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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 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
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
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
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
Macroscopic appearance • chronic eczema-like rash of the perianal skin • Erythematous plaque, may be ulcerative, crusty or papillary • Anal itching & burning sensation with bleeding
Microscopic appearance • Paget’s cell • Large vacuolated cell with basophilic cytoplasm • Arranged individually or in next at basal layer of epidermis
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
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
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.
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.
Treatment • Abdominoperineal Resection (APR) – for invasive lesion or disease with synchronous anorectal malignancy
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
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
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.
