Key learning points

Introduction

The most common vascular tumors encountered by the otolaryngologist are rare chromaffin cell tumors termed paragangliomas. Within the head and neck region, they commonly arise from the carotid body ( Fig. 1 ), vagus nerve (glomus vagale) ( Fig. 2 ), and jugular vein (glomus jugulare). Other vascular head and neck tumors include sinonasal malignancies, because of proximity to or involvement of the pterygoid plexus as well as the rich vascularity of the sinonasal mucosa, juvenile nasopharyngeal angiofibroma, a vascular tumor of male adolescents; unusual vascular tumors such as hemangiopericytoma; and metastatic renal cell cancer, which has a proclivity for an unusually rich blood supply.

( A ) MRI of a left carotid body tumor (CBT). ( B ) Postresection bed demonstrating splaying of the internal and external carotid artery. Inset depicts resected CBT. ECA, external carotid artery; ICA, internal carotid artery; IJV, internal jugular vein.

( A ) Axial MRI demonstrating a left vagal nerve paraganglioma (glomus vagale). Note the lateral position to the great vessels. ( B ) Coronal MRI of the same tumor. Note the absence of internal and external carotid artery splaying, which is seen in the setting of a carotid body tumor.

The location and histology of the tumor are key points in considering how to minimize blood loss before surgery begins. CT ( Fig. 3 ) or MRI ( Fig. 4 ) can be obtained as a first step, and often both are obtained for planning purposes because these are complementary modalities. CT is superior for demonstrating bone involvement, but MRI is superior in demonstrating the soft tissue characteristics of the tumor, delineating tumor from muscle and vascular relationships, and demonstrating classic flow voids (see Fig. 4 ) in paragangliomas.

CT scan of a right glomus vagale ( large arrow ). Note the splaying of the internal jugular vein (IJV) from the common carotid artery (CCA). This patient presented with right vocal cord paralysis and associated hoarseness.

T2 fat-saturated axial MRI of a left carotid body tumor demonstrating the classic flow voids, which give a salt-and-pepper appearance to the tumor when contrasted imaging is used. The large arrow points to the internal carotid artery; the small arrow points to the external carotid artery. Note how the 2 branches are splayed by the tumor (lyre sign).

Angiography ( Fig. 5 ) has classically been used in the workup of most vascular lesions. Increasingly, catheter angiography has been replaced by MRA and CTA in the preoperative workup of vascular lesions. However, angiography can be useful when embolization is being considered, and angiography with balloon occlusion testing is necessary if malignancy is suspected and/or if carotid sacrifice or injury is anticipated during resection, such as in large carotid body tumors. Embolization has been reported to reduce blood transfusion requirements. However, embolization of paragangliomas remains controversial with some investigators reporting no effect on blood transfusion, an increase in cranial nerve injury, and increased costs.

Angiogram demonstrating a well-vascularized carotid body tumor. Note the hallmark lyre sign in which the internal and external carotid vessels are splayed.

When a paraganglioma is suspected, 24-hour urine collection should be performed to detect catecholamine secretion (vanillylmandelic acid, metanephrines). Secreting tumors can cause an intraoperative hypertensive crisis or cardiac arrythmias from manipulation and must be identified preoperatively so α-cardiovascular and β-cardiovascular blockade can be instituted. Approximately 10% of paragangliomas secrete catecholamines. If urine catecholamine levels are elevated, an MIBG scan should be obtained. MIBG scans use a radioisotope similar to norepinephrine and can localize catecholamine uptake and storage, which is important to rule out the primary tumor as the source. Because 10% of paragangliomas are multiple, when catecholamines are elevated, a CT of the abdomen should also be performed to detect an adrenal pheochromocytoma. Paraganglioma metastasis is estimated to occur in 10% to 36% of cases ( Fig. 6 ). Patients with malignant paragangliomas are often asymptomatic. The diagnosis is commonly rendered following pathologic evaluation of an enlarged lymph node, which demonstrates chromaffin cells. In such cases, a full metastatic workup is warranted to evaluate both regional and distant metastasis. Clinical examination will direct the need for imaging. Both PET and octreotide scintigraphy have proven helpful in this setting.

Transcervical approach to a carotid body tumor. The incision is made at least 2 fingerbreadths beneath the mandible to avoid injury to the marginal mandibular nerve.

Treatment options for metastatic disease include surgical resection, targeted therapy with radioactive metaiodobenzylguanidine ( ¹³¹ I-MIBG), or chemotherapy. Timely diagnosis and treatment are imperative to avoid myocardial infarction, severe hypertension, stroke, and arrhythmia.

Under most circumstances, a presumptive diagnosis can be made on the basis of the above-named imaging studies. Under no circumstances should biopsy be performed before obtaining the radiologic studies. FNA biopsy can be a useful adjunct when the mass is readily accessible, either transcervically or transorally, and can provide useful information if a diagnosis of malignancy is suspected. However, if imaging studies suggest a vascular lesion, FNA provides little if any useable information and is not indicated. Incisional biopsy should only be considered if the patient is not an operative candidate and FNA is inconclusive, and only if a diagnosis of malignancy or lymphoma is strongly suspected. Transoral open biopsy has been described but carries a risk of hemorrhage and contamination of the pharyngeal mucosa by tumor, which will require excision of that site during subsequent definitive resection.

Introduction

The most common vascular tumors encountered by the otolaryngologist are rare chromaffin cell tumors termed paragangliomas. Within the head and neck region, they commonly arise from the carotid body ( Fig. 1 ), vagus nerve (glomus vagale) ( Fig. 2 ), and jugular vein (glomus jugulare). Other vascular head and neck tumors include sinonasal malignancies, because of proximity to or involvement of the pterygoid plexus as well as the rich vascularity of the sinonasal mucosa, juvenile nasopharyngeal angiofibroma, a vascular tumor of male adolescents; unusual vascular tumors such as hemangiopericytoma; and metastatic renal cell cancer, which has a proclivity for an unusually rich blood supply.

Adequate imaging is a prerequisite to surgical planning to determine:

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  Location of tumor

  
  
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  Likely diagnosis

  
  
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  Assess vascularity

  
  
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  Types of imaging modalities

  
  
  
  
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    Diagnostic imaging

    
    
    
    
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      Computed tomography (CT)

      
      
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      MRI

      
      
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      Computed tomography angiography (CTA)

      
      
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      Magnetic resonance angiography (MRA)

      
      
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      Angiography

    
    

  
  

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