We here distinguish three different clinical presentations of BPDCN. A nodular pattern is a more common feature than the originally reported 'bruise-like' pattern. Despite the fact that BPDCN may initially appear as a localized skin tumour, aggressive management including allogeneic bone marrow transplantation should be considered immediately, as it is currently the only option associated with long-term survival.

Blastic plasmacytoid dendritic cell neoplasm is a rare clinicopathologic entity, characterized by strong skin tropism and a poor prognosis. The diagnosis is generally made by skin biopsy with appropriate immunohistochemical studies. To identify potential biological prognostic factors for blastic plasmacytoid dendritic cell neoplasm, we performed an extended clinico-immunohistochemical study on a series of 91 well-documented cases collected since 1995 by the French Study Group on Cutaneous Lymphomas. Skin biopsies were analyzed using a panel of 12 immunohistochemical markers (CD4, CD56, CD123, CD303, TCL1, CD68, CD2, CD7, TdT, Ki-67, S100, and MX-1). The results were correlated with survival. The 5 most characteristic markers of this entity (CD4, CD56, CD123, CD303, and TCL1) were expressed simultaneously in only 46% of patients. However, when 4 markers were expressed the diagnosis could still be reliably made without resorting to any additional stains. Expression of TdT and/or S100 correlated with varying degrees of maturation. Statistical survival analyses showed that CD303 expression and high proliferative index (Ki-67) were significantly associated with longer survival.

Metastatic melanoma is a very aggressive cancer. For decades, although dacarbazine has been the standard of care as first-line therapy, new therapies have now been shown their superiority either alone or in association with dacarbazine. Ipilimumab (anti-CTLA4 monoclonal antibody) and vemurafenib (BRAF V600E inhibitor) have been recently approved by the Food and Drugs Association and European Medicine Agency for their use in metastatic melanoma patients. Other compounds targeting the MAP kinase pathway (anti-MEK, anti-ERK, other anti-BRAF) are under clinical evaluation as well as molecules targeting alternate pathways. Along with the development of these targeted agents, an initial molecular characterization of each patient melanoma has become the first step to define the best therapeutic options. The recent published data shed the light on advanced melanoma management. Herein we review the latest development of these molecules and their impact on the treatment strategy.