J-LSMS 2018 | Archive | Issues 1 to 4

JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY

Combined Surgical Oncology and Neurosurgery Approach for Resection of Sacral Chordoma

Racheal Wolfson, MD, Richard Menger, MD, MPA, Ouyen Dinh Chu, MD, MBA, Anthony Sin, MD

Sacral chordomas are rare neoplasms of the sacral spine arising from remnants of the notochord. They are poorly responsive to chemotherapy and radiation therapy. The recommended treatment of sacral chordomas is en bloc resection with wide margins. We present a case of a 46-year-oldman with a sacral chordoma who underwent surgical resection of the mass. A combined anterior-posterior approachwas used, with a surgical oncologist freeing the pelvic cavity through an abdominal laparotomy followed by a neurosurgeon resecting the mass and fusing the lumbar spine to the pelvis posteriorly. The patient underwent a laparotomy the second day for exploration of the pelvis and final closure. The procedure was well tolerated the the patient retained ambulation postoperatively. Complete resection of sacral chordomas with wide margins offers the best prognosis for patients. Sacral chordomas should be referred to a tertiary care center with experience treating these lesions. Our center recommends a combined surgery-neurosurgery approach for safe resection of sacral chordomas.

INTRODUCTION

Chordomas are rare but malignant tumors arising from vestigial remnants of the embryological notochord. They account for 1.4% of all primary malignant bone tumors and 17% of primary bone tumors of the spine. 1 The incidence of chordomas is <0.1 per 100,000 people per year 2,3 and increases with age. 4 Chordomas are most commonly found within the sacrococcygeal area (40- 50%), followed by the skull base (35-40%) and the vertebral bodies (15-20%). 5 Patients often present with vague symptoms mimicking lumbosacral stenosis and advanced disease due to the slow growth of these tumors deep within the pelvis. 6 Because chordomas are poorly responsive to chemotherapy, the treatment modality of choice is radical resection. Local recurrence remains common (43-85%) even with complete resection with negative margins. 5 Sacrectomy with wide resection margins also carries a high risk of postoperative morbidity due to extensive nerve root and musculoskeletal resection leading to ambulatory, sexual, bowel, and urinary symptoms. 2 Despite this, wide resection margins provides the best long-term prognosis in terms of progression-free survival and thus the gold standard for surgery is en bloc resection of the chordoma. In sacral chordomas this can be achieved with a combined anterior-posterior approach for lesions extending above S3, whereas a posterior approach alone is usually sufficient for tumors below this level. 6 We present a patient who underwent resection of a sacral chordoma with a combined anterior-posterior approach with an interdisciplinary team of surgeons from surgical oncology and neurosurgery.

Figure 1 : Post-gadolinium T1-weighted MRI showing a contrast-enhancing sacral mass with infiltration into the spinal canal and pelvic cavity.

6 J La State Med Soc VOL 170 JANUARY/FEBRUARY 2018

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