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Hypopharyngeal Amyloidoma

Cliniques Universitaires St. Luc Brussels 1200, Belgium

A 76-year-old woman reported dyspnea, dysphagia, vocal changes, and a vague discomfort in her throat that had persisted for several months. Nasofibroscopy showed a submucosal tumor had developed on the posterior side of the left arytenoid cartilage. A barium swallow study showed a smooth-surfaced submucosal mass in the left piriform sinus that caused an eccentric narrowing of the hypopharynx (Fig. 3A). While the patient performed quiet breathing, MDCT scans were obtained before and after IV administration of contrast medium; they revealed a well-defined, slightly enhancing, 16-mm round mass that bulged into the left piriform sinus and the laryngeal vestibule and had contact with the left aryepiglottic fold (Fig. 3B). No calcification was seen within the lesion, and laryngeal cartilages were intact. An additional MDCT scan of the patient's vocal cords obtained during the Valsalva's maneuver showed that the mass originated in the area of the left postarytenoid cartilage (Fig. 3C). Endoscopy performed with the patient under general anesthesia confirmed the tumoral extension (Fig. 3D).

View larger version (120K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A. —76-year–old woman with hypopharyngeal amyloidoma. Anteroposterior image from barium swallow study shows smooth-surfaced submucosal mass (arrow) with slight lobular contour in left piriform sinus. Mass causes eccentric narrowing of hypopharynx.

View larger version (122K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B. —76-year–old woman with hypopharyngeal amyloidoma. Coronal reformatted image obtained from contrast-enhanced MDCT scan of neck acquired during quiet breathing shows well-defined round mass (arrow) bulging into left piriform sinus and laryngeal vestibule. No calcification is seen, and laryngeal cartilages are intact.

View larger version (99K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3C. —76-year–old woman with hypopharyngeal amyloidoma. Axial reformatted image from contrast-enhanced MDCT scan obtained at level of arytenoid cartilage during Valsalva's maneuver reveals that mass (arrow) has bulged into left postarytenoid area.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3D. —76-year–old woman with hypopharyngeal amyloidoma. Photograph of aspect of tumor was obtained during laryngoscopy performed with patient under general anesthesia. Tumor has caused submucosal nonulcerated thickening of left arytenoid cartilage.

The entire tumor was resected using a carbon dioxide laser. Histologic examination revealed a submucosal nodule covered by a slightly parakeratotic, squamous epithelium. It was made of dense acellular soft tissue containing abundant interstitial deposits of amyloid that, when stained with Congo red, showed the characteristic orange and apple-green birefringence and dichroism under polarized light. Immunohistochemical analysis revealed positive results for lambda light chain and negative results for ß₂-microglobulin, kappa light chain, and amyloid A serum protein. Serum electrophoresis revealed a monoclonal band of kappa light chain fragment of IgA. No sign of multiple myeloma or B-cell lymphoma was found at bone marrow biopsy or on skeletal radiography. Neither clinical nor biochemical workup revealed any hematologic disease after 8 months of follow-up. The final diagnosis was localized light chain–associated AL amyloidosis, with coexisting monoclonal gammopathy of undetermined significance.

Amyloidosis is a benign, slowly progressive disease characterized by extracellular deposition of insoluble fibrillar protein with a ß-pleated-sheet configuration in tissues and organs throughout the body [1]. AL amyloidosis refers to amyloid derived from the lambda or kappa light chain fragments of immunoglobulin. Localized amyloidosis is usually associated with AL amyloid, primarily of the lambda type. AL amyloid is also associated with multiple myeloma and B-cell lymphoma.

The larynx is the most common site of involvement in the upper aerodigestive tract [1]. AL-type hypopharyngeal amyloidosis resulting from multiple myeloma is rare; to our knowledge, it has reported only once [2]. We believe that ours is the first reported case of localized AL-type hypopharyngeal amyloidoma mimicking a submucosal tumor.

CT findings of amyloidomas are nonspecific. The lesions are described as well-defined submucosal homogeneous soft-tissue masses, with no or only a slight degree of contrast enhancement. Occasionally, areas of localized calcification within the mass are present [3]. CT provides excellent information on the anatomic location and topography of different benign lesions [4] but cannot be used to differentiate inflammatory masses from benign neoplasms. A patient with a localized amyloidoma has a better prognosis than a patient with systemic amyloidosis. Treatment of localized laryngeal amyloid deposits is surgery using microendoscopic carbon dioxide laser resection [1].

References

1. Lewis JE, Olsen KD, Kurtin PJ, Kyle RA. Laryngeal amyloidosis: a clinicopathologic and immunohistochemical review. Otolaryngol Head Neck Surg 1992;106:372 –377[Medline]
2. Chadwick MA, Buckland JR, Mason P, Randall CJ, Theaker J. A rare case of dysphagia: hypopharyngeal amyloidosis masquerading as a post-cricoid tumour. J Laryngol Otol2002; 116:54 –56[Medline]
3. Hegarty JL, Rao VM. Amyloidoma of the nasopharynx: CT and MR findings. AJNR 1993;14 : 215–218[Abstract]
4. Aspestrand F, Kolbenstvedt A, Boysen M. CT findings in benign expansions of the larynx. J Comput Assist Tomogr1989; 13:222 –225[Medline]

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This Article Figures Only Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ghekiere, O. Articles by Coche, E. Search for Related Content PubMed PubMed Citation Articles by Ghekiere, O. Articles by Coche, E. Social Bookmarking                      What's this? Hotlight (NEW!) What's Hotlight?