Brain Tumor Res Treat.  2022 Oct;10(4):270-274. 10.14791/btrt.2022.0026.

Spheno-Orbital Plasmacytoma as an Initial Presentation of Multiple Myeloma

• Ahn S
• Cha SH
• Cho WH

• KMID: 2534726
• DOI: http://doi.org/10.14791/btrt.2022.0026

Abstract

Intracranial plasmacytoma is a rare neoplasm and a subtype of malignant plasma cell tumor. Most patients with plasma cell tumors are diagnosed with multiple myeloma, but 5%–10% of patients are not. This report includes descriptions of radiologic and clinical findings in a patient with intracranial plasmacytoma. Intracranial extra-axial plasmacytomas can be easily misdiagnosed as meningioma in radiologic and clinical findings. A 69-year-old woman presented with exophthalmos and diplopia, and MRI indicated meningioma. Thus, she underwent gross total resection, and her pathologic diagnosis was plasmacytoma. Exophthalmos and diplopia were fully recovered. She was finally diagnosed with multiple myeloma based on systemic evaluation and treated with targeted chemotherapy. MRI conducted at 3 months after surgery showed no local recurrence or remnant tumor. Although intracranial plasmacytomas are difficult to distinguish from meningiomas in preoperative evaluation, gross total resection is recommended for the same purposes as meningiomas. If the pathologic diagnosis is a plasmacytoma, it is essential to have a systemic evaluation for multiple myeloma.

Keyword

Plasmacytoma; Multiple myeloma; Meningioma; Exophthalmos

Figure

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  Fig. 1 Preoperative CT scanning. Axial (A) and coronal (B) views of brain CT revealed a mass lesion which has destroyed the lateral wall of the right orbit, zygomatic process of right frontal bone, and squamous part of the right temporal bone and extend to temporal muscle.

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  Fig. 2 Preoperative MRI. A: T2-weighted MR coronal image shows a lobulated extra-axial mass extend to exocranial space. B: Apparent diffusion coefficient map MR axial image shows low signal intensity, which indicate more aggressive tumor behavior. C and D: T1-weighted enhanced MR axial image demonstrates a homogenous hyperintense mass and thickened dura mate, which meningioma-like radiological appearance.

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  Fig. 3 Digital subtraction angiography. A and B: Pre-embolization angiography shows tumor-like vascular brush. C and D: Post-embolization angiography shows decreased blood flow.

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  Fig. 4 Pathologic examination of the surgical specimen (×400). A: Hematoxylin-eosin staining shows high cellularity of well-differentiated plasma cells. B and C: Immunohistochemical staining shows diffuse staining CD79a and CD138. D: Epithelial membrane antigen staining shows negative staining.

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  Fig. 5 Postoperative MRI. T1-weighted enhanced MR axial images shows no local recurrence or remnant tumor.

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