title: Systemic amyloidosis associated with pleomorphic sarcoma of the spleen and remission of nephrotic syndrome after removal of the tumor.
abstract: We report a case of a 57-year-old woman who was diagnosed with a systemic AA amyloidosis associated with a pleomorphic sarcoma of the spleen. Although the association and causality between chronic inflammatory states and systemic AA amyloidosis have been well established, the evidence linking solid malignancies to reactive AA amyloidosis is scarce. Our patient had a significant systemic amyloid deposition including biopsy-proven renal and cardiac AA amyloidosis. Subsequent evaluation uncovered the presence of a large splenic mass, which was treated by splenectomy. Histologically the splenic tumor was classified as pleomorphic sarcoma. The removal of the tumor resulted in a marked decline in proteinuria, stable renal and cardiac functions, and symptomatic improvement at 1-year follow-up. Based on the noted improvements, we speculate that the pleomorphic sarcoma of the spleen caused secondary amyloidosis in our case. Stromal tumors, although rare, may be associated with and should be considered in the differential diagnosis of the cause of AA amyloidosis.
authors.affiliation: Renal Division and Division of Cardiology, Department of Internal Medicine, Washington University School of Medicine and The Barnes-Jewish Hospital, St. Louis, MO, USA.
authors.forename: Irfan
authors.initials: I
authors.lastname: Agha
authors.forename: Robert
authors.initials: R
authors.lastname: Mahoney
authors.forename: Michael
authors.initials: M
authors.lastname: Beardslee
authors.forename: Helen
authors.initials: H
authors.lastname: Liapis
authors.forename: Randy G
authors.initials: RG
authors.lastname: Cowart
authors.forename: Irmantas
authors.initials: I
authors.lastname: Juknevicius
mesh_terms: D000686:Amyloidosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000098:blood supply / Q000473:pathology / Q000648:ultrastructure; D008854:Microscopy, Electron; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000209:etiology / Q000473:pathology / Q000628:therapy; D012074:Remission Induction; D012509:Sarcoma / Q000150:complications / Q000601:surgery; D000685:Serum Amyloid A Protein / Q000032:analysis; D013160:Splenic Neoplasms / Q000150:complications / Q000601:surgery
chemical_list: D000685:Serum Amyloid A Protein