Oncologic and Functional Outcome Following Sacrectomy for Sacral Chordoma

Hulen, Christopher A.; Temple, H. Thomas; Fox, William P.; Sama, Andrew A.; Green, Barth A.; Eismont, Frank J.

doi:10.2106/jbjs.d.02533

Cited by 153 publications

(99 citation statements)

References 28 publications

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“…Thus, the expected toxicity after radical resection would have been a complete urinary and bowel incontinence in at least 80% of the patients [20,21]. Furthermore chronic neuropathic pain, wound complications and walking difficulties are also possible side effects of sacrectomy [30,31]. Against this background, the published side effects after hypofractionated carbon ion therapy should be evaluated.…”

Section: Discussionmentioning

confidence: 99%

Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

et al. 2014

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BackgroundChordomas are relatively rare lesions of the bones. About 30% occur in the sacrococcygeal region. Surgical resection is still the standard treatment. Due to the size, proximity to neurovascular structures and the complex anatomy of the pelvis, a complete resection with adequate safety margin is difficult to perform. A radical resection with safety margins often leads to the loss of bladder and rectal function as well as motoric/sensoric dysfunction. The recurrence rate after surgery alone is comparatively high, such that adjuvant radiation therapy is very important for improving local control rates. Proton therapy is still the international standard in the treatment of chordomas. High-LET beams such as carbon ions theoretically offer biologic advantages in slow-growing tumors. Data of a Japanese study of patients with unresectable sacral chordoma showed comparable high control rates after hypofractionated carbon ion therapy only.Methods and designThis clinical study is a prospective randomized, monocentric phase II trial. Patients with histologically confirmed sacrococcygeal chordoma will be randomized to either proton or carbon ion radiation therapy stratified regarding the clinical target volume. Target volume delineation will be carried out based on CT and MRI data. In each arm the PTV will receive 64 GyE in 16 fractions. The primary objective of this trial is safety and feasibility of hypofractionated irradiation in patients with sacrococygeal chordoma using protons or carbon ions in raster scan technique for primary or additive treatment after R2 resection. The evaluation is therefore based on the proportion of treatments without Grade 3–5 toxicity (CTCAE, version 4.0) up to 12 months after treatment and/or discontinuation of the treatment for any reason as primary endpoint. Local-progression free survival, overall survival and quality of life will be analyzed as secondary end points.DiscussionThe aim of this study is to confirm the toxicity results of the Japanese data in raster scan technique and to compare it with the toxicity analysis of proton therapy given in the same fractionation. Using this data, a further randomized phase III trial is planned, comparing hypofractionated proton and carbon ion irradiation.Trial registrationClinicalTrials.gov Identifier: NCT01811394.

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Section: Discussionmentioning

confidence: 99%

Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

et al. 2014

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“…Our results confirm that chances to obtain an R0 resection are higher for patients who have an accurate preoperative diagnosis and who undergo an aggressive multidisciplinary treatment rather than for patients who have been misdiagnosed and are in need of secondary surgery [3, 4, 11, 13]. The quality of surgical margins at initial surgery is the primary prognostic factor for LR [7, 11, 12, 17, 18, 19], with the 10-year OS rates ranging between 30% and 65% depending on the surgical margins [5, 7, 8, 9, 10, 11, 12]. A review of the recent literature shows LR rates ranging between 0% and 60% for wide margins, between 31% and 71% for marginal margins, and between 67% and 100% for intralesional resection margins (Table 3).…”

Section: Discussionmentioning

confidence: 99%

“…Distant disease is less frequent (20%-40%), but metastases to the bone, lung, liver, lymph-nodes and skin have been described [3, 4, 5, 6, 7]. The 10-year overall survival (OS) rate ranges from 30% to 65% [5, 7, 8, 9, 10, 11, 12]. Radical surgery is the only hope for cure as no chemotherapy has been demonstrated to be effective against a chordoma, and radiotherapy is only marginally effective [5, 7, 9, 13, 14, 15].…”

Section: Introductionmentioning

confidence: 99%

The Unresolved Case of Sacral Chordoma: From Misdiagnosis to Challenging Surgery and Medical Therapy Resistance

et al. 2014

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PurposeA sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status.MethodsEight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension.ResultsSeven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years).ConclusionOur experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center.

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“…In adult cases, the clinical course generally shows indolent progression (1,2,7). Certain studies have reported that the local recurrence rates in adult cases were 33–71% (8,9) and Hulen et al (10) identified that following a surgical procedure the mean time of first recurrence was 29 months (range, 12–66 months) and the mean time of metastases was 50 months (range, 16–122 months). In addition, previous studies demonstrated that the five- and 10-year overall survival rates of chordoma patients were 74–83 and 50–56%, respectively (8,11,12).…”

Section: Introductionmentioning

confidence: 99%

Sacral chordoma in an adult showing an aggressive clinical course: A case report

et al. 2014

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The current report presents a case of a 78-year-old male with sacral chordoma, showing an aggressive clinical course. The patient underwent sacral resection, however, nine months later, multiple metastases were detected by magnetic resonance imaging. The metastases progressed rapidly and 15 months following surgery the patient succumbed to respiratory dysfunction. An autopsy revealed multiple metastases of the lung, liver, heart, kidneys and vertebrae. Pathologically, the tumors did not show proliferation of anaplastic cells or dedifferentiation; however, the metastatic tumor cells were smaller than the primary tumor cells. The Ki-67 labeling indices were <5% in all of the patient’s tumors, therefore, the capacity for cellular proliferation of the tumors was considered to be low. Chordoma in adults are generally slow-growing tumors and are associated with a relatively prolonged course and frequent local recurrences. Therefore, it must be recognized that chordoma may grow rapidly and show an aggressive clinical course, even when the Ki-67 labeling index is low.

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Oncologic and Functional Outcome Following Sacrectomy for Sacral Chordoma

Abstract: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.

Cited by 153 publications

References 28 publications

Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

Randomized phase II trial of hypofractionated proton versus carbon ion radiation therapy in patients with sacrococcygeal chordoma-the ISAC trial protocol

The Unresolved Case of Sacral Chordoma: From Misdiagnosis to Challenging Surgery and Medical Therapy Resistance

Sacral chordoma in an adult showing an aggressive clinical course: A case report

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