JOURNAL OF THE LOUISIANA STATE MEDICAL SOCIETY
diagnosis is made by core needle biopsy.5 Once a sacral chordoma is diagnosed, the patient should be referred to a tertiary care center for evaluation by experienced surgeons. The main prognostic factor across several studies is the extent of resection.1-3,7,8 Local recurrence remains common but is affected by the resection margins. Margin classifications are based on the classifications by Enneking in 1980. A wide margin involves resection of the surrounding healthy tissue without entering the reactive zone surrounding the tumor, whereas a marginal margin extends through the reactive zone, and an intralesional margin enters the tumor.9 With a wide margin the rate of local recurrence is 5-17%, compared to 71-81% for intralesional or marginal resection margins. Another potential complication is tumor seeding, which can occur due to intraoperative violation of the tumor capsule and can potentially increase the rate of local recurrence.8 Seeding can also occur from tumor biopsy, therefore every effort should be made to resect the biopsy tract during tumor resection. The choice of surgical approach will be largely dictated by the experience of the surgeon(s) involved. Some centers advocate the use of the anterior-posterior combined approach, while others have found no difference in outcomes using the posterior-only approach regardless of tumor level.3 A combined approach allows for carful dissection of the pelvic neurovascular structures, especially when the tumor extends high into the pelvis.8 The morbidity associated with extensive pelvic resection is generally quite high due to sacrifice of involved nerve roots and extensive muscular dissection. In our patient, using the combined approach allowed for a well-controlled resection with preserved ambulation using a walker, but at the expense of urinary continence. Classical chemotherapy has not been proven to be effective in these tumors; however there are some anecdotal reports of slowed tumoral progression with cisplatin, anthracycline, and alkylating agents. Current studies are aimed at identifying and targeting specific receptors expressed by the chordoma, with promising results from tyrosine kinase inhibitors such as imatinib and sumitinib. Cetuximab, gefitinib, and erlotinib target the epidermal growth factor pathway expressed by these tumors. Use of radiotherapy is controversial, and the radio- sensitivity of surrounding structures such as bladder, small bowel, and sacral plexus limits the extent of radiation therapy.5 Recent studies have shown a statistically significant increase in overall survival and progression-free survival for patients who undergo total resection followed by proton beam therapy compared to patients with incomplete resection with or without proton therapy, indicating that surgical resection remains the most important variable.1
flaps to decrease tension on the abdominal wound. The patient remained intubated and was admitted to the ICU overnight. The following day the patient returned to the operating room with the surgical oncology team for planned exploratory laparotomy of the abdominal cavity in order to inspect the resection bed, achieve hemostasis, and final close the abdomen. The patient was extubated on post-operative day two and discharged to home on post-operative day 13. Postoperatively the patient was able to ambulate with a rolling walker but experienced persistent urinary incontinence and erectile dysfunction with perineal numbness. At one year follow-up, the patient continued to have urinary incontinence and radicular leg pain. His ambulation had improved and he was able to walk without assistive device with only a small limp. His surgical scar was well-healed (Figure 6).
Figure 6: Surgical scar after healing of posterior incision.
DISCUSSION
While sacral chordomas are rare, they present a unique challenge to the treating physician. Their insidious presentation requires careful attention to the patient’s symptoms, with the diagnosis often being made based on imaging workup for lumbar radiculopathy, incontinence, or erectile dysfunction. Often the symptoms are non-specific and the diagnosis is made after the tumor has grown to a large size, rendering the resection of such tumors more difficult. In contrast to osteosarcomas or chondrosarcomas, chordomas locally invade the intervertebral disc space as they spread, which can be visualized on computed tomography (CT) or MRI. Calcifications are present in 30-70% of chordomas. They are iso- or hypo-intense compared to muscle on T1 and hyperintense on T2-weighted MRI images. Definitive
CONCLUSION
The gold standard for treatment of sacral chordoma remains en bloc resection with wide margins. These tumors are often diagnosed after they have grown to considerable size in the
74 La State Med Soc VOL 170 MAY/JUNE 2018
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