Some studies have shown that patients with HS may carry mutations in genes encoding the γ-secretase complex: nicastrin (NCSTN), presenilin 1 (PSEN1), presenilin enhancer 2 (PSENEN) and POGLUT1, an O-glucosyltransferase involved in the Notch pathway. This evidence concerns the gene NCSTN and histiocytic sarcoma.