Extramedullary plasmacytoma: Clinical behaviour and response to treatment

Abstract

The clinical features and response to treatment of 35 patients (pts) with extramedullary plasmacytoma (EMP) were retrospectively analysed. The median age at diagnosis was 49 years (28-72). Twenty-two pts (63%) had stage I disease (localized to the primary site) 12 of whom (34%) had stage I-E (locally extended). Three pts (9%) had stage II (regional lymph nodes involved) and 10 (29%) stage III (disseminated disease). In locoregional disease (stages I, I-E, II) complete local control was achieved in 22 of 25 pts (88%), while in diffuse disease (stage III) complete remission (CR) was obtained in 5 of 10 pts (50%) (p = 0.05). In 9 of 18 pts treated with surgery, local control was achieved, and in 8 of the 9 patients with incomplete resection local control was obtained with additional radiation and/or chemotherapy. In 8 (66%) of the 12 pts treated with radiation complete local control was achieved. In 11 (58%) of 19 pts evaluable for initial chemotherapy CR was obtained. Three of these pts were treated with chemotherapy only and were alive and disease-free after a minimum follow-up of 8 years. The median time to relapse in local disease was 48 months versus 13 in disseminated disease. For pts with local disease the median survival time was 114 months and for disseminated disease 16 months (p = 0.0000). We conclude that in stage I chemotherapy is curative per se. In local stages (I, I-E and II) adjuvant chemotherapy should be considered, while in stage III only palliative therapy is feasible.

Extramedullary plasmacytoma of the head and neck region: clinicopathological correlation in 25 cases

“…Corwin and Lindberg (1979) and Mendenhall et al (1980) required less than 10% plasma cells in the bone marrow, while Knowling et al (1983) required normal bone marrow biopsy for the diagnosis of EMP. Soesan et al (1992) has accepted the diagnosis of EMP for those plasma cell tumours which presented in an extramedullary site and did not arise from bone marrow with a breach through the bone cortex. Most authors agree that the detection of a monoclonal band on serum protein electrophoresis or urinary Bence Jones protein does not necessarily preclude the diagnosis.…”

“…Subsequent development of a paraprotein may signal a recurrence (Kapadia et al, 1982;Mock et al, 1987). Holland et al (1992) felt that patients with paraprotein at the time of diagnosis fared worst, while Harwood et al (1981), Soesan et al (1992) and Shih et al (1995) suggested no effect on prognosis provided they return to normal after treatment. Only one patient, in our series, had an IgA monoclonal band on serum protein eletrophoresis at diagnosis, and he has remained disease-free.…”

“…Very few reported cases of local recurrence have been treated primarily by surgery (Wiltshaw, 1976;Soesan et al, 1992). Surgery is recommended if there is local failure after XRT in a resectable tumour (Kotner and Wang, 1972).…”

“…Kapadia et al (1982) advocates chemotherapy in the adjuvant setting in large or poorly differentiated tumours. Wiltshaw (1978) and Soesan et al (1992) found better results when they treated the patients with locally invasive disease with adjuvant alkylating chemotherapy. The patient with a high-grade tumour who had a local recurrence was treated with high-dose chemotherapy and peripheral blood stem cell rescue.…”

Late and localized extramedullary relapse of a light chain kappa myeloma after syngeneic bone marrow transplantation

“…Soft tissue localized plasmacytoma occurs in 80% of cases in the oral cavity or upper respiratory tract and prognosis is excellent after local treatment only. 7 In our patient, response to local treatment was complete and maintained for over 2 years at the time of writing. Extramedullary relapse without bone marrow infiltration seems to occur more frequently after autologous BMT as compared to conventional chemotherapy.…”