Extramammary Paget's disease secondary prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Secondary prevention strategies following extramammary Paget's disease include an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.[1]

Secondary Prevention

  • As some patients develop recurrences more than 15 years after initial treatment, follow up needs to be long term. Long-term follow up is necessary to exclude both local recurrence and the development of associated internal malignancies.[2][3]
  • It is suggested that follow up for perianal extramammary Paget's disease (EMPD) should involve an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.[1]
  • Because of multifocality of lesions, local recurrences are common even when the margins of resection are negative.
  • Vulval EMPD may be similarly followed up with regular inspection of the vulva, the use of punch biopsies to exclude invasive disease in any recurrent lesion and regular pelvic ultrasound scans and hysteroscopy.[4]
  • Some physicians recommend lifelong follow-up after radical treatment of perianal disease, and the control examinations include a complete physical examination, proctosigmoidoscopy, double-contrast bowel enema, and biopsy of the perianal region once a year.[5]
  • Patients with EMPD require follow-up examination every three months after surgery to assess recurrence. This routine follow-up should continue for at least 24 months, after which follow-up examinations may be done annually.

References

  1. 1.0 1.1 Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM (1997). "Paget's disease of the perianal region--an aggressive disease?". Dis Colon Rectum. 40 (10): 1187–94. PMID [ 9336114 [ Check |pmid= value (help).
  2. DiSaia PJ, Dorion GE, Cappuccini F, Carpenter PM (1995). "A report of two cases of recurrent Paget's disease of the vulva in a split-thickness graft and its possible pathogenesis-labeled "retrodissemination"". Gynecol Oncol. 57 (1): 109–12. doi:10.1006/gyno.1995.1106. PMID 7705692.
  3. Oster MW, Magun A, Herter FP, Wolff M (1979). "Colorectal carcinoma 15 years after the diagnosis of perianal Paget disease". J Surg Oncol. 12 (4): 379 84. PMID 230392.
  4. Jensen SL, Sjølin KE, Shokouh-Amiri MH, Hagen K, Harling H (1988). "Paget's disease of the anal margin". Br J Surg. 75 (11): 1089–92. PMID 2850073.
  5. Beck, David E.; Fazio, Victor W. (1987). "Perianal Pagetʼs disease". Diseases of the Colon & Rectum. 30 (4): 263–266. doi:10.1007/BF02556169. ISSN 0012-3706.


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