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


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

DDx

Stains

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:

See also

References

  1. 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. 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.
  3. 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.
  4. 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.
  5. RS. May 2010.