Interestingly, among TSC patients receiving systemic mTOR inhibitors for the treatment of other TSC-related tumors, modest beneficial effects have been observed in decreasing the growth rate of concomitant PNETs or pathologically undefined pancreatic lesions.6,20 Similarly, one of our TSC patients with a biopsy-proven PNET received an mTOR inhibitor (sirolimus) for renal angiomyolipomas and a subependymal giant cell tumor of the brain. This evidence concerns the gene MTOR and primitive neuroectodermal tumor.