대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Amyloidosis

Radiologic Findings

Plain chest radiograph shows bilateral paratracheal and bilateral hilar bulging shadows. Chest CT shows extensive calcified lymphadenopathy in the mediastinum and hilar and interlobar area.

Mediastinoscopic biopsy was performed. Congo red staing and birefringence microscopic examination reveal amyloidosis.

Brief Review

Lymph node involvement is uncommon in amyloidosis, occurring in less than 20% of cases (1). Only a few cases have been reported in the world literature to my knowledge.

Amyloidosis is a disease characterized by the accumulation of amyloid (a protein) in various body tissues. Amyloidosis may occur secondary to recognized predisposing diseases such as rheumatoid arthritis, tuberculosis, and multiple myeloma. In their absence, it is termed primary.

Only a few of demonstrable lymph node invovement in amyloidosis have been reported previously. All showed enlargement of the hilar, mediastinal, and/or paratracheal lymph nodes. The rarity of radiographically demonstrable lymph node involvement in amyloidosis causes this entity to be omitted from consideration in cases of lymph node enlargement and/or calcification. In addition, there is nothing diagnostic about these manifestations. Lymph node enlargement is usually bilateral, but may be unilateral. Biopsy of enlarged nodes will only be diagnostic if Congo red staining is performed. Thus, the radiologist is advised to add this entity to the differential diagnosis of lymph node enlargement.

Lymph node enlargement, nonspecific calcification, and eggshell calcification are manifestations of lymph node Involvement in amyloidosis. These fIndings are not diagnostic, and very rare. Nevertheless, the possibility of amyloidosls deserves consideratIon in cases of lymph node enlargement with a negative diagnostic work-up, particularly In the presence of diseases which predispose the patient to amyloidosis.

References

1.Borge MA, Parker LA, Mauro MA. Amyloidosis: CT appearance of calcified, enlarged periaortic lymph nodes. J Comput Assist Tomogr 1991;15:855-857.

2. Gross BH. Radiographic manifestations of lymph node involvement in amyloidosis. Radiology 1981;138:11-14.

3. Wilson SR. Sanders DE, Delarue NC. Infrathoraclc manifestations of amyloid disease. Radiology 1976;120:283-289

4. Dlrlcse PR. Primary amyloldosis of the lungs. AJR 1946;56:577-585

5. Wang CC. Robbins Li. Amylokl disease. Its roentgen manifes tations. Radiology 1956;66:489-501.

6. Mackenzie DA. AmyloldosIs presenting as lymphadenopathy. BrMedJ 1963:1449

7. Brown J. Primary amyloldosls. ClIn Radiol 1964;15:358-367.

8. Scheurlen PG. Haun W, MaUsle E, et aI. Generalisierte tumorfOrmlge Lymphknotenamyloldose mit Polyneuropathie und Makroglobulin. Dtsch Med Wochenschr 1973;98:1947-1951.

9. Bottomley JP, Bradley J, Whftehouse GH. Waldenstrom’s macroglobulinaemla and amyloldosls with subcutaneous calcificationand lymphographic appearances. BrJ Radiol 1974;47:232-235.

10. Kidd CR, Fred HL, Natelson EA. Bilateral hilar adenopathy In amyloidosis [letter]. N EngI J Med 1974;290:972.

11. Garcia Gallego F, Calleja Canelas JL. Hilarenlargement In amyloidosls [letter].N Engl J Med 1974;291:531.

12. Glenner GG, lgnaczak TF, Page DL. The inherited systemic amyloidoses and localized amylold deposits. [In] Stanbury JB, Wyngaarden JB, Fredrickson DS, eds. The Metabolic Basis of Inherited Disease. New York, McGraw-Hill, 1978, pp 1308- 1339

Keywords

Mediastinum, Connective tissue diseases,