Extramammary Paget’s Disease

Herrel LA, Weiss AD, Goodman M, Johnson TV, Osunkoya AO, Delman KA, Master VA. Extramammary Paget’s disease in males: survival outcomes in 495 patients. Ann Surg Oncol. 2015 May;22(5):1625-30.

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Results: Incidence rates of EMPD in men have been increasing with an annual percent change of +3.2 % (p < .0002) since 1978. Incidence of EMPD in blacks was nearly four times lower (p = .0003) and in Asians/Pacific islanders four times higher (p < .0001), relative to whites. Overall survival among 495 patients was 60.2 % at 120 months post-diagnosis. On multivariate analysis, significant factors negatively impacting survival were primary site in the perianal region compared to penoscrotal and truncal lesions (both p < .001), age older than 75 years (p < .001), and presence of distant versus localized disease (p = .018). Survival did not differ by race or presence of additional cancer.

Conclusions: Survival in men with EMPD is lower among those with distant disease and primary tumors located in the perianal region. The reasons for increasing EMPD incidence over time and for the racial disparities in disease occurrence require further study.

Asel M, LeBoeuf NR. Extramammary Paget’s Disease. Hematol Oncol Clin North Am. 2019 Feb;33(1):73-85.

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  • In all patients with EMPD, a thorough review of systems, full skin, breast, abdominal, pelvic, and lymph node examination should be followed by complete diagnostic testing; this extends beyond standard age-appropriate malignancy screenings.
  • Although there is no standard approach to the required workup, studies suggested by Lam and Funaro, [3] in a comprehensive review from 2010 in addition to the above, include gynecologic evaluation (including colposcopy, Pap smear, and pelvic ultrasound), urologic evaluation (including cystoscopy with or without uroscan), colonoscopy, and, in the case of invasive EMPD, serum CEA.
  • Many use advanced imaging, such as computed tomography or PET scan.
  • An algorithm for cancer screening was more recently proposed, which calls for, in addition to history, examination and age-appropriate cancer screenings, and the following investigations: serial sectioning with staining to evaluate for invasive disease and underlying carcinoma, urine cytology and colonoscopy plus either prostate-specific antigen and digital rectal examination for men or Pap smear and mammography for women.
  • The algorithm also recommends lymph node evaluation when invasive EMPD or contiguous invasive malignancy is present.[4] (Adapted from p. 75)

Wollina U, et al. Surgical Treatment for Extramammary Paget’s Disease. Curr Treat Options Oncol. 2018 May 3;19(6):27.

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Extramammary Paget’s disease (EMPD) is a rare, slow growing non-melanoma skin cancer.Diagnosis is often significantly delayed, since clinical presentation may resemble common benign dermatoses. Treatment is characterized by high recurrence rates. This is in part due to ill-defined margins and by frequent development of satellites. Improvement of outcome needs a better preoperative planning with fluorescence diagnostics or scouting biopsies. Mohs micrographic surgery has some advantages for patients, such as reduced relapse rates and prolonged relapse-free survival. Improved identification of Paget cells in cryosections by immunostainings or alternatively the use of delayed Mohs with formalin-fixed material reduces the rate of false-negative results. Surgery remains the cornerstone of treatment.

More PubMed results on EMPD.


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