Clinical characteristics and treatment outcome of primary mediastinal lymphoma / 대한내과학회지

ABSTRACT

BACKGROUND: The primary mediastinal lymphoma (PML) is defined as the non-Hodgkin's lymphoma that presents primarily within the mediastinum, and primary mediastinal diffuse large B cell lymphoma (PMLBL) is defined as primary mediastinal lymphoma of which histology shows diffuse large B cell lymphoma. There is no available clinical study yet about the clinical characteristics and treatment outcome of PMLBL in Korea. Here the authors report our experience of 21 cases of PML including 11 cases of PMLBL. METHODS: The authors reviewed retrospectively medical records of 21 cases with PML from January 1992 to January 2003, and analyzed clinical characteristics, response to induction therapy, and survival. The authors subsequently performed subset analysis in 11 cases with PMLBL. Median follow-up period was 16 months (range, 4~80 months). RESULTS: Male to female ratio was 813 and the median age of 21 PML cases was 33.5 years. Of 21 PML cases, histology of most cases was diffuse large B cell lymphoma (11/21) and lymphoblastic lymphoma (8/21). Cell lineage was B cell in 13 cases (61.9%). Thirteen cases (62.0%) were in stage I and II. Initial induction therapy was chemotherapy alone in 19 cases, combined chemoradiotherapy in 1 case and no treatment in remaining 1 case. Response rate to initial therapy was 70% in 20 PML cases (complete response [CR] 50%, partial response 20%) with CR of 50% in 10 PMLBL. Median progression-free survival and overall survival for 11 PMLBL cases were 11 months and 16 months, respectively. CONCLUSION: Incidence of PML showed slight predominance in female and in relatively young age with median age of less than 40 years. Most patients presented with a chief complaint of superior vena cava syndrome including dyspnea. The most common histology of PML was diffuse large B cell lymphoma. PMLBL represented low complete response rate to conventional chemotherapy, low progression-free and overall survival rates compared with peripheral diffuse large B cell lymphoma by historical review.