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The irreplaceable image: Plasmacytoma with cutaneous and pleural involvement.

1. Wiltshaw E. The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine 1976; 55: 21738. 2. Carere R, Stewart K, Bailey D, Baker MA. Extensive cutaneous plasmacytomas. Leuk Lymphoma 1993; 10:493-5. 3. Sasser RL, Yam LT, Li CY. Myeloma with involvement of the serous cavities. Cytologic and immunochemical diagnosis and literature review. Acta Cytol 1990; 34:479-85. 4. Abbate SL, Jaff MR, Fishleder AJ, Meeker DP. Lambda light chain myeloma with pleural involvement. Cleve Clin J Med 1991; 58:235-9. Extramedullary plasmacytomas (EP) are rare tumors that occur preferentially in the upper respiratory tract, even though involvement of other sites has been described.1 Their characteristics at onset may play a prognostic role; in particular primary lesions of the skin seem to have a better prognosis than secondary cutaneous plasmacytomas,2 while cavitary involvement carries a poor prognosis.3,4 We report a case of EP, involving two different sites, that has shown a good response to high dose chemotherapy. A 58-year-old man presented in January 1998 with diffuse purple mushroom-shaped cutaneous lesions varying in diameter from 0.5 to 8 cm (Figure 1) mainly involving the trunk and the arms. An excisional biopsy revealed a monomorphic plasma-cell population and immunohistochemical studies identified the secretion of a monoclonal l chain, consistent with a diagnosis of plasmacytoma. A staging procedure was started: a skeletal X ray showed lytic lesions involving ribs (VI on the right and V on the left), the left clavicle and the left ischiopubic branch. Bone marrow trephine biopsy was negative, serum M component was absent, while a small amount of Bence Jones protein (l chain) was detected in the urine. In the meantime the patient developed severe dyspnea; the chest X ray film showed a right pleural effusion. Fluid cytology revealed numerous plasma-cells, indicating that the pleura had also been involved by the disease. The patient was initially treated with VAD (vincristine, adriamycin and dexamethasone), but a poor response was observed. Therefore a high-dose chemotherapy program was started including cyclophosphamide 7 g/m2 with stem cell collection, followed by double autologous transplant. Both the cutaneous lesions and the pleural effusion progressively improved from the beginning of the therapeutic program (Figure 2). The patient is presently undergoing the second autologous transplant, in a good clinical condition.