Concurrent Pituitary Adenoma and Chordoma

Objective: To describe a patient with multiple recurrent pituitary macroadenoma and concurrent skull base chordoma.

Method: In a tertiary care academic medical center, a 64-year-old man underwent transnasal transsphenoidal removal of a nonsecretory pituitary macroadenoma. Five months later, he presented with intermittent diplopia, and imaging confirmed extensive recurrent disease. He subsequently underwent transnasal transsphenoidal removal of the mass, and pathology confirmed recurrent pituitary adenoma. One year later, he developed severe headache and diplopia. Imaging revealed a heterogeneous appearing mass in the sella and sphenoid sinus. Retrospectively, two distinct masses could be seen with different MR imaging characteristics, one mass in the suprasellar space and one involving the sphenoid and ethmoid air cells, eroding the clivus. He was again taken to the operating room and no tumor was found in the sella itself via a revision right-sided transsphenoidal approach. Blood and clots were identified in the sella and sphenoid sinus and were removed. These were not sent for pathology. One year later, the patient developed progressive diplopia and progressive left visual loss, and MR imaging showed a large, aggressive pituitary fossa lesion filling the sphenoid and posterior ethmoid sinus air cells with suprasellar, parasellar, and anterior cavernous involvement, affecting the optic chiasm. He was subsequently referred to the University of Michigan multidisciplinary cranial base team for consideration of further resection. He presented to the operating room for expanded endoscopic radical resection of the recurrent disease.

Result: An intraoperative frozen section of the mass favored chordoma. On final analysis, the majority of the specimen contained pathologic findings consistent with chordoma. There also was a small intrasellar fragment consistent with residual pituitary adenoma. Imaging and histopathologic slides are available for presentation.

Conclusion: This report highlights the possibility of concurrent skull base neoplasms. Though rare, this possibility should be entertained when the clinical course differs from the natural history of the original pathologic diagnosis.