Abstract
Background
Extramammary Paget’s disease (EMPD) is a rare disease in Chinese males. Our objectives were to examine the results of our experience, to correlate them with the published literature, and thus to formulate improved management guidelines.
Methods
This was a retrospective analysis of all male patients with EMPD treated at our institution. We reviewed the patients and their case notes for data such as presenting symptoms, delay to diagnosis, incidence of concurrent malignancies, treatments given, and a variety of outcome measures, including the number of patients with recurrent or residual disease.
Results
The overall recurrence rate was 35%. The risk of recurrence was reduced if intraoperative frozen section analysis was used (25%) compared to simple wide local excision (60%). Wide local excision failed to excise the disease completely in 38%. There were no associated internal malignancies and no disease-related deaths in our sample.
Conclusions
We recommend intraoperative frozen sections to reduce the risk of incomplete resection and to reduce the rate of recurrence. In our population, screening for internal malignancies can be restricted to select patients, e.g., those with perianal disease, those with invasive disease, and young patients.
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References
Pitman GH, McCarthy JG, Perzin KH, et al. (1982) Extramammary Paget’s disease. Plast Reconstr Surg 69:238–244
Zollo JD, Zeitouni NC (2000) The Roswell Park Cancer Institute experience with extramammary Paget’s disease. Br J Dermatol 142:59–42
Besa P, Rich TA, Delclos L, et al. (1992) Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys 24:73–78
van Randenburgh H, Paul R, Nahrig J, et al. (2002) Extramammary Paget’s disease of the penis and scrotum. J Urol 168:2540–2541
Park S, Grossfeld GD, McAninch JW, et al. (2001) Extramammary Paget’s disease of the penis and scrotum: excision, reconstruction and evaluation of occult malignancy. J Urol 16:2112–2117
Bansal D, Bowman CA (2004) Extramammary Paget’s disease masquerading as lichen sclerosus. Int J STD AIDS 15:141–142
De Blois M, Patterson JW, Hunter SB (1984) Extramammary Paget’s disease. Arch Pathol Lab Med 108:713–716
Jones RE, Austin C, Ackerman AB (1979) Extramammary Paget’s disease: Arising in knee region in association with sweat gland carcinoma. Am J Dermatopathol 1:101–132
Urabe A, Matsukuma A, Shimizu N, et al. (1990) Extramammary Paget’s disease. J Cutan Pathol 17:257–265
Koss LG, Ladinsky S, Brockunier A (1968) Paget’s disease of the vulva: report of ten cases. Obstet Gynecol 31:513–525
Hamm H, Vroom TM, Czarnetzki BM (1986) Extramammary Paget’s cells: Further evidence of sweat gland derivation. J Am Acad Dermatol 15:1275–1281
Helwig EB, Graham JH (1963) Anogenital (extramammary) Paget’s disease: a clinicopathological study. Cancer 16:387–403
Merot Y, Mazoujian G, Pinkus G, et al. (1985) Extramammary Paget’s disease of the perianal and perineal regions. Arch Dermatol 121:750–752
Lloyd J, Flanagan AM (2000) Mammary and extramammary Paget’s disease. J Clin Pathol 53:742–749
Smith AA, Silver TM (2003) Zirconyl haematoxylin vs alcian blue staining in a case of Extramammary Paget’s disease. J Cutan Pathol 30:401–404
Anthony PP, Freeman K, Warin AP (1986) Extramammary Paget’s disease. Clin Exp Dermatol 11:387–395
Brown RS, McCormack M, Lankester KJ et al. (2000) Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget’s disease: response to topical 5-fluorouracil. Cutis 66:454
Ramalingam P, Hart WR, Goldblum JR (2001) Cytokeratin subset immunostaining in rectal adenocarcinoma and normal anal glands. Arch Pathol Lab Med 125:1074–1077
Ogawa T, Nagashima Y, Wada H, et al. (2005) Extramammary Paget’s disease: analysis of growth signal pathway for human epidermal growth factor receptor 2 protein. Human Pathol 36:1273–1280
Reich O, Liegl B, Tamussino K, et al. (2005) P185 (HER 2) overexpression and HER 2 oncogene amplication in recurrent vulvar Paget’s disease. Mod Pathol 18:354–357
Kuivanen T, Tanskanen M, Jahkola T, et al. (2004) Matrilysin-1 (MMP-7) and MMP-19 are expressed by Paget’s cells in extramammary Paget’s disease. J Cutan Pathol 31:483–491
Kuan SF, Montag AG, Hart J, et al. (2002) Differential expression of mucin genes in mammary and extramammary Paget’s disease. Am J Surg Pathol 26:617–623
Ojeda VJ, Heenan PJ, Watson SH (1987) Paget’s disease of the groin associated with adenocarcinoma of the urinary bladder. J Cutan Pathol 14:227–231
Salamania J, Benito A, Garcia-Penalver C, et al. (2004) Paget’s disease of the glans penis secondary to transitional cell carcinoma of the bladder: a report of 2 cases and a review of the literature. J Cutan Pathol 31:341–345
Young DC, Nam HC, Young SP, et al. (2005) Lymphovascular and marginal invasion as useful prognostic indicators and the role of c-erbB-2 in patients with male extramammary Paget’s disease: a study of 31 patients. J Urol 174:561–565
Chang YT, Liu HN, Wong CK (1996) Extramammary Paget’s disease: A report of 22 cases in Chinese males. J Dermatol 23:320
Chanda JJ (1985) Extramammary Paget’s disease: prognosis and relationship to internal malignancy J Am Acad Dermatol 13:1009–1014
Allan SJ, McLaren K, Aldridge RD (1998) Paget’s disease of the scrotum: a case exhibiting positive prostate-specific antigen staining and associated prostatic adenocarcinoma. Br J Dermatol 138:689–691
Archer B, Lonback JB, MacDonald DM (1998) Spontaneous regression of perianal extramammary Paget’s disease after partial surgical excision. Arch Dermatol 123:379–382
Gunn RA, Gallager HS (1980) Vulvar Paget’s disease: a topographic study. Cancer 46(3):590–594
Fanning J, Lambert HC, Hale TM, et al. (1999) Paget’s disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget’s disease, and recurrence after surgical excision. Am J Obstet Gynecol 180:24–27
DeVita VT Jr, Hellman S, Rosenberg SA (1997) Cancers of the skin. Cancer, principles and practice of oncology. New York, Lippincott Raven, pp 1565–1566
Hendi A, Brodland DB, Zitelli JA (2004) Extramammary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 51:767–773
Coldiron BM, Goldsmith BA, Robinson JK (1991) Surgical treatment of extramammary Paget’s disease. Cancer 67:933–938
Misas JE, Cold CJ, Wesley Hall F (1991) Vulvar Paget disease: fluorescein-aided visualization of margins. Obstet Gynaecol 77:156–160
Gibson JR, Baker H, Pegum JS, et al. (1983) Multifocal extramammary Paget’s disease. J R Soc Med 76:426–427
Lai YL, Yang WG, Tsang PK, et al. (2003) Penoscrotal extamammary Paget’s disease: A review of 33 cases in a 20 year experience. Plast Reconstr Surg 112:1017–1023
Molinie V, Paniel BJ, Lessana-Leibowitch M, et al. (1993) Paget’s disease of the vulva. 36 cases. Ann Dermatol Venereol 120:522–527
Shieh S, Dee AS, Cheney RT, et al. (2002) Photodynamic therapy for the treatment of extramammary Paget’s disease. Br J Dermatol 146:1000–1005
Burrows NP, Jones DH, Hudson PM, et al. (1995) Treatment of extramammary Paget’s disease by radiotherapy. Br J Dermatol 132:970–972
Xu H, Li N, Li W (1996) Observations on signet cell invasion and metastasis in extra-mammary Paget’s disease. Chin J Pathol 25:294–295
Mohs FE, Blanchard L (1979) Microscopically controlled surgery for extramammary Paget’s disease. Arch Dermatol 115:706–708
Shutze WP, Gleysteen JJ (1990) Perianal Paget’s disease: classification and a review of management. Report of two cases. Dis Colon Rectum 33:502–507
Ray B, Whitmore WF (1977) Experience with carcinoma of the scrotum. J Urol 117(6):741–745
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Chiu, T.W., Wong, P.S.Y., Ahmed, K. et al. Extramammary Paget’s Disease in Chinese Males: A 21-year Experience. World J Surg 31, 1941–1946 (2007). https://doi.org/10.1007/s00268-007-9189-x
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DOI: https://doi.org/10.1007/s00268-007-9189-x