Clinicopathologic analysis of extramammary Paget’s disease

Purpose: To retrospectively analyze the clinicopathologic characteristics of patients with extramammary Paget’s disease who were surgically treated in a single institution. Method: The charts of 14 patients with extramammary Paget’s disease were retrospectively reviewed, and the clinicopathologic data were collected and analyzed. Results: From January 1990 to July 2009, 14 patients were treated at our institution. Most patients (11/14 patients) had delayed diagnosis. Two patients (14.3%) had associated malignant neoplasms. Eight of 14 patients (57.1%) had positive surgical margins; of these patients, five patients had no evidence of recurrence. In the six patients with negative surgical margins, two patients (33.3%) developed recurrence. Conclusions: The diagnosis of extramammary Paget’s disease is commonly delayed. Because of the possible association with other malignancies before or after the diagnosis of extramammary Paget’s disease, thorough examinations are recommended. Disease recurrence is common regardless of the surgical margin status, so long-term monitoring of patients is recommended.

Extramammary Paget’s disease; Retrospective; Clinicopathologic analysis

[1] Paget J.: “On disease of the mammary areola preceding cancer of the mammary gland”. St. Barth Hospital Rep., 1874, 10, 87.

[2] Dubreihl W.: “Paget’s disease of the vulva”. Br. J. Dermatol., 1901, 13, 407.

[3] Curtin J.P., Rubin S.C., Jones W.B., Hoskins W.J., Lewis J.L.: “Paget’s disease of vulva”. Gynecol. Oncol., 1990, 39, 374.

[4] Bilings S.D., Roth S.C.: “Pseudoinvasive, nodular extramammary Paget’s disease of the vulva”. Arch. Pathol. Lab. Med., 1998, 122, 471.

[5] Sieling S., Elferink M.A., van Dijck J.A., Pierie J.P., Blokx W.A.: “Epidemiology and treatment of extramamary Paget’s diseasein Netherlands”. Eur. J. Surg. Oncol., 2007, 33, 951.

[6] Black D., Tornos C., Sosclow R.A., Awtrey C.S., Barakat R.R., Chi D. S.: “The outcomes of patients with positive margins after excision for intraepithelial Paget’s disease of vulva”. Gynecol. Oncol., 2007, 104, 547.

[7] Pierie J.P., Choudry U., Muzikansky A., Finkelstein D.M., Ott

M. J.: “Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies”. J. Am. Coll. Surg., 2003, 196, 45.

[8] Jensen S.L., Sjolin K.E., Shokouh-Amiri M.H., Hagen K., Harling H.: “Paget’s disease of the anal margin”. Br. J. Surg., 1988, 75, 1089.

[9] Sarmiento J.M., Wolff B.G., Burgart L.J., Frizelle F.A., Istrup

D. M.: “Paget’s disease of the perianal region - An aggressive disease?” Dis. Colon Rectum, 1997, 40, 1187.

[10] Yoon S.N., Park I.J., Kim H.C., Yu C.S., Lee M.W., Koh J.K., et al.: “Extramammary Paget’s disease in Korea: its association withgastrointestinal neoplasms“. Int. J. Colorectal Dis., 2008, 23, 1125.

[11] Hatta N., Yamada M., Hirano T., Fujimoto A., Morita R.: “Extramammary Paget’s disease: treatment, prognostic factors and outcome in 76 patients”. Br. J. Dermatol., 2008, 158, 313.

[12] Parker L.P., Parker J.R., Bodurka-Beavers D., Deavers M., Bevers

M. W., Shen-Gunther J. et al.: “Paget’s disease of the vulva: pathology, pattern of involvement, and prognosis”. Gynecol. Oncol., 2000, 77, 183.

[13] Victoria S., Emma J.D., John D.H.: “Extramammary Paget’s disease”. BJOG., 2004, 112, 273.

[14] Bakalianou K., Salakos N., Lavazzo C., Paltoglou G., Papadias K., Gregoriou O. et al.: “Paget’s disease of the vulva. A ten-year experience”. Eur. J. Gynaecol. Oncol., 2008, 29, 368.

[15] Chanda J.J.: “Extramammary Paget’s disease: prognosis and relationship to internal malignancy”. J. Am. Acad. Dermatol., 1985, 13, 1009.

[16] Petkovic S., Jeremic K., Vidakovic S., Jeremic J., Lazovic G.: “Paget’s disease of the vulva - a review of our experience”. Eur. J. Gynaecol. Oncol., 2006, 27, 611.

[17] Balducci L., Crawford E.D., Smith G.F., Lambuth B., McGehee R., Hardy C.: “Extramammary Paget’s disease: an annotated review”. Cancer Invest., 1988, 6, 293.

[18] Besa P., Rich T.A., Delclos L., Edwards C.L., Ota D.M., Wharton J. T.: “Extramammary Paget’s disease of the perineal skin: role of radiotherapy”. Int. J. Radiat. Oncol. Biol. Phys., 1992, 24, 73.

[19] Fanning J., Lambert H.C., Hale T.M., Morris P.C., Schuerch C.: “Paget’s disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget’s disease, and recurrence after surgical excision”. Am. J. Obstet. Gynecol., 1999, 180, 24.

[20] Goldblum J.R., Hart W.R.: “Perianal Paget’s disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma”. Am. J. Surg. Pathol., 1998, 22, 170.

[21] Marchesa P., Fazio V.W., Oliart S., Goldblum J.R., Lavery I.C., Milsom J.W.: “Long-term outcome of patients with perianal Paget’s disease”. Ann. Surg. Oncol., 1997, 4, 475.

[22] Gunn R.A., Gallager H.S.: “Vulvar Paget’s disease: a topographic study”. Cancer, 1980, 46, 590.

[23] Adamsons Jr. K., Resfield D.: “Observations on intradermal migration of Paget cells”. Am. J. Obstet. Gynecol., 1964, 90, 1274.

[24] Kodama S., Kaneko T., Saito M., Yoshiya N., Honma S., Tanaka K.: “A clinicopathologic study of 30 patients with Paget’s disease of the vulva”. Gynecol. Oncol., 1995, 56, 63.

[25] Fishman D.A., Chambers S.K., Schwartz P.E., Kohorn E.I., Chambers J.T.: “Extramammary Paget’s disease of the vulva”. Gynecol. Oncol., 1995, 56, 266.