Extramedullary plasmocytoma associated with a massive deposit of amyloid in the duodenum

Abstract

We report a rare case of extramedullary plasmocytoma associated with a massive deposit of amyloid in the duodenum. A 72-year-old Japanese man was admitted to our hospital presenting with a 3-mo history of epigastric pain, vomiting and weight loss. On computed tomography (CT) a wall thickening of the fourth part of the duodenum was observed. Multiple biopsies obtained from the lesion showed infiltration of plasma cells and lymphocytes, but they were not conclusive. The patient underwent resection of the lesion and, on histopathological examination, the lesion consisted of a dense and diffuse infiltrate of plasma cells and a few admixed lymphocytes with reactive follicles extending to the muscular propria. An extensive deposition of amyloid was also observed. Immunohistochemical stains revealed that a few plasmacytoid cells showed lambda light chain staining, though most were kappa light chain positive. These cells also were positive for CD138 and CD56 but negative for CD20 and CD79. The findings were consistent with extramedullary plasmocytoma associated with a massive deposit of amyloid in duodenum. A subsequent workup for multiple myeloma was completely negative. The patient showed no signs of local recurrence or dissemination of the disease after 12 mo follow-up. Because of the association of plasmocytoma and amyloidosis, the patient must be followed up because of the possible systemic involvement of the neoplasm and amyloidosis in future.

Figure 1

CT showing a mass on the fourth part of the duodenum (arrow).

Figure 2

Gross appearance of the surgical specimen containing the tumor (arrow).

Figure 3

Histopathological examination displaying a dense and diffuse infiltrate of plasma cells and amyloid deposit (arrow). (HE × 400).

Figure 4

Immunohistochemical findings (positive cells in brown, × 400). A: Most plasmocytoid cells were positive for κ light chain; B: Few plasmacytoid cells showing λ light chain staining; Plasmocytoid cells were positive for CD56 (C) and negative for CD20 (D).