Abstract
Background
Extramammary Paget’s disease is a rare cutaneous malignancy, which occurring frequently in the elderly and affecting primarily the genital, perianal, and axillary regions. Unfortunately, surgical and ablative treatment modalities for extramammary Paget’s disease have a high recurrence rate and are often associated with significant morbidity.
Methods
We present a case of a 51-year-old Caucasian man with Paget’s disease of the right groin. The isolated localization of Paget’s disease in the groin is extremely rare. Wide local excision of the tumor is currently the standard of care treatment. All publications found in the literature reporting this rare entity were reviewed for the purpose of further delineating a treatment regimen for this rare pathology.
Results
Six years after surgery, the patient remains disease free with a very acceptable aesthetic result. Currently, only 11 cases of Paget’s disease have been reported with an isolated localization to the groin.
Conclusions
Paget’s disease of the groin has an extremely low incidence. Various treatments are described in the literature, early wide local excision may be the treatment of choice.
Similar content being viewed by others
References
Crocker HR. Paget’s disease, affecting the scrotum and penis. Trans Pathol Soc (Lond) 1889;40:187–91
Balducci L, Crawford ED, Smith GF, Lambuth B, McGehee R, Hardy C. Extramammary Paget’s disease: an annotated review. Cancer Invest 1988;6:293–303
Yugueros P, Keeney GL, Bite U. Paget’s disease of the groin: report of seven cases. Plast Reconstr Surg 1997;100:336–9
Zampogna JC, Flowers FP, Roth WI, Hassenein AM. Treatment of primary limited cutaneous extramammary Paget’s disease with topical imiquimod monotherapy: two case reports. J Am Acad Dermatol 2002;47(4 Suppl):S229–35
Zollo JD, Zeitouni NC. The Roswell Park Cancer Institute experience with extramammary Paget’s disease. Br J Dermatol 2000;142:59–65
Pierie JP, Choudry U, Muzikansky A, Finkelstein D, Ott M Prognosis and management of extramammary Paget's disease and the associationwith secondary malignancies. J Am Coll Surg 2003;196(1) :45–50
Coldiron BM, Goldsmith BA, Robinson JK. Surgical treatment of extramammary Paget’s disease. A report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision. Cancer 1991;67:933–8
Ojeda VJ, Heenan PJ, Watson SH. Paget’s disease of the groin associated with adenocarcinoma of the urinary bladder. J Cutan Pathol 1987;14:227–31
Fu M, Gao S, Wang P. 19 cases of Paget’s disease. Zhonghua Wai Ke Za Zhi 1999;37:429–31
Pitman GH, McCarthy JG, Perzin KH, Herter FP. Extramammary Paget’s disease. Plast Reconstr Surg 1982;69:238–44
Burrows NP, Jones DH, Hudson PM, Pye RJ. Treatment of extramammary Paget’s disease by radiotherapy. Br J Dermatol 1995;132:970–2
Shieh S, Dee AS, Cheney RT, Frawley NP, Zeitouni NC, Oseroff AR. Photodynamic therapy for the treatment of extramammary Paget’s disease. Br J Dermatol 2002;146:1000–5
Van Hamme C, Marot L, Dachelet C, Dumont M, Salamon E, Lachapelle JM. Paget’s extramammary disease of the axillae and perineum. Ann Dermatol Venereol 2002;129:717–9
Chanda JJ. Extramammary Paget’s disease. Prognosis and relationship to internal malignancy. J Am Acad Dermatol 1985;13:1009–14
Hartley EL, Nambisan RN, Rao U, Karakousis CP. Extramammary Paget disease of the inguinoscrotal area. N Y State J Med 1988;88:546–8
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 1997;4:475–80
Chandawarkar RY, Ricchuiti D, Amjad I, Marsico RE, Wells MD. Extramammary Paget’s disease of the perineum: avoiding pitfalls in diagnosis and management. Can J Plast Surg 2003;11:205–8
Tsutsumida A, Yamamoto Y, Minakawa H, Yoshida T, Kokubu I, Sugihara T. Indications for lymph node dissection in the treatment of extramammary Paget’s disease. Dermatol Surg 2003;29:21–4
Park S, Grossfeld GD, McAninch JW, Santucci R. Extramammary Paget's disease of the penis and scrotum: excision, reconstruction and evaluation of occult malignancy. J Urol 2001; 166(6):2112–2116; discussion 2117
Yang WJ, Kim DS, Im YJ, Cho KS, Rha KH, Cho NH, Choi YD. Extramammary Paget's disease of penis and scrotum. Urology 2005; 65(5):972–975
Dufourmentel CL. La fermeture des pertes des substance cutanée limitées. Le “lambeau de rotation en L pour losange,” dit ‘LLL.’ Ann Chir Plast Esthet 1962;7:61–6
Servant JM, Dufourmentel CL. (1984) Treatment of facial lesions. In: Goldwyn, eds. The Unfavorable Result in Plastic Surgery. Boston: Brown & Co, 1(19):348–50
Coit DG, Peters M, Brennan MF. A prospective randomized trial of perioperative cefazolin treatment in axillary and groin dissection. Arch Surg 1991;126:1366–71
Tonuchi H, Ohmori Y, Kobayashi M, et al. Operative morbidity associated with groin dissections. Surg Today 2004;34:413–8
Acknowledgments
The authors thank Professor A. Mylonas, MD, Ph.D for gastrointestinal investigation and K. Syllas, MD, Pathologist for the microphotograph.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Dionyssopoulos, A., Mandekou-Lefaki, I., Vakirlis, E. et al. Extramammary Paget’s Disease: Isolated Localization on the Groin—Wide Local Excision With Immediate Reconstruction. Ann Surg Oncol 13, 1235–1240 (2006). https://doi.org/10.1245/s10434-006-9003-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1245/s10434-006-9003-2