Nasal paraganglioma: differential diagnosis from a radiologic and pathologic perspective

Abstract

A 33-year-old Asian male presented with spontaneous nosebleeds and olfactory sense problems for the past several years. CT scan and MRI demonstrated a large soft tissue mass in the nasal cavity and paranasal sinus with avid and homogeneous contrast enhancement, focal osseous destruction and a non-enhancing cyst at the intracranial tumour–brain margin. After complete endonasal resection, histopathological examination revealed a paraganglioma. This case highlights the non-specific imaging features of a rare paraganglioma of the anterior skull base and the differential diagnosis from both radiological and pathological perspective.

Nasal Cavity Paraganglioma: Literature Review and Discussion of a Rare Case

“…The 4 most common sites of development are the carotid bifurcation, jugular foramen, vagus nerve, and middle ear. Due to the extensive distribution of the autonomic nervous system, they can appear in other locations, such as the orbit, thyroid, larynx, nasopharynx, tongue, and paranasal sinus [1,4,5,6]. Despite its cytoplasmic neurosecretory granules, only 1-3% are functioning paragangliomas with catecholamine secretion [1].…”

“…They have a higher predisposition to females, and patients are usually diagnosed in their fifth to seventh decade, and pediatric cases suggest genetic predisposition [1,5]. Hereditary paraganglioma incidence is between 10 and 50%, which is often multicentric or bilateral, and is more than a decade younger [8,9].…”

“…Sinonasal paragangliomas are infrequent, with only approximately 40 cases described [5]. Patients often complain about recurrent epistaxis, nasal obstruction, facial swelling, frontal headache, and loss of olfaction [4,13].…”

“…These tumors must be differentiated from other sinonasal masses, benign and malignant, such as esthesioneuroblastomas, gliomas, angiofibromas, hemangiopericytomas, rhabdomyosarcomas, and metastases from neuroendocrine tumors [14]. They most commonly arise from the ethmoid sinus, followed by lateral nasal wall and middle turbinate, and are slow-growing tumors, tending to present in older patients [5]. Sinonasal paragangliomas are most often diagnosed in middle-aged women with no personal or family history of paragangliomas.…”

“…Sinonasal paragangliomas are most often diagnosed in middle-aged women with no personal or family history of paragangliomas. The average age at presentation is 45 years, and most patients are between 40 and 60 years old [5,14,15]. Only 3 cases of functioning sinonasal paragangliomas have been described: the first presented as Cushing syndrome and had elevated ACTH levels; the second had a late onset of Cushing's syndrome, presenting 10 years after the first resection; and the last was a catecholamine-secreting tumor in a patient with previous diagnosis of abdominal paragangliomas [16,17,18].…”