O'Connor William J, Lim Katherine K, Zalla Mark J, Gagnot Maureen, Otley Clark C, Nguyen Tri H, Roenigk Randall K
Department of Dermatology, Mayo Clinic, Scottsdale, Arizona Mayo Clinic, Rochester, Minnesota, USA.
Dermatol Surg. 2003 Jul;29(7):723-7. doi: 10.1046/j.1524-4725.2003.29184.x.
Extramammary Paget's disease is a rare cutaneous adenocarcinoma that occurs in an apocrine gland distribution mainly in the anogenital region.
To formulate treatment recommendations for this rare disease, we examined clinical and follow-up data of patients with it.
A retrospective review is given about the treatment and outcome for 95 patients at Mayo Clinic, Rochester, Minnesota, and Scottsdale, Arizona, between 1976 and 2001. The literature regarding diagnosis and treatment of this disease is also reviewed.
Of the 95 patients, 86 had primary disease and 9 had recurrent disease. At mean follow-up (wide excision, 65 months; Mohs surgery, 24 months), disease had recurred in 18 of 83 (22%) who underwent standard wide excision, compared with recurrence in 1 of 12 (8%) who had the Mohs micrographic excision.
Mohs micrographic surgery compares favorably with wide excision. Intraoperative immunostaining with cytokeratin 7 is helpful in delineating disease, as are preoperative scouting biopsies and photodynamic diagnosis.
乳腺外佩吉特病是一种罕见的皮肤腺癌,主要发生于大汗腺分布区域,多见于肛门生殖器部位。
为制定针对这种罕见疾病的治疗建议,我们研究了其患者的临床及随访数据。
回顾性分析了1976年至2001年间明尼苏达州罗切斯特市及亚利桑那州斯科茨代尔市梅奥诊所95例患者的治疗情况及预后。同时也回顾了有关该疾病诊断和治疗的文献。
95例患者中,86例为原发性疾病,9例为复发性疾病。平均随访时间(广泛切除组为65个月;莫氏手术组为24个月),83例行标准广泛切除的患者中有18例(22%)疾病复发,而12例行莫氏显微描记手术切除的患者中仅1例(8%)复发。
莫氏显微描记手术与广泛切除相比效果更佳。术中细胞角蛋白7免疫染色有助于明确病变范围,术前的探索性活检及光动力诊断也有同样作用。