Characteristics and management of sinonasal paragangliomas: a systematic review

Abstract

Background: Paragangliomas of the nasal cavity and paranasal sinuses, although exceedingly rare, can exhibit frequent and aggressive recurrences. Our objective was to evaluate tumor characteristics, clinical course, management, and associated complications of sinonasal paragangliomas Methods: A systematic review of the literature was performed utilizing the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines, searching for sinonasal paraganglioma. Studies meeting inclusion criteria were assessed for level of evidence. Patient demographics, tumor characteristics, primary intervention, and other clinical characteristics were evaluated.Results: Forty-five relevant studies encompassing 54 patients with sinonasal paraganglioma were identified. The most common tumor locations were the nasal cavity (66.7%), ethmoid sinuses (22.2%), maxillary sinuses (7.4%), and sphenoid sinuses (3.7%). Common presenting symptoms included recurrent epistaxis (68.5%), nasal obstruction (53.7%), and headache (13.0%). Tumors were malignant in 28.6% of patients. Only 4 cases (7.4%) involved functional tumors. Initial management was always surgical, via either an open (63.0%) or endoscopic (33.3%) approach. Radiotherapy was used as adjunctive treatment in 10 cases (18.5%). Recurrence rate was 21.7% and occurred between 12 to 156 months a er initial resection. The overall survival was 87.0% with a metastatic rate of 8.7%. Conclusion:Sinonasal paragangliomas are vascular neoplasms manifesting clinically with recurrent epistaxis and nasal obstruction. Management goals are total resection with clear margins and long-term follow-up due to tendency for local recurrence. Radiotherapy has been utilized as adjuvant therapy with variable results. Further randomized controlled studies may be invaluable in elucidating these findings. C 2018 ARS-AAOA, LLC.

Preliminary exploration of functional MRI in sinonasal paragangliomas

“…DCE was available in patient 1 and patient 2, and indicated a type II pattern and type III pattern, respectively (Figures 1g, 2f). 1 H-MRS spectra were acquired in patient 1. The Cho/Cr ratio was 12.9, and another peak of the compound succinate was detected at 2.4 ppm (Figure 1f).…”

“…Hence, DCE-MRI may be used to assess the abundant microvessel perfusion and neoangiogenesis in SNPGLs. 1 H-MRS is a tool with potential to relate cellular and molecular biochemistry to tumoral pathology by providing metabolic information. It enables the analysis of specific metabolites semi-quantitatively and the non-invasive detection of peaks of specific compounds, thus facilitating the diagnosis of head and neck diseases [27].…”

“…Paragangliomas are rare neuroendocrine tumors derived from neural crest cells that located outside the adrenal glands. They are usually distributed along the paraganglia, and approximately 3% occur in the head and neck region [1]. The most recent edition of the WHO Classification of head and neck tumors suggests that paragangliomas have undetermined biologic potential and should not be considered benign [2].…”

“…The most recent edition of the WHO Classification of head and neck tumors suggests that paragangliomas have undetermined biologic potential and should not be considered benign [2]. Sinonasal paragangliomas (SNPGLs), which are located in the nasal cavity and paranasal sinus, ae rarely found, and approximately 50 SNPGLs have been reported to date [1]. The appearance of SNPGLs under conventional CT and MRI has been published in several case reports [3][4][5][6][7][8][9][10].…”

“…By providing noninvasive morphologic and functional information on the tumor microvasculature, DCE-MRI can be useful for discriminating malignancies from benign lesions and monitoring treatment response in head and neck malignancies [19]. 1 H-MRS is a powerful tool that can detect changes in biological compounds within tumors, and can be used for providing tumor-associated metabolic information [20]. Yet, none of these methods have been reported in the evaluation of SNPGLs.…”