Extramammary Paget Disease



Extramammary Paget Disease


Elsa F. Velazquez, MD










Large pale-staining neoplastic cells extensively infiltrate all levels of a hyperplastic epidermis in EMPD. There is a chronic inflammatory infiltrate in the upper dermis.






Cytokeratin 7 immunohistochemical stain strongly highlights the neoplastic cells throughout the epidermis. The use of CK7 may also assist in defining the margins of the disease.


TERMINOLOGY


Abbreviations



  • Extramammary Paget disease (EMPD)


Definitions



  • Adenocarcinoma involving epidermis and commonly extending to epithelium of eccrine glands &/or hair follicles (adenocarcinoma in situ)


  • May involve dermis in minority of cases


ETIOLOGY/PATHOGENESIS


Pathogenesis



  • Uncertain in most cases


  • Cases limited to epidermis are postulated to originate from intraepidermal portion of sweat glands or from intraepidermal stem cells


  • Heterogeneous condition; can be classified as primary (cutaneous origin) and secondary (extracutaneous origin)



    • Rarely, primary EMPD is associated with underlying sweat gland adenocarcinoma (usually apocrine)


    • Secondary EMPD may represent extension from urethral, bladder, anal/rectal, prostatic, or endocervical carcinoma



      • Incidence of association with internal carcinoma is about 15%


      • Especially true in perianal EMPD, which is associated with rectal carcinoma in 1/3 of cases


CLINICAL ISSUES


Epidemiology



  • Age



    • 6th-8th decade


  • Gender



    • Female predominance


Site



  • Skin regions rich in apocrine glands



    • Most common sites are vulva and perianal region


    • Other sites include scrotum, perineum, penis, axilla, umbilicus


    • Exceptional sites include eyelid (accompanying carcinoma of Moll gland), external auditory meatus (associated with ceruminous carcinoma), face, scalp, chest, abdomen, and extremities


Presentation



  • Circinate or annular moist erythematous scaly patches or plaques


  • Gray-white eczematous patches are also common


  • Hypopigmented macules, ulceration, crusting, or palpable tumor may be present


  • Pruritus is frequent


  • Bleeding, oozing, tenderness, or burning sensation can occur


  • EMPD may mimic eczema


  • Lesions may be multifocal


Treatment



  • Complete surgical excision is treatment of choice for primary EMPD


  • Treatment in secondary lesions will depend on associated carcinoma


Prognosis



  • Primary EMPD confined to epidermis and epithelium of adnexal structures (in situ carcinoma) has good prognosis when completely excised


  • Patients require long-term follow-up because of multifocal nature of condition and high recurrence rate


  • Dermal invasion is associated with worse prognosis


  • Prognosis in secondary Paget disease is related to underlying carcinoma and is usually poor


Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Extramammary Paget Disease
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