Extramammary Paget disease
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| Extramammary Paget disease | |
|---|---|
| Diagnosis in short | |
 Extramammary Paget's disease. H&E stain. | |
|
| |
| LM | large epithelioid cells - nested or single - in the epidermis, clear/pale cytoplasm (occasionally eosinophilic), large nucleoli |
| LM DDx | benign Toker cell hyperplasia, malignant melanoma, Bowen's disease, apocrine carcinoma of the skin |
| IHC | CK7 +ve, CEA +ve, S-100 -ve, CK5/6 -ve, HER2 +ve |
| Gross | erythema, +/-weeping, +/-crusted |
| Site | vulva, penis, scrotum, others |
|
| |
| Symptoms | pruritis (itchy) |
| Prognosis | typically benign - usually not associated with an underlying malignancy (unlike Paget's disease of the breast) |
| Clin. DDx | contact dermatitis, lichen sclerosus |
Extramammary Paget disease, abbreviated EMPD, is a skin disease. As the name suggests, there is also a Paget disease of the breast.
There is also a Paget disease of the bone - just to make things confusing. This is dealt with in the bone article and has nothing (from a pathologic perspective) to do with the Paget disease discussed in this article
General
- Two types
- Primary Extramammary Paget disease - a malignancy of the cutaneous apocrine glands
- Arises in apocrine rich areas - usually the vulva but also the groin, inguinal area, perineum, penis[1] or scrotum.[2] and rarely axilla or eye.
- Usually entirely intraepidermal but may be associated with an underlying apocrine gland carcinoma (in contrast to mammary Paget disease which is usually associated with underlying mammary carcinoma).
- Secondary Extramammary Paget disease - intraepidermal spread from a distant tumor
- Usually of urothelial or colorectal origin.
- Arises in the perineal areas near these organs
- Primary Extramammary Paget disease - a malignancy of the cutaneous apocrine glands
Clinical:
- Pruritis.
- R/O VIN
- R/O vulvitis
Gross
Features:[2]
- Plaque with an irregular border.
- Erythematous or white.
Clinical DDx:
- Lichen sclerosus.[3]
- Vulvar intraepithelial neoplasia
- Vulvar squamous cell carcinoma in situ
- Other vulvitis
Images
Microscopic
Features:
- Epitheliod morphology (round/ovoid).
- Cells nested or single.
- Classically Paget cells ride above the basal cell layer
- But the process can fill the entire epidermis
- Clear/pale cytoplasm key feature - may also be eosinophilic.
- Large nucleoli.
Images
Anus Pagets Disease - (SKB)
Anus Pagets Disease - (SKB)
Anus Pagets Disease - (SKB)
DDx
- Malignant melanoma in situ.
- Bowen disease - Pagetoid squamous cell carcinoma in situ.
- Pagetoid vulvar intraepithelial neoplasia
- Vulvar squamous cell carcinoma.
Stains
- Mucicarmine stain +ve.
IHC
Panel:
- CEA +ve (-ve in Bowen's disease, -ve in Toker cells).
- CK7 +ve.
- Toker cells CK7 +ve.[4]
- S100 -ve, HMB-45 -ve (both typically +ve in melanoma).
Additional:
- HER2/neu - usually +ve.
- CK5/6 -ve.[5]
- Usu. +ve in squamous cell carcinoma.
- CAM 5.2 +ve.
See also
References
- ↑ Ekwueme, KC.; Zakhour, HD.; Parr, NJ. (2009). "Extramammary Paget's disease of the penis: a case report and review of the literature.". J Med Case Reports 3: 4. doi:10.1186/1752-1947-3-4. PMID 19126202.
- ↑ 2.0 2.1 2.2 Guerra, R.; Misra, S. (2013). "Management of Extramammary Paget's Disease: A Case Report and Review of the Literature.". Case Rep Dermatol Med 2013: 436390. doi:10.1155/2013/436390. PMID 24349803.
- ↑ Bansal, D.; Bowman, CA. (Feb 2004). "Extramammary Paget's disease masquerading as lichen sclerosus.". Int J STD AIDS 15 (2): 141-2. doi:10.1258/095646204322764361. PMID 15006079.
- ↑ Nofech-Mozes, S.; Hanna, W.. "Toker cells revisited.". Breast J 15 (4): 394-8. doi:10.1111/j.1524-4741.2009.00743.x. PMID 19601945.
- ↑ RS. May 2010.