|Year : 2017 | Volume
| Issue : 2 | Page : 21-23
Weledji's clinicopathological classification of perianal paget's disease
Elroy Patrick Weledji
Department of Surgery, Faculty of Health Sciences, University of Buea, Cameroon
|Date of Web Publication||23-May-2017|
Elroy Patrick Weledji
Department of Surgery, Faculty of Health Sciences, University of Buea
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Weledji EP. Weledji's clinicopathological classification of perianal paget's disease. IJS Short Rep 2017;2:21-3
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., 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]. 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. Regression of perianal Paget's disease (Type 1) has been observed following removal of an associated sigmoid colon carcinoma. 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).,,, 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]. The third group (Type 3) represents the true primary intraepithelial cutaneous apocrine adenocarcinoma.,, The fourth group may represent primary Paget's disease with an associated malignancy but with discordant immunoprofile.,
|Figure 1: Perianal Paget's disease with raised edges (with permission)|
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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. 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.,,,,, 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). 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,,,,,,). Clinically, occult Paget's disease may be found on histological examination following rectal excision for carcinoma (Type 2).,, 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). Latent perianal Paget's disease from contiguous spread have appeared several years following excision of rectal carcinoma (Type 2). Conversely, Paget's disease with no apparent associated malignancy may be followed several years later by the development of an adenocarcinoma (Type 2).,, 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. The staging system is the basis for deciding appropriate treatment of perianal Paget's disease as shown in [Table 2].
|Table 1: Weledji' s clinicopathological classification of perianal Paget' s disease|
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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.,,,,,
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.
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[Table 1], [Table 2]