Paget's Disease

Kenneth R. Blank, MD
Last Modified: November 1, 2001

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Question
Dear OncoLink "Ask the Experts,"
As I am sure you are aware, Paget's disease is primarily a bone or breast disease. Unfortunately, there are several people in the Bay Area, specifically women, who are afflicted with Paget's of the anus, rectum or vulva. There is very little information of Paget's disease on these parts of the anatomy and I was wondering if you could let me know who might know more about this and treatment of the disease in those areas. I know someone who has had this condition for the last 10 years and it appears, even though she has had numerous laser treatments & wide excisions, the Paget's has gone deeper into the flesh, and she may now require radiation and chemo.

Do you have any information about who may have treated others with this disease or what types of treatment would be recommended in a case such as this.

Thank you for any help you can give me!!  
Regards,
DP


Answer
Kenneth R. Blank, MD, OncoLink Editorial Assistant, responds:

Dear DP:
Thank you for your question.

Paget's disease of the anal margin and vulva is an intraepithelial adenocarcinoma characterized by the presence of Paget's cells—large vacuolated cells positive for periodic acid-Schiff. Paget's disease of the anus and vulva is often treated by wide local excision but extensive disease may require radiation for adequate local control. Recurrence of the disease can occur and may necessitate more aggressive therapy. 15-20% of cases are associated with an underlying carcinoma requiring careful clinically and radiographic examinations to rule-out this possibility. Information on paget's disease of the anus and vulva can be found in the following OncoLink sections:

Vulvar Cancer
Anal Cancer

Sincerely,
Kenneth R. Blank, MD



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