The extramedullary plasmacytomas is a plasma cell tumour arising outside the bone marrow and constitutes around 4 % of all plasma cell neoplasms. The Extramedullary Plasmacytoma of the Trachea (EPT) is an extremely rare condition and presents as a tracheal expanding mass. The most common symptoms consist of coughing, dyspnoea, voice change, stridor and expiratory wheezing. The diagnosis is done by computed tomography, bronchoscopy and histological examination which demonstrates neoplastic monoclonal plasma cells expressing specific antigens. The treatment of the EPT remains still speculative, current options are radiotherapy or surgery alone and surgery followed by radiotherapy. The adjuvant chemotherapy is considered only in case of relapse or systemic spread. The prognosis is comforting: the complete remission occurs in 60 % of cases, the recurrence in 22 %, the progression to multiple myeloma in 16 %. We report the case of a 51-year-old female affected by EPT and treated with the removal of the first four tracheal rings and subsequent PAD chemotherapy (bortezomib, adriamycin and dexamethasone). Although the stage of the tumour was early, we decided to administer chemotherapy conceiving it as a preventive systemic treatment.
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