Itami Jun, Hara Ryusuke, Komiyama Takafumi, Kato Daiki, Saito Kiyoshi
Department of Radiation Therapy and Oncology, International Medical Center of Japan, Shinjuku-ku.
Radiat Med. 2002 Nov-Dec;20(6):311-8.
PMLBL is a rare disease in Japan, and its optimal management and prognosis remain to be examined. A retrospective analysis of combined modality treatment in PMLBL was performed.
Three women and four men (mean age, 36 years) were found to have PMLBL. Six patients had stage II disease, and one patient stage III disease. Each patient had a mediastinal tumor over 9 cm in diameter. The numbers of risk factors according to the international prognostic index (IPI) and modified tumor score (mTS) were 2 and 2 or 3, respectively. All patients were treated by doxorubicin-based chemotherapy. Two patients underwent tumor resection. Radiation therapy of 27.3 to 40 Gy (mean, 32 Gy) was delivered, after chemotherapy in six patients, and before chemotherapy in one.
Only one stage IIE patient recurred in the bilateral kidneys and had a fatal outcome. The remaining six patients remain disease-free at follow-up ranging from seven to 126 months. Positive accumulation of gallium scintigraphy after chemotherapy was converted to negative by radiation therapy.
The favorable prognosis of PMLBL was confirmed in this study. Radiation therapy should preferably be delivered to all patients with PMLBL after chemotherapy.
原发性纵隔大B细胞淋巴瘤（PMLBL）在日本是一种罕见疾病，其最佳治疗方法和预后仍有待研究。对PMLBL的综合治疗进行了回顾性分析。
发现3名女性和4名男性（平均年龄36岁）患有PMLBL。6例患者为II期疾病，1例为III期疾病。每位患者都有一个直径超过9厘米的纵隔肿瘤。根据国际预后指数（IPI）和改良肿瘤评分（mTS）的危险因素数量分别为2和2或3。所有患者均接受了以阿霉素为基础的化疗。2例患者接受了肿瘤切除术。6例患者在化疗后、1例患者在化疗前接受了27.3至40 Gy（平均32 Gy）的放射治疗。
仅1例IIE期患者双侧肾脏复发并死亡。其余6例患者在7至126个月的随访中无疾病复发。化疗后镓闪烁显像的阳性积聚通过放射治疗转为阴性。
本研究证实了PMLBL的良好预后。放射治疗最好在化疗后应用于所有PMLBL患者。
