficient swallowing function, allowing the patient to resume a normal lifestyle. CONCLUSION Vagal paragangliomas are rare, vascular, slow-growing neoplasms presenting with subtle signs and symptoms related to a mass effect in the parapharyngeal space. Careful preoperative assessment for cate2. cholamine excess should be undertaken. 3. Evaluation with angiography is necessary prior to surgery. Multicentric lesions are common and must 4. be pursued diagnostically. Surgical resection is most commonly done 5. via a lateral cervical approach. Meticulous dissection and hemostasis are necessary. Preparation for a vascular interposition graft or reanastamosis is imperative. 6. Although vagal sacrifice is universal, care must be taken not to injure other cranial nerves, thereby lessening postoperative morbidity. Postoperative evaluation of cranial nerve 7. deficits and appropriate attention to the rehabilitation of swallowing and speech are vital to the well-being of the patient. Adjunctive tracheostomy, vocal cord augmentation, feeding tubes, and cricopharyngeal myotomy may be needed. a. Lifelong follow-up for recurrent or multicentric disease is essential. 1.

References 1. Black FO, Myers EN, Parnes SM: Surgical management of vagal chemodectomas. Laryngoscope 1977; 67:1259-1269 2, Jackson CG, Glasscock ME III, Harris PF: Glomus tumors:

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Diagnosis, classification and management of large lesions. Arch Otolaryngol 1982; 108:401-4063. Iackson CG. Glasscock ME III. Nissen Al. et al: Glomus tumors surgery: The approach, results and problems. Otolaryngo1 Clin North Am 1982; 15:897-916 4. Biller HF, Lawson W, Som P, et al: Glomus vagale tumors. Ann Otol Rhino1 Laryngol 1989; 98:21-26 5. Lack EE, Cubilla AL, Woodruff JM, et al: Paragangliomas of the head and neck region: A clinical study of 69 patients. Cancer 1977; 39:397-409 6. Spector GJ, Ciralsky R, Maisel RI-I, et al: Multiple glomus tumours in the head and neck. Laryngoscope 1975; 85:10661075 7. Batsakis JG: Tumors of the Head and Neck: Clinical and Pathological Considerations (2nd ed). Baltimore, MD, Williams & Wilkins, 1979, pp 369-380 8. Saldana MJ, Salem LE, Travezan R: High altitude hvpoxia __ and chemodectomas. Hum Path01 1973; 4:251-263 9. Wetmore RF. Tronzo RD. Lane RI. et al: Nonfunctional paraganglioma of the larynx: Clinical and pathological considerations. Cancer 1981; 48:2717-2723 10. Glenner GG, Grumley PM: Tumors of the Extra-Adrenal Paraganglion System (Including Chemoreceptors). Atlas of Tumor Pathology, series 2, fascicle 9. Washington, DC, Armed Forces Institute of Pathology, 1974 11. Lawson W: The neuroendocrine nature of the glomus cells: An experimental ultrastructural and histochemical tissue culture study. Laryngoscope 1980; 90:120-144 12. Green JD, Olsen KD, DeSanto LW, et al: Neoplasms of the vagus nerves. Laryngoscope 1988; 98:648-654 13. Heinrich MC, Harris AE, Bell WR: Metastatic intravagal paraganglioma: Case report and review of the literature. Am J Med 1985; 78:1017-1024 14. Lawson W: Glomus bodies and tumors. N Y State J Med 1980; 80:1567-1574 15. Scully RE, McNeely BU: Case records of the Massachusetts General Hospital. N Engl J Med 1975; 292:741-745 16. Leonetti JP, Brachmann DE: Glomus vagale tumor: The significance of early vocal cord paralysis. Otolaryngol Head Neck Surg 1989; 100:533-537 17. Fernandez BB, Hernandez FJ, Staley CJ: Chemodectoma of the vagus nerve. Report of a case with ultrastructure study. Cancer 1975; 35:263-269 18. Barnes L, Taylor SR: Carotid body paragangliomas: A clinicopathologic and DNA analysis of 13 tumors. Arch Otolaryngol Head Neck Surg 1990; 116:447-453 19. Korat 0, Trojanowski JQ, LiVolsi VA, et al: Antigenic expression in normal paraganglia and paragangliomas. Surg Path01 1988; 1:33-40 20. Eyyunni U, Lowry LD: Chemodectoma of the neck presenting with hemoptysis and a thyroid mass. Trans Pa Acad Ophthalmol Otolaryngol 1985; 38:392-395

287 21. Persson AV, Frusha JD, Dial PF, et al: Vagal body tumor: Paraganglioma of the head and neck. CA 1985; 35:232-237 22. Moore G, Yarington CT, Mangham GA, Jr: Vagal body tumors: Diagnosis and treatment. Laryngoscope 1986; 96:533536 23. Endicott JN, Maniglia AJ: Glomus vagale. Laryngoscope 1980; 90:1604-1611 24. Sataloff RT, Myers DL, Kremer FB: Management of cranial nerve injury following surgery of the skull base. Otolaryngo1 Clin North Am 1984; 17:577-589 25. Mikaelian DO, Sataloff RT, Lowry LD: Lipoinjection for unilateral vocal cord paralysis. Laryngoscope 101:465-468, 1991 26. Druck NS, Spector GJ, Ciralsky KI-I, et al: Malignant glomus vagale: Report of a case and review of the literature. Arch Otolaryngol 1976; 102:634-636 27. Khan LB: Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association. Cancer 1976; 38:2367-2377 28. Karusseit VOL, Lodder JV: Functioning vagal body tumor. Br J Surg 1987; 74:1184 29. Spector GJ, Sobol S: Surgery for glomus tumors at the skull base. Otolaryngol head Neck Surg 1980; 88:524-530 30. Tannir NM, Cortas N, Allam C: A functioning catecholamine secreting vagal body tumor. A case report and review of the literature. Cancer 1983; 52:932-935 31. Schwaber MK, Glasscock ME, Nissin AJ, et al: Diagnosis and management of catecholamine secreting glomus tumors. Laryngoscope 1984; 94:1008-1015 32. Olsen WL, Dillion WP, Kelly WM, et al: MR imaging of paragangliomas. Am J Roentgen01 1987; 148:201-204 33. DeMarino DP, Mueller DP, Yuh WTC, et al: Imaging studv of the month: Multinle: oaraeaneliomas. Ann Otol rhino1 Laryngol 1990; 99:85-86 _ A - y 34. Laird JD. Ferguson WR, McIlrath EM, et al: Radionuclide angiography as the primary investigation in chemodectoma: Concise communication. I Nucl Med 1983: 24:475-478 35. Marruyana Y: Radiotherapy of tympanojugular chemodectoma. Radiology 1972; 105:659-663 36. Brachman DE, House WF, Terry R, et al: Glomus jugulare tumors: Effect of irradiation. Trans Am Acad Ophthalmol Qtolaryngol 1972; 76:1423-1431 37. Myers EN, Newman J: Glomus jugulare tumors: A radiographic-histologic correlation. Laryngoscope 1975; 85:690-696 38. Spector GJ, Compagnano J, Perez CA, et al: Glomus jugulare tumor: Effects of radiotherapy. Cancer 1975; 35:13161321 39. Logemann JA: The role of the speech language pathologist in the management of dysphagia. Otolaryngol Clin North Am 1988; 21:783-788