Primary Lymphoma of the Pituitary: An Emerging Clinical Entity

Primary pituitary lymphoma in an immunocompetent patient: a rare clinical entity

“…Out of 1,120 patients who underwent transsphenoidal resection for pituitary masses, only one case (0.1%) was reported to be PPL [5]. Interestingly, recent data show an increase in the number of cases of PPL in the immunocompetent population [6,7]. In our review of 29 immunocompetent patients with PPL, there is an 18:11 male/female ratio.…”

“…Twenty-three (23/27, 85.2%) patients exhibited anterior hypopituitarism, and nine (9/27, 33.3%) had diabetes insipidus ( Table 2). The heterogeneous endocrine findings suggest different origins of lymphoproliferative tissue [7]. Clinically, these patients with hypopituitarism presented with various symptoms, including fatigue, muscle weakness, loss of libido, amenorrhea, thirst and polyuria [12].…”

“…The clinical and laboratory presentations in our patient were consistent with diabetes insipidus and panhypopituitarism, which may result from posterior and anterior pituitary involvement or compression of the pituitary stalk. Together with hypopituitarism, headache (about 75% of patients) [7] and cranial nerve abnormalities were also common presenting symptoms. Eleven patients (37.9%) with PPL had diplopia due to the compression of the cranial nerves through cavernous sinus, and ten (34.5%) had visual field defects owing to optic chiasm involvement.…”

“…They are always of the non-Hodgkin's type [21]. The majority of PCNSLs are high-grade immunoblastic or diffuse large-cell type with a B-cell immunophenotype [7,22]. Similar to PCNSLs, PPLs are predominant B-cell nonHodgkin's lymphoma.…”

“…In a review of the English language literature, most patients underwent tumor resection through transsphenoidal approach ( Table 2). It is feasible to try systemic lymphoma regimen for PCNSL because of the histological similarity between PCNSL and systemic non-Hodgkin's lymphoma [7]. High-dose MTX is the only independent treatment-related factor that clearly improved the prognosis [29].…”