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Amyloidosis of the Nasopharynx and Nasal Cavity Presenting as Severe Epistaxis and Sphenoid Sinus Dehiscence
Author(s) -
Harirchian Sanaz,
Mirani Neena,
Baredes Soly
Publication year - 2011
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.21943
Subject(s) - nasal cavity , dept , medicine , citation , art , surgery , library science , computer science , immunology
Amyloidosis is a collection of diseases characterized by the extracellular deposition of the insoluble fibrillar proteinaceous material, amyloid. Its symptoms vary depending on whether the pathology is systemic or localized, and on the organ systems involved. While localized amyloidosis is rare, constituting 10-20% of amyloidosis cases, it most commonly affects the head and neck.1,2 The larynx is most frequently affected, followed by the orbit, salivary glands, oral cavity and oropharynx.3 Nasopharyngeal and nasal cavity amyloidosis is extremely rare, with only 14 prior case reports in the literature.3-5 Presenting symptoms typically include epistaxis, nasal obstruction, and otologic findings due to Eustachian tube obstruction. CT findings are nonspecific, typically revealing a non or minimally enhancing soft tissue mass with areas of calcification and no bony erosion.6-8 It is typically treated with surgical resection and carries an excellent prognosis, despite recurrences. We present a patient with localized amyloidosis of the nasal cavity and nasopharynx who presented with severe epistaxis and sphenoid sinus dehiscence, and eventually had spontaneous amyloidoma resolution with conservative management. We will review the clinical and radiographic features of localized nasopharyngeal and nasal cavity amyloidosis, as well as its management and a review of the literature. Our case highlights the possibility that nasopharyngeal amyloidosis may be managed conservatively with spontaneous regression.