Extramammary Paget's/Paget's Disease
Thursday, February 11, 2010
The diagnosis of Paget's disease or extramammary Paget's is made
when Paget cells are identified in histopathologic specimens from
involved skin. The term Paget's disease is used when the cutaneous
lesion is identified involving the nipple of the breast, whereas
extramammary Paget's is used when the skin lesion is identified in
an anogenital location.
- Evaluate the patient for an underlying carcinoma of the breast
(Paget's) or sweat gland, lower genitourinary (GU) tract, or lower
gastrointestinal (GI) tract (extramammary Paget's). Mammary Paget's
is almost always associated with an underlying carcinoma. The data
on extramammary Paget's are less clear, but between 25% and 50%
have an associated carcinoma. A careful lymph node evaluation is
essential. Immunoperoxidase markers on paraffin-fixed tissue can be
used to identify the source of the primary carcinoma, or confirm
that the lesion arose in the skin locally.
- Refer patients with Paget's disease of the breast to a general
surgeon with experience in the management of breast cancer.
- If underlying carcinoma is found in a case of extramammary
Paget's referral to an appropriate surgeon (ie, GU tract to a
urologist, female genital tract to a gynecologic cancer specialist,
and GI tract to a general surgeon) is indicated.
- Extramammary Paget's lesions, if small and not associated with
underlying carcinoma, may be totally excised. Careful evaluation of
the margins is essential. Some dermatologic micrographic surgeons
believe that they can remove these lesions and spare tissue by
tracing the involvement.
- If surgery is not possible due to the size of the lesion or
patient's overall health, a trial of imiquimod 5% cream applied
daily (or as maximally as tolerated) should be considered. Apply
the cream 2 cm beyond the visible lesion. After the lesion has
clinically resolved, a biopsy to confirm cure is suggested.
- If imiquimod alone is ineffective, imiquimod plus tretinoin
cream plus 5% 5-fluoruracil cream can be used.
- Photodynamic therapy can be considered in patients who are not
candidates or if the above treatments fail.
- Careful follow-up is essential, as the recurrence rate is high
following all forms of therapy.
- Failure to diagnose a recalcitrant, apparently eczematous,
lesion involving the nipple or anogenital area as Paget's disease
or extramammary Paget's is possible.
- Failure to evaluate and follow up regularly for underlying
- All forms of therapy are associated with high recurrence rates.
Therapy should be determined in consultation with appropriate
surgical specialists in a Tumor Board setting.