Perianal Paget's disease: presentation of six cases and literature review.
OBJECTIVE
Extramammary Paget's disease (EMPD) is frequently associated with adnexal or visceral synchronous or metachronous malignancies. Our purpose was to evaluate, retrospectively, the results obtained in six cases of EMPD and to review the literature.


METHODS
Six patients with the perianal Paget's disease had been treated in our division between March 1996 and December 2006. In three cases, the disease was confined in the epidermis; in one case, there was a microinvasion of the dermis, while in another one the dermis was infiltrated. The last case was associated to a low rectal adenocarcinoma. All patients underwent wide perianal excision and reconstruction with skin graft. We performed a transanal resection of the rectal adenocarcinoma. A review of the literature from 1990 to 2008 revealed 193 cases of perianal EMPD, 112 were intraepithelial/intradermal while 81 were associated with malignancies. Anorectal adenocarcinoma was already existing in two cases, synchronous in 48, and subsequent to diagnosis in 11.


RESULTS
In three cases, the disease recurred locally, but no patient developed metastatic spread. Five patients survived and are free of disease. The review of the literature allows a clear identification of the primitive EMPD and the form associated to anorectal adenocarcinoma and little information about cases associated with synchronous adnexal adenocarcinoma. The Paget's disease can relapse after radical surgery and has a capacity of metastatic spread.


CONCLUSIONS
Up to now, no clear guidelines have been established for the diagnosis of EMPD. The association with synchronous or metachronous carcinomas imposes a long-term follow-up with frequent clinical, radiological, and endoscopical controls.
Minicozzi
Annamaria
A
II Scuola di Specializzazione in Chirurgia Generale, Ospedale Civile Maggiore, Università degli Studi di Verona, Italy. annaminicozzi@hotmail.com
Borzellino
Giuseppe
G
Momo
Rostand
R
Steccanella
Francesca
F
Pitoni
Federica
F
de Manzoni
Giovanni
G
D000368:Aged; D000369:Aged, 80 and over; D001003:Anal Canal / Q000473:pathology*; D004817:Epidermis / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000473:pathology*
Acta Gastroenterol Belg. 2004 Apr-Jun;67(2):228-31
15285581
J Am Coll Surg. 2003 Jan;196(1):45-50
12517548
Dermatol Surg. 2004 Oct;30(10):1329-34
15458530
Am J Surg Pathol. 1998 Feb;22(2):170-9
9500217
Dis Colon Rectum. 1990 Jun;33(6):502-7
2161727
Am J Dermatopathol. 1996 Apr;18(2):118-23
8739985
Am J Dermatopathol. 1992 Oct;14(5):402-7
1384380
South Med J. 2000 Jul;93(7):713-5
10923963
Ann R Coll Surg Engl. 2004 Nov;86(6):W26-31
16749961
Br J Dermatol. 2000 Jan;142(1):59-65
10651695
J R Soc Med. 2001 Mar;94(3):137-8
11285798
Dis Colon Rectum. 2003 May;46(5):612-6
12792436
Pathol Int. 2004 Aug;54(8):630-5
15260855
G Chir. 2008 Nov-Dec;29(11-12):469-74
19068182
Dis Colon Rectum. 1996 Feb;39(2):227-31
8620793
Am Surg. 2007 Mar;73(3):307-9
17375797
Arch Pathol Lab Med. 1998 Dec;122(12):1077-81
9870855
Med Sci Monit. 2007 Jun;13(6):CS75-7
17534239
Dermatol Surg. 2003 Jul;29(7):723-7
12828695
Surg Today. 2006;36(7):666-9
16794808
Histopathology. 1997 Oct;31(4):384-5
9363457
Dermatology. 2005;210(4):315-8
15942218
J Eur Acad Dermatol Venereol. 2007 May;21(5):581-90
17447970
Int J Radiat Oncol Biol Phys. 1992;24(1):73-8
1324902
Surg Today. 1996;26(9):711-4
8883244
Acta Pathol Jpn. 1991 May;41(5):386-93
1651042
Am J Surg Pathol. 2001 Dec;25(12):1469-77
11717535
Colorectal Dis. 2004 May;6(3):206-9
15109389
Dermatol Surg. 1997 Nov;23(11):1032-4
9391559
J Eur Acad Dermatol Venereol. 2008 Apr;22(4):522-3
18363931
Histopathology. 2002 Aug;41(2):177-9
12147102
Am J Gastroenterol. 1990 Feb;85(2):199-202
2154090
J Plast Reconstr Aesthet Surg. 2007;60(11):1208-13
17409038
J Dermatolog Treat. 2006;17(3):167-71
16854759
J Cutan Pathol. 2002 Mar;29(3):185-9
11972718
Eur J Surg Oncol. 1993 Oct;19(5):469-73
8405484
J Cutan Med Surg. 2006 Jul-Aug;10(4):197-200
17234119
Clin Oncol (R Coll Radiol). 2002 Aug;14(4):272-84
12206637
Eur J Dermatol. 2003 Jan-Feb;13(1):93-4
12609793
Br J Dermatol. 2006 Feb;154(2):375-6
16433815
Dis Colon Rectum. 2003 Nov;46(11):1563-5
14605581
Dis Colon Rectum. 1997 Oct;40(10):1187-94
9336114
Cancer. 1991 Feb 15;67(4):933-8
1846769
BJOG. 2005 Mar;112(3):273-9
15713139
Ann Surg Oncol. 1997 Sep;4(6):475-80
9309336
Dis Colon Rectum. 2000 Apr;43(4):499-502
10789745
Dis Colon Rectum. 1990 Feb;33(2):150-2
2153511
Int J Colorectal Dis. 2005 Mar;20(2):199-200
15449079
Br J Dermatol. 1999 Nov;141(5):951-3
10583201
Mod Pathol. 2006 Feb;19 Suppl 2:S93-S126
16446719
Gastroenterol Jpn. 1993 Oct;28(5):719-24
8224624
J R Soc Med. 1991 Feb;84(2):112-3
1847974
Am J Surg. 2001 Apr;181(4):363-5
11438274
Dis Colon Rectum. 2001 Jun;44(6):868-70
11391150
Br J Radiol. 1999 Jun;72(858):610-2
10560346
Jpn J Clin Oncol. 1998 May;28(5):347-50
9703865
Cancer. 1992 Apr 15;69(8):2166-71
1311986
J Am Acad Dermatol. 2004 Nov;51(5):767-73
15523356
Pathology. 1998 Feb;30(1):1-3
9534198
Int J Colorectal Dis. 1992 Sep;7(3):167-9
1328429
Dermatol Surg. 2003 Jan;29(1):21-4
12534507
J Dermatol. 2005 Aug;32(8):632-7
16334862
Eur J Surg Oncol. 2000 Sep;26(6):612-4
11034816
Br J Plast Surg. 1999 Sep;52(6):471-5
10673924
Int J Colorectal Dis. 2008 Nov;23(11):1125-30
18535830
Eur J Dermatol. 2005 May-Jun;15(3):168-70
15908300
Am J Surg. 2004 Mar;187(3):413-6
15006574