Vulva
Malignant neoplasms
Paget disease

Author: Priya Nagarajan, M.D., Ph.D. (see Authors page)

Revised: 9 October 2017, last major update March 2015

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] vulva

Cite this page: Nagarajan, P. Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvapagetsdisease.html. Accessed October 22nd, 2017.
Definition / general
  • First described in 1901 by Dubreuilh (Case Rep Oncol Med 2012;2012:854827)
  • Constitutes < 1 - 2% of vulvar malignancies (Gynecol Oncol 2011;122:42)
  • Based on origin, several classification schemes have been proposed:
    • Type 1: primary vulvar cutaneous origin (Int J Gynecol Cancer 2006;16:1212)
    • Type 2: secondary to primary anal or rectal adenocarcinoma
    • Type 3: secondary to urothelial carcinoma
  • OR
    • Type I: Paget disease of primary cutaneous origin (Hum Pathol 2002;33:549)
      • Ia: as a primary intraepithelial neoplasm
      • Ib: as an intraepithelial neoplasm with invasion
      • Ic: as a manifestation of underlying adenocarcinoma of skin appendage or vulval glandular origin
    • Type II: Paget disease of noncutaneous origin
      • IIa: secondary to anorectal carcinoma
      • IIb: secondary to urothelial neoplasia
      • IIc: as a manifestation of another noncutaneous carcinoma: endocervical, endometrial or ovarian adenocarcinoma, etc.
Terminology
  • Synonyms: extramammary Paget (Paget's) disease
Epidemiology
  • Common in elderly, postmenopausal, Caucasian women
Sites
  • Labia majora > clitoris
Etiopathogenesis
  • Intraepithelial adenocarcinoma that can be:
    • Primary, arising from intraepidermal glandular cells or pluripotent cells of epidermis / folliculosebaecous or eccrine units
    • Secondary, due to either direct spread from an underlying in situ or invasive adenocarcinoma or rarely epidermotropic metastasis
  • Underlying neoplasms may be colorectal, urothelial, ovarian, endometrial or cervical in origin
  • May become invasive and eventually metastasize to regional lymph nodes
Clinical features
  • Slowly expanding, erythematous or eczematous, focally eroded or crusted plaques
  • Lesions are frequently sharply demarcated
  • Often pruritic, may be painful or associated with burning sensation, rarely asymptomatic
  • Often accompanied by discharge
  • Frequently multifocal
Diagnosis
  • Must be confirmed by histologic examination
  • Underlying neoplasm should be ruled out by complete pelvic examination, colposcopy and radiologic examination
Prognostic factors
Case reports
Treatment
Clinical images

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Lesion on perineal area

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Preoperative

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Erythemato-squamous lesion

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Before and after treatment

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Asymmetrical brown lesion

Gross description
  • Sharply demarcated plaque
Microscopic (histologic) description
  • Microscopic disease almost always extends well beyond the grossly visible lesion
  • Epidermis is frequently acanthotic, accompanied by hyperkeratosis, parakeratosis, erosion or ulceration
  • Intraepidermal scattering of single or clusters of large cells characterized by abundant eosinophilic, clear or amphophilic cytoplasm, round / ovoid vesicular nuclei, open chromatin and small nucleoli
  • These cells may be seen in combinations of several histologic patterns: glandular, acantholysis-like, suprabasal nests, vertical nests, budding and sheet-like (J Dermatol 2011;38:1054)
  • Basal layer is preserved but may be attenuated
  • Clefting between tumor cells and the surrounding epidermis may be present, due to absence of desmosomes
  • Fibroepithelioma-like hyperplasia or papillomatous hyperplasia may be seen (Am J Surg Pathol 2000;24:543)
  • May resemble invasive disease due to marked intraepithelial proliferation within deep epithelial invaginations, tangential sectioning of rete and papillary dermal fibrosis (Arch Pathol Lab Med 1998;122:471)
  • Sometimes, the glandular nature of the infiltrating cells is highlighted by mucinous differentiation and signet ring-like morphology
  • Rarely, the cytologic atypia can be marked with anaplastic features; these are seen more frequently in secondary Paget disease
  • Exceptionally, the cells may be pigmented
  • There may be an associated lichenoid lymphohistiocytic inflammatory infiltrate in the superficial dermis
Microscopic (histologic) images

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Paget cells

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Epidermis with keratinization


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PAS

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CK7

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CK7+, CK20-



Images contributed by Dr. Priya Nagarajan:
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Low power - subtle disease on left

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Eyeliner sign

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Invasive


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Medium power

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High power

Cytology description
Positive stains
Negative stains
Differential diagnosis