Sinonasal Globular Amyloidosis Simulating Malignancy: A Rare Presentation

Abstract

Primary localized amyloidosis in the head and neck region is a rare entity. The most commonly involved organ is larynx. Primary amyloidosis localized to the sinonasal tract is extremely rare. We report one such case along with a brief review of the associated literature. The aim of reporting this case is to emphasize the fact that sometimes nasal amyloidosis can also present with signs and symptoms of nasal and nasopharyngeal malignancy. The definitive diagnosis in such cases depends upon histopathology and further confirmed by immunohistochemistry. A 55-year old male presented with recurrent episodes of nasal bleed, bilateral nasal obstruction, and bilateral hearing loss from last 7 years. On clinical examination a mass was found in the nasal cavity on both sides reaching up to the nasopharynx. Contrast enhanced CT scan revealed that the mass was extending up to the skull base and destroying bony landmarks of the nasal cavity and paranasal sinuses. Mass was proved to be amyloidosis after histopathological examination. It showed multiple blotches of globular submucosal deposit of amyloid, on staining with Congo red. Immunohistochemistry confirmed AL amyloidosis with expression of mixed kappa and lambda light chain immunoglobulin (κ > λ). No evidence of systemic amyloidosis was found after proper work up. It was managed by conservative surgery.

Keywords: Amyloidosis; Globular; Nose.

Fig. 1

Images from rigid endoscopy demonstrating a pale pinkish looking nasal mass between the septum and the lateral wall above the inferior turbinate

Fig. 2

CECT of the nose and PNS showing enhancing mass extending from nasal cavity to sella and supra seller region with destruction of medial wall of maxillary sinus, nasal septum, hard palate, pterygoid process on both sides

Fig. 3

Blotches of extracellular globular lamellated, homogenous fragmented eosinophilic material in subepithelial and perivascular region (H & E; 100×)

Fig. 4

Apple green birefringence on polarized microscopy (Congo red stain; 100×)

Fig. 5

Immunohistochemistry for amyloid light chain protein show kappa overexpression in amyloid material and nearby lympho-plasmacytic infiltrate (AL-κ; 100×)

Fig. 6

Immunohistochemistry for amyloid showing lambda light chain immunoglobulin expression in amyloid material and nearby lympho-plasmacytic infiltrate (AL-λ; 100×)

Fig. 7

Immunohistochemistry for amyloid light chain protein show kappa expression in nearby lympho-plasmacytic infiltrate at other foci (AL-κ; 400×)

Fig. 8

Immunohistochemistry for amyloid showing lambda light chain immunoglobulin expression in nearby lympho-plasmacytic infiltrate at other foci (AL-λ; 400×)