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 Table of Contents  
Year : 2017  |  Volume : 2  |  Issue : 2  |  Page : 21-23

Weledji's clinicopathological classification of perianal paget's disease

Department of Surgery, Faculty of Health Sciences, University of Buea, Cameroon

Date of Web Publication23-May-2017

Correspondence Address:
Elroy Patrick Weledji
Department of Surgery, Faculty of Health Sciences, University of Buea
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijssr.ijssr_8_17

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How to cite this article:
Weledji EP. Weledji's clinicopathological classification of perianal paget's disease. IJS Short Rep 2017;2:21-3

How to cite this URL:
Weledji EP. Weledji's clinicopathological classification of perianal paget's disease. IJS Short Rep [serial online] 2017 [cited 2019 Jan 18];2:21-3. Available from: http://www.ijsshortreports.com/text.asp?2017/2/2/21/206756

Perianal Paget's disease is a heterogeneous entity which may fall into four clinicopathological groups (Weledji's classification). In the first group (Type 1), there is a high frequency of an associated distant malignancy with similar immunoprofile (lysozyme/leu1–M1), requiring an aggressive search for the primary. This is akin to a paraneoplastic syndrome and has the worse prognosis. In the second group (Type 2), perianal Paget's disease represents a cutaneous manifestation through the intraepithelial spread of an underlying anorectal or vulvar adenocarcinoma (i.e., secondary). The third group (Type 3) represents true primary intraepithelial cutaneous apocrine adenocarcinoma. The fourth group (Type 4) represents a primary perianal Paget's disease with an associated malignancy but with discordant immunoprofile. Perianal Paget's disease is exceedingly rare. First discovered by Paget in 1874 as a breast lesion, similar findings in the perianal area were reported 20 years later.[1],[2] It is important to distinguish true Paget's disease (a primary lesion of the apocrine glands) from the pagetoid spread of signet ring cells from a nearby carcinoma (a secondary lesion) by immunohistochemical studies. As perianal Paget's disease is a heterogeneous entity, the author attempted to simplify the conundrum by proposing a clinicopathological classification system of perianal Paget's disease (Weledji's classification). The author classifies perianal Paget's disease into four clinicopathological groups [Table 1].[3] In the first group (Type 1), there is a high frequency of associated malignancies with similar immunoprofile (lysozyme/leu1–M1) and resultant poor outcome highlighting the importance of an aggressive search for a second malignancy. This is akin to a paraneoplastic syndrome.[4] Regression of perianal Paget's disease (Type 1) has been observed following removal of an associated sigmoid colon carcinoma.[5] In the second group (Type 2), perianal Paget's disease merely represents a cutaneous manifestation through intraepithelial spread of an underlying anorectal or vulvar adenocarcinoma (i.e., secondary).[6],[7],[8],[9] This type may be made latent by neoadjuvant and adjuvant chemotherapy to slowly re-emerge as the evidence of local rectal recurrence after anterior resection [Figure 1].[3] The third group (Type 3) represents the true primary intraepithelial cutaneous apocrine adenocarcinoma.[10],[11],[12] The fourth group may represent primary Paget's disease with an associated malignancy but with discordant immunoprofile.[13],[14]
Figure 1: Perianal Paget's disease with raised edges (with permission)[3]

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It is important to compare Paget's disease of the breast with perianal Paget's disease histopathologically. Paget's disease of the breast is always accompanied by intraduct carcinoma, and direct continuity at the nipple can usually be demonstrated.[1] The affected skin shows reactive changes in the dermis, with exudation, formation of vascular fibrous tissue, and infiltration with lymphocytes and plasma cells. These changes produce a characteristic eczematous condition of the nipple and areola named Paget's disease of the breast. The cancer cells are larger than those of the epidermis, with pale cytoplasm and a large vesicular, hyperchromatic nucleus and prominent nucleolus (pagetoid cells). In some patients, intraduct cancer has already invaded the breast stroma; in others, it is still contained within the duct system. In either case, mastectomy is essential. Similarly, extramammary Paget's disease of the skin may occur at the vulva, perianal region, axilla, etc., by intraepithelial spread of carcinoma of the sweat glands in these sites.[5],[6],[7],[8],[9],[10] As Paget's cells are probably of apocrine origin, corroborated by the breast itself being a modified sweat gland, these are “true” Paget's disease with usually low-grade neoplasia when unassociated with adenocarcinoma elsewhere (Type 3).[15] In most cases of perianal Paget's disease (50-80%), there is an underlying adenocarcinoma usually of the rectum or vulva (Type 2) although the lesion may rarely be a distant organ (Type 1[4],[5],[6],[7],[8],[9],[10]). Clinically, occult Paget's disease may be found on histological examination following rectal excision for carcinoma (Type 2).[5],[6],[9] Perianal Paget's disease have occurred 4 years after anterior resection of rectal carcinoma associated with local recurrence of rectal carcinoma, necessitating an abdomino-perineal excision (Type 2).[3] Latent perianal Paget's disease from contiguous spread have appeared several years following excision of rectal carcinoma (Type 2).[16] Conversely, Paget's disease with no apparent associated malignancy may be followed several years later by the development of an adenocarcinoma (Type 2).[5],[6],[9] The differential diagnosis of perianal Paget's disease is wide and includes intraepithelial squamous cell carcinoma (Bowen's disease), squamous cell carcinoma, dermatitis, lichen planus, melanoma, psoriasis, hidradenitis suppurativa, and eczema. Thus, the diagnosis must be confirmed from several biopsies around the periphery of the lesion under general anesthesia.[17] The staging system is the basis for deciding appropriate treatment of perianal Paget's disease as shown in [Table 2].[18]
Table 1: Weledji' s clinicopathological classification of perianal Paget' s disease[3]

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Table 2: Staging and treatment for perianal Paget's disease[18]

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Clinicians and pathologists should carefully examine the perianal epidermis in anorectal carcinoma and vice versa if the anorectal tumor shows intraepithelioid pagetoid (signet ring) cells. Preoperative staging of perianal Paget's disease should include the Weledji's clinicopathological classification before deciding treatment. Long-term follow-up after definitive treatment is required as local recurrence may occur many years later with the risk of metastatic spread.[3],[4],[5],[6],[16],[19]


The author would like to acknowledge his clinical fellowship award on pelvic floor surgery granted by the European Society of Coloproctology to Kantonsspital St Gallen, Switzerland, where my study on perianal Paget's disease evolved. I acknowledge Dr. Lukas Marti and Dr. Walter Brunner of the coloproctology unit at Kantonsspital St Gallen.

  References Top

Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholomew Hosp Res Lond 1874;10:87-9.  Back to cited text no. 1
Darier J, Couillard P. On a case of Paget's disease of the perineo-anal scrotal region. Soc Franc Dermatol Syph 1893;4:25-31.  Back to cited text no. 2
Weledji EP, Hummel B, Marti L, Gruenert J, Malkert E, Brunner W. Perianal Paget's disease: Is it a primary or secondary disease? World J Colorectal Dis 2016;6:1.  Back to cited text no. 3
Le Fur R, Mears L, Dannawi Z. A peri-anal extramammary Paget's disease associated with two well-differentiated invasive intramucosal sigmoid carcinomas, a very rare case: An immunohistochemical and clinical review of extramammary Paget's disease. Ann R Coll Surg Engl 2004;86:W26-31.  Back to cited text no. 4
Lertprasertsuke N, Tsutsumi Y. Latent perianal Paget's disease associated with mucin-producing rectal adenocarcinoma. Report of two cases. Acta Pathol Jpn 1991;41:386-93.  Back to cited text no. 5
Kubota K, Akasu T, Nakanishi Y, Sugihara K, Fujita S, Moriya Y. Perianal Paget's disease associated with rectal carcinoma: A case report. Jpn J Clin Oncol 1998;28:347-50.  Back to cited text no. 6
Louis-Sylvestre C, Haddad B, Paniel BJ. Paget's disease of the vulva: Results of different conservative treatments. Eur J Obstet Gynecol Reprod Biol 2001;99:253-5.  Back to cited text no. 7
Kim NR, Cho HY, Baek JH, Jeong J, Ha SY, Seok JY, et al. Rare case of anal canal signet ring cell carcinoma associated with perianal and vulvar pagetoid spread. J Pathol Transl Med 2016;50:231-7.  Back to cited text no. 8
Sasaki M, Terada T, Nakanuma Y, Kono N, Kasahara Y, Watanabe K. Anorectal mucinous adenocarcinoma associated with latent perianal Paget's disease. Am J Gastroenterol 1990;85:199-202.  Back to cited text no. 9
Armitage NC, Jass JR, Richman PI, Thomson JP, Phillips RK. Paget's disease of the anus: A clinicopathological study. Br J Surg 1989;76:60-3.  Back to cited text no. 10
Clark ME, Schlussel AT, Gagliano RA Jr. Management of adenocarcinoma in the setting of recently operated perianal Paget's disease. Case Rep Surg 2013;2013:510813.  Back to cited text no. 11
Goldblum JR, Hart WR. Perianal Paget's diseases: A histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. Int J Surg Pathol 2009;17:42-5.  Back to cited text no. 12
Chanjuan S, Argani P. Synchronous primary perianal Paget's diseases and rectal adenocarcinoma: Report of a hitherto undescribed phenomenon. Dis Colon Rectum 2014;57:747-51.  Back to cited text no. 13
Liao X, Mao W, Lin A. Perianal Paget's disease co-associated with anorectal adenocarcinoma: Primary or secondary disease? Case Rep Gastroenterol 2014;8:186-92.  Back to cited text no. 14
Merot Y, Mazoujian G, Pinkus G, Momtaz-T K, Murphy GF. Extramammary Paget's disease of the perianal and perineal regions. Evidence of apocrine derivation. Arch Dermatol 1985;121:750-2.  Back to cited text no. 15
Koashi Y, Kitajima S, Schwartz RA, Tsuji T. Perianal Paget's disease years after rectal adenocarcinoma removal. Dermatol Surg 1997;23:1032-4.  Back to cited text no. 16
Jabir S, Khatib M, Ali S, Niranjan N. Perianal Paget's disease: A diagnostic dilemma. BMJ Case Rep 2013;2013. pii: Bcr2013200504.  Back to cited text no. 17
Wietfeldt ED, Thiele J. Malignancies of the anal margin and perianal skin. Clin Colon Rectal Surg 2009;22:127-35.  Back to cited text no. 18
Carbotta G, Sallustio P, Prestera A, Laforgia R, Lobascio P, Palasciano N. Perineal Paget's disease: A rare disorder and review of literature. Ann Med Surg (Lond) 2016;9:50-2.  Back to cited text no. 19


  [Figure 1]

  [Table 1], [Table 2]


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