Metastatic Disease to the Clivus Mimicking a Clival Chordoma

Objective: This study presents two cases of clival metastases, which presented as clival chordomas, and reviews the literature of this rare phenomenon.

Study Design: Two cases from a university medical center are reported, as well as a review of the literature.

Methods: The history, radiology, and pathology of two patients with clival metastases are presented and the literature is reviewed for this rare clinical entity.

Results: Case 1: A 52-year-old woman with no significant past medical history presented with diplopia and was found to have a right sixth cranial nerve palsy. Imaging was obtained and demonstrated a clival tumor measuring 1.8 × 2.2 × 2.3 cm with MR imaging consistent with a chordoma. The patient was taken for endoscopic resection via a transnasal transsphenoidal approach, and the tumor was resected successfully without complication. Intraoperative frozen section analysis showed findings consistent with olfactory neuroblastoma, but metastatic carcinoma could not be ruled out. Final pathology demonstrated mucinous colloid type adenocarcinoma. A postoperative PET/CT scan revealed left breast and axilla hypermetabolism as well as numerous metastatic foci throughout the axial skeleton. A biopsy of the lesion on the breast revealed invasive mammary carcinoma with mucinous colloid features. Case 2: A 62-year-old woman with a history of a leiomyosarcoma and clival chordoma, which were both treated with surgical resection and radiation 4 years prior, presented to the neurosurgical department with an intermittent right sixth nerve palsy. MR imaging was obtained that showed a clival mass consistent with a clival chordoma. The patient was taken to the operating room and underwent an uncomplicated endoscopic resection of the clival mass. Final pathology revealed metastatic leiomyosarcoma.

Conclusion: To date, there are only 34 previously reported cases of metastatic disease to the clivus. Although clival metastases are extremely rare, they are an important part of the differential diagnosis of clival masses. Clival metastases can occur in the context of a known history of a distant malignancy; however, clival metastases can be the presenting symptom of a distant malignancy and must be maintained in the differential diagnosis regardless of past medical history.