Dermatológia pre prax 4/2023

Rare skin tumours: Merkel cell carcinoma and rare tumours of melanocytic and epidermal origin – 2. part

The incidence of skin tumors has annually a constantly increasing trend .The majority of skin cancer are non-melanocytic types – basal cell carcinomas and squamous cell carcinomas, but there is also a rising incidence of malignant melanoma. However, in the tangle of tumors, the dermatologist must not neglect and properly manage the less common skin tumors, whose incidence ranges from 0.3% to 3.3%. However, these figures are approximate as many of them are not separately coded according to the International Classification of Diseases (ICD). In addition to rarely occurring tumours of epidermal and melanocytic origin, these include tumours of cutaneous adnexa and Merkel cell carcinoma, cutaneous tumours of haematopoietic and lymphoid origin, cutaneous mesenchymal and neural tumours, and finally cutaneous tumours of uncertain differentiation. The most aggressive skin tumor with the worst survival prognosis is Merkel cell carcinoma. The inconspicuous and ambiguous clinical picture of this tumor in the initial stages delays accurate diagnosis, which is based on histological and immunohistological examinations. This holds true for rarely occurring tumors of melanocytic and epidermal origin. The focus of the issue is on the main clinical, dermatoscopic, differential-diagnostic, and prognostic data of these tumors and their subsequent management, including the nevoid basal cell carcinoma syndrome.

Keywords: Merkel cell carcinoma, melanocytoma, rarely occurring tumors of melanocytic and epidermal origin, nevoid basal cell carcinoma syndrome, clinic, diagnosis, management