ONCOLOGY.
No. 7
REVIEW ARTICLE
The Multidisciplinary Management of Paragangliomas of the Head and Neck, Part 1
By Kenneth Hu, MD1, Mark S. Persky, MD2 |
July 1, 2003
1Assistant Professor of Radiation Oncology, Albert Einstein College of Medicine, Beth Israel Medical Center
2Vice Chairman, Department of Otolaryngology-Head and Neck Surgery; Associate Director, Continuum Cancer Center, Codirector, Institute for Head and Neck Cancer, Beth Israel Medical Center, New York, New York; Professor of Clinical Otolaryngology–Head and Neck Surgery, Albert Einstein College of Medicine, Bronx, New York
Traditionally, surgical resection has been the mainstay of treatment for these tumors, but the outcome is dependent on many factors that may influence the ideal result of total tumor removal and minimal postoperative complications. Relative contraindications to surgery include extensive skull-base or intracranial involvement, advanced age of the patient, medical comorbidities, and bilateral or multiple paragangliomas, which may result in unacceptable postoperative morbidity and bilateral lower cranial nerve palsies. If surgery is the chosen course of treatment, preoperative embolization is performed. Embolization of paragangliomas has been an extremely useful adjunct to our treatment protocol. Although not uniformly accepted,[ 52-56] there are major advantages to performing endovascular embolization prior to subsequent surgery, assuming that certain criteria have been met before the embolization is performed.[41,57-61] An experienced vascular radiology team must be thoroughly familiar with the complexities and possible variations in the vascular anatomy of the head and neck. Many anastomoses exist between the external and internal carotid system, and without this knowledge, disastrous neurologic consequences may result.[62] The safe performance of this procedure by interventional vascular radiology must be established and documented with an acceptable rate of morbidity and mortality.[60,63] Surgery is performed within 2 days of angiography and embolization to avoid recruitment of collateral tumor blood supply and prior to the onset of a significant postinflammatory effect.[ 28] If there is concern about tumor edema, which may compromise the dissection, short-term steroids are administered. The anesthesiologist must be prepared to counteract the alpha- and beta-adrenergic catecholamine- induced cardiovascular effect when dealing with "secreting" tumors. Adequate removal of the tumor requires a subadventitial dissection of the carotid artery. Nerves adherent to but not infiltrated by these tumors can usually be mobilized; these include the vagus, hypoglossal, and, occasionally, glossopharyngeal nerves. The sympathetic chain and the superior laryngeal nerve are often adherent to tumor, especially in cases with medial extension into the parapharyngeal space. Tympanic paragangliomas confined to the middle ear (Glasscock- Jackson type I, Fisch class A) can be accessed via a transcanal approach.[ 64] These small tumors do not require preoperative embolization. Involvement of the jugular bulb requires a combined transmastoid and transcervical approach.[65] Tumor dissection is meticulously performed off the internal carotid artery. The exiting cranial nerves are preserved if they can be adequately separated from the tumor. Additional exposure into the infratemporal fossa can be accomplished with resection of the mandibular condyle and zygomatic arch. Such an approach allows access to the posterior and middle cranial fossa, and, if necessary, further tumor resection can be accomplished through a neurosurgical, intracranial approach. A pedicled temporalis, temporal- parietal, or sternomastoid flap is used to reconstruct and obliterate the defect. The external auditory canal is closed at the meatus. Spinal drainage catheters are used postoperatively for significant cerebrospinal fluid leaks. Vagal paragangliomas may vary in the extent of skull-base or intracranial involvement. Most vagal paragangliomas originate in the nodose (inferior) ganglion, approximately 2 cm below the jugular foramen. When these tumors grow, they will extend to the skull base and involve the post styloid parapharyngeal space as well. Vagal paragangliomas arising in the middle or superior vagal ganglia are associated with early skull-base involvement with intracranial extension. As with all paragangliomas, a complete radiographic evaluation with contrast-enhanced MRI and CT stud- ies will define the extent of involvement. A combined cervical-mastoid approach to the skull base is best for achieving safe and wide exposure. The tumor almost always involves sacrifice of the vagus nerve and additional cranial nerves according to its size and local involvement.[6] The complications associated with head and paraganglioma surgery can be grouped into three main categories: cranial nerve injury, vascular injury, and injury to the carotid body/ carotid sinus complex. Lower cranial nerve dysfunction is a common complication of surgical resection of head and neck paragangliomas and often requires postoperative rehabilitation.[6] Indeed, larger tumors that involve the temporal bone can infiltrate between the fascicles of the cranial nerves, even with normal nerve function. These involved nerves should be sacrificed to effect a total tumor resection.[66] Our experience with postoperative cranial nerve dysfunction reflects a combination of total paralysis, due to nerve sacrifice and paresis, with ultimate recovery of function due to nerve traction. The frequency of affected nerves is related to tumor type and size and the surgical approach used (Table 5).[28] Although isolated injury to one of the lower cranial nerves (IX-XII) sometimes causes only temporary minor difficulty in swallowing, aspiration, phonation, shoulder mobility, or tongue motion, vagus nerve and multiple cranial nerve injury may result in significant morbidity.[ 67] Multiple cranial nerve deficits are particularly poorly tolerated by elderly patients. Bilateral lower cranial nerve palsies most often represent a severe, potentially life-threatening situation. Paraganglioma surgery generally produces postoperative nerve dysfunction, and familiarity with rehabilita tion techniques is necessary for proper patient care. If tumor resection results in nerve transection, then primary anastomosis, if possible, results in acceptable function. This is especially true of the facial, accessory, and hypoglossal nerves. Cable grafts using the greater auricular or sural nerve are also an option, especially in the case of facial and accessory nerve dysfunction. Rehabilitation for patients with postoperative nerve dysfunction depends on the functional deficit and may require additional surgical intervention, including upper-lid gold-weight implants for corneal protection, transoral hemipalatal adhesion for palatal insufficiency,[68] temporalis and masseter muscle/ fascial sling or adynamic fascial or allograft slings for facial muscle weakness, and gelatin (Gelfoam) injection/ silastic implant of a paralyzed vocal cord for medialization to potentiate swallowing and improve voice quality. The incidence of intraoperative or postoperative stroke has decreased dramatically as surgical and anesthetic techniques have improved. This improvement has been attributed to many factors, including detailed preoperative imaging and angiographic evaluation to determine vessel involvement by tumor, carotid occlusion testing, correlation of bilateral cerebral angiography findings with postocclusion cerebral function, and advances in surgical arterial revascularization techniques. Recent series report a 0% to 2% rate of major strokes as a complication of carotid body tumor resection[ 1,50,55,69-72]; earlier series reported a rate of 10% to 20%.[72,73] Compared to carotid body paragangliomas, vagal paragangliomas are usually not as intimately associated with the great vessels, making vascular injury less likely.[74] Netterville et al reported carotid artery encasement in 5 of 46 patients with vagal paragangliomas.[6] Other paragangliomas associated with vascular injury include large jugular paragangliomas, which commonly involve the internal carotid artery, especially if there is erosion of the bony partition between the internal jugular vein and the internal carotid artery. Internal carotid artery damage occurred in only 1% of patients with large paragangliomas of the skull base.[75] Resection of bilateral carotid body tumors can result in baroreceptive dysfunction due to bilateral denervation of the carotid sinus. This dysfunction manifests as sustained hypertension and tachycardia. Netterville et al reported that 10 of 11 patients who underwent bilateral carotid sinus denervation demonstrated severe labile hypertension/hypotension, headache, diaphoresis, and emotional instability.[19] As the parasympathetic response is lost, unopposed sympathetic stimuli result in cardiovascular morbidity, which is usually successfully managed postoperatively with alphaadrenergic antagonists. Sodium nitroprusside is administered in the early postoperative period to prevent hypertension. The long-term cardiovascular effects are controlled with clonidine or phenoxybenzamine (Dibenzyline). Financial Disclosure: The authors have no significant financial interest or other relationship with the manufacturers of any products or providers of any service mentioned in this article.
The Multidisciplinary Management of Paragangliomas of the Head and Neck
1. Williams MD, Phillips MJ, Nelson WK,
et al: Carotid body tumor. Arch Surg 127:963-
968, 1992.B
2. Gaylis H, Davidge-Pitts K, Pantanowitz
D: Carotid body tumours. A review of 52
cases. S Afr J Surg 72:493-496, 1987.
3. Jackson CG, Harris PF, Glassock ME 3rd,
et al: Diagnosis and management of paragangliomas
of the skull base. Am J Surg 159:389-
393, 1990.
4. Lawson W: Glomus bodies and tumors. N
Y State J Med 80:1567-1575, 1980.
5. Moore G, Yarington CT Jr, Mangham CA
Jr: Vagal body tumors: Diagnosis and treatment.
Laryngoscope 96:533-536, 1986.
6. Netterville JL, Jackson CG, Miller FR, et
al: Vagal paraganglioma: A review of 46 patients
treated during a 20-year period. Arch
Otolaryngol Head Neck Surg 124:1133-1140,
1998.
7. Pearse A: The cytochemistry and ultrastructure
of the polypeptide hormone-producing
cells of the APUD series and the embryonic,
physiologic and pathologic implications of the
concept. J Histochem Cytochem 17:303-313,
1969.
8. Pearse A: The diffuse neuroendocrine system:
Historical review. Front Horm Res 12:1-
7, 1984.
9. Thirlwall AS, Bailey CM, Ramsay AD, et
al: Laryngeal paraganglioma in a five-year-old
child—the youngest case ever recorded. J
Laryngol Otol 113:62-64, 1999.
10. Venkataramana NK, Kolluri VR, Kumar
DV, et al: Paraganglioma of the orbit with extension
to the middle cranial fossa: A case
report. Neurosurgery 24:762-764, 1989.
11. Kronz JD, Argani P, Udelsman R, et al:
Paraganglioma of the thyroid: Two cases that
clarify and expand the clinical spectrum. Head
Neck 22:621-625, 2000.
12. Nielsen TO, Sejean G, Onerheim RM:
Paraganglioma of the tongue. Arch Pathol Lab
Med 124:877-879, 2000.
Paraganglioma of the tongue. Arch Pathol Lab
Med 124:877-879, 2000.
13. Welkoborsky HJ, Gosepath J, Jacob R,
et al: Biologic characteristics of paragangliomas
of the nasal cavity and paranasal sinuses. Am
J Rhinol 14:419-426, 2000.
14. Sharma HS, et al: Malignant paraganglioma
of frontoethmoidal region. Auris Nasus
Larynx 26:487-493, 1999.
15. Skinner LJ, Curran AJ, Barnes C, et al:
Paraganglioma of the external auditory canal:
An unusual case. J Laryngol Otol 114:370-
372, 2000.
16. Sambaziotis D, Kontogeroges G, Kovacs
K, et al: Intrasellar paraganglioma presenting
as nonfunctioning pituitary adenoma.
Arch Pathol Lab Med 123:429-432, 1999.
17. Yamauchi T, Kubota M, Saeki N, et al:
Paraganglioma in the frontal skull base—case
report. Neurol Med Chir 39:308-312, 1999.
18. Grufferman S, Gillman MW, Pasternak
LR, et al: Familial carotid body tumors: Case
report and epidemiologic review. Cancer
46:2116-2122, 1980.
19. Netterville JL, ReillyKM, Robertson D,
et al: Carotid body tumors: A review of 30
patients with 46 tumors. Laryngoscope 105:115-
126, 1995.
20. Oosterwijk J, Jansen JC, van Schothorst
EM, et al: First experience with genetic counselling
based on predictive DNA diagnosis in
hereditary glomus tumors (paragangliomas). J
Med Genet 33:379-383, 1996.
21. van der Mey AG, Maaswinkel-Mooy
PD, Cornelissa CJ, et al: Gentic imprinting in
hereditary glomus tumours: Evidence for a new
genetic theory. Lancet 2:1291-1894, 1989.
22. Heutink P, van der Mey AG, Sankuijl
LA, et al: A gene subject to genomic imprinting
and responsible for hereditary paragangliomas
maps to chromosome 11q23-qter. Hum
Mol Genet 1:7-10, 1992.
23. Sykes J, Ossoff R: Paragangliomas of
the head and neck. Otolaryngol Clin North Am
19:755-767, 1986.
24. Jackson G: Diagnosis for treatment planning
and treatment options. Laryngoscope
103:17-22, 1993.
25. Lack EF, Cubilla AL, Woodruff JM, et
al: Paragangliomas of the head and neck region:
A clinical study of 69 patients. Cancer
39:397-409, 1977.
26. Kliewer KE, Wen DR, Cancilla PA, et
al: Paragangliomas: Assessment of prognosis
by histologic, immunohistochemical, and ultrastructural
techniques. Hum Pathol 20:29-39,
1989.
27. Rao AB, Koeller KK, Adair CF: From
the archives of the AFIP. Paragangliomas of
the head and neck: Radiologic-pathologic correlation.
Armed Forces Institute of Pathology.
Radiographics 19:1605-1632, 1999.
28. Persky MS, Setton A, Niimi Y, et al:
Combined endovascular and surgical treatment
of head and neck paragangliomas—A team approach.
Head Neck 24:423-431, 2002.
29. Batsakis J: Paragangliomas of the head
and neck, in Tumors of the Head and Neck:
Clinical and Pathological Consideration,
pp 369-380. Baltimore, Williams & Wilkins,
1979.
30. Shamblin WR, Mine WH, Sheps SG, et
al: Carotid body tumor (chemodectoma): Clinicopathologic
analysis of ninety cases. Am Surg
122:732-739, 1971.
31. Manolidis S, Shohet JA, Jackson CG, et
al: Malignant glomus tumors. Laryngoscope
109:30-34, 1999.
32. Zbaren P, Lehman W: Carotid body
paraganglioma with metastasis. Laryngoscope
95:450-454, 1985.
33. El Fiky FM, Paparella MM: A metastatic
glomus jugulare tumor. A temporal bone
report. Am J Otol 5:197-200, 1984.
34. Pacheco-Ojeda L: Malignant carotid body
tumors: Report of three cases. Ann Otol Rhinol
Laryngol 110:36-40, 2001.
35. Jansen JC, van den Berg R, Kuiper A, et
al: Estimation of growth rate in patients with
head and neck paragangliomas influences the
treatment proposal. Cancer 88:2811-2816, 2000.
36. Schwaber MK, Glasscock ME, Nissen
AJ, et al: Diagnosis and management of catecholamine
secreting glomus tumors. Laryngoscope
94:1008-1015, 1984.
37. Zak FG, Lawson W: The Paraganglionic
Chemoreceptor System. New York, Springer-
Verlag, 1982.
38. Irons GB, Weiland LH, Brown WL:
Paragangliomas of the neck: Clinical and pathologic
analysis of 116 cases. Surg Clin North
Am 57:575-583, 1977.
39. Parkin JL: Familial multiple glomus tumors
and pheochromocytomas. Ann Otol Rhinol
Laryngol 90:60-63, 1981.
40. Revak CS, Morris SE, Alexander GH:
Pheochromocytoma and recurrent chemodectomas
over a twenty-five year period. Radiology
100:53-54, 1971.
41. Maier W, Marangos N, Laszig R: Paraganglioma
as a systemic syndrome: Pitfalls and strategies.
J Laryngol Otol 113:978-982, 1999.
42. Cheng A, Niparko JK: Imaging quiz
case 2. Glomus tympanicum tumor of the temporal
bone. Arch Otolaryngol Head Neck Surg
123:549, 551-552, 1997.
43. Swartz JD, Harnsberger HR, Mukherji
SK: The temporal bone. Contemporary diagnostic
dilemma. Radiol Clin North Am 36:819-
853, 1998.
44. Som PM, Braun IF, Shapiro MD, et al:
Tumors of the parapharyngeal space and upper
neck: MR imaging characteristics. Radiology
164:823-829, 1987.
45. Olsen WL, Dillon WP, Kelly WM, et al:
MR imaging of paragangliomas. AJR Am J
Roentgenol 148:201-204, 1987.
46. van Gils AP, van der May AG, Hoogma
RP, et al: MRI screening of kindred at risk of
developing paragangliomas: Support for genomic
imprinting in hereditary glomus tumours.
Br J Cancer 65:903-907, 1992.
47. van den Berg R, Wasser MN, van Gils
AP, et al: Vascularization of head and neck
paragangliomas: Comparison of three MR angiographic
techniques with digital subtraction
angiography. Am J Neuroradiol 21:162-170,
2000.
48. Telischi FF, Bustillo A, Whiteman ML,
et al: Octreotide scintigraphy for the detection
of paragangliomas. Otolaryngol Head Neck
Surg 122:358-362, 2000.
49. Lasjaunias AP, Berenstein K: Surgical
Neuro-angiography: Endovascular Treatment
of Craniofacial Lesions, vol 2. New York,
Springer-Verlag, 1987.
50. Anand VK, Alemar GO, Sanders TS:
Management of the internal carotid artery during
carotid body tumor surgery. Laryngoscope
105(3 pt 1):231-235, 1995.
51. Sakurai H, Hayakawa K, Mitsuhashi N,
et al: Chemodectoma of the carotid body treated
with radiation therapy: A case report. Radiat
Med 13:191-194, 1995.
52. Biller H, Lawson W, Som P, et al: Glomus
vagale tumors. Ann Otol Rhinol Laryngol
98(1 pt 1):21-26, 1989.
53. Brackman D, Kinney S, Fu K: Glomus
tumor: Diagnosis and management. Head Neck
Surg 9:306-311, 1987.
54. Leonetti JP, Donzelli JJ, Littooy FN, et
al: Perioperative strategies in the management
of carotid body tumors. Otolaryngol Head Neck
Surg 117:111-115, 1997.
55. Matticari S, Credi G, Pratesi G, et al:
Diagnosis and surgical treatment of the carotid
body tumors. J Cardiovasc Surg 36:233-239,
1995.
56. Litle VR, Reilly LM, Ramos TK: Preoperative
embolization of carotid body tumors:
When is it appropriate? Ann Vasc Surg 10:464-
468, 1996.
57. Merland JJ, Reizine D, Guimaraens L, et
al: Diagnostic and therapeutic angiography in
the evaluation and treatment of glomus jugulare
tumors. Apropos of 32 cases (French). Neuro-
Chirurgie 31:358-366, 1985.
58. Ogura JH, Spector GJ, Gado M: Glomus
jugulare and vagale. Ann Otol 87(5 pt 1):622-
629, 1978.
59. Smith RF, Shetty PC, Reddy DJ: Surgical
treatment of carotid paragangliomas presenting
unusual technical difficulties. The value
of preoperative embolization. J Vasc Surg
7:631-637, 1988.
60. LaMuraglia GM, Fabian RL, Brewster
DC, et al: The current surgical management of
carotid body paragangliomas. J Vasc Surg
15:1038-1045 (incl discussion), 1992.
61. Ward PH, Liu C, Vinuela F, et al: Embolization:
An adjunctive measure for removal of
carotid body tumors. Laryngoscope 98:1287-
1291, 1988.
62. Lasjaunias P: Nasopharyngeal angiofibromas:
Hazards of embolization. Radiology
136:119-123, 1980.
63. Murphy TP, Brackmann DE: Effects of
preoperative embolization on glomus jugulare
tumors. Laryngoscope 99:1244-1247, 1989.
64. House WF, Glasscock ME: Glomus tympanicum
tumors. Arch Otolaryngol 87:550,
1968.
65. Brackmann D, Arriaga M: Surgery for
glomus tumors, in Brackmann D, Shelton C,
Arriaga M (eds): Otologic Surgery. Philadelphia,
WB Saunders, 1994.
66. Sen C, Hague K, Kacchera R, et al:
Jugular foramen: Microscopic anatomic features
and implications for neural preservation
with reference to glomus tumors involving the
temporal bone. Neurosurgery 48:838-848,
2001.
67. Jackson CG: Skull base surgery. Am J
Otol 3:161-171, 1981.
68. Netterville JL, Vrabec JT: Unilateral palatal
adhesion for paralysis after high vagal
injury. Arch Otolaryngol Head Neck Surg
120:218-221, 1994.
69. Dickinson PH, Griffin SM, Guy AJ, et
al: Carotid body tumour: 30 years experience.
Br J Surg 73:14-16, 1986.
70. Hallett JW Jr, Nora JD, Hollier LH, et al:
Trends in neurovascular complications of surgical
management for carotid body and cervical
paragangliomas: A fifty-year experience
with 153 tumors. J Vasc Surg 7:284-291,
1988.
71. Wax MK, Briant TD: Carotid body tumors:
A review. J Otolaryngol 21:277-285,
1992.
72. Muhm M, Polterauer P, Gstottner W, et
al: Diagnostic and therapeutic approaches to
carotid body tumors. Review of 24 patients.
Arch Surg 132:279-284, 1997.
73. Monro R: The natural history of carotid
body tumours and their diagnosis and treatment.
Br J Surg 37:4445-453, 1950.
74. Urquhart AC, Johnson JT, Myers EN, et
al: Glomus vagale: Paraganglioma of the vagus
nerve. Laryngoscope 104:440-445, 1994.
75. Woods CI, Strasnick B, Jackson CG:
Surgery for glomus tumors: The Otology Group
experience. Laryngoscope 103:65-70, 1993.
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Postoperative Cranial Nerve Deficits in Paraganglioma Patients
