Basal cell carcinoma (incl. subtypes)

Last Updated: 2024-10-25

Author(s): Anzengruber F., Navarini A.

ICD11: 2C32.Z

Krompecher 1900.

Basalioma, BCC, basalioma, basal cell carcinoma, basal cell epithelioma, basal cell epithelioma, ulcus terebrans, epithelioma basocellulare terebrans.

Most common epithelial neoplasia with basaloid differentiation. Low-grade malignancy, minimal metastatic potential, but significant local invasiveness.

  • Incidence (Germany): 170/100000/year
  • Most common skin cancer (Caucasians)
  • Most common cancer in the USA and Australia
  • Average age: approx. 60 years
  • Incidence: men > women (30% higher in the USA)
  • In the USA, the incidence has risen by 10% per year in the last 10 years
  • Lifetime risk: 30% (USA)
  • The risk of developing the disease is 100 times higher at the age of 55-75 years compared to a 20-year-old person

Clinical/etiological classification:

  • Nodular basal cell carcinoma
  • Superficial basal cell carcinoma
  • Premalignant fibroepithelioma
  • Sclerodermiform basal cell carcinoma
  • Pigmented basal cell carcinoma
  • Destructive basal cell carcinoma (ulcus terebrans)
  • Scarring basal cell carcinoma
  • X-ray basal cell carcinoma
  • Polypoid basal cell carcinoma

 

Histological classification:

  • Solid nodular basal cell carcinoma
  • Micronodular basal cell carcinoma
  • Superficial basal cell carcinoma
  • Infiltrative (cirrhotic) basal cell carcinoma
  • Sclerodermiform basal cell carcinoma
  • Basal cell carcinoma of the fibroepithelioma pinkus type
  • Adenoid basal cell carcinoma
  • Cystic basal cell carcinoma
  • Metatypical basal cell carcinoma
  • Basosquamous carcinoma

  • The BCC arises from epidermal stem cells or undifferentiated cells of the hair follicle bulge or the outer root sheath
  • BCCs arise de novo, not via precancerous lesions

 

Aetiologically important, predisposing factors:

  • Genetics (p53 mutation)
  • Cumulative UVB exposure
  • Exposure to arsenic
  • Radiation therapy
  • Immunosuppression
  • Medicinal UV sensitisation
  • Light skin type
  • Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome)
  • Rombo syndrome
  • Bazex-Dupré-Christol syndrome
  • Oculocutaneous albinism
  • Xeroderma pigmentosum
  • Albinism
  • Arsenic exposure
  • Strict scars
  • Nevus sebaceus
  • Epidermolysis bullosa simplex, Dowling-Meara
  • Actinic keratoses (suggests ↑ UV exposure)

Glassy, pearly macules, plaque / papule or ulcer. Often encrusted with blood, as spontaneous bleeding occurs. Arborising capillaries often visible to the eye. With progression, sharply defined, sometimes spontaneously bleeding, progressive ulcer, often with indurated rim.

  • Pigmented variant: Inhomogeneously pigmented, reddish-brownish, sharply defined coarse tumour with varying melanin content
  • Sclerodermiform variant: Atrophic, slightly raised, scarred, partly keloid-like, rarely ulcerating, yellowish, waxy or ivory-coloured, blurred, coarse plaque with telangiectasia. There is an increased tendency to infiltrate deep structures.

Arborising vessels, shiny-white spots and stripes, blue-grey non-aggregated globules, large blue-grey ovoid nests, leaf-like areas, wheel-spoke-like structures, shotgun-like distributed dots.

  • Anamnesis (predisposing factors?)
  • Clinic
  • Biopsy

  • Chronic light-exposed skin areas (capillitium, face, neck, décolleté)
  • 80% of basal cell carcinomas occur in the head and neck area
  • 70% of basal cell carcinomas occur on the face
  • 15% of basal cell carcinomas occur on the trunk
  • Very rarely do basal cell carcinomas occur on the penis, vulva or perianal area

The tumour cells are derived from stem cells of the hair follicle epithelium and from the stratum basale of the interfollicular epidermis. They show a basaloid morphology with small, hyperchromatic nuclei and narrow cytoplasm. Typical for BCC is the palisading of the cells at the edges of the tumour nests, whereby the cells are arranged in a columnar fashion. Another feature is the peritumoural retraction gap, a distance between the tumour cells and the surrounding stroma, which is histologically visible as a gap, as well as a characteristic peritumoural desmoplastic stroma. Increased mucin deposits are also frequently found in the peritumoural stroma or in the cystic intratumoural cavities. There are different growth patterns of BCCs, including nodular, superficial and infiltrative forms, each with a different clinical course. Infiltrative basal cell carcinomas are considered more aggressive and penetrate deeper into the surrounding tissue, making treatment more difficult. Perineural sheath infiltration is rarely observed, particularly in the basosquamous and metatypical types. Immunohistochemical markers such as BerEP4 and bcl-2 are usually positive in basal cell carcinomas and support the histological differentiation from other skin tumours.

  • Excellent, provided there has been no metastasis. This is extremely rare
  • Probability of recurrence:
    • Different authors estimate the recurrence rates differently.
Years after initial diagnosis of a BCC Probability of recurrence of a BCC
1 Ca. 20%
2 approx. 50%
3 approx. 66%
5 Ca. 40-80% (depending on the data)

Source: Hauschild, A. (2016). Long version of the guideline "Basal cell carcinoma of the skin". Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/032-021.html

  • Patients who have already undergone basal cell carcinoma are four times more likely to develop basal cell carcinoma again.
  • The risk decreases if the first basal cell carcinoma occurs after the 75th birthday.
  • If an upper extremity is the first affected localisation, this indicates a poorer prognosis.
  • 6-10% of patients with a BCC develop an SCC (3 times higher risk than in the normal population).
  • The risk of subsequently developing malignant melanoma is 2-4 times higher in BCC patients.
  • Check-ups should be carried out every 6 months in the first year and then once a year.
  1. Leibovitch I, Huilgol SC, Selva D, Richards S, Paver R. Basal cell carcinoma treated with Mohs surgery in Australia II. Outcome at 5-year follow-up. Journal of the American Academy of Dermatology 2005;53:452-7.
  2. Peris K, Campione E, Micantonio T, Marulli GC, Fargnoli MC, Chimenti S. Imiquimod Treatment of Superficial and Nodular Basal Cell Carcinoma. Dermatologic Surgery 2005;31:318-23.
  3. Epstein EH. Basal cell carcinomas: attack of the hedgehog. Nature Reviews Cancer 2008;8:743-54.
  4. Geist DE, Garcia-Moliner M, Fitzek MM, Cho H, Rogers GS. Perineural Invasion of Cutaneous Squamous Cell Carcinoma and Basal Cell Carcinoma: Raising Awareness and Optimising Management. Dermatologic Surgery 2008;34:1642-51.
  5. Gudbjartsson DF, Sulem P, Stacey SN, et al. ASIP and TYR pigmentation variants associate with cutaneous melanoma and basal cell carcinoma. Nature Genetics 2008;40:886-91.
  6. Pincus LB, McCalmont TH, Neuhaus IM, Kasper R, Oh DH. Basal cell carcinomas arising within multiple trichoepitheliomas. Journal of Cutaneous Pathology 2008;35:59-64.
  7. Redondo P, Marquina M, Pretel M, Aguado L, Iglesias ME. Methyl-ALA-Induced Fluorescence in Photodynamic Diagnosis of Basal Cell Carcinoma Prior to Mohs Micrographic Surgery. Arch Dermatol 2008;144.
  8. Szeimies RM, Ibbotson S, Murrell DF, et al. A clinical study comparing methyl aminolevulinate photodynamic therapy and surgery in small superficial basal cell carcinoma (8-20 mm), with a 12-month follow-up. Journal of the European Academy of Dermatology and Venereology 2008;22:1302-11.
  9. Telfer NR, Colver GB, Morton CA. Guidelines for the management of basal cell carcinoma. Br J Dermatol 2008;159:35-48.
  10. Love WE, Bernhard JD, Bordeaux JS. Topical Imiquimod or Fluorouracil Therapy for Basal and Squamous Cell Carcinoma. Arch Dermatol 2009;145.
  11. Muller FM, Dawe RS, Moseley H, Fleming CJ. Randomised Comparison of Mohs Micrographic Surgery and Surgical Excision for Small Nodular Basal Cell Carcinoma. Dermatologic Surgery 2009;35:1349-54.
  12. Swanson EL, Amdur RJ, Mendenhall WM, Morris CG, Kirwan JM, Flowers F. Radiotherapy for basal cell carcinoma of the medial canthus region. The Laryngoscope 2009;119:2366-8.
  13. Basset-Seguin N. Topical photodynamic therapy for superficial and nodular basal cell carcinoma. Expert Review of Dermatology 2010;5:61-6.
  14. Saran A. Basal cell carcinoma and the carcinogenic role of aberrant Hedgehog signalling. Future Oncology 2010;6:1003-14.
  15. Youssef KK, Van Keymeulen A, Lapouge G, et al. Identification of the cell lineage at the origin of basal cell carcinoma. Nature Cell Biology 2010.
  16. Sekulic A, Migden MR, Oro AE, et al. Efficacy and Safety of Vismodegib in Advanced Basal-Cell Carcinoma. New England Journal of Medicine 2012;366:2171-9.
  17. Sezer E, Erkek E, Sahin S. A Case Report of Syringocystadenoma Papilliferum Mimicking Basal Cell Carcinoma on the Face. Turkish Journal of Dermatology / Türk Dermatoloji Dergisi 2012;6:21-3.
  18. Dreier J, Felderer L, Barysch M, Rozati S, Dummer R. Basal cell carcinoma: a paradigm for targeted therapies. Expert Opinion on Pharmacotherapy 2013;14:1307-18.
  19. Harms KL, Dlugosz AA. Harnessing Hedgehog for the Treatment of Basal Cell Carcinoma. JAMA Dermatol 2013;149:607.
  20. Iarrobino A, Messina JL, Kudchadkar R, Sondak VK. Emergence of a squamous cell carcinoma phenotype following treatment of metastatic basal cell carcinoma with vismodegib. Journal of the American Academy of Dermatology 2013;69:e33-e4.
  21. Sekulic A, Mangold AR, Northfelt DW, LoRusso PM. Advanced basal cell carcinoma of the skin. Current Opinion in Oncology 2013:1.
  22. McCusker M, Basset-Seguin N, Dummer R, et al. Metastatic basal cell carcinoma: Prognosis dependent on anatomic site and spread of disease. European Journal of Cancer 2014;50:774-83.
  23. Majores M, Bierhoff E. [Actinic keratosis, Bowen's disease, keratoacanthoma and squamous cell carcinoma of the skin]. Pathologist 2015;36:16-29.
  24. Hauschild, A. (2016). Long version of the guideline "Basal cell carcinoma of the skin". Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/032-021.html
  25. Lebwohl, Mark. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. Elsevier, 2014. print.