Management of Jugular Paragangliomas in Otolaryngology Practice

Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity and should be considered in the evaluation of all lateral neck masses. The aim of this study was to review an institutional experience in the management of jugular paragangliomas. Thirteen jugular paragangliomas (Fisch classifications C and D glomus jugulare) were treated in our institution during a period of 5 years (2003-2008). All patients with adequate follow-up and complete records (13 cases) were reviewed with emphasis on the results of surgical management and the factors influencing them. Coupled with the residual tumors in 2 patients, the surgical control achieved was 82%. No recurrence was seen. All patients were subjected to digital subtraction angiography to define the vascular supply of the tumor. Microcoil embolization of 11 jugular paragangliomas (all [100%] of the patients who accepted the operation) was performed during the initial digital subtraction angiography. There was no perioperative mortality. There was one case of perioperative cerebrospinal fluid leak, which was repaired during the surgery. The facial nerve was preserved in all of the patients. The overall preservation rate of lower cranial nerves was 54% (7 of 13). Two patients with residual jugular paraganglioma were posttreated with irradiation (gamma knife) owing to skull base extension with significant symptomatic relief. Two patients who refused the surgical treatment were managed by stereotactic radiosurgery. The primary therapeutic option for jugular paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. The combined therapeutic approach with preoperative selective embolization followed by surgical resection is the safe and effective method for complete excision of tumors with a reduced morbidity rate. Irradiation (gamma knife) is an effective therapy method for residual jugular paraganglioma.

[1]  C. Erem,et al.  Paragangliomas of the Head and Neck , 2007, Medical Principles and Practice.

[2]  J. Netterville,et al.  Paragangliomas of the head and neck. , 2004, Oral oncology.

[3]  M. van Buchem,et al.  Head and neck paragangliomas: improved tumor detection using contrast-enhanced 3D time-of-flight MR angiography as compared with fat-suppressed MR imaging techniques. , 2004, AJNR. American journal of neuroradiology.

[4]  D. Frank,et al.  Combined endovascular and surgical treatment of head and neck paragangliomas—A team approach , 2002, Head & neck.

[5]  A. V. D. van der Mey,et al.  Management of carotid body tumors. , 2001, Otolaryngologic clinics of North America.

[6]  S. Schmid,et al.  An Update on the Surgical Treatment of Temporal Bone Paraganglioma , 2000, Skull base surgery.

[7]  E. Roeder,et al.  Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management. , 1999, The American journal of otology.

[8]  R. Jackler,et al.  Clinicopathologic presentation and diagnostic imaging of jugular foramen tumors , 1996 .

[9]  D. Haynes,et al.  Hearing conservation in surgery for glomus jugulare tumors. , 1996, The American journal of otology.

[10]  M. Mack,et al.  Glomus tumors of the skull base: combined use of MR angiography and spin-echo imaging. , 1994, Radiology.

[11]  N. F. Jensen Glomus Tumors of the Head and Neck: Anesthetic Considerations , 1994, Anesthesia and analgesia.

[12]  A. Valavanis,et al.  Preoperative Embolization of Paragangliomas (Glomus Tumors) of the Head and Neck: Histopathologic and Clinical Features , 1993, Skull base surgery.

[13]  John C. Dailey,et al.  Familial Multiple Cervical Paragangliomas: Report of a Kindred and Review of the Literature , 1990, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

[14]  T. Vogl,et al.  Paragangliomas of the jugular bulb and carotid body: MR imaging with short sequences and Gd-DTPA enhancement. , 1989, AJR. American journal of roentgenology.

[15]  P. Phelps,et al.  Glomus jugulare or tympanicum? , 1988, The Journal of Laryngology & Otology.

[16]  T. H. Newton,et al.  MR imaging of paragangliomas. , 1986, AJR. American journal of roentgenology.

[17]  U. Fisch Infratemporal Fossa Approach for Glomus Tumors of the Temporal Bone , 1982, The Annals of otology, rhinology, and laryngology.

[18]  M. Gillman,et al.  Familial carotid body tumors: Case report and epidemiologic review , 1980, Cancer.

[19]  S. Sheps,et al.  Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. , 1971, American journal of surgery.

[20]  B. Alford,et al.  A comprehensive study of tumors of the glomus jugulare. , 1962, The Laryngoscope.

[21]  M. Uğuz,et al.  Malignant carotid body tumor: a case report. , 2004, Kulak burun bogaz ihtisas dergisi : KBB = Journal of ear, nose, and throat.

[22]  J. C. Sniezek,et al.  Vagal paragangliomas. , 2001, Otolaryngologic clinics of North America.

[23]  J. Bloem,et al.  MR diagnosis of paraganglioma of the head and neck: value of contrast enhancement. , 1994, AJR. American journal of roentgenology.

[24]  A. Gulya The glomus tumor and its biology. , 1993, The Laryngoscope.

[25]  G. Valvassori Benign tumors of the temporal bone. , 1974, Radiologic clinics of North America.

[26]  W. G. S. Smitt [Glomus tumors]. , 1958, Nederlands tijdschrift voor geneeskunde.