Weledji's clinicopathological classification of perianal paget's disease

Abstract

P erianal 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).[6‐9] 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.[10‐12] The fourth group may represent primary Paget’s disease IJS Publishing Group Ltd www.ijsshortreports.com INTERNATIONAL JOURNAL OF SURGERY SHORT REPORTS