Besa P, Rich T A, Delclos L, Edwards C L, Ota D M, Wharton J T
Department of Clinical Radiotherapy, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Int J Radiat Oncol Biol Phys. 1992;24(1):73-8. doi: 10.1016/0360-3016(92)91024-h.
We have reviewed our treatment results in 65 patients with extramammary Paget's disease arising in the vulva, perianal area, or scrotum. In 30 patients with primary disease, positive surgical margins were found in 53%, and there was an actuarial local recurrence rate of 40% within 5 years. The median follow-up period for primary extramammary Paget's disease patients treated with surgery alone was 198 months, and none died of this disease. Three patients treated with definitive radiotherapy were without recurrence at 12, 21, and 60 months after 56 Gy of supervoltage x-rays. In 22 patients with extramammary Paget's disease and associated adnexal or rectal adenocarcinoma, nine treated with surgery alone had a 75% local control rate. Three patients treated with surgery and adjuvant radiotherapy all had local control; of two patients treated with radiotherapy alone, one had persistent adenocarcinoma. The median survival for all patients with extramammary Paget's disease and adenocarcinoma was 22 months. We conclude that patients with extramammary Paget's disease have excellent survival but that local recurrence and morbidity from surgery, especially in the elderly, can be high. Radiotherapy greater than 50 Gy as primary treatment for extramammary Paget's disease in those medically unfit for surgery, or as an alternative to further surgery for recurrence after surgery and for anyone wishing to avoid mutilating surgery, is indicated. For those with adenocarcinoma and extramammary Paget's disease, the use of adjuvant postoperative radiotherapy in doses greater than 55 Gy is indicated because of the high risk of local recurrence after surgery alone.
我们回顾了65例发生在外阴、肛周或阴囊的乳房外佩吉特病患者的治疗结果。在30例原发性疾病患者中，53%发现手术切缘阳性，5年内的精算局部复发率为40%。单纯接受手术治疗的原发性乳房外佩吉特病患者的中位随访期为198个月，无患者死于该病。3例接受根治性放疗的患者在接受56 Gy的超高压X线照射后12、21和60个月均无复发。在22例乳房外佩吉特病合并附件或直肠腺癌的患者中，9例单纯接受手术治疗的患者局部控制率为75%。3例接受手术及辅助放疗的患者均实现了局部控制；2例单纯接受放疗的患者中，1例仍有持续性腺癌。所有乳房外佩吉特病合并腺癌患者的中位生存期为22个月。我们得出结论，乳房外佩吉特病患者的生存率很高，但局部复发以及手术带来的并发症，尤其是在老年患者中，可能会很高。对于那些因身体原因不适宜手术的患者，或作为手术后复发时进一步手术的替代方案，以及任何希望避免进行致残性手术的患者，建议采用大于50 Gy的放疗作为乳房外佩吉特病的主要治疗方法。对于那些患有腺癌和乳房外佩吉特病的患者，由于单纯手术后局部复发风险高，建议使用大于55 Gy的辅助术后放疗。
