The islet/insulinoma tumors are the less aggressive subtype characterized by the expression of insulinoma-associated genes (INS, IAPP, INSM1); the MLP subtypes are associated with a high rate of liver or lymph node metastasis and enriched for genes correlated with stroma, hypoxia and pancreatic progenitor-specific genes; the intermediate subtype is more similar to islet/insulinoma tumors but more aggressive with a high frequency of MEN1 and DAXX/ATRX mutations [33]. The gene discussed is INSM1; the disease is pancreatic insulinoma.