Abstract
Mastocytosis is a heterogeneous disease characterized by clonal proliferation and abnormal mast cells accumulation in various tissues, particularly in the skin and bone marrow. The clinical spectrum of systemic mastocytosis (SM) includes, among others, SM with associated hematologic neoplasm (SM-AHD) and mast cell leukemia (MCL). However, little is known about the incidence and pathogenesis of non-hematological malignancies in patients with mastocytosis. Increased risk of carcinogenesis in this group of patients could be associated with treatment methods of this disease. PUVA-therapy, as one of cutaneous mastocytosis (CM) treatment options, and the influence of immunosuppression and cytoreductive treatment in advanced forms of SM may influence carcinogenesis. According to the recent publications, increased risk of malignant neoplasms of the skin and internal organs in patients with mastocytosis has been observed. The current state of knowledge suggests a holistic approach and the need of oncological assessment of mastocytosis patients, particularly as far as malignant melanoma is concerned.
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