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

Abstract

Primary pituitary lymphoma in immunocompetent patients is an exceedingly rare clinical entity. We report the case of a 41-year-old woman with initial polydipsia and polyuria. When laboratory evaluation revealed panhypopituitarism, a pituitary enhanced MRI was performed and was suggestive of a pituitary adenoma. Obstructive hydrocephalus provoked by enlarged sellar mass was also demonstrated during observation, and a shunt procedure following lesion resection was carried out. Histopathological examination and immunophenotyping verified diffuse large B-cell non-Hodgkin's lymphoma. No systemic disease was found on staging of the patient. We will also review the clinical, radiological features and outcomes of 28 cases that have been reported since 1993.

Case Report: Identification of Potential Prognosis-Related TP53 Mutation and BCL6-LPP Fusion in Primary Pituitary Lymphoma by Next Generation Sequencing: Two Cases

“…We also identified that gene mutation of TP53 and BCL6 was responsible for prognostic difference. Normally, the treatment of PPL often follows the management protocol of PCNSL consisting of surgery, chemotherapy and/or radiotherapy ( 6 ). However, it is said that surgical intervention suggests no obvious benefits in the outcome of PCNSL and the neurotoxic effects of radiotherapy should be noted ( 3 , 5 ).…”

“…Whether BCL6 can be considered as a prognostic factor of PCNSL is still controversial. Cady et al investigated that BCL6 was associated with inferior OS alone or concomitant with del ( 6 ) (q22) ( 18 ). However, the expression of BCL6 was paradoxically correlated with the prognosis of PCNSL.…”

Primary lymphoma of the pituitary gland: an unusual cause of hemianopia in an immunocompetent patient

“…Endocrine dysfunction appears to occur in roughly 50% of the studies reviewed in this study. 4,5,8,11,15–17,19,21,23,24,29 Studies of non-functioning pituitary macroadenomas suggest that pituitary dysfunction is present in more than 30% 30 which would correlate well with lymphomas. By far the most common lymphoma subtype reported was B-cell (65%), the other cases were made up of mixed cell, T-cell, Burkitt cell and mucosa-associated lymphoid tissue cell types.…”

Case Report: Primary pituitary non-Hodgkin’s lymphoma developed following surgery and radiation of a pituitary macroadenoma

“…11,12 The majority of cases are associated with congenital and acquired immunodeficiency, whereas around 29 case reports with sellar and suprasellar location concern immunocompetent patients, like ours. 4,5,13 Regarding radiologic evaluation, there are no distinct radiological features of sellar and suprassellar lymphomas that can aid the differential diagnosis. On MRI, they usually appear as iso-or hypointense on T1 and T2-weighted images and tend to have homogeneous enhancement following radio-contrast administration.…”