Case of the Month: June, 2012, Page 2

Answer: All of the above

Discussion:

Glomus vagale pargangliomas (aka glomus intravagale tumors or vagal paragangliomas) are relatively uncommon benign tumors. They are derived from neural crest remnants in the nodose ganglion which is one of the vagal ganglia in the upper neck which lies within the carotid sheath. They are the least common of all the major paragangliomas. In order from most to least common: carotid body tumors, glomus tympanicum, glomus jugulare, glomus vagale.

They most commonly arise at the level of the angle of mandible above the hyoid bone. Cranial nerves IX-XII may be affected or  they may present with Horner syndrome. The most common symptoms are related to vagus neuropathy  in addition to vocal cord paralysis with hoarseness. There is a slight female predominance and the most common age range at presentation is 30-50.

They are highly vascular masses with dramatic enhancement. They may be differentiated from carotid body tumors based on displacement patterns of the adjacent vessels. Glomus vagale tumors tend to displace the internal and external carotid arteries anteromedially and the jugular vein posterolaterally. Carotid body tumors tend to splay the external and internal carotid arteries apart from one another.

They may be distinguished from vagus nerve schwannomas by the presence of flow voids on MRI. Glomus tumors also enhance rapidly whereas shwannomas tend to have slow enhancement. On CT schwannomas are usually hypodense while glomus tumors are usually isodense. Indium-111 octreotide scans may also distinguish glomus tumors from schwannomas with intense activity seen in glomus tumors.

Treatment of glomus vagale paragangliomas almost always results in sacrifice of the vagus nerve and vocal cord paralysis with surgical resection. In the case of bilateral tumors surgical resection is completed on one side with radiation therapy reserved for the other in attempt to preserve unilateral vagus nerve function.

Always check for a second paragamglioma once one is found. Paragangliomas have been associated with thyroid carcinomas and MEN syndromes and multiple paragangliomas are nearly always familial in origin.

References:

  • Harnsberger, H. “Paraganglioma, Glomus Vagale.” STATdx. 13 Sept. 201. https://my.statdx.com/STATdxMain.jsp?rc=false#dxContent;glomus_vagale_paraganglioma
  • Yousem, Zimmerman, and Robert Grossman. Neuroradiology: The Requisites. Philadelphia: Mosby, Inc., 2010.

 

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