Keywords
Multifocal amyloidoma
Skull base
Orbit
Diagnostic imaging
How to Cite
Copyright © | All rights reserved.
The journal holds the exclusive copyright for the publication of this article under the terms of Brazilian law 9610/98.
Partial reproduction
The use of parts of the texts, figures and questionnaire of the article is free, being mandatory the citation of the authors and journal.
Total reproduction
It is expressly prohibited and must be authorized by the journal.
Abstract
Objective: To report a rare case of extensive multifocal amyloidoma involving the skull base, nasopharynx, and intraorbital extension, emphasizing the imaging criteria that contributed to the differential diagnosis against neoplastic lesions. Case detail: A 66-year-old female patient presented with progressive visual loss culminating in bilateral blindness. Magnetic Resonance Imaging (MRI) investigation revealed a voluminous extra-axial expansive lesion in the skull base and orbit, featuring heterogeneous enhancement and T2 hypointensity, findings that mimicked a meningioma. The definitive diagnosis was established by endoscopic biopsy of the nasopharynx, which demonstrated amyloid deposition with Congo Red positivity, confirming an amyloidoma. Final considerations: This case highlights the challenge of radiological differential diagnosis and the need for a high index of suspicion to include amyloidosis in complex skull base lesions. The recognition of atypical imaging patterns is crucial, and histopathological confirmation via a low-risk biopsy was essential to avoid a high-morbidity neurosurgical intervention.
References
ALOBAIDAN O, et al. Eyelid amyloidosis: a rare condition with diagnostic challenges. Cureus, 2025; 17(8): e90107.
BENNASSI A, et al. Orbital amyloidosis and radiotherapy: a case report and review of literature. Cancer Radiother, 2022; 26(8): 1070-1074.
BRAY DP, et al. Minimally invasive resection of intracerebral amyloidoma: case report and systematic review of the literature. World Neurosurgery, 2020; 138: 205-213.
CROSETTI E, et al. A rare nasopharyngeal presentation of amyloidosis. Ear Nose Throat Journal, 2021; 100(8): 562–565.
DESAI SS, et al. Amyloidoma: a review and case report. Skeletal Radiology, 2021; 50(2): 437-444.
ELGHETANY MT, et al. The Congo red stain revisited. Annals of Clinical and Laboratory Science, 1989; 19(3): 190–195.
FIGUEIREDO E, et al. Amiloidose nodular: relato de rara apresentação da amiloidose localizada cutânea. Diagn Tratamento, 2023; 28(3): 108-111.
GALLUZZI F, GARAVELLO W. Surgical treatment of laryngeal amyloidosis: a systematic review. Eur Arch Otorhinolaryngol, 2023; 280(7): 3065-3074.
GANDHI D, et al. CT and MR imaging of intracerebral amyloidoma: case report and review of the literature. American Journal of Neuroradiology, 2003; 24(3): 519–522.
GEORGE J, et al. Localized nasopharyngeal amyloidosis: A case report. Radiology Case Reports, 2022; 17(12): 4506–4509.
GOLDIS R, et al. Diagnostic challenges and solutions in systemic amyloidosis. International Journal of Molecular Sciences, 2023; 24(5): 4655.
GOMES S, et al. Amyloidosis localized to the sinonasal tract: from the diagnosis to management of disease. Cureus, 2023; 15(1): e33480.
HALL J, et al. Treatment of localized amyloid light chain amyloidosis with external beam radiation therapy. Practical Radiation Oncology, 2022; 12(6): 504–510.
HOWARD S, et al. Multimodality imaging in amyloidosis. Cancer Imaging, 2012; 12(1): 109-117.
JAMARUN SA, WONG EHC. Primary nasopharyngeal amyloidosis with nasal polyposis: case report of a diagnostic challenge. International Journal of Surgery Case Reports, 2022; 94: 107056.
JAMSHIDI P, et al. Clinicopathologic and proteomic analysis of amyloidomas involving the ocular surface and adnexa. American Journal of Clinical Pathology, 2022; 157(4): 620–627.
JOSEPH D, et al. Amyloidosis of the head and neck region – an infrequent entity in an uncommon site: report of three cases and review of literature. European Journal of Cardiovascular Medicine, 2025; 15(4): 576-581.
KAPOOR A, et al. A rare case of cerebral amyloidoma mimicking a hemorrhagic malignant central nervous system neoplasm. Cureus, 2020; 12(3): e7245.
MATHEW R, ZEPPIERI M. Ocular Amyloidosis. StatPearls, 2025.
NISHI S, et al. The features of bone articular lesions in dialysis-related amyloidosis (DRA) and criteria for the clinical diagnosis of DRA. Ren Replace Ther, 2019; 5: 10.
PARK SI, RYU G. A Case of Bilateral Trigeminal Amyloidoma Diagnosed Through an Endoscopic Transsphenoidal Approach. J Rhinol, 2023; 30(3): 177-180.
PARMAR H, et al. Imaging of focal amyloid depositions in the head, neck, and spine: amyloidoma. AJNR Am J Neuroradiol, 2010; 31(7): 1165-1170.
RAMANI NS, KRISHNAN B. Fine-needle aspiration of amyloidoma: a critical analysis. Cancer Cytopathol, 2024; 132(3): 179-185.
SAKAGIANNIS G, et al. Localized nasopharyngeal amyloidosis: a clinicopathologic series of 7 cases with a literature review. Head Neck Pathol, 2018; 12(4): 542-547.
SINGH A, et al. Clinical profile of patients with head and neck amyloidosis: a single-institution retrospective chart review. Int Arch Otorhinolaryngol, 2020; 24(4): 450-456.
TAKUMI K, et al. Amyloidosis in the head and neck: CT findings with clinicopathological correlation. Eur J Radiol, 2020; 128: 109034.
ZUO W, et al. Nasopharyngeal amyloidoma: report of three cases and review of the literature. J Cancer Res Clin Oncol, 2024; 150: 337.