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Perianal Paget’s disease

Perianal Paget’s disease. CHOW SHEUNG MING QUEEN ELIZABETH HOSPITAL. Sir James Paget (1814-1899). British Surgeon & Pathologist One of the founder of modern medical pathology “Paget’s disease” Paget’s disease of bone Paget’s disease of nipple Extramammary Paget’s disease.

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Perianal Paget’s disease

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  1. Perianal Paget’s disease CHOW SHEUNG MING QUEEN ELIZABETH HOSPITAL

  2. Sir James Paget (1814-1899) • British Surgeon & Pathologist • One of the founder of modern medical pathology • “Paget’s disease” • Paget’s disease of bone • Paget’s disease of nipple • Extramammary Paget’s disease

  3. Extramammary Paget’s disease • Radcliffe Crocker recognised & reported it as distinct disease entity in 1889 • Uncommon neoplastic condition • Apocrine gland bearing area • Vulva • Scrotum • Groin • Perineum • Perianal area

  4. Perianal Paget’s disease • First reported by Darier & Coulillaud in 1893 • About 300 cases were reported¶,§ • Male & female are equally predominant¥ • Mean age of onset: 59-64 ¶ Berardi RS, Lee S, Chen HP. Perianal extramammary Paget’s disease. Surg Gynecol Obstet 1988;167:359–66 § Minicozzi A. et al., Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal Dis 2010,Jan,01;25(1):1-7 ¥ McCarter et al., Long-Term Outcome of Perianal Paget’s Disease. Dis Colon Rectum. May 2003; 46(5):612-616

  5. Pathogenesis • 2 different categories of pathological process with common clinical & histological features§ • Intra-epithelial adenocarcinoma arising from apocrine sweat gland at perianal region • Extends into contiguous epithelium of hair follicles & eccrine sweat glands • Extends into dermis (invasive, from where it metastasizes) • Adenocarcinoma begins at anal canal/rectum, followed by spreading into contiguous epidermis § Goldblum JR, Hart WR. Perianal Paget’s disease. A histologic and immunohistochemical study with and without associated rectal adenocarcinoma. Am J Surg Pathol 1998; 22: 170–9

  6. Macroscopic appearance • chronic eczema-like rash of the perianal skin • Erythematous plaque, may be ulcerative, crusty or papillary • Anal itching & burning sensation with bleeding

  7. Microscopic appearance • Paget’s cell • Large vacuolated cell with basophilic cytoplasm • Arranged individually or in next at basal layer of epidermis

  8. Workup • Biopsy of the lesion: to identify Paget’s cell • Other differential diagnosis: contact dermatitis, tinea cruris, Bowen’s disease, Basal cell carcinoma • Multiple punch biopsy : to map the extend of involvement • at 1cm from edge of lesion in 4 quadrants including dentate line, anal verge & perineum¶ • Paget’s cell may extend beyond the gross visible edge of lesion ¶ Beck D. and Fazio V. Perianal Paget’s Disease. Dis Colon Rectum. 1987; 30:263-6

  9. Workup • Colonoscopy: to identify associated anorectal malignancy • ~25% of patients with perianal Paget’s disease got synchronous anorectal malignancy§ • Assessment of genitourinary tract: necessity guided by involvement of related region § Minicozzi A. et al., Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal Dis 2010,Jan,01;25(1):1-7

  10. Prognosis • Survival after adequate surgical treatment should be similar as that of normal age-matched population§ • Progression to invasive carcinoma: 100% (after local excision) vs 12.5% (after wide local excision ie>1cm resection margin)¶ • Recurrence rate after wide local excision 30%-61%§ • Survival for patients with invasive disease at presentation would be much groomy. § Sarmiento JM, Wolff BG, Burgart LJ, et al: Paget's disease of the perianal region -- an aggressive disease? Dis Colon Rectum 1997; 40:1187-1194 ¶ Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC, Milsom JW. Long-term outcome of patients with perianal Paget's disease. Ann Surg Oncol. Sep 1997;4(6):475-80.

  11. Treatment • Wide local excision – Standard for non-invasive lesion¥ • Aim at preserving anal function while archiving local control • Resection margin: 1cm₤ • Frozen section of the margin • Besa et al., and Pierie et al. found +ve resection margin in 53% & 56% of patient with Perianal Paget’s disease§,¶ • Covering enterostomy • Skin coverage with flap or grafting ¥ Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Paget’s disease of the perianal region—an aggressive disease? Dis Colon Rectum 1997;40:1187–94 ₤ Minicozzi A. et al., Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal Dis 2010,Jan,01;25(1):1-7 ¶ Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT. Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 1992;24:73–8. § Pierie JP, Choudry U, Muzikansky A, Finkelstein DM, Ott MJ. Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 2003; 196: 45–50.

  12. Prone Jack-knife Position

  13. Before excision…

  14. After excision…

  15. Specimen

  16. Applying stay stitches…

  17. Planning of advancement flap…

  18. Elevation of flaps…

  19. Advancing of flap…

  20. Formation of mucocutaneous junction…

  21. Post-operative…(followed by transverse colostomy)

  22. Treatment • Abdominoperineal Resection (APR) – for invasive lesion or disease with synchronous anorectal malignancy

  23. Chemotherapy & radiotherapy • External radiation as primary treatment • Associated with relative high rate of local complication¥ & high recurrence rate¶ • Adjuvant chemoradiation • Not associated with local control or improved survival§ ¶ Jensen SL, Sjolin KE, Shokouh-Amiri MH, et al. Paget’s disease of the anal margin. Br J Surg 1988;75:1089–1092. ¥ Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT. Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 1992;24:73–8. § McCarter et al., Long-Term Outcome of Perianal Paget’s Disease. Dis Colon Rectum. May 2003; 46(5):612-616

  24. Bring home message • Perianal Paget’s disease is a rare intraepithelial neoplasm involving perianal region • Diagnosis required high index of suspicion, especially lack of response to classical therapy • Non-invasive disease could be treated by wide local excision, while more advanced disease required more radical treatment • The prognosis of non-invasive disease would be excellent

  25. Reference • Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholomew Hosp Res London 1874;10:87-9. • Crocker HR. Paget’s disease affecting the scrotum and penis. Trans Pathol Soc Lond 1888–1889; 40: 187–91. • Darier J, Coulillaud P. Sur un case de maladie de Paget de la region perineo-anal et scrotale. Ann Dermatol Syphiligraphie 1893;4:33. • Berardi RS, Lee S, Chen HP. Perianal extramammary Paget’s disease. Surg Gynecol Obstet 1988;167:359–66 • Minicozzi A. et al., Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal Dis 2010,Jan,01;25(1):1-7 • McCarter et al., Long-Term Outcome of Perianal Paget’s Disease. Dis Colon Rectum. May 2003; 46(5):612-616 • Goldblum JR, Hart WR. Perianal Paget’s disease. A histologic and immunohistochemical study with and without associated rectal adenocarcinoma. Am J Surg Pathol 1998; 22: 170–9 • Beck D. and Fazio V. Perianal Paget’s Disease. Dis Colon Rectum. 1987; 30:263-6 • Goldman S, Ihre T, Lagerstedt U, Svensson C. Perianal Paget's disease: report of five cases. Int J Colorect Dis 1992;7:167-9. • Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT. Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 1992;24:73–8. • Pierie JP, Choudry U, Muzikansky A, Finkelstein DM, Ott MJ. Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 2003; 196: 45–50. • Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Paget’s disease of the perianal region—an aggressive disease? Dis Colon Rectum 1997;40:1187–94 • Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC, Milsom JW. Long-term outcome of patients with perianal Paget's disease. Ann Surg Oncol. Sep 1997;4(6):475-80. • Shieh S et al. Photodynamic therapy for the extramammary Paget’s disease. Br J Derm 2002;146:1000-5 • Jensen SL, Sjolin KE, Shokouh-Amiri MH, et al. Paget’s disease of the anal margin. Br J Surg 1988;75:1089–1092.

  26. Perianal Paget’s disease Q&A…

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