Value of PAX 8 immunostaining in tumor diagnosis: a review and update.

PAX 8 is a transcription factor involved in the regulation of organogenesis of the thyroid gland, kidney, and Müllerian system. Recent studies have shown that, among tumors, PAX 8 is commonly expressed in epithelial tumors of the thyroid and parathyroid glands, kidney, thymus, and female genital tract. Although PAX 8 expression has also been reported in certain neuroendocrine tumors, including well-differentiated pancreatic neuroendocrine tumors, and duodenal and rectal carcinoids, as well as in B-cell lymphomas, it has recently been shown that the PAX 8 positivity reported in these tumors was due to a cross-reactivity of the antibody used with the N-terminal region of PAX 6 and PAX 5, respectively. Owing to its restricted expression, PAX 8 has proved to be a useful immunohistochemical marker with a wide range of diagnostic applications in surgical pathology, some of which are briefly reviewed.