Acta Clin Croat, Vol. 47, No. 1, 2008 # Marija Buljan et al.: Variations in clinical presentation of basal cell carcinoma VARIATIONS IN CLINICAL PRESENTATION OF BASAL CELL CARCINOMA Marija Buljan, Vedrana Bulat, Mirna itum, Liborija Lugoviæ Mihiæ and Sandra Staniæ-Duktaj University Department of Dermatology and Venereology, Sestre milosrdnice University Hospital, Zagreb, Croatia SUMMARY  Basal cell carcinoma (basalioma, BCC) is the most common skin cancer and the most common human malignancy in general, with a continuously increasing incidence. In most cases, BCC develops on chronically sun-exposed skin in elderly people, most commonly in the head and neck region. Besides chronic UV radiation, other risk factors for the development of BCC include sun bed use, family history of skin cancer, skin type 1 and 2, a tendency to freckle in childhood, immunosuppression, previous radiotherapy, and chronic exposure to certain toxic substances such as inorganic arsenic. There are numerous variations in clinical presentation of BCC, such as nodular BCC, ulcerating BCC, pigmented BCC, sclerosing BCC, superficial BCC, and fibroepithelioma of Pinkus. Each varies in terms of clinical presentation, histopathology and aggressive behavior. Treatment modalities for BCC include surgical excision, cryosurgery, curettage, electrodessication, radiotherapy, photodynamic therapy, topical cytostatics, and immunomodulators. If left untreated or inadequately treated, BCC may become invasive and locally destructive, although it very rarely metastasizes. Due to the extremely high incidence of BCC, medical professionals should be familiar with its manifold clinical presentations. Key words: Carcinoma, basal cell  etiology; Carcinoma, basal cell  diagnosis; Skin neoplasms  etiology; Skin neoplasms  pathology; Skin neoplasms  diagnosis Acta Clin Croat 2008; 47:25-30 Review Correspondence to: Marija Buljan, MD, University Department of Dermatology and Venereology, Sestre milosrdnice University Hospital, Vinogradska c. 29, HR-10000 Zagreb, Croatia E-mail: buljan.marija@gmail.com Received December 21, 2007, accepted February 12, 2008 Introduction Basal cell carcinoma (BCC) is undoubtedly the most common malignant cancer in fair-skinned adults. It is a relatively slowly expanding, painless, locally aggressive, and recurrent malignant epidermal neoplasm. BCC aris- es from undifferentiated cells in the basal cell layers of the lower epidermis or from outer root sheet of the hair follicle 1-3 . Recent studies have shown that two-thirds of the tumors are located in the head and neck region. The incidence of BCC worldwide has increased substantial- ly over the past several decades and shows geographical variation 4,5 . Several epidemiological studies have clear- ly demonstrated that the incidence of BCC is lower in more polar latitudes than in equatorial latitudes. BCC may occur at any age, but is more common after the age of 60. According to gender, BCC is more common in men than in women 1 . It has been shown that patients with BCC have an increased risk of developing further BCC 5 . Clinically, this tumor usually presents as a slowly growing, flesh-colored, well-defined papule or nodule with telangiectasias located on the upper two-thirds of the face, above the line connecting the angle of the mouth and earlobe. However, according to clinical ap- pearance, histopathologic analysis, and aggressiveness there are several variants including nodular, ulcerated, pigmented, sclerosing, cystic, superficial, fibroepitheli- oma, metatypical BCC, and basal cell nevus syndrome (Gorlin-Goltz syndrome) 1-3 . Although rarely metastatic, its malignant nature is sometimes emphasized by the local tissue destruction, disfigurement, and even death if left untreated 4 . Etiology and Pathogenesis Various endogenous and exogenous factors, or a com- bination of both may be responsible for the develop- 06 Buljan.p65 27. 06. 08, 18:32 25