2000;20:242C245. signs and symptoms, some of which may be vague. Consequently, demonstration and appropriate diagnostic investigations may be delayed. 2 Cranial nerve palsy may be a significant complication of plasmaCcell neoplasms including this anatomic site.3 Treatable skull foundation lesions, including plasmacytomas and multiple myelomas, must be considered in the differential analysis of individuals who demonstrate a progressive program or multiple cranial neuropathies,4 even those more youthful than 40 years older.5 We present a patient with light chain multiple myeloma who became symptomatic with signs and symptoms associated with plasmacytoma of the skull base. ILLUSTRATIVE CASE A 39CyearCold man was admitted having a 2Cyr history of progressive paresthesia of the remaining side of the face and remaining arm, intermittent diplopia, dysphagia, and hoarseness. His physical exam was unremarkable. His neurological exam exposed ninth and tenth cranial nerve palsy on the right. Routine laboratory evaluation was normal except for slight anemia (hemoglobin, 12.8 gr/dL) and an elevated erythrocyte sedimentation rate (31 mm/hr). Serum calcium, alkaline phosphatase levels, and renal function checks were normal. Cranial magnetic resonance imaging (MRI) disclosed a large midline mass extending from your ethmoid and sphenoid sinuses into the clivus NIBR189 and petroclival junctions. The tumor also invaded the atlantoaxial joint and odontoid process (Fig. 1). A presumptive analysis of hematological malignancy, chordoma, or invasive adenoma was made. Open in a separate window Number 1A T1Cweighted sagittal gadoliniumCenhanced MRI shows homogenously enhancing softCtissue mass in the ethmoid and sphenoid sinuses and clivus and extending into top two cervical vertebrae. Transnasal transCsphenoidal biopsy of the tumor was performed to obtain a definitive analysis. Intraoperative imprint smear and paraffin sections of the biopsy specimen showed considerable infiltration of plasma cells (Fig. 2), which stained positive for CD 38 and kappa light chain, immunohistochemically. The lambda light chain stain was bad. Bone marrow biopsy showed almost diffuse neoplastic plasma cell infiltration. Serum immunoglobulin (Ig) levels were as follows: IgG, 453 mg/dL; IgA, 35.5 mg/dL; and IgM, 45.5 NIBR189 mg/dL. BetaC2 microglobulin level was 2.6 mg/L. Urine immunelectrophoresis showed improved (8 gr/day time) light chain excretion. Light chain multiple myeloma stage II A NIBR189 according to the DurieCSalmon staging system was diagnosed. Open in a separate window Number 2 Neoplastic plasma cell infiltration (hematoxylin and eosin 500). The patient received radiation (52 Gy) for the skull base mass followed by systemic vincristine, adriamycine, and dexamethasone (VAD) combination chemotherapy. Cranial MRI performed after the patient completed four cycles of chemotherapy showed a partial response with some regression of the tumor (Fig. 3). A followCup exam showed no residual neurological deficit and the patient was symptom free. The patient is definitely Rabbit Polyclonal to SRPK3 expected to receive highCdose melphalan and an autologous stem cell transplant. Open in a separate window Number 3 After radiotherapy, a T2Cweighted sagittal MRI shows regression of the tumor and some treatmentCrelated tumor necrosis. Conversation Multiple myeloma has been reported in only 2?% of individuals under 40 years of age.6 Our patient was in this rare age range for disease presentation. Skull foundation lesions are usually asymptomatic, but larger tumors can cause symptoms. Multiple myeloma hardly ever entails the skull foundation. Cranial nerves II, III, IV, and VI are most often involved, but large lesions can affect additional cranial nerves.3, 4, 5, 7 Plasma cell neoplasms should be considered in the differential analysis of skull foundation tumors associated with cranial nerve palsy. Our individual experienced ninth and tenth cranial nerve palsy due to the considerable involvement of the skull foundation from the lesion. His intermittent diplopia was probably related to improved intracranial pressure.8 He did not exhibit the common initial findings of myeloma such as bone pain, anemia, renal insufficiency, and hypercalcemia. Extramedullary plasmacytoma can be a harbinger of multiple myeloma6 in 20 to 30?% of the patients1 and may develop several years after disease onset.8, 9 The risk for any plasmacytoma of the skull foundation to progress to multiple myeloma over the course of longCterm followCup is high.10 The longstanding symptoms and relatively.