Paget's Disease of the Perianal Region--An Aggressive Disease? Juan M. Sarmiento, M.D.,* Bruce G. Wolff, M.D.,*t Lawrence J. Burgart, M.D.,t Francis A. Frizelle, M.D.,* Duane M. Ilstrup, M.S.t From the Divisions of* Colon & Rectal Surgery, tPathology, and tBiostatistics, Mayo Clinic and Mayo Foundation, Rochester, Minnesota BACKGROUND: Perianal Paget's disease is a rare entity, often associated with internal malignancies and a poor prog- nosis. METHODS: A chart review of patients with perianal Paget's disease who presented consecutively to Mayo dur- ing 25 years (starting in January 1970) was made. Patients included had Paget's disease located in or around the anus (3 cm). Patients were excluded for evidence of spread of vulvaperineal lesions or pagetoid extension of a rectal ade- nocarcinoma. Histology slides were reviewed, and immuno- histochemistry was applied to confirm diagnoses. Follow-up was updated in all patients. Recurrence and survival curves were generated by the Kaplan-Meier method. Survival was compared with an age-matched population by the log-rank test. RESULTS: Thirteen patients, eight females, were diag- nosed (age _+ standard deviation of 68.3 -+ 10.6 years). All histologic diagnoses were confirmed with immunohisto- chemical staining results. Mean follow-up was 6.7 years, 8.8 for living patients. One patient had associated extramam- mary Paget's disease (scrotum). Lesions were located ran- domly at the dentate line, anal verge, and/or perianal area. Four patients had associated carcinomas; none of them were visceral. Eleven patients underwent local resection, without adjuvant therapy. Almost all recurrences were treated by wider local excision. The five-year recurrence rate was 61 percent. Overall five-year and ten-year survival was 67 percent, no different from the age-matched popula- tion (P = 0.546). CONCLUSIONS: These results do not reflect an aggressive nature of perianal Paget's disease, de- spite a high rate of local recurrence. Both primary lesions and recurrences are susceptible to treatment by wider local resection. Long-term survival is no different from that of the normal age-matched population. [Key words: Paget's dis- ease; Anal neoplasia; In situ adenocarcinoma] Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Paget's disease of the perianal region--an aggressive dis- ease? Dis Colon Rectum 1997;40:1187-1194. T he first description of Paget's disease (PD) was made by Sir James Paget in 18741 in a publication of 15 cases of the disease limited to the nipple. All patients had underlying carcinoma of the breast.1 Al- though Paget suggested the possibility of an extra- mammary location of the disease, it was not until 1889 that Crocker reported a patient with scrotal and penile Poster presentation at the meeting of The American Society of Colon and Rectal Surgeons, Seattle, Washington, June 9 to 14, 1996. Address reprints requests to Dr. Wolff: Service of Colon & Rectal Surgery, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 559O5. involvement, which Paget himself agreed with the diagnosis of PD. 2 In addition to the above anatomic sites, PD has been described in other regions of the body: axilla, perineum, groin, thigh, vulva, and perianal region. 3 Distribution of the lesions typically follows an in- creased density of apocrine glands, and this associa- tion could reflect the origin of the disease in many patients. 4 PD of the perianal region is an infrequent diagno- sis. As of 1988, the total available cases from the literature was 96. 5 Because of its rarity, many cases are misdiagnosed during the initial evaluation of pa- tients. 6 One of the most discussed topics of this dis- ease process rests in its association with underlying or internal visceral carcinomas, which perhaps has been overestimated. 7 The following report shows the ex- periences at the Mayo Clinic with perianal PD for more than two decades. MATERIALS AND METHODS Charts of patients with a diagnosis of PD of the perianal region during a period of 25 years (beginning in January 1970) were reviewed. The criteria for in- clusion to the study was as follows: 1) first treatment performed at Mayo during the review period; 2) dis- ease located in the perianal region; 3) histologic proof of PD, as defined by Jones et al. 3 The perianal region was defined as 3 cm around the anus. Cases with downward pagetoid extension of ade- nocarcinoma of the anal canal or rectum were exclud- ed. 8 Also, patients with extensive involvement of vul- var lesions down to the perineum were considered to have a different origin of disease and, thus, were not included. The histology slides of patients who met the inclu- sion criteria (hematoxylin and eosin (H&E)) were reexamined by one experienced pathologist (LJB). Cytochemical stains (PAS-D, Mucicarmine, colloidal iron) and immunohistochemistry (immunoperoxidase AE1/AE3, Cam 5.2, carcinoembryonic antigen, Brst-2, 1187