Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan.
Int J Radiat Oncol Biol Phys. 2011 May 1;80(1):47-54. doi: 10.1016/j.ijrobp.2010.01.073. Epub 2010 Oct 15.
Extramammary Paget's disease (EMPD) is a relatively rare malignancy that usually arises in the genital areas. Wide surgical excision remains the standard and most reliable curative treatment of EMPD. However, surgery is sometimes not possible, because many patients are elderly, and complete excision can be difficult owing to the tumor location. We, therefore, performed a review to determine the role of radiotherapy (RT) for EMPD.
A total of 22 patients with EMPD in their external genitalia (4 men and 18 women, age 52-94 years at RT) underwent RT with curative intent. Nine patients had regional lymph node metastases. A total dose of 45-70.2 Gy (median, 60) was delivered to the pelvis, including the tumors, in 25-39 fractions (median, 33).
In all but 3 patients, the irradiated tumors were controlled during a follow-up period of 8-133 months (median, 42). Of the 22 patients, 13 developed recurrences, including local progression within the radiation field in 3 and lymph node and/or distant metastases outside the radiation field in 10, at 3-43 months after treatment. The 2- and 5-year local progression-free rates were 91% and 84%, respectively. Of the 22 patients, 7 patients had died at 33-73 months after RT. The cause of death was tumor progression in 4, infectious pneumonia in 2, and renal failure in 1 patient. The overall and cause-specific survival rates were 100% for both at 2 years and 53% and 73% at 5 years, respectively. No therapy-related Grade 3 or greater toxicity was observed.
RT is safe and effective for patients with EMPD. It appears to contribute to prolonged survival as a result of good tumor control.
外阴派杰氏病（EMPD）是一种相对罕见的恶性肿瘤，通常发生在生殖器区域。广泛的手术切除仍然是 EMPD 的标准和最可靠的治愈性治疗方法。然而，由于许多患者年龄较大，且由于肿瘤位置，完全切除可能较为困难，因此手术有时无法进行。因此，我们进行了一项回顾性研究，以确定放疗（RT）在外阴派杰氏病中的作用。
共有 22 名患有外阴 EMPD 的患者（4 名男性和 18 名女性，RT 时年龄为 52-94 岁）接受了根治性 RT。9 名患者有区域淋巴结转移。盆腔包括肿瘤共接受 45-70.2 Gy（中位数，60）的总剂量，25-39 次分割（中位数，33）。
除 3 例患者外，所有接受放疗的肿瘤在 8-133 个月（中位数，42）的随访期间均得到控制。22 例患者中，13 例出现复发，包括治疗后 3-43 个月时放疗野内局部进展 3 例和放疗野外淋巴结和/或远处转移 10 例。2 年和 5 年局部无进展生存率分别为 91%和 84%。22 例患者中，有 7 例在 RT 后 33-73 个月死亡。死亡原因分别为肿瘤进展 4 例，感染性肺炎 2 例，肾衰竭 1 例。2 年和 5 年总生存率和疾病特异性生存率分别为 100%和 53%和 73%。未观察到与治疗相关的 3 级或更高级别的毒性。
RT 对外阴派杰氏病患者是安全有效的。它似乎通过良好的肿瘤控制来延长生存时间。
