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© 2003 American Society for Clinical Oncology
Hematologic Malignancies With Extramedullary Spread of DiseaseCase 1. Multiple Myeloma With Extramedullary Involvement of the Pleura and TestesHematology, University of Parma, Parma, Italy
A 62-year old man was admitted to our hospital for fatigue. Laboratory tests revealed the presence of anemia (hemoglobin, 10.5 g/dL), with total serum protein of 12.1 g/dL and M-component IgG immunoglobulin (8,520 mg/dL). Serum immunoelectrophoresis demonstrated a spike of IgG lambda chains. Other results were a sedimentation rate of 137 mm (normal value, < 4 mm) and beta-2 microglobulin of 6.8 mg/dL (normal value, 1.2 to 2.5 mg/dL). Bence Jones lambda chains were detected in the urine. Bone marrow biopsy showed a high number of plasma cells (range, 70% to 75%) with a diffuse type of infiltration. Bone marrow (BM) aspirate showed 80% of plasma cells with hypocellular erythroid, myeloid, and megakaryocyte components. Radiographic examination demonstrated the presence of a single osteolytic lesion in distal humerus. A diagnosis of multiple myeloma (MM) was made, and the patient received five cycles of vincristine, doxorubicin, and dexamethasone (VAD) chemotherapy. After the third cycle, a partial response was observed with a reduction of BM plasmacytosis (45%). Relapse occurred after the last VAD treatment with increase in BM plasma cells to 75%. Physical examination revealed dullness and decreased breath sounds in the right lung base. Radiographic studies showed a right pleural effusion (Fig 1
MM is a plasma cell malignancy usually restricted to the bone marrow. Extramedullary involvement (plasmacytoma) can occur occasionally, particularly in the nasopharynx, upper respiratory tract, or gastrointestinal tract.1,2 Our patient showed an unusual extramedullary spread including testis and pleural localization with a rapid and aggressive evolution. In addition, the development of duodenal obstruction with ascites that was rapidly fatal indicated a potential intestinal localization of plasmacytoma. The pleura is an unusual site for plasmacytoma.3,4 Only 0.8% of MM patients were reported to have myelomatous pleural effusion in a review from the Mayo Clinic.5 The majority are caused by IgA myeloma and, less frequently, IgG. Similarly, testicular involvement in patients with MM is a rare event, with a small number of cases reported in the literature.2,6,7 It may occur as an occasional finding during autopsy, as a primary clinical manifestation, or as a metastatic lesion in patients with multiple myeloma or plasma cell leukemia2,7
REFERENCES
1. Bataille R, Harousseau JL: Multiple myeloma. N Engl J Med 336:1657–1663, 1997 2. Dolin S, Dewer J: Extramedullary plasmacytoma. Am J Pathol 32:83–103, 1955
3. Safa AM, Van Orstrand HS: Pleural effusion due to multiple myeloma. Chest 64:246–248, 1973
4. Juan NR, Antonio P, Juan CM, et al: Pleural effusion in multiple myeloma. Chest 105:622–624, 1994 5. Kintzer JS, Rosenow EC III, Kyle RA: Thoracic and pulmunary abnormalities in multiple myeloma: a review of 958 cases. Arch Intern Med 138:727–730, 1978[Abstract] 6. Ferry JA, Young RH, Scully RE: Testicular and epididymal plasmacytoma: A report of 7 cases, including three that were the initial manifestation of plasma cell myeloma. Am J Surg Pathol 21:590–598, 1997[CrossRef][Medline] 7. Castagna M, Gaeta P, Cecchi M, et al: Bilateral synchronous testicular involvement in multiple myeloma. Case report and review of the literature. Tumori 83:768–771
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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