Nasal paraganglioma: A case report and literature review

Abstract

Summary Introduction: Paragangliomas are neuroendocrine tumors that most commonly originate in the adrenal gland, a type that is called pheochromocytoma; however, 5–10% of paragangliomas are extra-adrenal and may arise in any area between the neck and pelvic region along the sympathetic nervous system. Those located in the head and neck comprise 3% of extra-adrenal tumors, with the majority originating in the tympanic–jugular region and carotid body. Objective: To present a rare case of nasal paraganglioma and review the literature. Case report: The patient was submitted to medial subtotal maxillectomy, and her clinical findings, diagnostic data, and treatment outcome were recorded. Conclusion: Paragangliomas are considered benign tumors, but they occasionally display a malignant character. The most important finding in this case was the need for total resection of the tumor to avoid recurrence.

Nasal paraganglioma: differential diagnosis from a radiologic and pathologic perspective

“…The classical paraganglioma has a “salt and pepper” appearance on both T 1 and T 2 weighted images, which is of high diagnostic value when seen; the salt-like areas are high signal areas secondary to subacute haemorrhage, whereas the pepper-like areas are low signal intensity flow voids or vascular channels. 6 – 8 A similar appearance may be seen on T 2 sequences or post-contrast T 1 sequences in patients with juvenile nasopharyngeal angiofibroma. 3 Owing to slow growth, adjacent bone erosion and remodelling occur frequently.…”

“…Paragangliomas of the sinonasal tract are extremely rare, with less than 40 case reports described. 7 , 8 The exact origin is mostly the ethmoid sinus, followed by the lateral nasal wall and middle turbinate. 6 Because of its slow growing and expansive behaviour, patients present at an older age and late in the course of the disease with nasal obstruction, epistaxis, facial swelling, pain and loss of olfaction.…”

“… 3 Owing to slow growth, adjacent bone erosion and remodelling occur frequently. 3 , 6 – 8 Larger masses can have degenerated cystic parts, not to be mistaken for necrosis. On angiography, a prolonged tumour blush is seen owing to hypervascularity.…”

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

“…These are all hypervascular tumors, with contrast enhancement, presenting the classic “salt-and-pepper” appearance and variable patterns of intensity on MRI [5,10,20,21,22,23,24,25,26,27,28,29,30,31,32]. …”

Review Paraneoplastic syndromes in olfactory neuroblastoma

“…Because of the proximity to the sella, ONBs must be distinguished from pituitary adenomas that have extended downward into the nasal cavity or mentioned intracranial “ectopic” pituitary adenomas which originate from remnant cells deposited along the Rathke's pouch developmental route and may develop into hormonally active adenomas in the ectopic regions, such as the sphenoid or cavernous sinus [ 14 ]. ACTH-secreting neuroendocrine tumors such as nasal paragangliomas [ 15 , 16 ], ACTH-secreting nasopharyngeal carcinoma and meningioma [ 13 ] are other described paranasal and cranial ectopic sources of ACTH.…”