病例報告：陰莖會陰部乳房外柏哲德氏病16年之經驗
張琮昕1、陳建志1、林文州1、張奐光1、楊志東1、許炯明1、
蔡維恭1、江百凱1、周永強1、林文榮1、邱文祥1,2
1台北馬偕紀念醫院 泌尿科；2國立陽明大學 醫學院
Penoscrotal extramammary Paget’s disease:
A report of a single center experience over 16 years.
Tsung-Hsin Chang, MD1, Chien-Chih Chen, MD, PhD1, Wen-Chou Lin, MD1, Huang-Kuang Chang, MD1, Stone Yang, MD1, Jong-Ming Hsu, MD1, Wei-Kung Tsai, MD1, Pai-Kai Chiang, MD1, Yung-Chiong Chow, MD, PhD1, Wun-Rong Lin, MD1, Wen-Hsiang Chiu, MD, PHD1,2,
1Department of Urology, Mackay Memorial Hospital, Taipei, Taiwan
2 School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC.
Abstract
Objectives: This study retrospectively reported a series of 9 male patients with a rare intraepithelial malignancy - penoscrotal extramammary Paget’s diseases (EMPD) from a single center experience over the past 16 years. We described the clinical presentation, histopathology, surgical method and follow up course, thus to improve our experience and management to this rare disease.
Material and Methods: 9 patients with extramammary Paget’s disease of the scrotum, penis and/or perineal were seen between January 2000 and November 2016. The history, clinical presentation, pathology, treatment, and prognosis of 9 patients were analyzed. Any patients with concomitant symptoms especially related with urogenital or anorectal disease will receive thorough and detailed investigation to find occult cancer. All cases underwent wide local excision to remove the involved skin and subcutaneous tissue. The resected tissue were sent and examined by pathology specialists. The defective wound was reconstructed using local flap or split thickness skin graft. The final diagnosis was confirmed by pathology report.
Results: Of the 9 cases, 1 was treated at other hospital after diagnosis. As for the other 8 patients, mean age at diagnosis was 64.8 years old. Mean time from onset to clinical diagnosis was 4.4 years. Metastasis to lymph nodes occurred in 1 patient during pre-op CT. All underwent wide local excision surgery. Surgical margins ranges from 0.5 to 3 cm were decided by surgeons depending on the location and the possibility of wound closure. Final pathology reported surgical margin free in 5 out of 8 patients (67.5%), those patients with surgical margin over 1cm all showed margin free. Mean follow up time was 20 months postoperatively. Local tumor recurrence was found in 2 patients, both received wide excision to remove the tumor, no more recurrence was reported after second operation.
Discussion: In previous studies, wide local excision with intraooperative frozen section could provide patients with favored prognosis. As for our study, we recommended a surgical margin for at least 1cm in order to decrease the recurrence rate. Patients with surgical margin greater than 1 cm all had final clear pathology margin. The accuracy of intra-operative frozen section analysis for margin status was limited due to the subclinical extension of Paget's cell over the margin. Patients with associated internal malignancies are more likely to develop disease recurrence, and we therefore suggest any patient with concomitant symptoms especially related with urogenital or anorectal disease should receive thorough and detailed investigation to find occult cancer and a closer follow up.
Conclusions: EMPD is a rare disease and almost always delayed diagnosed due to insidious onset and similar presentation to chronic eczema or dermatitis. Surgical wide local excision with adequate surgical margin is essential to complete the treatment of EMPD. Plastic surgeon consultation for reconstruction is suggested for large lesion or disease in difficult location. Intra-operative frozen section is recommended, however it provides limited diagnostic value. Final pathology margin involvement of tumor cell could be associated with higher tumor recurrence rate. Wide local excision with close follow up may provide patients with a favored prognosis.