Treatment Options for Peri-anal Paget's Disease

Li Liu, MD
Last Modified: November 1, 2001

In 1986 and l987 I was operated on twice to excise Peri-anal Paget's disease and obtain clean margins. Pathology revealed underlying adenocarcinoma and the sphincter was excised, but not destroyed. After the second operation there were still some underlying cancer cells, but I was told I would just be watched--and I was for every three months for five years and then every 6 months for ten. And I thought I was cured.

A recent exam revealed a return of the Paget's and surgery is scheduled. Needless to say I am distraught at having to go through more painful surgery.

It was very difficult at that time to find any oncologist or doctor who had seen, or was aware of this disease. I did go to USC who advised the second operation.

I am writing to inquire if new treatments have been discovered in the past ten years and if this time the surgery reveals internal carcinoma, (as it did last time) if more aggressive treatments can be used with losing my sphincter function?

Do you have more information on the rare cancer?  

Li Liu, MD, OncoLink Editorial Assistant, responds:

Dear E:  
Thank you for your interest and question.

Paget's disease is a relatively rare disease which occur more frequently on the areola and nipple (mammary Paget's disease) and much less commonly on the vulva, scrotum, or perineal and perianal areas (extramammary Paget's disease). Extramammary Paget's disease is a rare form of adenocarcinoma. Perianal Paget's disease is a very uncommon clinical entity. Wide local excision has been used in most of the reported series. Mayo Clinic reported 13 cases of perianal Paget's disease treated with local excision (Sarmiento JM, et al. Diseases of the Colon and Rectum, 1997; 40:1187-1194). The five-year recurrence rate was 61%. Almost all recurrences were treated by wider local excision. Long-term survival is no different from that of the normal age-matched population. The report from Cleveland Clinic Foundation revealed that survival of patients treated by wide local excision was higher than that for those treated with a less aggressive local excision (Marchesa P, et al. Annals of Surgical Oncology, 1997, 4:475-480).

In addition to surgical resection, high dose rate brachytherapy was also used in a case report (Kwan WH, et al. Clinical Oncology (Royal College of Radiologists), 1995; 7:400-401). Although radiation therapy is commonly used for other cancers of the anus, the rarity of Paget's disease of the anus and generally good results with surgery have left radiation therapy largely unexplored for this disease. If there are serious concerns about losing sphincter function with additional surgery, you may want to consider discussing your case with an oncologist as well as a radiation oncologist.