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Unusual Sites of Involvement by Hematologic Malignancies

Case 2. Meningeal Mycloma With Cord Compression

Huron Hospital and Cancer Center, South Pointe Hospital, Cleveland Clinic Health System, Cleveland, OH

A 55-year-old woman presented with pathologic rib fractures, multiple osteolytic lesions, and anemia (hemoglobin level, 5.5 g/dL). She had hypergammaglobulinemia (5.4 g/dL), normal calcium level, and normal renal function. Serum protein electrophoresis revealed a monoclonal spike of gamma globulin. Serum immunofixation identified an immunoglobulin (Ig) G lambda-type M protein. A quantitative Ig assay showed an increase in the IgG level (3.54 g/dL), with diminished IgA and IgM levels. Urine immunofixation electrophoresis revealed Bence-Jones proteinuria (lambda light chain). Bone marrow biopsy showed an increased amount of plasma cells with mitotic features and pleomorphism. Multiple myeloma was diagnosed, and the patient was treated with RBC transfusions, dexamethasone, and pain medications. Three months later, the patient presented with midback pain, progressive bilateral leg weakness, and urinary retention. A neurologic examination revealed weakness of both the upper and lower extremities, bilaterally diminished ankle jerks, impaired fine touch, vibratory and position sensations in both legs, and unresponsive plantar reflexes.

A computed tomographic scan of the head showed lytic bone lesions in the calvarium but no intracranial abnormality. A magnetic resonance imaging scan of the cervical, thoracic, and lumbar spine showed an intradural but extramedullary soft tissue mass posterior and lateral to the spinal cord, which was enhanced with gadolinium contrast. The mass extended from the C2 to the S1 level (Fig 1, A and B). Treatment with melphalan and prednisone was started. An upper thoracic laminectomy performed at the T4 level revealed an epidural, friable gray mass constricting the spinal cord posteriorly. A histologic examination showed typical plasma cells with a few binucleated and mitotic forms (Fig 2).

View larger version (88K): [in this window] [in a new window]   Fig 1.

View larger version (120K): [in this window] [in a new window]   Fig 2.

Meningeal involvement is a rare complication of multiple myeloma.¹ The true incidence is unknown since only 41 cases have been reported in the English literature, the majority of which were localized to intracranial meninges or presented as disseminated disease.^1-7 Meningeal spread can occur either by local invasion from the bone or by hematogenous dissemination.^2-4 The natural history of meningeal myeloma is aggressive, characterized by rapid progression and resistance to therapy.^4-7 Petersen et al⁷ reported a median survival of 1.5 months from the time of diagnosis of meningeal involvement. The therapeutic options are decompressive surgery and/or radiotherapy. Our patient is the fourth reported case of extensive spinal myeloma with cord compression. Meningeal involvement should be considered in myeloma patients who present with progressive paraplegia.

REFERENCES

1. Maldonado JE, Kyle RA, Ludwig J, et al: Meningeal myeloma. Arch Intern Med 126: 660-663, 1970[Abstract/Free Full Text]

2. Slager UT, Taylor WF, Opfell RW, et al: Leptomeningeal myeloma. Arch Pathol Lab Med 103: 680-682, 1979[Medline]

3. Quint DJ, Levy R, Krauss JC: MR of myelomatous meningitis. Am J Neuroradiol 16: 1316-1317, 1994[Abstract]

4. Shalay KM, Parikh JR: Meningeal myelomatosis. Can Assoc Radiol J 45: 460-462, 1994[Medline]

5. Mäntylä R, Kinnunen J, Böhling T: Intracranial plasmocytoma: A case report. Neuroradiology 38: 646-649, 1996[Medline]

6. Lolin YI, Lam CW, Lo WH, et al: IgD multiple myeloma with thoracic spine compression due to epidural extra-osseous tumour spread. J Clin Pathol 47: 669-671, 1994[Abstract/Free Full Text]

7. Petersen SL, Wagner A, Gimsing P: Cerebral and meningeal multiple myeloma after autologous stem cell transplantation: A case report and review of the literature. Am J Hematol 62: 228-233, 1999[Medline]

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This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Solti, M. Articles by Abraksia, S. Search for Related Content PubMed PubMed Citation Articles by Solti, M. Articles by Abraksia, S. Social Bookmarking                            What's this?