,2 More recently, GPV in telomere maintenance genes (TMG) POT1, TERF2IP, ACD, and TERT have been observed in up to 6% of melanoma-prone families.3, 4, 5, 6, 7POT1 GPV also predispose to lymphoid and myeloid malignancies, angiosarcoma, and glioma.8 The gene discussed is TERF2IP; the disease is melanoma.