Low-Grade Fibromyxoid Mesenchymal Tumor Mimicking a Chordoma of the Clivus. Case Report

We report the case of a male patient, 28 years old, who attends to the emergency room with severe headache associated with nausea and vomiting without visual changes. The CT scan and MRI show a well defined tumor that occupies the clivus and the sphenoid sinus and which erodes the floor of the sella, the dorsum sellae and clivus with lateral displacement of the cavernous sinuses and cavernous carotid arteries, rostral displacement of the pituitary gland and the optic pathways, mild extension to the prepontic cistern and total compromise of the suprasellar cistern. Its CT and MRI characteristics suggested a chordoma of the clivus. The patient had panhypopituitarism prior to surgery which had to be corrected with hormone replacement therapy. We performed an endoscopic transsphenoidal and transclival approach with extension on the coronal plane up to the pterygoid processes with the aid of neuronavigation achieving a complete resection. The pathology and immunohystochemistry report were compatible with a low-grade fibromyxoid mesenchymal tumor with a Ki67 of 1%. We perform a 6 month follow-up without recurrence of the tumor.