Six patients (24.0%) had tumors or evidence of malignancy with two patients presented with high blood level of CA-125 (anti-Yo, PNMA2) and the other four patients each presented with germ cell tumor (anti-Yo), ovarian tumor (anti-CV2), neuroblastoma (anti-GAD) and pancreatic cancer (PNMA2). The gene discussed is PNMA2; the disease is familial pancreatic carcinoma.