Three-Dimensional Volume Imaging to Increase the Accuracy of Surgical Management in a Case of Recurrent Chordoma of the Clivus
Challenging differential diagnosis, Rare disease
João Pedro Perez Gomes, José de Ribamar Castro Veloso, Albina Messias de Almeida M. Altemani, Carlos Takahiro Chone, João Mauricio Carrasco Altemani, Claudio Fróes de Freitas, Carmen Silvia Passos Lima, Paulo Henrique Braz-Silva, Andre Luiz Ferreira Costa
Division of General Pathology, Department of Stomatology, School of Dentistry, University of Sao Paulo (USP), São Paulo, SP, Brazil
Am J Case Rep 2018; 19:1168-1174
The clivus is a depression in the anterior occipital bone of the skull base, posterior to the dorsum sellae, at the junction with the sphenoid bone. Chordoma is a rare tumor arising from embryonic remnants of the notochord and can be locally aggressive with a tendency to recur. The optimal management of this rare tumor remains controversial. A report of a case of recurrent chordoma of the clivus is presented to illustrate the value of volumetric three-dimensional (3-D) reconstruction with computed tomography (CT) and magnetic resonance imaging (MRI) to determine optimal surgical management.
CASE REPORT: A 53-year-old man presented with pain in the right orbital cavity, right proptosis, swelling of the right cheek, and bilateral loss of vision. He also had adrenal insufficiency. CT and contrast-enhanced (gadolinium) T1-weighted MRI with multiplanar acquisition were performed with volumetric 3-D reconstruction of the tumor, to increase the chances of treatment success. Surgical resection was performed to remove the tumor and reduce the risk of recurrence. Histology of the tumor was consistent with chordoma, supported by positive immunohistochemical staining for S-100 and epithelial membrane antigen (EMA).
CONCLUSIONS: This report highlighted the value of 3-D volume imaging in the diagnosis and treatment planning in a rare case of recurrent chordoma of the clivus. Analysis of tumor volume may be an indicator of the efficacy of surgery, complementing the Response Evaluation Criteria In Solid Tumors (RECIST) system and as a valuable tool to predict treatment outcome.
Keywords: Head and Neck Neoplasms, Imaging, Three-Dimensional, Magnetic Resonance Imaging