Scrotal and Orbital Involvement in Extramedullary De-differentiated Multiple Myeloma

Abstract

A 71-year-old Indian male presented with a five-month history of progressive bilateral testicular swelling and swelling of the left orbit or one month duration. Nine months ago, he had undergone four cycles of chemotherapy (bortezomib, cyclophosphamide, and dexamethasone) for an immunoglobulin A (IgA) lambda multiple myeloma (3.8 grams per decilitre [g/dL]). This was stage 3 according to the International Staging System (ISS) and had no adverse cytogenetics. The patient had undergone six more cycles of treatment prior to presentation. Physical examination revealed gross distension of the left orbital tissues with violaceous colouring (Figure 1A). The abdomen was distended with ascites. Genitourinary examination revealed testicular enlargement (Figure 1B). There was no erythema or warmth associated with either area. Laboratory tests failed to demonstrate monoclonal paraproteinemia, but his lactate dehydrogenase (LDH) level was elevated at 2068 units per litre (units/L). Abdominal paracentesis revealed a fluid cell count of 16,180 106/L and a fluid LDH of 10,591 units/L. Cytology showed CD138 positivity, 80% for Ki-67, and lambda light chain restriction. These findings supported a diagnosis of dedifferentiated extramedullary multiple myeloma. The prognosis and available management plans were discussed with the patient, and he was discharged to the community to be placed in the care of the palliative care team per his wishes.