Primary pituitary lymphomas (PPLs) are very rare tumors, often challenging for preoperative diagnosis, and only 39 cases have been reported in the English literature till date.Most of the PPLs usually show extension to involve adjacent structures such as the suprasellar region, cavernous sinuses, optic chiasma, sphenoid sinus, and cranial nerves.Several non-validated hypotheses have been proposed for the pathogenesis of PPLs.These tumors are much more heterogeneous in comparison with primary central nervous system lymphomas (PCNSLs).Primary pituitary lymphomas comprise of large spectrum of both low-grade and high-grade lymphomas.Some local factors, such as hormonal release, growth factors, and cytokines secreted from the pituitary gland (PG), may be responsible for the heterogeneity of PPLs.Overall survival, remission, and outcomes in PPLs are much better than PCNSLs.A personalized treatment is more suitable in PPLs, unlike a specific regimen in PCNSLs, due to their heterogeneity.This review paper aims to summarize all the PPL cases reported in the English literature with emphasizing their pathogenesis, characteristic features, and diversity of cases.
Rakesh K Gupta (Tue,) studied this question.