阴囊Paget病的治疗和预后(附23例报告)

目的:探讨阴囊Paget病的临床特点、治疗方式和预后。方法:回顾性分析23例阴囊Paget病患者的资料,分析其临床、病理、手术治疗和预后的特点。患者平均年龄62.4(49~72)岁,从出现症状到确诊的间隔时间平均为67.6(12~132)个月。A1期9例,A2期12例,B期1例,D期1例。首诊误诊率91.3%(21/23)。手术治疗20例,放疗2例,化疗1例。结果:失访1例,随访22例,平均随访时间119(7~233)个月。第1次治疗后,因肿瘤死亡1例,非肿瘤死亡3例。复发4例,复发伴腹股沟淋巴结转移1例,单独腹股沟淋巴结转移1例,12例无瘤生存。复发或转移患者均接受第2次手术治疗,非肿瘤死亡1例,5例无瘤生存。结论:阴囊Paget病在治疗上应以彻底切除病灶为首选治疗方式,术前或术中确定病灶的范围十分重要。肿瘤侵及真皮时易发生转移,切缘阳性是术后局部复发的关键因素。本病高龄患者居多,进展缓慢,早期治疗预后尚佳,一旦发生远处转移,预后差。

Treatment and Prognosis of Scrotal Extramammary Paget's Disease: A Report of 23 Cases

Objective: To evaluate the clinical manifestation, management and prognostic characteristics of scrotal extramammary Paget's disease (EMPD). Methods: Twenty-three cases of EMPD were identified and retrospectively reviewed, and the clinical findings, surgical treatment, pathologic features and prognostic characteristics were evaluated. The patients ranged in age from 49 to 72 years (mean 62.4 years). The diagnoses were delayed by 12 to 132 months ( 67.6 on average) in 9 cases at Stage A1, 12 at A2, 1 at B and 1 at D. The rate of initial misdiagnosis was 91.3 %(21/23). Twenty of the patients underwent operations, 2 received radiotherapy and 1 chemotherapy. Results: Twenty-two patients of the total number were followed up for 7 to 223 months, averaging at 119, of whom 12 remained cancer free, 1 died of tumor, 3 died of intercurrent disease, 4 experienced local recurrence, 1 relapsed with inguinal lymph node metastasis and 1 developed inguinal lymph node metastasis exclusively. Those with relapses received the second surgery, and 5 of them survived without cancer and 1 died of intercurrent disease. Conclusion: The primary treatment of choice for scrotal EMPD is radical excision. Precise, histological examination before and during the operation is of crucial importance. The positive excisal margins are one of the key causes of local recurrence, and the involvement of dermis by Paget's cells may suggest possible metastasis. Scrotal EMPD tends to occur as a slowly growing lesion, mainly in the elderly, and has a good prognosis in cases of non-invasion. Otherwise the prognosis is poor.