General
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• Similar to cutaneous basal cell carcinoma

Epidemiology
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• 2-8% of all vulvar malignancies
• Usually women >60 years of age (Indian J Pathol Microbiol 2012;55:583)
• Mean age at diagnosis: 70 years (range 20-96 years) (Gynecol Oncol 2005;97:192)
• Development of BCC correlates to ultraviolet light exposure, but non sun exposed areas can also develop BCC (Gynecol Oncol 2005;97:192)
• Caucasian women are affected more than those with pigmented skin

Sites
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• Tumors usually arise from cutaneous surface, but rarely affect mucosal epithelia of vulva

Pathophysiology
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• Chronic vulvar irritation (e.g. long term use of diapers) appears to be the most important contributing factor
• Other proposed factors: ionizing radiation, arsenic, chronic inflammation, hamartomas, immune deficiency
• Molecular alterations:
  • Activation of the Hedgehog signaling pathway leading to overexpression of GLI1 or GLI2 gene products
  • Loss of function mutations of PTCH1 or PTCH2 (My Cancer Genome - BCC)
  • Activating mutations of SMO
  • p53 mutations identified in many cases
  • Not related to HPV

Clinical features
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• Slow growing, painless lesion that may ulcerate
• Itching, discomfort, bleeding, mass or swelling, ulcer and pain
• Younger women (< 50 years) may not have many symptoms
• Often mimics eczema, psoriasis or other inflammatory dermatoses that do not respond to standard therapies
• Diagnosis of vulvar BCC almost never made at clinical examination

Radiology
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• MRI: (J Dermatol 2011;38:97)
  • T1-weighted: hypointense mass compared to the skeletal muscle

Radiologic images
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Low intensity area

Prognostic factors
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• Early diagnosis and complete excision are the most important prognostic factors
• When diagnosed at an advanced stage (which happens frequently), patients are at a high-risk for local recurrence and distant metastases (Int J Gynecol Pathol 1997;16:319)
• Incomplete resection with positive margins is common with the infiltrative, micronodular and morpheaform types of BCC
• Presence of perineural invasion can predict local recurrence
• Metastasis is common in patients with lymphovascular space invasion

Case reports
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• 72 year old woman with vulvar basal cell carcinoma with clitoral involvement (Acta Derm Venereol 2009;89:191)
• 79 year old woman with basal cell carcinoma of the vulva with lymph node and skin metastasis (J Dermatol 1995;22:36)
• 86 year old woman with basal cell carcinoma of the vulva with groin node metastasis (Gynecol Oncol 1994;53:366)
• 86 year old and 90 year old women with vulvar basal cell carcinoma (Gynecol Oncol 2002;85:532)

Treatment
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• Surgical resection: (Dermatol Online J 2011;17:8)
  • Partial or total vulvectomy or conservative wide excision
  • Mohs micrographic surgery
  • Inguinofemoral lymph node dissection may also be considered for deep or large tumors
• Adjuvant or palliative radiation therapy
• Immunomodulators such as topical Imiquimod

Clinical images
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Irregularly shaped and ulcerated tumor

Well-limited plaque with a pigmented border

Multiple indurated nodules

Gross description
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• Vegetating, ulcerated, pedunculated, infiltrative, nodular or pigmented lesions

Micro description
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• Histologic examination is essential for diagnosis
• Features are similar to BCC elsewhere
• Proliferation of nests of small basal cells with high nuclear to cytoplasmic ratio, peripheral palisading and no obvious intercellular bridges
• Epidermal connection can often be identified
• Mitotic figures and apoptosis are frequently seen within the same nest
• The surrounding stroma is loose and mucin-rich (hyaluronic acid), leading to retraction artifacts due to mucin shrinkage during tissue processing
• Presence of perineural infiltration and lymphovascular space involvement should be documented
• A wide variety of histologic types have been described; the following patterns are also clinically significant for management and prognosis:
  • Nodular/ulcerative
  • Diffuse (infiltrating, micronodular and morpheaform)
  • Superficial (multifocal)
  • Pigmented
  • Fibroepithelioma of Pinkus

Micro images
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Vuvlar BCC

 

Vulvar BCC

Adenoid differentiation

Basophilic nodules with peripheral palisading

Nodular BCC

Dermal tumor masses

Infundibulocystic BCC

With Paget's disease

Diffuse bcl2+

Positive stains
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• Epithelial cells: HMW CK, BerEP4, bcl2, p53, p63

Negative stains
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• EMA, CEA

Electron microscopy description
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• Usually not performed

Differential diagnosis
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• Clinical differential diagnosis:
  • Bowen's disease
  • Contact dermatitis
  • Lichen sclerosus
  • Lichen simplex chronicus
  • Melanoma
  • Paget's disease
  • Psoriasis
• Histological differential diagnosis:
  • Adnexal tumors including desmoplastic trichoepithelioma
  • Trichoblastoma

Additional references
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• McKee's Pathology of the Skin - Chap.24:Tumors of the Surface Epithelium, Obstet Gynecol 1997;90:765, J Surg Oncol 1999;70:172, J Skin Cancer 2011;2011:650258, Curr Dermatol Rep 2014;3:40

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