Primary pituitary lymphoma: an update of the literature

Abstract

Primary intracranial lymphomas (Weller et al. in Neuro Oncol 14(12):1481-1484, 2012) are an emerging disease and an isolated localization in the pituitary gland i.e. primary pituitary lymphoma (PPL) represents a rare condition. We present an update of the most recent evidence for PPL through a systematic review of the literature. A systematic literature review was conducted using PubMed database up to October 2015. The population was defined as immunocompetent patients with a pathologically confirmed diagnosis of PPL. Patients' characteristics, clinical presentation, radiological features, pathology reports, adjuvant treatment and follow-up data were analyzed. We reported one case of PPL and included our data in this analysis. A total of 33 cases of PPL were identified, including ours. A slight not significant female prevalence was evident, with a mean age of 59 years at diagnosis. Visual troubles and headaches were the most common presenting symptoms. About 80 % of patients presented a cranial nerve (CN) deficit. The most frequently involved were the II and III CN. Anterior hypopituitarism was present in 70 % of cases and a diabetes insipidus in 36 % of cases. PPL was rarely limited to the sella and most often extended to the suprasellar and parasellar space. 70 % of cases underwent resection, 21 % a biopsy. A B-cell lymphoma was isolated in 82 % of cases, a T-cell lymphoma in 15 % and a NK/T cell lymphoma in one case. Overall mean survival rate was 14.4 months (95 % confidence interval 9.0-19.8 months) and there was no difference in terms of survival rates when patients were stratified according to the treatment they received. PPL is an emerging clinical entity. Literature data are too scarce to allow the definition of specific protocols of treatment and the management is based on the guidelines present for PCNSL. The role of surgery aiming at a complete resection of PPL should be reevaluated in wider studies including only this category of patients, to establish the real role of each therapeutic strategy.

Primary Pituitary Lymphoma As Rare Cause Of A Pituitary Mass And Hypopituitarism In Adulthood

“…Mean age at diagnosis was 58.9 years (range 18-86 years), without gender difference (M: F=1:1). Analyzing epidemiological features, the mean age at diagnosis is in line with previous findings, as the peak of incidence was reported around the sixth decade of age (5,9). Regarding gender difference, the systematic review by Tarabay et al (5) reported a slight prevalence in female subjects, but the data were in contrast with those of the previous analysis by Giustina et al (9) that, however, included immunocompromised patients in their investigation.…”

“…Among tumours causing hypopituitarism, an emerging clinical entity is primary pituitary lymphoma (5). Although it is a rare disease in last years an increasing frequency of primary intracranial lymphoma has been described worldwide.…”

A Unique Case of Central Hypopituitarism and Central Diabetes Insipidus Caused By Diffuse Large B-Cell Lymphoma

“…Central DI related to infundibular infiltration is highly associated with anterior pituitary deficiencies, which were also demonstrated in this patient. While malignancy may be the most common cause of infundibular thickening, with lymphoma being a frequent offender (22), there remains a paucity of cases in the literature of diffuse large B-cell lymphoma causing pituitary infundibular thickening and multiple pituitary deficiencies.…”

DIAGNOSIS OF ENDOCRINE DISEASE: Expanding the cause of hypopituitarism

“…We reported a rare case of hypopituitarism in a patient with intravascular large B-cell lymphoma who after complete hematological remission, reversed hypopituitarism after more than two years (8). Anterior pituitary deficiencies are present in 70% of cases (most have panhypopituitarism), diabetes insipidus is frequent (in 36% of cases), unlike in pituitary adenomas (7). MRI usually shows diffusely enlarged pituitary gland with invasion of both cavernous sinuses mimicking a pituitary adenoma.…”

“…Recently published systematic review of primary sellar lymphoma reported a total of 33 cases of this isolated localization of lymphoma in the sella (7). The vast majority of CNS lymphomas are non-Hodgkin B-cell lymphomas.…”