ABSTRACT: BACKGROUND: Adult granulosa cell tumors of the ovary (GCTs) are sex cord stromal tumors of unpredictable behaviour. Up to now, the prediction of the relapsing/malignant potential remains difficult. CD56 (NCAM) in GCTs was previously described in only two studies. However, the expression of its isoforms was not examined. METHODS: 30 GCTs (16 primaries, 14 relapses) were investigated immunohistochemically with antibodies against Pan-CD56 (CD56Pan) and the isoform with 140/180 kDa length (CD56140/180 kDa). The reaction was assessed with respect to percentage of positive cells and intensity of staining. RESULTS: In all GCTs, CD56Pan was expressed, but differences were found between primaries and relapses. The percentage of CD56Pan positive tumor cells was lower in relapses, whereas CD56140/180 kDa showed a higher staining intensity in the latter. CONCLUSION: Expression of CD56 is an additional sensitive and helpful immunohistochemical tool for histopathologists diagnosing a GCT. It does not seem possible to provide a validly individual risk assessement. However, the different expression of CD56 isoforms might indicate important changes in the course to a more malignant behaviour.