Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum

Abstract

Study Design Systematic review. Objective To determine evidence-based guidelines for the management of locally recurrent spinal chordoma. Summary of Background Data Chordoma of the spine is a low-grade malignant tumor with a strong propensity for local recurrence. Salvage therapy is challenging due to its relentless nature and refractoriness to adjuvant therapies. There are currently no guidelines regarding the best management of recurrent chordoma. Methods We combined the results of a systematic review with expert opinion to address the following research questions: (1) For locally recurrent chordoma of the spine without systemic disease, if surgery is planned, should en bloc resection be attempted if technically feasible with acceptable morbidity? (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, what is the treatment of choice? (2) Should adjuvant or neoadjuvant radiation therapy be used in the treatment of locally recurrent chordoma? Results A total of nine surgical and seven radiation therapy articles met study criteria. Evidence quality was low or very low. Recurrent disease is associated with predominantly poor outcome, regardless of treatment modality. As for primary chor-doma, resection with wide margins appears to confer an advantage with respect to local control, although this effect is attenuated in the setting of relapse. Postoperative radiation therapy likely reduces the rate of further relapse. Conclusion (1) For locally recurrent chordoma of the spine without systemic disease, when surgery is planned, wide en bloc resection should be performed if technically feasible with acceptable morbidity. Strong recommendation, Low Quality of Evidence. (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, partial resection is the treatment of choice. Weak recommendation, Very Low Quality of Evidence. (3) For the treatment of locally recurrent chordoma, high-dose conformal radiation therapy should be administered postoperatively to reduce the risk of further recurrence, and may be considered as a primary therapy. Strong recommendation, Very Low Quality of Evidence.

Role of the Apparent Diffusion Coefficient as a Predictor of Tumor Progression in Patients with Chordoma

“…Most of the reports related to chordoma evaluated primary chordomas and recurrent chordomas en bloc, but we focused on residual or recurrent chordomas. 21,29,30 Ailon et al 6 suggested that further complete surgical resection can be considered for local recurrent chordoma, even if the management of recurrent chordoma is challenging and may be palliative. We supposed that follow-up MR imaging using ADC mapping could discriminate small chordomas with an aggressive potential from those without it; this discrimination could allow a short follow-up or early salvage therapy (further surgical resection) that would likely be successful or effective.…”

“…It is important to recognize recurrence early to ensure adequate salvage therapy. 6,13 Although radiologic evaluation is important in oncology, assessment of tumor progression or response to treatment is based on changes in the residual tumor size on imaging. 14 ADC can identify further characteristics of many tumors with malignant potential.…”

“…Surgical resection is the standard therapy for chordoma, though there have been recent advances in radiation therapy (RT). [6][7][8][9][10][11] En bloc surgical resection with negative margins and no intraoperative spill is associated with a reduced rate of local recurrence. However, the location of the chordoma may limit the ability to perform a gross total resection.…”

Benign-appearing intra-spinal malignant peripheral nerve sheath tumors (mpnsts): treatments and outcomes of 14 consecutive patients

“…As a result, whether postoperative radiotherapy was beneficial for patients with an intraspinal tumor that was incompletely resected was still controversial (2,17). Tumor resection method (tumor margin) was a universally acknowledged prognostic factor for almost all resectable malignant tumors (1,4,13). In our study, most of the benign-appearing intraspinal MPNSTs were intradural extramedullary tumors (accounting for 78.6%).…”

Surgical Consideration for Patients with Repeatedly Recurrent Spinal Chordoma: A Systematic Analysis of Prognostic Factors and Quality of Life

“…Owing to its rarity, limited information regarding to clinical experience and prognostic analysis of spinal chordoma has been reported, but most of published information only focused on prognosis of primary spinal chordoma, with less attention on recurrent cases especially repeatedly recurrent cases. Tamir Ailon et al [10] systematically reviewed main published articles about recurrent spinal chordoma and obtained guidance in regard to the management of recurrent cases. Wide en bloc resection should be achieved if technically feasible with appropriate cases, and high-dose radiation therapy was suggested to administer postoperatively to reduce the risk of local recurrence.…”