Multiple Liver Nodules Mimicking Metastatic Disease as Initial Presentation of Multiple Myeloma

Abstract

Multiple myeloma is a malignant clonal proliferation of plasma cells in the bone marrow preceded by monoclonal gammopathy of undetermined significance. Initial presentation of multiple myeloma as extramedullary spread in soft tissues particularly in the liver is uncommon. We report a case of a 74-year-old African American female who presented with epigastric pain, hematemesis, elevated alkaline phosphatase, and gamma-glutamyl transferase. Initial impression was peptic ulcer disease; however, ultrasound and CT scan of the abdomen showed multiple liver nodules and perihepatic lymphadenopathy suggestive of metastatic disease. Biopsy of the liver nodules showed CD138 and kappa light chain-restricted positive cells consistent with extramedullary spread of multiple myeloma to the liver. The patient achieved partial response after 6 months of treatment with Velcade, cyclophosphamide, and dexamethasone (VCD). Due to severe neutropenia from cyclophosphamide, regimen was switched to Velcade, Revlimid, and dexamethasone (VRD) which resulted to very good partial response in 1 year which eventually persisted after 4 years. No controlled prospective studies have defined the standard treatment for multiple myeloma with extramedullary spread particularly to the liver. Treatment of multiple myeloma with extramedullary disease follows guidelines for multiple myeloma.

A Case with Hepatic Involvement Mimicking Metastatic Disease in Multiple Myeloma

“…omas et al reported that, in an autopsy series including 64 patients, 40% had plasma cell infiltration of the liver [2,3]. Liver involvement may be nodular, sinusoidal, or diffuse or may be in a mixed pattern [4]. Soft tissue involvement may…”

“…Thomas et al reported that, in an autopsy series including 64 patients, 40% had plasma cell infiltration of the liver [ 2 , 3 ]. Liver involvement may be nodular, sinusoidal, or diffuse or may be in a mixed pattern [ 4 ]. Soft tissue involvement may be seen due to decreased expression of adhesion molecules, downregulation of chemokine receptors, downregulation of tetraspanins, increased heparanase-1 expression, angiogenesis, and mutations in alternative or classical nuclear factor-kB pathways [ 4 , 5 ].…”

“…Liver involvement may be nodular, sinusoidal, or diffuse or may be in a mixed pattern [ 4 ]. Soft tissue involvement may be seen due to decreased expression of adhesion molecules, downregulation of chemokine receptors, downregulation of tetraspanins, increased heparanase-1 expression, angiogenesis, and mutations in alternative or classical nuclear factor-kB pathways [ 4 , 5 ]. Liver involvement in extramedullary myeloma is found as hypoechoic nodules on the CT scan.…”

Liver failure due to relapsed myeloma and hepatic iron overload

“…This type of presentation is often described in imaging studies as multiple nodular hepatic lesions mimicking metastatic tumours. It is often associated with poor prognosis, despite treatment efforts [2][3][4]. Infiltrative liver disease, due specifically to malignant infiltration by plasma cells, is also rare at the time of diagnosis, unless the presentation is due to an aggressive form of multiple myeloma manifesting as primary plasma cell leukemia [5,6].…”

Extramedullary multiple myeloma involving the liver and periportal lymph node, diagnosed by EUS‐FNA in a patient with cirrhosis

“…Extramedullary multiple myeloma (EMM) is a malignancy of clonal plasma cells affecting extraosseous sites. Liver involvement can be seen in the late‐stage of disease . Most of the cases present as diffuse infiltrative lesions other than a focal space‐occupying mass .…”