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PAX2 is a member of the PAX family of transcription factors that, together with PAX8, is involved in the regulation of the organogenesis of the kidney and the Müllerian system. Recent investigations have demonstrated that, among tumors, PAX2 is commonly expressed in epithelial tumors of the kidney and female genital tract. Although PAX2 expression has also been reported in B-cell lymphomas and rhabdomyosarcomas, especially alveolar rhabdomyosarcomas, it has been suggested that the positivity in these tumors was most probably due to a cross-reactivity of the anti-PAX2 antibody used in those investigations with other members of the PAX protein family. An analysis of published studies indicates that PAX2 sensitivity for epithelial renal neoplasms and epithelial tumors of the female genital tract is lower than that of PAX8. In contrast to the latter marker, however, PAX2 does not appear to be expressed in epithelial tumors of the thyroid gland or thymus. Because of its restricted expression, PAX2 has proved to be a useful immunohistochemical marker with a wide range of diagnostic applications in surgical pathology, some of which will be briefly reviewed.