chow sheung ming queen elizabeth hospital perianal paget’s disease
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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 conditionApocrine gland bearing area
Vulva Scrotum Groin Perineum Perianal area
Perianal Paget’s disease
First reported by Darier & Coulillaud in 1893About 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
1. Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholomew Hosp Res London 1874;10:87-9.
2. Crocker HR. Paget’s disease affecting the scrotum and penis. Trans Pathol Soc Lond 1888–1889; 40: 187–91.3. Darier J, Coulillaud P. Sur un case de maladie de Paget de la region perineo-anal et scrotale. Ann Dermatol
Syphiligraphie 1893;4:33.4. Berardi RS, Lee S, Chen HP. Perianal extramammary Paget’s disease. Surg Gynecol Obstet 1988;167:359–665. Minicozzi A. et al., Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal
Dis 2010,Jan,01;25(1):1-76. McCarter et al., Long-Term Outcome of Perianal Paget’s Disease. Dis Colon Rectum. May 2003; 46(5):612-
6167. 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: 170–98. Beck D. and Fazio V. Perianal Paget’s Disease. Dis Colon Rectum. 1987; 30:263-69. Goldman S, Ihre T, Lagerstedt U, Svensson C. Perianal Paget's disease: report of five cases. Int J Colorect Dis
1992;7:167-9.10. 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. 11. 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. Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM. Paget’s disease of the perianal region—an
aggressive disease? Dis Colon Rectum 1997;40:1187–9413. 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):475-80.14. Shieh S et al. Photodynamic therapy for the extramammary Paget’s disease. Br J Derm 2002;146:1000-515. Jensen SL, Sjolin KE, Shokouh-Amiri MH, et al. Paget’s disease of the anal margin. Br J Surg 1988;75:1089–
1092.