Amyloidosis Concurrently Involving the SinonasalCavities and Larynx

Shy-Chyi Chin, Girish Fatterpeckar, Chuan-Hsiang Kao, Cheng-Yu Chen, and Peter M. Som

Summary: Localized amyloidosis is an uncommon benigndisorder. The purpose of this report is to present the caseof a 21-year-old man who had localized amyloidosis simul-taneously involving the sinonasal cavities and the larynx. Therarer sinonasal lesion demonstrated CT findings of adjacent“fluffy” bone changes, possibly representing a new findingsuggestive of this disorder. At MR imaging, the amyloid hadsignal intensity similar to that of skeletal muscle on T1- andT2-weighted images. After contrast material administration,the amyloid enhanced at most minimally, but peripheral en-hancement about the mass was present. The importance ofthis case lies in the multifocal presentation of this uncommondisorder, and the imaging findings herein may provide a newsign of this paranasal sinus disease.

Amyloidosis is characterized by extracellular de-posits of amyloid (1). Although uncommon, about150 cases of amyloidosis have been documented since1935 in the otolaryngology literature (2–5). Therehas, however, been a paucity of descriptions of itsimaging findings in the head and neck (6). The pur-pose of this report is to present the CT and MRfindings of this rare disorder concurrently involvingthe sinonasal cavities and the larynx.

Case ReportA 21-year-old man presented to the ear, nose, and throat

clinic with a 1-year history of slowly progressive nasal stuffinessand dysphonia. Clinical examination demonstrated whitish, ul-cerative, and swollen nasal conchae with serous secretionspresent in the adjacent osteomeatal infundibulum, ethmoid,and maxillary sinuses. Laryngoscopy disclosed a submucosalfirm mass involving the left false fold and effacing the ipsilat-eral laryngeal ventricle. The patient denied any history offamilial or hereditary disease or any notable allergies. CT andMR imaging studies were performed. The paranasal sinus CTshowed that the affected bony conchae and sinus walls had a“fluffy” and somewhat hyperplastic reaction adjacent to thesoft tissue mass that filled both nasal fossae (right greater thanleft) and the ethmoid sinuses (Fig 1A). At MR imaging, thelesion had low to intermediate signal intensity on both T1- and

T2-weighted images and demonstrated peripheral enhance-ment on the contrast-enhanced study. The remaining soft tis-sues within the paranasal sinuses had high T2-weighted signalintensity, which is typical of obstructive secretions (Fig 1B andC). The MR study demonstrated a submucosal lesion in thelarynx involving the left false cord and obliteration of the leftlaryngeal ventricle. The lesion also had low T1- and T2-weighted signal intensities and enhanced primarily along itsperiphery (Fig 2 A and B). The patient underwent surgicalexcision of both lesions and had an uneventful recovery. His-topathologic analysis with Congo Red stain confirmed thediagnosis of amyloidosis. Immunohistochemical examinationrevealed a positive staining pattern for six and eight light chainimmunoglobulins. A thorough systemic workup for additionalamyloid deposits revealed no evidence of other disease.

DiscussionAmyloidosis refers to a heterogeneous group of dis-

orders that share the idiopathic extracellular accumula-tion of amyloid in tissues. If the deposition is extensive,it may interfere with organ or tissue function and evenlead to death (7). Amyloid is a linear, nonbranching,fibrillar proteinaceous material that is arranged in ahighly organized fashion (1). Amyloidosis is now classi-fied as primary amyloidosis, myeloma-associatedamyloid, localized amyloid (laryngeal), secondary amy-loidosis, familial amyloidosis, senile amyloidosis, anddialysis-associated amyloidosis (8, 9). In a large review,70% of the cases were primary, 19% were localized, 4%were familial, and 3% were secondary (7).

About 19% of the cases involved the head and neck,and the larynx is the most commonly affected area,although amyloid has been reported in virtually all headand neck sites (10, 11). To our knowledge, only two casereports have described the simultaneous involvement ofthe larynx and the sinonasal cavities (12, 13).

Although the otolaryngology literature has de-scribed amyloidosis in detail, little has been men-tioned regarding its imaging appearance in the radi-ology literature. Calcification has been mentioned asa nonspecific CT finding (6). In the paranasal sinuses,however, this case had a “fluffy” appearance in thesinonasal cavity bones adjacent to the amyloid depos-its. It is possible that the deposition of the protein-aceous amyloid fibrils in the submucosal layers of thesinonasal cavities incited an osteoblastic reaction thatresulted in the “fluffy” bone changes noted. Althoughsinonasal calcifications can also be seen in inspissatedsecretions, fungal mycetomas, cartilaginous tumors,and olfactory neuroblastomas, the “fluffy” bonechanges have not been seen with these diseases.

The MR findings of a prolonged T2 relaxation timeand signal intensity characteristics similar to those of

Received April 10, 2003; accepted after revision June 30.From the Departments of Radiology (S.-C.C., C.-Y.C.) and Oto-

laryngology—Head and Neck Surgery (C.-H.K.), Tri-Service Gen-eral, Hospital, Taipei, Taiwan; and Departments of Radiology(G.F., P.M.S.), Otolaryngology (P.M.S.), and Anatomy and Func-tional Morphology (P.M.S.), Mount Sinai School of Medicine, NewYork University, New York, New York.

Address correspondence to Peter M. Som, MD, Department ofRadiology, Box 1234, Mount Sinai School of Medicine, OneGustave Levy Place, New York, New York 10029.

© American Society of Neuroradiology

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skeletal muscle on T1-weighted and T2-weighted im-ages have been described in the radiology literature(6). This is not surprising in light of the fact that thatthe highly organized ?-pleated sheet ultrastructure ofamyloid is similar to the multilayered, myofibrillarultrastructure of skeletal muscle (14).

Although the amyloid itself does not enhance withcontrast material administration, peripheral enhance-ment in the region of the amyloid deposits has beennoted. This may be caused by the foreign-body giant-cell reaction that is evoked about the amyloid depos-its (15). The lack of enhancement of the amyloid

FIG 1. A, Coronal CT viewed at wide window settings showsfluffy calcifications (arrows) of the turbinates and sinus wallsadjacent to a nasal mass. Soft tissue windowing shows en-trapped secretions and inflammatory mucosal thickening in theright maxillary and sphenoid sinuses.

B, Axial T2-weighted MR image (TR/TE, 3000/96 ms) throughthe paranasal sinuses, demonstrates a predominantly hypoin-tense lesion (arrows) involving the right sinonasal cavity. In con-trast, entrapped secretions in the right maxillary and sphenoidsinuses have high T2-weighted signal intensity.

C, Contrast-enhanced, axial fat-suppressed T1-weighted im-age (TR/TE, 540/12 ms) demonstrates peripheral enhancementof the lesion (arrows).

FIG 2. A, Coronal T1-weighted image through the larynx demonstrates amass (arrows) involving the left false vocal cord with hypointensity similarto adjacent muscle. The lesion also has low signal intensity on T2-weighted images (not shown).

B, Contrast-enhanced axial T1-weighted, fat-suppressed image dem-onstrates a minimally enhanced submucosal lesion (arrow) surrounded byintense mucosal enhancement.

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deposits helps to distinguish them from cellular tu-mors, all of which enhance to varying degrees. Asmentioned above, CT findings of desiccated secre-tions and fungal mycetomas can be seen as hyperat-tenuated foci, and MR may disclose low T2-weightedsignal intensity or signal intensity voids. However,these inflammatory diseases do not cause the “fluffy”bone changes in the adjacent sinonasal walls noted inthe case of amyloidosis.

In the larynx, the amyloid deposits are submucosaland homogeneous and are not associated with thecartilage changes. They are firm lesions that tend tooccur in the supraglottic larynx, although all laryngealsites may be affected (7). The differential diagnosisincludes other submucosal diseases such as laryngealsarcoidosis, lymphoma, and pseudotumor. Submuco-sal lesions such as paragangliomas and hemangiomasare more localized on CT scans and MR images, andthe entire lesion enhances.

Although the final diagnosis of a mass requires thatthe pathologist be the final arbiter, in this patient the“fluffy” bone appearance in the paranasal sinus wallsoccurring concurrently in a patient with a submucosalsupraglottic mass may suggest the diagnosis of thisrare disorder.

References1. Cohen AS, Calkens E. Electron microscopic observations on a fibrous

component in amyloid of diverse origins. Nature 1959;183:1202–1203

2. Simpson GT, Skinner M, Strong MS, Coheb AS. Localized amy-loidosis of the head and neck and upper aerodigestive and lowerrespiratory tracts. Ann Otol Rhinol Laryngol 1984;93:374–379

3. Kramer R, Som ML. Local tumor-like deposits of amyloid. ArchOtolaryngol 1935;21:324–334

4. Johner CH, Widen AH, Sahgal S. Amyloidosis of the head andneck. Trans Am Acad Ophthalmol Otolaryngol 1972;76:1354–1355

5. Tsikoudas A, Martin-Hirsch DP, Woodhead CJ. Primary sinonasalamyloidosis. J Laryngol Otol 2001;115:55–56

6. Gean-Marton AD, Kirsch CF, Vezina LG, et al. Focal amyloidosisof the head and neck: evaluation with CT and MR imaging. Radi-ology 1991;181:521–525

7. Barnes, L. Miscellaneous disorders of the head and neck. In:Barnes L, ed. Surgical Pathology of the Head and Neck. Vol 3, 2nded. New York: Marcel Dekker;2001:2191–2193

8. Cohen AS, Jones LA. Amyloid and amyloidosis. Curr Opin Rheu-matol 1992;4:94–105

9. Husby G, Araki S, Benditt EP, et al. The 1990 guidelines fornomenclature and classification of amyloid and amyloidosis. In:Natvig JB, ed. Amyloid and Amyloidosis. Dordecht: Kluwer Aca-demic;1990:7–11

10. Lewis JE, Olsen KD, Kurtin PJ, Kyle RA. Laryngeal amyloidosis:a clinicopathologic and immunohistochemical review. OtolaryngolHead Neck Surg 1992;106:372–377

11. Thompson LDR. Pathology of the larynx, hypopharynx, and tra-chea. In: Fu YS, Wenig BM, Abemayor E, eds. Head and NeckPathology with Clinical Correlations. 1st ed. Philadelphia: ChurchillLivingstone;2001:379–380

12. Birchall D, Fields JM, Poon CL. Case report: focal amyloidosis ofthe maxillary antrum: plain film, CT and MR appearances. ClinRadiol 1997;52:392–394

13. Sataloff RT, Abaza M, Abaza NA. Amyloidosis of the larynx. EarNose Throat J 2001;80:369–370

14. Banker BQ, Girvin JP. The ultrastructure features of the mamma-lian muscle spindle. J Neuropathol Exp Neurol 1971;30:155–195

15. Barnes EL Jr, Zafar T. Laryngeal amyloidosis: clinicopathologicstudy of seven cases. Ann Otol Rhinol Laryngol 1977;86:856–863

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