Vulva, vagina & female urethra
Other carcinomas
Basal cell carcinoma
Last author update: 1 March 2014
Last staff update: 28 August 2023 (update in progress)
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PubMed Search: Basal cell carcinoma [title] vulva
Table of Contents
Definition / general | Epidemiology | Sites | Pathophysiology | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Nagarajan P, Peters SB. Basal cell carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vulvabasalcellcarc.html. Accessed September 26th, 2023.
Definition / general
- Similar to cutaneous basal cell carcinoma
Epidemiology
- 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 nonsun exposed areas can also develop BCC (Gynecol Oncol 2005;97:192)
- Caucasian women are affected more than those with pigmented skin
Sites
- Tumors usually arise from cutaneous surface but rarely affect mucosal epithelia of vulva
Pathophysiology
- 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: Molecular Profiling of Basal Cell Carcinoma [Accessed 6 October 2017])
- Activating mutations of SMO
- p53 mutations identified in many cases
- Not related to human papillomavirus (HPV)
Clinical features
- 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 description
- MRI: (J Dermatol 2011;38:97)
- T1 weighted: hypointense mass compared to the skeletal muscle
Radiology images
Images hosted on other servers:
Low intensity area
Prognostic factors
- 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
- 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
- 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
Images hosted on other servers:
Irregularly shaped and ulcerated tumor
Well limited plaque with a pigmented border
Multiple indurated nodules
Gross description
- Vegetating, ulcerated, pedunculated, infiltrative, nodular or pigmented lesions
Microscopic (histologic) description
- 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
- 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
- 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
Microscopic (histologic) images
Contributed by Priya Nagarajan, M.D., Ph.D.
Vulvar BCC
Vulvar BCC
Images hosted on other servers:
Adenoid differentiation
Basophilic nodules with peripheral palisading
Nodular BCC
Dermal tumor masses
Infundibulocystic BCC
With Paget disease
Diffuse BCL2+
Electron microscopy description
- Usually not performed
Differential diagnosis
- Clinical differential diagnosis:
- Histological differential diagnosis:
- Adnexal tumors including desmoplastic trichoepithelioma
- Trichoblastoma
