Photoclinic

 

 

 

 

 

Figure 1. Showing a bulging at the lateral pharyngeal wall and tonsillar pillar (arrows)

Figure 2. CT scan of the neck demonstrating an enhanced left parapharyngeal mass

Figure 3. Digital subtracted angiogram showing a mass above the left carotid bifurcation

 

Aliasghar Peyvandi MD•*, Habibolah Peyrovi MD**, Navid Ahmady-Roozbahany MD*

 

Authors’ affiliations: *Department of Otololaryngology Head and Neck Surgery, Loghman Hakim Hospital, Shahid Beheshti Medical University, **Department of Vascular Surgery, Taleghani Hospital, Shahid Beheshti Medical University, Tehran, Iran.

•Corresponding author and reprints: Aliasghar Peyvandi MD, Department of Otololaryngology Head and Neck Surgery, Loghman Hakim Hospital, Shahid Beheshti Medical University, Tehran, Iran. Fax: +98-218-833-1082

E-mail: aliapeyvandi@yahoo.com

 

Accepted for publication: 8 April 2009

 

 

 

A

41-year-old female complaining of an eight month history of dysphagia from 8 months ago presented to the Otolaryngology Department at Loghman Hakim Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, Iran. There was no history of any current infections of the upper respiratory tract, dental problems, and inflammations of the oral cavity, oropharynx, salivary glands, nose, or paranasal sinuses. There was no pain, malaise, fever, or weight loss.

Upon physical examination, she had a bulging area in the left lateral oropharyngeal wall which pushed the palatine tonsil and its pillars to midline (Figure 1). A slight fullness behind the left angle and ramous of the left mandible was noted. There was no pulsation or bruits at the site of the mass. No other mass or lymphadenopathy was found in the neck. Laboratory tests including CBC, ESR, and PPD were normal and toxoplasma and HIV serologic tests were negative. A chest radiography revealed no abnormality.

Contrast axial CT scan of the neck revealed a deeply enhancing mass located in the left parapharyngeal space, adjacent to the great vessels of the neck. Other areas of the neck were normal (Figure 2).

The patient underwent cervical vessel digital subtraction angiography (DSA) which showed a vascular mass with multiple nutrient vessels
 mainly originating from the external carotid (ascending pharyngeal branch) and a few smallerbranches from the internal carotid artery (Figure 3). Surgery was performed to remove the mass.

 

 

What is Your Diagnosis?

 

See the pages 59 – 60 for the diagnosis

 

 

 

 

 

 

Photoclinic Diagnosis:             Vagus Nerve Paraganglioma  (Glomus Vagale)

 

Figure 4. Intraoperative views of the tumor: The hypoglossal nerve (CN XII) is retracted away from the surface of the tumor. The common carotid and its bifurcation were evident.

Figure 5. Histopathology of vagus nerve paraganglioma (H&E, ×50)

 

 

T

he patient was diagnosed with a vagus nerve paraganglioma (glomus vagale). Paragangliomas are the most common benign vascular neoplasm of the neck. Paragangliomas arise from extra-adrenal paraganglionic cells which are derived from the neural crest. The paraganglionic system consists of a collection of neuroectoderm-derived chromaffin cells in extra-adrenal sites. Before adrenal medulla formation in the fetus, the paraganglionic system is a major source of catecholamines.1

The correct terminology of paragangliomas is based on their location. They are classified as carotid paragangliomas, jugulotympanic paragangliomas and vagal paragangliomas.

Approximately 90% of tumors arising from the paraganglion system are pheochromocytomas of the adrenal medula; the remaining 10% arise from extraadrenal sites with 85% originating in the abdomen, 12% in the thorax, and the remaining 3% in the head and neck area. The most common paraganglioma of the head and neck is the carotid body tumor, followed by jugulotympanic paragangliomas and vagal paragangliomas.2

Vagal paragangliomas are tumors derived from paraganglionic tissue associated with one ganglia of the vagus nerve. They most commonly arise from nodose or inferior vagal ganglion. Tumors arising from the superior vagal ganglion, or jugular ganglion, may be dumbbell-shaped and may extend intracranially through the jugular foramen.3,4

The etiology of paragangliomas appears to be multifactorial. Most paragangliomas are solitary but multiple pheochromocytomas and paragangliomas are seen in familial syndromes, especially multiple endocrine neoplasia types II (MEN II).4

Vagal paraganglioma usually presents as a palpable neck mass that is more mobile in a lateral direction than a craniocaudal orientation. Paralysis of the ipsilateral true vocal cord or Horner's syndrome, from involvement of the ipsilateral sympathetic chain, may be present as the tumor grows in size. True vocal cord paralysis can result in hoarseness. Large tumors arising from the jugular ganglion may be associated with cranial neuropathies of the lower cranial nerves. Diagnostic imaging, especially CT scanning with intravenous contrast, may demonstrate a hypervascular mass located above the carotid bifurcation with anterior displacement of the carotid artery from the tumor present in the posterior carotid sheath. The internal and external carotid arteries do not manifest a splayed configuration. Embolization is indicated for large tumors and in those rare instances where a malignant tumor may be suspected.4,5

The treatment of choice is surgical removal with meticulous extra-capsular dissection. An experienced vascular surgeon and appropriate instrumentation are needed.6

In our patient, surgery was performed to remove the mass. It was a well encapsulated yellowish mass measuring 5×5 cm in diameter located lateral and adherent to the left hypoglossal nerve. There was a major nutrient artery from the ascending pharyngeal branch of the left external
 carotid artery and a few smaller vessels extending  from the left internal and external carotid artery that were all ligated (Figure 4).

On histopathologic examination, the tumor consists of two cell types. The majority of cells are Type I, where they are arranged in a specific pattern which is called Zellballen. Type II cells surround Type I cells. Nuclear pleomorphism and cellular hyperchromatism are not considered as evidences of malignancy (Figure 5).7

 

References

 

1         Urquhart AC, Johnson JT, Myers EN, Schechter GL. Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope. 1994; 104: 440 – 445.

2         Walsh RM. Malignant vagal paraganglioma. J Laryngol Otol. 1997; 111: 83 – 88.

3         Goehler LE, Relton JK, Dripps D, Kiechle R, Tartaglia N, Maier SF, et al. Vagal paraganglia bind biotinylated interleukin-1 receptor antagonist: a possible mechanism for immune-to-brain communication. Brain Res Bull. 1997; 43: 357 – 364.

4         Lozano FS, Gómez JL, Mondillo MC, González-Porras JR, González-Sarmiento R, Muñoz A. Surgery of vagal paragangliomas: six patients and review of literature. Surg Oncol. 2008; 17: 281 – 287.

5         van den Berg R. Imaging and management of head and neck paragangliomas. Eur Radiol. 2005; 15: 1310 – 1318.

6         Antonitsis P, Saratzis N, Velissaris I, Lazaridis I, Melas N, Ginis G, et al. Management of cervical paragangliomas: review of a 15-year experience. Langenbecks Arch Surg. 2006; 391: 396 – 402.

7         Tischler AS, Kimura N, Mcnicol AM. Pathology of pheochromocytoma and extra-adrenal paraganglioma. Ann N Y Acad Sci. 2006; 1073: 557 – 570.

 

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