Non-Melanoma Skin Cancers: Biological and Clinical Features
Abstract
:1. Introduction
2. Normal Skin and the Mechanisms of Cancerogenesis
3. Basal Cell Carcinoma
3.1. The Genetic Landscape of BCC
3.2. Epidemiology, Classification and Clinical Features
3.2.1. Nodular
3.2.2. Superficial
3.2.3. Fibroepithelial
3.2.4. Morpheaform
3.2.5. Infiltrative
3.2.6. Micronodular
3.2.7. Basosquamous
3.3. Therapeutic Options
3.3.1. Radiotherapy
3.3.2. Systemic Treatments
3.3.3. Innovative Therapeutic Strategies
4. Squamous Cell Carcinoma
4.1. The Molecular Features of SCC
4.2. The Clinical Characteristics of SCC
4.3. The Immune System and SCC
4.4. Systemic Treatments
5. Merkel Cell Carcinoma
5.1. Pathogenesis of MCC
5.2. The Clinical Presentation and Diagnosis of MCC
5.3. The Role of Sentinel Lymph Node Biopsy and of Systemic Treatments
6. Conclusions and Future Perspectives
Funding
Acknowledgments
Conflicts of Interest
References
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Syndrome | Type of NMSC | Clinical Features |
---|---|---|
Xeroderma Pigmentosus | BCC SCC | Autosomal recessive disorder characterized by defects in the mechanisms of DNA repair. NMSCs are frequently developed by younger (≤20 years). The risk is directly associated to UVR exposition. |
Oculocutaneous Albinism | SCC | Autosomal recessive disease showing a pigmentary dilution of the skin, eyes, and hair. SCCs are frequently developed by young subjects exposed to UVR. A high incidence of metastatic SCC has been described in black population. |
Epidermodysplasia Verruciformis | SCC | Rare and usually recessively inherited disorder characterized by a colonization of the skin by HPV. The majority of patients develop NMSC as adults, usually in sun-exposed regions, but earlier with respect to the general population. Aggressive biological behavior includes perineural spread, metastases and death. |
Dystrophic epidermolysis bullosa | SCC | It is characterized by both dominant and recessive mutations of the type VII collagen gene. The majority of recessive patients develop SCCs, that occur during the third-fifth decade of life, frequently multiple and with high attitude to local recurrence and spreading to distant metastatic sites. |
Basal Cell Nevous Syndrome (Gorlin) | BCC | It is an autosomal dominant disorder caused by inactivating mutations of PTCH1 or rarely PTCH2. Clinical features include early NMSC, involvement of multiple sites on course of life, odontogenic keratocysts of the jaw, palmo-plantar pits calcifications of the falx cerebri and abnormalities of the skeleton. Patients also develop multiple cancers of the SNC, ovary and heart, as well as other skin defects such as epidermoid cysts and facial milia. |
Bazex-Dupré-Christol Syndrome | BCC | It is a rare condition characterized by follicular atrophoderma of the hands and feet, hypotrichosis, localized hypohidrosis, epidermoid cysts and multiple BCCs developed during the second decade of lifer showing a trichoepithelioma-like histology. The inheritance pattern is often X-linked. |
Rombo Syndrome | BCC | Patients have an atrophoderma vermiculatum-like appearance on the cheeks with evidence of sweat duct proliferation. They often suffer of hypotrichosis, blepharitis, peripheral erythema, trichoepithelioma and skin cancer. |
Clinical Features | LOW RISK | HIGH RISK |
Site and Size | Area L; <20 mm Area M; <10 mm | Area L; ≥20 mm Area M; ≥10 mm Area H; |
Margins | Well defined; R0 | Undefined or R1 |
Immune Suppression | Absence | Presence |
Exposition to radiotherapy or chronic inflammatory process | Absence | Presence |
Rapid growth | Absence | Presence |
Neurological symptoms | Absence | Presence |
Histology | LOW RISK | HIGH RISK |
Grading | G1 or G2 | G3 |
Adenoid-squamous, Adenoid-cystic, Desmoplastic, Metaplastic, Carcinosarcoma | Absence | Any Variant |
Thickness or level of invasion | ≤6 mm and no subcutaneous invasion | >6 mm or subcutaneous invasion |
Perineural, lymphatic or vascular invasion | Absence | Presence |
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Cives, M.; Mannavola, F.; Lospalluti, L.; Sergi, M.C.; Cazzato, G.; Filoni, E.; Cavallo, F.; Giudice, G.; Stucci, L.S.; Porta, C.; et al. Non-Melanoma Skin Cancers: Biological and Clinical Features. Int. J. Mol. Sci. 2020, 21, 5394. https://doi.org/10.3390/ijms21155394
Cives M, Mannavola F, Lospalluti L, Sergi MC, Cazzato G, Filoni E, Cavallo F, Giudice G, Stucci LS, Porta C, et al. Non-Melanoma Skin Cancers: Biological and Clinical Features. International Journal of Molecular Sciences. 2020; 21(15):5394. https://doi.org/10.3390/ijms21155394
Chicago/Turabian StyleCives, Mauro, Francesco Mannavola, Lucia Lospalluti, Maria Chiara Sergi, Gerardo Cazzato, Elisabetta Filoni, Federica Cavallo, Giuseppe Giudice, Luigia Stefania Stucci, Camillo Porta, and et al. 2020. "Non-Melanoma Skin Cancers: Biological and Clinical Features" International Journal of Molecular Sciences 21, no. 15: 5394. https://doi.org/10.3390/ijms21155394