Print version ISSN 0034-7299
Rev. Bras. Otorrinolaringol. vol.73 no.3 São Paulo May/June 2007
Adriano Sérgio Freire MeiraI; Lauro Otacílio Campos de SousaII; Camila Gomes Fernandes de SousaIII; Ricardo Marcio MoraisIV; Pedro de Oliveira Cavalcanti FilhoV; Jose Walter da Fônseca JúniorVI; Jose Diniz JúniorVII
Physician at the Pedro Cavalcanti Clinic/ UFRN University Hospital
IIResident Physician at the Pedro Cavalcanti Clinic/ UFRN University Hospital
IIIMedical Student - UFRN
IVResident Physician at the Pedro Cavalcanti Clinic/ UFRN University Hospital
VMD. ENT. Head of the Medical Residency Service at the Pedro Cavalcanti Clinic/ UFRN University Hospital
VIMD. ENT at the Pedro Cavalcanti Clinic/ UFRN University Hospital
VIIPhD in Otorhinolaryngology, Adjunct Professor of Otorhinolaryngology - UFRN
Keywords: bilateral, neck, paraganglioma, temporal bone.
Paragangliomas are tumors that originate from microscopic structures associated with the Central Nervous System (CNS) in the para-axial regions of the trunk, neighboring the branchiometric arches and CNS periphery, the paraganglia1.
N.F.S., 76, black, complained of dizziness that did not improve with medication, pulsatile tinnitus and progressive hearing loss for the past 3 years in his right ear, as well as left side neck bulging for 30 years, of slow growth, painless, of fibroelastic material, non-pulsatile.
On CT scan we noticed an extensive solid mass with ill-defined lobulated contours, occupying the carotid space in both sides, with right side temporal bone destruction by a tumor radiologically equal to the one previously described, and the patient was allergic to iodine. MRI showed hypervascularized lesions (see Figure), in the aforementioned regions, thus corroborating CT findings. Angiography confirmed the diagnosis.
Neck paragangliomas, because of their rare occurrence, are a difficult to diagnose and treat pathology. Among other causes of neck bulging we have: branchyal cysts, carotid aneurisms, neurofibromas, parotid tumors, tumor metastasis, inflammatory lymphadenopathy, amongst others.
They are mostly benign; paragangliomas can have malignant transformation in 2 to 10% of cases, accounting for 0.012% of head and neck tumors. The most frequent site is the carotid body; the less common sites are the middle ear, jugular, vagus nerve, larynx and aortic arch2.
CT scan is the most adequate diagnostic method for obtaining data on the tumors extension and location. MRI is considered superior to CT in documenting soft tissue details and tumor characterization3. Nonetheless, only selective angiography provides the definitive diagnosis, outlines the structure and looks for synchronic tumors. FNA should not be done; these tumors rarely secrete catecholamine, however one can look for vanyl-mandelic acid and urinary catecholamine levels.
Surgery is the treatment of choice; radiotherapy is questionable. Expectation approach and embolization with routine assessments are treatment options for those who do not fit the surgery profile4, as was our patient.
Surgery is the major therapeutic approach. Radiotherapy, controversial, did not prove to help in these tumors. To wait and use embolization also seems to be an option, and this was the approach preferred by our patient because of his cardiac condition, and it was an excellent outcome.
1. Austin MB, Mills SE. Neoplasms and neoplasm-like lesions involving the skull base. Ear, Nose Throat J 1986;65(2):57-73. [ Links ]
2. Burihan MC, Albuquerque RGB, Nascimento JFB, Nasser F, Ingrud JC. Tumor de corpo carotídeo: relato de dois casos. Cir Vasc Ang 1997;13:13-18. [ Links ]
3. Chakeres DW, La Masters DL. Paragangliomas of the temporal bone; hightresolution CT studies. Radiology 1984;150(3):749-53. [ Links ]
4. Dickinson PH, Griffin SM. "Carotid body tumor: 30 years experience".Br J Surg 1986;73:14-16. [ Links ]
Paper submitted to the ABORL-CCF SGP (Management Publications System) on August 22th, 2005 and accepted for publication on July 20th, 2006. cod. 687.
Clínica Pedro Cavalcanti / Hospital Universitário da UFRN.