Metastatic disease to the clivus mimicking clival chordomas

Abstract

Objectives/Hypothesis A comprehensive review of the literature of clival metastases and presentation of two additional cases. Study Design Literature review and report of two cases. Methods A literature review of the MEDLINE database (1950 to January 19, 2013) was performed to identify all cases of patients with metastatic disease to the clivus. Additionally, two novel cases are presented. Results In total, 47 cases were identified in the literature, including the two cases presented in this study. Metastatic disease to the clivus is the initial presenting symptom of the primary malignancy in 36% (13/36) of the cases. When there was a history of malignancy, the median interval of time to clival metastases was 24 months (range 1 to 172 months). Clinical symptoms manifested often as cranial neuropathies, with at least abducens palsies as the initial presenting symptom in 61.9% (26/42) of patients. Tumor pathology was diverse, but several pathologies were seen more commonly: prostate carcinoma (18.1%, 9/47), hepatocellular carcinoma (10.6%, 5/47), and thyroid follicular carcinoma (8.5%, 4/47). Conclusion Although clival metastases are extremely rare, they are an important part of the differential of clival masses as they can be the presenting symptom of distant malignancy. Level of Evidence 4.

Keywords: clival chordoma; clival metastases; clival tumor; clivus; skull base.

Fig. 1

Magnetic resonance imaging (MRI) of patient with a history of prior clival chordoma resection who presents with a new clival mass. The clival mass (white arrow) is isointense on T2-weighted axial (A) and coronal (B) images. T1-weighted coronal images demonstrate an isointense clival mass that enhances after gadolinium administration (C). Isointensity on T2-weighted images is atypical for chordomas; however, given the history, a transnasal transsphenoidal resection was undertaken. Final pathology was consistent with metastatic leiomyosarcoma, grade 2.

Fig. 2

Pathology from endoscopic transnasal transsphenoidal resection of a leiomyosarcoma clival metastasis. (A) Hematoxylin and eosin staining demonstrates a spindle cell neoplasm with moderately pleomorphic nuclei with mitotic figures. Immunohistochemistry was positive for desmin, smooth muscle action, and caldesmon as shown in figure (B) and negative for CKAE1/AE3, GFAP, Ki677, S100, EMA.

Fig. 3

Magnetic resonance imaging (MRI) of a patient with metastatic breast adenocarcinoma to the clivus. This clival metastasis (arrow) is hypointense on coronal cuts of a T1-weighted MRI (A) and enhances after administration of gadolinium (B). The mass demonstrates hyperintensity in this (C) axial T2-weighted MRI. Final pathology demonstrated metastatic mucinous adenocarcinoma of the breast.

Fig. 4

Pathology from endoscopic transnasal transsphenoidal resection of a breast mucinous adenocarcinoma clival metastasis. (A) Hematoxylin and eosin staining reveals nests of tumor separated by a richly vascularized abundant mucinous stroma. Immunohistochemistry staining demonstrated strong diffuse positivity for pankeratin (B), CAM5.2, CK7, mCEA, and synaptophysin and nonreactive with prolactin, chromogranin, CD56, S100, p62, CDX-2, CK 20, and CD117.