Matched blood samples and formalin-fixed paraffin-embedded tumor material from 20 untreated SI-NET patients at different stage of disease with primary tumors (P), lymph node metastasis (LNM) and liver metastasis (LM) were analyzed to detect a possible correlation between the serum titer of Ma2 autoantibodies and the tumor expression of Ma2 antigen. The gene discussed is PNMA2; the disease is lymphangioma.