The Coexistence of an Intrasellar Adenoma, Lymphocytic Hypophysitis, and Primary Pituitary Lymphoma in a Patient with Acromegaly

Abstract

The concomitant presence of three histopathologically different entities in the pituitary gland is a rare occurrence. Most publications identify at least two distinct pathologies, mainly, a pituitary adenoma coexisting with a second intrasellar lesion. We present a case of a 71-year-old female referred for evaluation and treatment of acromegaly. Questioning revealed she was experiencing facial palsy, visual disturbances, and syncopal spells for several weeks. When laboratory evaluation showed elevated somatomedin (IGF-I) levels and an oral glucose tolerance test failed to demonstrate any suppression of her growth hormone (GH) values, an MRI of the pituitary revealed a sellar mass. A presumptive diagnosis of pituitary adenoma was established. The patient underwent transsphenoidal resection of the sellar mass, which proved to be a large B-cell lymphoma (Stage I-E) associated with areas of adenoma and lymphocytic hypophysitis.

Primary pituitary diffuse large B-cell lymphoma with somatotroph hyperplasia and acromegaly: case report

“…A 71-year-old woman who presented with acromegaly was found to have evidence of 3 discrete pathological processes: primary pituitary lymphoma, lymphocytic hypophysitis, and an intrasellar adenoma. 21 The current case contrasts with this case, however, because of the lack of a discrete adenoma to explain the elevation in IGF-1.…”

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

“…Furthermore, 3 cases of lymphocytic hypophysitis were detected associated to lymphoma (9,26,37). We can speculate that, as demonstrated in primary thyroid B cell lymphoma and autoimmune Hashimoto's thyroiditis 59 Considering the rarity of the disease and the pitfalls in the diagnosis, a close collaboration between endocrinologist, neurosurgeon, neuroradiologist and hematologist is essential to ensure optimal delivery of care.…”

“…Notable, 5 concomitant pituitary adenomas and 2 cases of pituitary cell hyperplasia were diagnosed (19,20,31,37,43,45,46). This association could be explained by the stimulating effect of pituitary hormones on lymphoma cells that is known to express endocrine hormones receptors (57,58), but this association is speculative and remain to be elucidated so far.…”

Hypophysitis (Including IgG4 and Immunotherapy)

“…Recently, IgG4H either isolated or in the context of IgG4-RD has also been described [21, 22, 24]. Its incidence is 0.28–1.08/100,000 patients, comprising 30% of all hypophysitis and 4% of all hypopituitarism cases [17, 18]; however, it may be underreported, as histological/immunohistochemical analysis revealed that 41.4% of LH cases fulfilled the criteria for IgG4H [25].…”