CHOW SHEUNG MINGQUEEN ELIZABETH HOSPITAL

Perianal Paget’s disease

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.

Prone Jack-knife Position

Before excision…

After excision…

Specimen

Applying stay stitches…

Planning of advancement flap…

Elevation of flaps…

Advancing of flap…

Formation of mucocutaneous junction…

Post-operative…(followed by transverse colostomy)

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.

Q&A…

Perianal Paget’s disease