• Kyu Yun Jang and Ae Ri Ahn.
• Departments of Pathology, Jeonbuk National University Medical School, Research Institute of Clinical Medicine of Jeonbuk National University, Biomedical Research Institute of Jeonbuk National University Hospital, and Research Institute for Endocrine Sciences, Jeonju, Jeonbuk, Republic of Korea.
• Medicine (Baltimore). 2024 Dec 13; 103 (50): e40995e40995.

RationalePrimary pituitary lymphoma is defined as a lymphoma that develops only in the pituitary gland without involvement of other areas.Patient ConcernsWe present the case of a 61-year-old female patient who underwent an endonasal transsphenoidal approach for the preoperative diagnosis of a pituitary macroadenoma based on radiological findings.DiagnosesMicroscopically, the capillaries were distended by tumor cells. The high-magnification view showed large cells with vesicular nuclei and single or multiple prominent nucleoli, with mitotic figures often observed within the intravascular space.InterventionsImmunohistochemical staining showed strong positivity for CD45 and pan-B cell markers such as CD19 and CD20. The postoperative diagnosis was intravascular large B-cell lymphoma of the pituitary gland.OutcomesNext-generation sequencing revealed alterations in 12 genes: ARID5B, BCL2, CD79B, ETV6, HLA-B, LRRC7, MYD88, PIM1, POT1, PTPN11, RASA1, and SRSF2.LessonsTo our knowledge, this is the first case report of pituitary intravascular large B-cell lymphoma.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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