Myeloma and other monoclonal gammopathies - Clinical SINONASAL EXTRAMEDULLARY PLASMACYTOMA: PROPOSAL OF PROTOCOL OF FOLLOW-UP

Background: Plasmacytoma is characterized by malignant proliferation of a single clone of plasma cells producing monoclonal immunoglobulins and presenting as multiple lesions, multiple myeloma (MM), or a single lesion, solitary plasmacytoma (SP). SP is a single lesion of monoclonal plasma cells inside or outside the skeletal system, in this later case it is named, extramedullary plasmacytoma (EMP). Patient affected by EMP are commonly younger than MM (55- 60); male/female ratio is 3:1. A non unusual presentation of EMP is in the upper airways, and the most common presenting symptoms of sinonasal EMP are unilateral nasal obstruction, epistaxis, rhinorrhea, facial swelling and pain. The diagnosis is based on biopsy of lesions, unilateral bone marrow aspirate, laboratory studies, CT, MRI, and PET/CT. Radiotherapy is the treatment of choice, whereas surgery is performed for diagnostic biopsy or excision of residual disease. Conversely, the role of chemotherapy is still debated. Aims: Adapting timing of control to the behaviour of the disease. Methods: We report 4 cases (3 M, 1 F; mean age:58, range 37-72) of EMP extended to sinonasal cavities complaining of unilateral nasal obstruction and epistaxis. Results: Nasal endoscopy showed a soft, friable, and bloody tumor mass occupying the nasal cavity in 1 case, extended to the right maxilla in 2 and to right ethmoid in the last one. All subjects underwent CT scan, MRI,biochemical tests and bone marrow histology. Nasal biopsies revealed a diffuse infiltration by CD138 positive plasma cells with Ig light chain restriction. Subjects received radiotherapy by linear acceleration , with dose ranging from 40 to 60 Gy over a 1-month period. At 5 years follow-up, 3 patients had signs of recurrence (2 after 5 years and 1 after 2 years) and underwent chemotherapy and autologous bone marrow transplantation with long term control of EMP with complete remission. Summary/Conclusion: Compared to other lymphoproliferative disorders, EMP shows higher probability of late relapse, hence prolonged periodical controls should be schedule