Department of Gynecological Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China.
Gynecol Oncol. 2013 May;129(2):412-6. doi: 10.1016/j.ygyno.2013.02.029. Epub 2013 Feb 27.
To characterize the clinicopathological features and evaluate the treatment outcomes for cases of primary extramammary Paget's disease of the vulva (EMPDV).
The medical records and pathology slides were reviewed and analyzed for 43 patients with primary EMPDV.
The mean age of the patients was 68.6 years (range, 52-85). Intraepithelial EMPDV, invasive EMPDV and EMPDV with adnexal adenocarcinoma were observed in 33 (76.7%), 7 (16.3%) and 3 (7.0%) cases, respectively. Varied surgical procedures were initially performed in 35 (81.4%) cases. A positive incision margin was observed in 16 cases (47.0%). Definitive radiotherapy at a median dose of 60 Gy was performed in 8 (18.6%) patients. Six patients received postoperative radiotherapy due to a positive margin or lymph node metastasis after surgical excision. During a follow-up period of 6-169 months (median, 54), recurrence was observed in 12 (34.3%) patients. Nine (75.0%) patients underwent repeated surgery and 3 (25.0%) patients received radiotherapy. Long-term overall survival was observed in patients with intraepithelial EMPDV. The median overall survival was 124.5 months in intraepithelial cases, 70.8 months in invasive cases and 21.3 months in cases with adnexal adenocarcinoma (log rank, P=0.032).
Intraepithelial EMPDV accounted for the majority of primary cases and had a better prognosis. Surgical excision was the standard curative treatment for EMPDV. Radiotherapy was an alternative choice for patients with medical contradiction or surgical difficulties. Postoperative radiotherapy could be considered in cases with positive surgical margin or lymph node metastasis. Recurrence was common and repeated excision was often necessary.
描述外阴原发性派杰氏病（EMPDV）的临床病理特征并评估其治疗效果。
回顾性分析 43 例原发性 EMPDV 患者的病历资料和病理切片。
患者平均年龄为 68.6 岁（52-85 岁）。33 例（76.7%）为上皮内 EMPDV，7 例（16.3%）为侵袭性 EMPDV，3 例（7.0%）为伴有附件腺癌的 EMPDV。35 例（81.4%）患者初始接受了不同的手术治疗，16 例（47.0%）的切缘阳性。8 例（18.6%）患者接受了中位剂量为 60 Gy 的根治性放疗。6 例患者因手术切除后切缘阳性或淋巴结转移而接受术后放疗。中位随访时间为 6-169 个月（54 个月），12 例（34.3%）患者复发。9 例（75.0%）患者再次接受手术，3 例（25.0%）患者接受放疗。上皮内 EMPDV 患者长期总生存率较好。上皮内 EMPDV 患者的中位总生存期为 124.5 个月，侵袭性 EMPDV 患者为 70.8 个月，伴有附件腺癌的 EMPDV 患者为 21.3 个月（对数秩检验，P=0.032）。
上皮内 EMPDV 占原发性 EMPDV 多数，预后较好。手术切除是 EMPDV 的标准治疗方法。对于有医疗矛盾或手术困难的患者，放疗是一种替代选择。对于切缘阳性或淋巴结转移的患者，可以考虑术后放疗。复发较为常见，常需反复切除。
