3.3.5 Basal Cell Carcinoma
ICD-11
2C32
Synonyms
Basal cell epithelioma, basalioma.
Epidemiology
Incidence in Europe: 450 to 500/100 000/year, most common skin malignancy.
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The incidence in Europe is 450 to 500/100,000/year. Incidence in US is 1,019/100,000/y in women and 1,488/100,000/year in men. It is the most common skin malignancy.
Definition
Locally destructive malignant tumour derived from pluripotential epidermal stem cells, almost never metastasizes.
Aetiology & Pathogenesis
Risk factors: Skin types I and II (pale white and fair skin), UV exposure, immunosuppression, radiation, arsenic exposure/ingestion, specific genetic predisposition. Naevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome) is a genetic disease with predisposition for multiple basal cell carcinomas.
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Predisposing factors are skin types I and II, UV exposure, immunosuppression, radiation, arsenic exposure/ingestion, and genetic. Naevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome) is a genetic predisposition for multiple basal cell carcinomas. Most, if not all, cases of BCC demonstrate overactive Hedgehog signalling.
Signs & Symptoms
Pearly nodule with telangiectases, often raised border and central ulceration in superficial type (most common). Solid or nodular type. Sclerodermiform type infiltrating without sharp borders. Other types: cystic, rodent ulcer, metatypic (aggressive), pigmented.
Localisation
Face, trunk, extremities. In contrast to squamous cell carcinoma, not restricted to sun exposed area.
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Lesions are usually located on the face (85%), less often on trunk or extremities. In contrast to squamous cell carcinoma, they are not restricted to sun-exposed areas.
Classification
No precursor lesions. Typing by histology.
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There are no precursor lesions. Classification is done due to clinical (C) or histological (H) features.
Superficial BCC (C)
Nodular (solid) BCC (C)
Sclerodermiform (Morphaeic infiltrating) BCC (C and H)
Adenocystic BCC (H)
Pigmented BCC (C)
Baso-squamous BCC (H)
Dedifferentiated BCC (H)
Ulcus rodens (C)
Ulcus terebrans (C)
Genetically based BCC such as Gorlin-Goltz syndrome (basal cell nevus syndrome) (C)
Laboratory & other workups
Not necessary.
Dermatopathology
Proliferation of progenitor cells in the basal layer of the epidermis and acroinfundibulum. Tumor cells invade different layers of the dermis. Peripheral palisading (darker row of cells at periphery), mitoses, single cell necrosis (apoptosis).
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Histology shows proliferation of basaloid cells streaming downwards from the basal cell layer of the epidermis or from follicular epithelium in upper dermis, peripheral palisading cell layer (darker row of cells at periphery), mitoses, and single cell necrosis (apoptosis).
There are subtypes which mimic abnexal structures of the skin. In the infiltrating type may show dedifferentiation and carcinoma-like pattern.
Course
Slowly progressing. May be locally destructive of deeper tissues.
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Lesions run a slow progressive course of peripheral or vertical extension. They may become ulcerated and locally extensive. Metastases in BCC are extremely rare.
Complications
Recurrence rate after surgery or radiotherapy up to 5% within 5 years.
Diagnosis
Depending on clinical and histological type.
Differential Diagnosis
Sebaceous hyperplasia, dermal naevi , dermatofibroma, pseudolymphoma, melanoma, carcinoma in situ (Bowen disease or Paget disease), actinic keratosis, tumors of skin appendages.
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Basal cell carcinoma must be differentiated from dermal naevi, sebaceous gland hyperplasia, fibroma, pseudolymphoma, melanoma, carcinoma in situ (Bowen disease or Paget disease), actinic keratosis and tumors of skin adnexal tumors.
Prevention & Therapy
Depends on the type, size and location. Excision of nodular and sclerodermic type because of recurrences in particular sclerosing tumours with micrographic control of margins; Radiation therapy in nodular types, cryosurgery. For superficial variants, also consider: cryosurgery, photodynamic therapy, topical 5-fluorouracil, immunomodulators (imiquimod), ablative laser with monitoring follow up. Advanced lesions: hedgehog inhibitors.
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Prevention
UV-light protection is essential.
Treatment depends on the type, size and location. Surgical excision with appropriate margins is the main treatment but recurrences may occur. For the sclerosing tumour type, micrographic control of margins is mandatory. Curettage, radiation therapy and cryosurgery can also be used in the small superficial types. Superficial variants can also be treated with photodynamic therapy, topical 5-fluorouracil, immunotherapy (imiquimod) or ablative laser with monitoring follow up.
New targeted therapies with inhibition of hedgehog pathway may be indicated in advanced cases (Vismodegib, Sonidegib).
Special
None.
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