Primary sellar glomus tumor with BRAF K601E mutation: an aggressive tumor of uncertain malignant potential.

Glomus tumor (GT) is a mesenchymal neoplasm composed of modified perivascular cells exhibiting smooth muscle-like features, resembling those of the normal glomus body. The lesions often occur in areas rich in glomus vascularis, most of which occur in the distal limbs, while the sellar GT is extremely rare. Here, we present a case of primary sellar GT harboring a BRAF K601E mutation, identified through next-generation sequencing. The patient has been followed for 10 years, with tumor recurrence noted in the fourth year post-surgery. This report highlights the histological features, biological behavior, clinical manifestations, and prognosis of sellar GTs with a BRAF K601E mutation. Literature review suggests that determining the biological behavior of sellar GTs remains challenging, complicating diagnosis and treatment planning. We summarize the clinical and pathological characteristics of sellar GTs and propose considerations for pathological diagnosis based on our findings.