Extramedullary plasmacytoma of the dura mimicking meningioma

Abstract

In December 2005, a 59-year-old man presented at our Neurosurgery Department with a 2-month history of amnesia and gait disturbance. A solid mass lesion was detected in the right temporal region on magnetic resonance imaging (MRI), presumed to be a meningioma (Fig. 1). He underwent surgery via a right temporal frontal craniotomy and partial resection of the mass. Biopsy of the mass demonstrated a densely cellular tumor composed of plasma cells, positive for IgG and kappa light chain (Fig. 2), and negative for lambda chain, CD56 and cyclin D1. These findings were consistent with intracranial plasmacytoma.No additional sites of plasmacytoma were identified on either computed tomography or bone scintigraphy, and bone marrow plasmacytosis was absent (1.8% plasma cells, morphologically normal), and bone marrow cells showed a normal karyotype. Serum immunoglobulin levels were almost normal (IgG 1,374 mg/dL, IgA 443 mg/dL, IgM 54 mg/dL, IgE 81 IU/mL, IgD 7.6 mg/dL), and postoperative serum protein immunoelectrophoresis showed a small M-component only (IgG-j). Urine Bence-Jones protein was not detected. Other organopathies, such as nephropathy, hypercalcemia and bone region, were absent. Thus, a diagnosis of extramedullary plasmacytoma was made according to the classification of the International Myeloma Working Group (IMWG) [1]. He underwent external beam radiotherapy to the brain at a dosage of 50 Gy, which resulted in the disappearance of the remaining tumor region.In January 2007, he developed hoarseness and thoracic pain. Since CT scans demonstrated new masses at other sites (rib and vertebral bones), he was diagnosed with disease progression. Laboratory findings showed an increased total serum protein of 8.6 g/dL, IgG 3,629 mg/dL, and serum protein immunoelectrophoresis revealed an evident M-component of IgG-j. Bone marrow examination showed no evidence of plasmacytosis (2.4% plasma cells, without atypia) and chromosomal analysis revealed a normal karyotype. Thereafter, he received several courses of chemotherapy with vincristine, doxorubicin and dexamethasone. Despite treatment, regrowth of the mass was observed. He refused further therapy and was discharged from our hospital.Patients with a solitary dural plasmacytoma have been reported with a female predominance of 84% and a mean age of 50.2 years. Clinically, combination therapy including surgical resection followed by at least 50 Gy radiotherapy is recommended, and long-term survival has been observed. On the other hand, patients with myelomatous meningeal involvement have shown an extremely poor prognosis despite intensified treatment, including intrathecal and/or systemic chemotherapy and cranial radiotherapy [2]. Our patient showed a recurrence almost 1 year after the initial diagnosis. We consider that postoperative radiotherapy was delayed by about 2 months because of intensive care, which might have caused the early recurrence.

Dural plasmacytoma mimicking meningioma

“…The differential diagnosis of plasmacytoma in dura mater includes meningioma, which has a similar MRI appearance, metastasis and lymphoma of the dura mater. Meningioma is the most common primary nonglial brain tumor with typical radiological features: an extraaxial tumor with broad attachment to the dura, hyperdense on computerized tomography (CT), isointense with grey matter on T1-weighted MRI with enhancing 'dural tail' (1,5,6).…”

“…A sharp tumor border with lack of bony sclerosis is suggestive of solitary plasmacytoma. Metastatic tumors (most commonly from thyroid, renal, or bronchial cancers) are usually lytic lesions (3,5,6). However, metastatic disease should be considered in all patients who experience new neurological finding, even after a prolonged disease-free interval (3).…”

Solitary Plasmacytoma in the Internal Auditory Canal and Cerebellopontine Angle Mimicking Meningioma

“…The neuroradiological findings of SEP lack specificity and may mimic lymphoma, metastasis, sarcoma, or meningioma (3,5). Final diagnosis is based on the demonstration of a clonal proliferation of plasma cells usually with immunohistochemical staining (1,2).…”

Irradiation combined with surgery for function preservation in the treatment of extramedullary plasmacytoma of the left labia majora during pregnancy: A case report

“…EMP accounts for 3-5% of all plasma cell tumors, with an incidence of only 0.04 cases per 100,000 individuals (2). Approximately 80% of EMPs occur in the upper respiratory tract (3), but they can also occur in numerous other sites (3,4), including the gastrointestinal tract (5,6), brain (7), orbits (8), thyroid gland (9), breasts (10), lungs (11), pleura (12), kidneys (13),bladder (14), urethra (15), ovaries (16), testes (17) and skin (18). Usually, EMPs have no specific clinical manifestations.…”