Terada Tadashi
Department of Pathology, Shizuoka City Shimizu Hospital, Miyakami 1231, Shimizu-Ku, Shizuoka, 424-8636, Japan.
Cases J. 2009 Nov 30;2:9110. doi: 10.1186/1757-1626-2-9110.
Multiple myeloma presenting as an intracranial tumor (plasmacytoma) is very rare. An 81-year-old woman was admitted to our hospital because of gait disturbance. A blood laboratory test revealed a mildly increased lactate dehydrogenase (236 IU/L) and glucose (121 mg/dl). Blood protein fractions were normal. Brain computed tomography and magnetic resonance imaging revealed an intracranial mass (5 x 4 x 3 cm) in the brain base next to the clavus, and it was clinically diagnosed as chordoma. An excision of the brain tumor was performed. Imaging modalities including ultrasound, x-ray, computed tomography, magnetic resonance imaging and positron emission tomography did not reveal any tumors other than the brain tumor. The tumor was soft, fragile, and bloody. Microscopically, a monotonous proliferation of atypical plasma cells with hyperchromatic nuclei was recognized. Histochemically, the tumor cells were pyroninophilic and the congo-red stain revealed amyloidosis. Immunohistochemically, the tumor cells were positive for kappa-chain and negative for cytokeratin, epithelial membrane antigen, vimentin, CD45, CD20, CD45RO, lambda-chain, IgM, IgA, IgG, synaptophysin, chromogranin, S100 protein, desmin, alpha-smooth muscle antigen, myoglobin, p53 protein, and glial fibrillary acidic protein. The Ki-67 labeling was 11%. Intracranial plasmacytoma was pathologically diagnosed. The patient was treated by adjuvant chemoradiation, and entered into the complete remission stage. However, multiple metastases emerged in the vertebral bones and ribs six months after the remission. A diagnosis of multiple myeloma was made. The urine revealed Bence-Jones protein of monoclonal IgG kappa-chain type, but blood M protein was not recognized. The patient's condition gradually deteriorated. The patient died of respiratory failure due to bronchopneumonia 18 months after the admission. The present case indicates that multiple myeloma may manifest as an intracranial brain tumor (plasmacytoma).
表现为颅内肿瘤（浆细胞瘤）的多发性骨髓瘤非常罕见。一名81岁女性因步态障碍入住我院。血液实验室检查显示乳酸脱氢酶轻度升高（236 IU/L）和血糖升高（121 mg/dl）。血液蛋白组分正常。脑部计算机断层扫描和磁共振成像显示在靠近视乳头的脑底部有一个颅内肿块（5×4×3 cm），临床诊断为脊索瘤。进行了脑肿瘤切除术。包括超声、X线、计算机断层扫描、磁共振成像和正电子发射断层扫描在内的影像学检查未发现除脑肿瘤外的任何其他肿瘤。肿瘤质地柔软、易碎且有出血。显微镜下，可见核深染的非典型浆细胞呈单一性增殖。组织化学检查显示肿瘤细胞对派洛宁呈嗜染性，刚果红染色显示淀粉样变性。免疫组织化学检查显示肿瘤细胞κ链阳性，细胞角蛋白、上皮膜抗原、波形蛋白、CD45、CD20、CD45RO、λ链、IgM、IgA、IgG、突触素、嗜铬粒蛋白、S100蛋白、结蛋白、α平滑肌抗原、肌红蛋白、p53蛋白和胶质纤维酸性蛋白阴性。Ki-67标记率为11%。病理诊断为颅内浆细胞瘤。患者接受了辅助放化疗，进入完全缓解期。然而，缓解6个月后在椎骨和肋骨出现多处转移。诊断为多发性骨髓瘤。尿液检查显示为单克隆IgG κ链型本-周蛋白，但未检测到血液M蛋白。患者病情逐渐恶化。入院18个月后因支气管肺炎呼吸衰竭死亡。本病例表明多发性骨髓瘤可能表现为颅内脑肿瘤（浆细胞瘤）。
