Local and Distant Recurrence in Resected Sacral Chordomas: A Systematic Review and Pooled Cohort Analysis

Abstract

Study Design: Systematic review. Objectives: Sacral chordomas are rare, primary tumors of the spine, best treated with en bloc resection. The purpose of this study was to assess the literature for resected sacral chordoma and to quantify the prevalence of, risk factors for, and treatment outcomes of local and distant recurrence therein. Methods: We searched 5 online databases from January 1980 to May 2016 to find articles that report survival, recurrence outcomes, and/or prognostic factors for the resected sacral chordoma patient population. Characteristics and clinical outcomes of the pooled cohort are reported. Fisher exact tests, unpaired t tests, and one-way analysis of variance were used to investigate patient- and treatment-associated prognostic factors for local and distant recurrence. Survival analyses were performed for time to local recurrence and death. The protocol’s PROSPERO ID is CRD42015024384. Results: Fifty-seven studies, with 1235 unique sacral chordoma patients, were included in this review. Local and distant recurrence occurred in 42.6% and 22.4% of patients with adequate follow-up, respectively. Kaplan-Meier overall median survival for patients with and without recurrence were 98 and 209 months after surgery, respectively. Wide surgical margin was associated with a lower rate of local recurrence; and wide surgical margin, female sex, and patient age ≥65 years were associated with lower rates of distant recurrence. Conclusions: While surgical margin remains the most significant prognostic factor for local and distant recurrence, combined surgical approach may be associated with local recurrence. Male sex and age <65 years may be associated with distant recurrence. Patients with risk factors for recurrence should undergo close monitoring to maximize survival.

En bloc resection of a high cervical chordoma followed by reconstruction with a free vascularized fibular graft: illustrative case

“…The 2 patients who experienced local recurrences did so at 25 months and 83 months postoperatively, which corresponds with previous studies identifying the propensity of chordomas to recur late. 40 Historically, high cervical chordomas were most commonly treated with gross-total resection with or without adjuvant radiotherapy; however, this approach is associated with higher rates of local recurrence than en bloc excision. 4 , 9–12 , 28 , 29 , 41 Surgeons should engage patients in a detailed preoperative discussion of the risks and benefits of each approach to determine how each approach aligns with patients’ goals of care.…”

The Surgical Approach Combined With Minimally Invasive Surgery for Sacral Chordoma

“…The treatment of sacral chordoma is a surgical challenge, due to its location, size, infrequent presentation, and histological findings difficult to classify, which pose the need for a differential diagnosis from chondrosarcoma, plasmacytoma, giant cell tumor, or metastatic disease that may require a different therapeutic approach; thus, it must be treated in reference centers and by multidisciplinary groups [ 6 ].…”

“…They are usually asymptomatic or have non-specific symptoms related to compression or involvement of adjacent structures, which may delay diagnosis [5]. Recurrence and five-year overall survival rates of 63.1% and 41% have been reported, respectively [6].…”

Solitary Subcutaneous Nodule on the Eyelid: Answer

“…A recent systematic review of local and distant recurrence in resected sacral chordomas evaluated factors associated with local recurrence or distant metastasis. 3 Tumor size and surgical margin positivity were the most significant predictors of both local recurrence and distant metastasis. A patient age of less than 65 years, male sex, and use of adjuvant therapy were factors found to be significantly associated with distant metastasis.…”