Malignant chordoma with metastasis to the peritoneum and pelvic lymph nodes treated with cytoreductive surgery: A Case Report

Abstract Chordoma is a rare bone cancer originating from the residual notochord and overexpressing the nuclear transcription factor, brachyury, for which a positive immunostaining is a diagnostic hallmark. Patients, most commonly adult males in the fifth to sixth decade of life, usually present with an axial mass in the sacral region. Common symptoms are reflective of the site of involvement, including back pain and neuropathy. Complete surgical resection is the mainstay of treatment of localized chordomas. However, these tumors can be highly aggressive and metastasize to visceral organs, lymph nodes, and the peritoneum, often harboring a poor prognosis. This case report discusses a 46-year-old male with a sacral chordoma that was initially treated with suboptimal resection and eventually metastasized to regional lymph nodes and peritoneum after 5 years. The subsequent management heavily relied on an oncologic multidisciplinary tumor board and involved complete resection of the primary tumor and regional lymph nodes with metastasectomy of the involved peritoneal deposits, providing a favorable patient outcome.