Head & neck imagingCase Type
Elena Cebada Chaparro, Tomás Ganado Díaz, Salomé Merino Menéndez, Irene Ruiz Adelantado, José Carlos Plaza Hernández, Juan Arrazola GarcíaPatient
64 years, female
A 64-year-old female presented with complaints of several months of duration of nasal obstruction. The patient did not refer epistaxis, rhinorrhea, allergy, palpitation, tinnitus or allergy. She had a clinical history of arterial hypertension, hypercholesterolemia, type 2 diabetes mellitus and breast cancer two years before.
Non-enhanced computed tomography (CT) was performed, showing a homogeneous 3-centimetre soft tissue mass in the left nasal cavity extending through the roof down to inferior turbinate (Fig. 1). No focal erosive bone changes or calcifications were found. There were no other findings of note.
Pre-contrast magnetic resonance imaging (MRI) demonstrated a homogeneous mass isointense to muscle on T1-weighted images (wi) and moderately hyperintense on T2-wi, showing punctiform hypointensities that suggested flow voids. Dynamic contrast-enhanced (DCE) MRI revealed early intense homogeneous enhancement and washout of the mass (Fig. 2). There was not diffusion restriction. There were no signs of locoregional, vascular or perineural invasion. (Fig. 2).
68Ga-DOTATOC PET-CT revealed increased uptake in left nasal cavity, that indicated high expression of somatostatin receptors (Fig. 3).
Selective angiography of the left external carotid artery confirmed a hypervascular lesion in the nasal cavity (Fig. 4) that was partially embolized.
Paragangliomas are mostly benign slow-growing neuroendocrine neoplasms that arise from paraganglionic tissue of the extra-adrenal neuroendocrine system. When secreting catecholamines (1-3% of cases) these neoplasms may manifest with relevant symptoms . The presentation on the sinonasal tract is extremely rare [2, 3]. About 30% of all head and neck paragangliomas are hereditary and they associate with different tumour syndromes (paraganglioma syndromes) characterized by distinct clinical features and gene mutations .
Nasal paraganglioma occurs more frequently in 40 - 60 years old patients with slight female predilection. Patients may present with nasal obstruction, epistaxis, facial swelling, pain and anosmia. Secretory neoplasms associate plasma or 24-h urinary metanephrine or catecholamine elevations.
When there is suspicion of nasal cavity mass, CT scan and MRI are indicated to detect and characterize the lesion and it extension.
Clinical inspection revealed a bluish lesion, which triggered further imaging. Multimodal imaging was performed for better assessment of the extension and characterization of the lesion. In this case, this action avoided a potential bleeding complication if biopsy would have been performed first.
As in our case, paragangliomas in the nasal cavity usually appear as well-circumscribed and strongly enhancing masses on MRI, which indicates abundant blood supply. DCE-MRI washout or type III time-intensity curve (TIC), with early enhancement and washout, is characteristic of paragangliomas . Salt-and-pepper appearance of the tumour, expansive growth and remodelling of adjacent bone are frequent findings.
As in most head and neck tumours, diffusion-weighted imaging (DWI) is useful for prognostic evaluation and suggest if a lesion is benign or malignant. Lower ADC values should suggest malignant lesion.
Gallium-68 PET/CT is an accurate imaging tool for localization of SSTR-positive NETs, as it is paraganglioma .
Paragangliomas are neuroendocrine neoplasm derived from paraganglionic system composed of chief cells (cells containing catecholamine granules) and sustentacular cells arranged in a characteristic (Zellballen) pattern nested (Fig. 5a).
Immunohistochemical analysis of our patient specimen demonstrated positivity for synaptophysin and chromogranin (chief cell) (Fig. 5b), and S-100 (sustentacular cells). Thus compatible for head and neck paraganglioma.
Standard treatment of nasal paragangliomas consists on complete resection. As this case, when tumours are larger than 3 cm pre-operative embolization in order to reduce the risk of bleeding and size of the tumour is recommended . A long term follow-up is important after resection, as recurrences or metastasis could occur after many years of initial diagnosis [6, 8].
Take-home Message / Teaching Points
Nasal paragangliomas are an infrequent cause of nasal cavity mass and lack specificity of radiologic findings. Although pathological analysis is mandatory to confirm the diagnosis, an early and intense enhancement and the presence of flow voids within the lesion may suggest the diagnosis.
 Apple D, Kreines K. Cushing’s syndrome due to ectopic ACTH production by a nasal paraganglioma. American Journal of the Medical Sciences 1982; 283(1):32-5. (PMID: 6275703)
 Nguyen B, Patel N, Arianpour K. Characteristics and management of sinonasal paragangliomas: a systematic review. International Forum of Allergy and Rhinology 2019; 9(4):413-426. (PMID: 30570216)
 Tolman C, Stam O. Nasal paraganglioma: differential diagnosis from a radiologic and pathologic perspective. British Journal of Radiology 2016; 2(4). (PMID: 30460037)
 Yuan Y, Shi H, Tao X. Head and neck paragangliomas: diffusion weighted and dynamic contrast enhanced magnetic resonance imaging characteristics. BMC Med Imaging 2016; 16:12. (PMID: 26833065)
 Itani M, Mhlanga J. Chapter 3 Imaging of Pheochromocytoma and Paraganglioma. Paraganglioma: A Multidisciplinary Approach. 2019. (PMID: 31294940)
 Papaspyrou K, Welkborosky H, Gouveris H, Mann WJ. Malignant and Benign Sinonasal Paragangliomas. Laryngoscope. 2013; 123:1830-1836. (PMID: 23371358)
 Tischler AS, De Krijger RR: Pathology of pheochromocytoma and paraganglioma. Endocr Relat Cancer 2015; 22:T123–T133. (PMID: 26136457)
 Hu K, Persky MS. The multidisciplinary management of paragangliomas of the head and neck, Part 2. Oncology (Williston Park) 2003;17(8):1143-53. (PMID: 12966680)
 Boedeker CC. Paragangliomas and paraganglioma syndromes. GMS Curr T Top Otorhinolaryngol Head Neck Surg. 2011;10:Doc03. (PMID: 22558053)
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