Multiple Myeloma and CNSAbdominal Manifestations of Extraosseous Myeloma

Multiple Myeloma and CNS/Abdominal Manifestations of Extraosseous Myeloma Pam O-charoen 11 -26 -14

Multiple Myeloma • neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin • 1 % of all cancers and 10 % of hematologic malignancies • More common in African American • Median age is 66 yo (2% younger than 40 yo)

Clinical presentation • • Anemia - 73 percent Bone pain - 58 percent Elevated creatinine - 48 percent Fatigue/generalized weakness - 32 percent Hypercalcemia - 28 percent Weight loss - 24 percent Recurrent bacterial infection

Extramedullary myeloma deposits outside the skeletal system, rare 7 % at the time of diagnosis additional 6 % later in the disease course Autopsy series 63. 5% of myeloma patients who died as a result of complications of the disease • lymph nodes, pleura, liver, CNS • •

Extramedullary myeloma • • • Increase incidence in younger patients Seen in aggressive type of myeloma eg. Ig. D MM Diagnosed and confirmed by tissue biopsy Poor prognosis; median survival is 38 days Difficult to diagnose The response to chemotherapy is poor

Extramedullary myeloma • Recent trend: increase E-MM - widespread use of sensitive imaging techniques - the use of high-dose therapy and the novel agents (thalidomide, bortezomib, and lenalidomide) increase survival - decreasing the adhesion of malignant plasma cells, lose the cell-to-cell interactions within the bone marrow disseminate and infiltrate extramedullary sites

CNS involvement • Intracranial plasmacytomas rare - extensions of lesions of the skull • Leptomeningeal myelomatosis with abnormal CSF uncommon, advance stage, poor prognosis • Hyperviscosity syndrome • Elevated ammonia levels in the absence of liver involvement

Abdominal involvement • • Liver Pancreas Spleen peritoneum and omentum GI tract renal and perirenal space lymph nodes soft tissues/muscles

Liver involvement • • • 28 -30% in autopsy studies, Ig. A MM Two types: diffuse infiltration, most common, hepatomegaly Nodular involvement (plasmacytoma) Poor prognosis Combination chemotherapy or steroid alone Stem cell transplant

Liver involvement • US: hypoechoic, target appearance, hyperechoic (rare) CT: hepatomegaly, hypodense lesion, no contrast enhancement, one case report – hyperdense MRI: hyperintense in T 1 weighted images • imaging findings may be nonspecific, can be confused for metastatic liver lesions

Liver involvement • Wide range of clinical manifestation - asymptomatic , elevated liver enzyme - present with hepatomegaly, jaundice, ascites, or fulminant liver failure • DDx – amyloidosis, light chain deposition disease • Need tissue biopsy

Take home message • • Extramedullary myeloma rare but incidence increasing Need high index of suspicion Poor prognosis Need tissue biopsy and aggressive treatment