Postoperative radiation therapy for extramammary Paget's disease
Postoperative radiation therapy for extramammary Paget's disease
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DOI：
10.1111/bjd.13357
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发表时间：
2015-04-01
影响因子：
10.3
通讯作者：
Inoue, T.
中科院分区：
文献类型：
--
作者：
Hata, M.;Koike, I.;Inoue, T.
乳房外佩吉特病（EMPD）是一种罕见的皮肤恶性肿瘤，通常采用手术治疗。手术切缘阳性的患者需要辅助治疗，但很少有关于使用放射治疗的报道。目的探讨EMPD术后放射治疗的有效性。材料与方法21例EMPD患者，涉及生殖器接受放射治疗作为术后辅助治疗。10例患者在放射治疗前有腹股沟淋巴结受累，但无远处转移。中位总剂量为59.4戈伊（范围，45-64.8戈伊），提供给肿瘤床30个分数（范围，23-36 fractions）。然而，6名患者在放射治疗后6-43个月发生远处转移。无远处转移率3年为66%，5年为55%。腹股沟淋巴结受累是远处转移的重要危险因素。4名患者在照射后33-58个月死亡;死亡原因是3名患者的肿瘤进展和1名感染性肺炎。3年的总生存率和病因特异性生存率均为92%，5年分别为62%和71%。结论EMPD患者术后放射治疗能有效维持局部控制，安全性高。
Background Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy that is usually treated with surgery. Patients with positive surgical margins require adjuvant therapy, but there have been few reports on the use of radiation therapy.Objectives To investigate the effectiveness of postoperative radiation therapy in EMPD.Materials and methods Twenty-one patients with EMPD involving the genitalia underwent radiation therapy as adjuvant therapy after surgery. Ten patients had inguinal lymph node involvement before radiation therapy, but none had distant metastases. A median total dose of 59.4 Gy (range, 45-64.8 Gy) was delivered to the tumour bed in 30 fractions (range, 23-36 fractions).Results At a median follow-up period of 38 months, all patients had local control. However, six patients had developed distant metastases 6-43 months after radiation therapy. The distant metastasis-free rates were 66% at 3 years and 55% at 5 years. Inguinal lymph node involvement was a significant risk factor for distant metastases. Four patients died 33-58 months after irradiation; the causes of death were tumour progression in three patients and infectious pneumonia in one. The overall and cause-specific survival rates were both 92% at 3 years, and 62% and 71% at 5 years, respectively. No therapy-related toxicities of grade >= 3 were observed.Conclusions Postoperative radiation therapy is safe and effective in maintaining local control in patients with EMPD.