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74 (); 186-194
doi:
10.1016/j.jor.2026.01.007

Nonoperative management of sacral chordomas: A systematic review of the literature

, , , , , , , , ,
a Georgetown University School of Medicine, 3800 Reservoir Rd. NW, Washington, DC, 20007, USA
b MedStar Health Research Institute, Washington, DC, 20010, USA
c Department of Orthopaedic Surgery, MedStar Georgetown University Hospital, 3800 Reservoir Rd. NW, Washington, DC, 20007, USA
d Department of Orthopaedic Surgery, MedStar Washington Hospital Center, 110 Irving St. NW, Washington, DC, 20010, USA

⁎Corresponding author: Addisu Mesfin. amesfin@gmail.com

Received: , Accepted: ,
Professor P K Surendran Memorial Education Foundation
Disclaimer:
This article was originally published by Reed Elsevier India Pvt. Ltd. and was migrated to Scientific Scholar after the change of Publisher.

Abstract

Abstract

Sacral chordomas are rare malignant tumors arising from notochordal remnants that can be identified incidentally or present with sacral pain. Although surgical resection is the accepted standard treatment, some patients forgo surgery due to associated morbidity and complications. Nonoperative alternatives, including radiotherapies, have emerged, but literature on their outcomes remains scarce. The purpose of this study was to evaluate the outcomes of nonoperative management of sacral chordomas.

We conducted a systematic review of the literature utilizing PubMed. Search keywords included “nonoperative treatment,” “sacral chordomas,” “radiotherapy,” and “functional outcomes.” Studies involving sacral chordoma patients who underwent nonoperative treatment were included; patients with surgical treatment and hybrid therapies that included surgery were excluded from the analysis.

Eleven clinical studies on nonoperative management of sacral chordomas with 52 patients aged between 35 and 85 years (mean = 62.8) were identified. Males were affected at higher rates than females (73.1 % vs 26.9 %). S1 (15.4 %) and S2 (25 %) were the most affected spinal segments. CT-guided biopsy was the most common method of tumor diagnosis (82.7 %). Carbon ion radiotherapy (CIRT) and proton beam therapy were the most common treatment methods, with 57.7 % of patients reporting recurrence-free survival at a mean follow-up of 24 months.

Nonoperative management of sacral chordomas shows promising outcomes for patients who are not candidates for surgery. Although recurrence remains a risk, nonoperative management may offer meaningful functional preservation and local disease control.

Keywords

Sacral chordoma
Spine tumor
Proton beam therapy
Carbon ion radiotherapy
Nonoperative management
Functional outcomes
1

1 Introduction

Chordomas are rare, locally aggressive tumors originating from embryonic remnants of the notochord, a mesodermal structure that proceeds to form the nucleus pulposus within the intervertebral discs of the spine. These tumors are typically found along the axial skeleton, with over 50 % of chordomas occurring in the sacrococcygeal region.1 Sacral chordomas are the most common primary malignant tumors of the sacrum, predominantly affecting males at an average age of 60-years-old at presentation.2 Since these tumors grow slowly, it is often difficult to diagnose them accurately and intervene in a timely fashion. By the time these tumors become symptomatic, they often grow to such a large size that surgical resection may no longer be possible, further complicating their management.3 While chordomas are typically categorized as low-grade malignancies, their locally aggressive nature and high recurrence rates after incomplete resection makes management challenging. Metastases are uncommon but can occur late in the clinical course, further complicating treatment.4

The current mainstay of treatment is wide, en-bloc resection with or without adjuvant radiotherapy for resectable tumors.5 Resection of large sacral chordomas carries significant risk due to the tumor's close proximity to sacral nerve roots, where wide en-bloc excision can result in severe motor, sensory, and sphincter dysfunction.6 Therefore, there is interest in nonoperative treatment options for patients with unresectable tumors or those who decline surgery because of the high risk of postoperative complications.

High-dose fractionated particle radiotherapy has emerged as a potential alternative for surgical resection in cases of unresectable sacral chordomas.7 This modality delivers precise, high linear energy transfer radiation using proton or carbon ions to maximize tumoricidal effects while minimizing damage to nearby neurovascular structures with superior local control rates and overall survival compared to conventional photon radiotherapy.7

Currently, little is known regarding the long-term efficacy of nonoperative management of sacral chordomas, and no standard of care has been established for these cases. We further describe the pathology, diagnosis, imaging characteristics, and nonsurgical treatment options for sacral chordomas. Clarifying the long-term efficacy of these approaches is essential for establishing a standardized management strategy and guiding future treatment decisions for patients with sacral chordoma.

2

2 Methods

A systematic search of PubMed was conducted to identify studies published between 1998 and 2023 reporting nonoperative management of sacral chordomas. The primary search combined the key terms “sacral chordomas” AND “radiotherapy”, followed by another search including “nonoperative treatment” and “functional outcomes”; PubMed was last consulted on October 12, 2025. We analyzed all clinical studies published before 2024.

Titles and abstracts were screened for relevance, and full-text articles were then assessed for eligibility. Studies were included if they reported patients with sacral chordomas managed nonoperatively and reported clinical outcomes. Exclusion criteria included studies in which patients underwent any surgery or chordomas affecting the rest of the spine. After full-text review, studies meeting all inclusion criteria were included in the final analysis. The study selection process, including the number of records excluded at each stage with reasons, is summarized in the PRISMA flow diagram (Fig. 1).

Article selection for the literature review of nonoperative management of sacral chordomas using PubMed.
Fig. 1 Article selection for the literature review of nonoperative management of sacral chordomas using PubMed.

Data extracted from the final studies included patient demographics, affected sacral level, symptom duration, tumor size, and diagnostic and management details. Outcome measures included survival, recurrence, and functional status.

3

3 Results

Our primary search resulted in a total of 313 records. After applying inclusion and exclusion criteria, a total of 11 studies: 6 case reports,8–13 1 case series,14 3 retrospective analyses,15–17 and 1 review18 resulted. Ultimately, 52 patients with nonoperative management of sacral chordomas were identified and included in our analysis (Table 1).

Table 1 Summary of key findings.
Author Patient age (years) Patient sex Vertebral level Symptom duration (months) Tumor size Diagnostic tool Follow-up period (months) Type of Management Outcomes
Nejmeddine Jelleli et al. (2023)8 67 M S2-S5 12 NA Biopsy NA Palliative radiotherapy, intermittent urinary self-catheterization combined with anticholinergic - No neurological disorder affecting lower limbs after 9 months.
- Rectal stenosis associated with severe constipation, increased sacral pain, improved lower urinary tract symptoms
- Increased size of the gluteal portion of sacral chordoma after 1 year.
Brooke T. Kennamer et al. (2020)9 65 M S1-S2 5 1290 cm3 Biopsy 29 Managed with supportive care only - Excellent quality of life with adequate pain management and no deterioration of neurologic status.
- Some tumor regression, but the bulk of the lesion continued to progress.
- Patient survived for 7.5 years. Died of urosepsis and new onset peritoneal metastasis.
Yuichiro Ukon et al. (2019)10 1) 73 1) M 1) S1-S5 1) NA 1) NA 1) Biopsy, MRI 1) 108 1) Carbon ion radiotherapy (70.4 GyE in 16 fractions) 1) Tumor shrank markedly and partially ossified 6 years after CIRT. Colposcopy revealed rectotumoral fistula formation 7 years after CIRT. Died of disease due to bleeding from the tumor 9 years after CIRT.
2) 55 2) M 2) S1-S5 2) 84 2) NA 2) Biopsy 2) 121 2) Carbon ion radiotherapy (70.4 GyE in 16 fractions), IV antibiotics for sepsis 2) Tumor shrank markedly and partially ossified 8 years after CIRT. Follow-up MRI and CT revealed tumor regrowth and lung metastasis 10 years after CIRT. Died of uncontrolled sepsis.
Taylor D'Amore et al. (2018)18 69 M NA NA NA MRI 96 Chemotherapy (Imatinib) - Increase in size of lesion on MRI after 7 years.'- Died of disease after 8 years.
Ziv Neeman et al. (2008)11 62 F NA 18 22 cm2 CT NA CT-guided percutaneous radiofrequency ablation - Progressive reduction in size of tumor and no evidence of local recurrence on CT scans at 2- and 6-months
- Substantial pain relief, near-complete tumor ablation.
- No clinical or radiographic evidence of rectal or nerve injury 13-months post ablation.
Esra Giray et al. (2023)12 55 M NA 2 NA EMG, MRI NA Palliative radiotherapy - Patient returned to clinic with 2 months of impaired walking and foot drop.
- MRI from 3 months prior did not show tumor recurrence.
- Electromyographic evaluation determined radiation plexitis rather than recurrent neoplastic invasion.
- One month later, patient presented with more severe pain and was re-evaluated by MRI, confirming tumor recurrence.
Bryan Shihan Ho et al. (2023)13 79 M S3-S5 6 1194 cm3 CT, MRI, Biopsy 30 Photon-based stereotactic body radiotherapy (6-fraction course) - Complete relief of symptoms after 21 months
Reiko Imai et al. (2004)15 1) 81 1) M 1) S3 1) 48 months 1) 1019 cm3 All patients were diagnosed with biopsy 1) 56 All patients were managed with carbon ion radiotherapy with 52.8–73.6GyE (gray equivalent, median 70.4) in 16 fixed fractions over 4 weeks 1) Died of gastric cancer
2) 71 2) M 2) S2-S4 2) 33 months 2) 491 cm3 2) 63 2) NA
3) 71 3) M 3) S2 3) NA 3) 714 cm3 3) 45 3) Died of pneumonia
4) 85 4) M 4) S2 4) NA 4) 902 cm3 4) 47 4) Continuous disease free
5) 65 5) M 5) S1 5) NA 5) 510 cm3 5) 44 5) Continuous disease free
6) 71 6) F 6) S1 6) NA 6) 841 cm3 6) 35 6) Continuous disease free
7) 41 7) M 7) S2 7) NA 7) 707 cm3 7) 34 7) Continuous disease free
8) 65 8) M 8) S2 8) NA 8) 997 cm3 8) 13 8) Died of brain hemorrhage
9) 51 9) M 9) S2 9) 1 month 9) 659 cm3 9) 31 9) Continuous disease free
10) 51 10) M 10) S2 10) 13 months 10) 691 cm3 10) 27 10) Continuous disease free
11) 57 11) M 11) S1 11) NA 11) 438 cm3 11) 27 11) Continuous disease free
12) 64 12) F 12) S2 12) NA 12) 413 cm3 12) 24 12) Continuous disease free
13) 69 13) M 13) S3 13) NA 13) 408 cm3 13) 21 13) Continuous disease free
14) 66 14) M 14) S2 14) NA 14) 169 cm3 14) 21 14) Continuous disease free
15) 60 15) M 15) S2 15) NA 15) 670 cm3 15) 19 15) Continuous disease free
16) 70 16) M 16) S2 16) NA 16) 407 cm3 16) 19 16) Continuous disease free
17) 51 17) M 17) S1 17) NA 17) 575 cm3 17) 17 17) Continuous disease free
18) 68 18) F 18) S1 18) NA 18) 502 cm3 18) 16 18) Continuous disease free
19) 81 19) M 19) S2 19) 10 months 19) 260 cm3 19) 15 19) Continuous disease free
20) 71 20) M 20) S1 20) NA 20) 536 cm3 20) 15 20) Continuous disease free
21) 46 21) M 21) S3 21) NA 21) 370 cm3 21) 15 21) Continuous disease free
22) 47 22) F 22) S1 22) NA 22) 293 cm3 22) 14 22) Continuous disease free
23) 63 23) M 23) S2 23) NA 23) 306 cm3 23) 11 23) Continuous disease free
24) 65 24) M 24) S2 24) NA 24) 745 cm3 24) 9 24) Continuous disease free
Lily Park et al. (2006)16 1) 67 1) M 1) S2-S5 NA 1) 6.3 cm NA 1) 91 1) High-dose proton/photon beam radiation therapy 1) No evidence of disease
2) 70 2) M 2) S2-S4 2) 8.5 cm 2) 66 2) High-dose proton/photon beam radiation therapy 2) Died of disease. Bone metastasis present.
3) 52 3) F 3) S1-S2 3) 5.6 cm 3) 59 3) High-dose photon-only beam radiation therapy 3) Alive with disease. Liver metastasis present.
4) 74 4) M 4) S1 4) 4.5 cm 4) 35 4) High-dose proton/photon beam radiation therapy 4) Alive with disease. Liver metastasis present.
5) 60 5) M 5) S3 5) 2 cm 5) 56 5) High-dose proton/photon beam radiation therapy 5) Died of disease. Lung metastasis present.
6) 84 6) F 6) S4-S5 6) 6.5 cm 6) 61 6) High-dose proton/photon beam radiation therapy 6) Died of disease. Bone metastasis present.
PS Prabhakaran et al. (1998)17 1) 55 1) F NA NA NA All patients were diagnosed with biopsy 1) 8 1) Pain-relief medication 1) Died of disease
2) 65 2) M 2) 39 2) Radical radiotherapy 2) Died of disease
3) 53 3) M 3) 36 3) Radical radiotherapy 3) Died of disease
4) 80 4) M 4) 6 4) Pain-relief medication 4) Died of disease
5) 69 5) M 5) 19 5) Radical radiotherapy 5) Alive with disease
Liang Li et al. (2020)14 1) 35 1) F 1) S1-S3 1) 35 days 1) 8.6 cm × 4.8 cm All patients were diagnosed with MRI, contrast-enhanced pelvic CT, CT-guided biopsy, and patient history. 1) 41 All patients were managed with an argon-helium based CryoHit cryoablation system. 1) Alive with local recurrence.
2) 58 2) F 2) Sacrococcygeal region 2) 30 days 2) 8.0 cm × 5.2 cm 2) 21 2) Alive with no evidence of disease.
3) 55 3) F 3) Sacrococcygeal region 3) 12 days 3) 12.5 cm × 9.1 cm 3) 6 3) Alive with no evidence of disease.
4) 60 4) F 4) Sacrococcygeal region 4) 28 days 4) 7.8 cm × 6.3 cm 4) 46 4) Alive with no evidence of disease.
5) 57 5) M 5) Sacrococcygeal region 5) 19 days 5) 11.2 cm × 7.1 cm 5) 40 5) Alive with no evidence of disease.
6) 47 6) F 6) Sacrococcygeal region 6) 11 days 6) 8.8 cm × 4.5 cm 6) 35 6) Alive with local recurrence.
7) 65 7) M 7) Sacrococcygeal region 7) 22 days 7) 8.5 cm × 5.3 cm 7) 33 7) Alive with no evidence of disease.
8) 51 8) M 8) Sacrococcygeal region 8) 28 days 8) 8.3 cm × 5.2 cm 8) 20 8) Alive with no evidence of disease.
9) 55 9) F 9) Sacrococcygeal region 9) 23 days 9) 8.6 cm × 5.5 cm 9) 10 9) Alive with no evidence of disease.

The mean age at presentation was 62.8 years (range = 35–85 years) with a clear male predominance; males accounted for 73.1 % of nonoperatively managed cases compared to 26.9 % in females (Table 2). Males also presented at a later age on average (64.6 years) compared to females (58.1 years). Anatomically, the S2 region was the most common involved level (25 %), followed by S1 and the sacrococcygeal region (15.4 % each, Table 2).

Table 2 Patient characteristics.
Demographics (n = 52)
Variable n (%)
# of Men 38 (73.1 %)
# of Women 14 (26.9 %)
Mean Male age 64.6 years (range = 41–85)
Mean Female age 58.1 years (range = 35–84)
Sacral Segment Affected
Sacral Segment n (%)
S1 8 (15.4 %)
S2 13 (25 %)
S3 4 (7.7 %)
S1-S2 2 (3.8 %)
S1-S3 1 (1.9 %)
S1-S5 2 (3.8 %)
S2-S4 2 (3.8 %)
S2-S5 2 (3.8 %)
S3-S5 1 (1.9 %)
S4-S5 1 (1.9 %)
Sacrococcygeal region 8 (15.4 %)
N/A 8 (15.4 %)

Diagnosis was primarily made via CT-guided core biopsy (78.8 %), typically using a posterior approach.18 Carbon ion radiotherapy (CIRT) was the most common treatment modality (50 %), followed by argon-helium based CryoHit cryoablation (17.3 %), high-dose proton/photon beam radiation therapy (9.6 %), supportive care (5.8 %), unclassified radical radiotherapy (5.8 %), high-dose photon-only beam radiation therapy (3.8 %), and 1 case each of chemotherapy and CT-guided percutaneous radiofrequency ablation (1.9 % each).

Final outcomes included nine patients (17.3 %) who died of sacral chordoma, five (9.6 %) who died of secondary complications, seven (13.5 %) were alive with disease, and 30 (57.7 %) were alive with no evidence of disease. Secondary complications resulting in death included urosepsis with peritoneal metastasis,9 uncontrolled sepsis,10 gastric cancer,15 pneumonia,15 and brain hemorrhage.15 Median follow-up by outcome category was 56 months for patients who died of sacral chordoma, 45 months for those who died of secondary complications, 35 months for patients alive with disease, and 21 months for patients alive with no evidence of disease. Final outcomes are summarized in Table 3.

Table 3 Patient outcomes and follow up duration after nonoperative management (n = 52).
Patient outcome n (%) Mean follow-up (months) Median follow-up (months) Range follow-up (months)
Died of sacral chordoma (primary disease) 9 (17.3 %) 52.9 56 102 (6–108)
Died of secondary complications 5 (9.6 %) 52.8 45 108 (13–121)
Alive with disease 7 (13.5 %) 37.8 35 40 (19–59)
Alive with no evidence of disease 30 (57.7 %) 24 21 85 (6–91)
No outcomes reported 1 (1.9 %) NA NA NA

Despite heterogeneous reporting of symptom resolution across studies, four studies reported instances of improvement of lower limb function and lower urinary tract symptoms,8 adequate pain management with no deterioration of neurologic status,9 near-complete tumor ablation with substantial pain relief,11 and complete relief of symptoms after 21 months.13

4

4 Discussion

4.1

4.1 Pathophysiology and molecular targets

Three histologic subtypes of chordomas are recognized: conventional, chondroid, and dedifferentiated. Conventional chordomas, accounting for roughly 75 % of cases, consistently express cytokeratin, epithelial membrane antigen (EMA), S100, and brachyury, a nuclear protein essential for notochord differentiation.19,20 Although a definitive genetic driver has not been identified for chordomas, the T-box transcription factor Brachyury, encoded by the TBXT gene on chromosome 6q27, has emerged as a defining immunohistochemical marker and supplementary diagnostic marker for chordomas.21–23 In a study by Jambhekar et al. (2010) including 51 chordomas and 58 non-chordomas, brachyury was positive in 90.2 % of chordomas with 100 % specificity and negative in all non-chordoma cases.24,25 However, it can be positive in benign notochordal tumors and absent in dedifferentiated chordomas, so its interpretation must be combined with imaging and biopsy findings.23

These tumors are relatively indolent but locally aggressive, with high rates of recurrence after incomplete resection. Chondroid chordomas also contain physaliferous cells embedded in a myxoid matrix, but may also exhibit hyaline cartilage, most commonly at the skull base.19,26 Prognosis for choroid chordomas is similar to conventional chordomas, with some reports indicating modestly improved outcomes due to their less aggressive local behavior and local recurrence rates, particularly in skull base disease.27,28 Dedifferentiated chordomas are high-grade variants with abrupt transitions to undifferentiated sarcoma, frequent metastasis (∼60 % of cases), and median overall survival of approximately 20 months.23 They lack physaliferous cells and the characteristic immunoprofile of conventional subtypes.19,23

4.2

4.2 Nonoperative management of sacral chordomas

High-dose radiotherapy refers to the delivery of a large, concentrated dose of charged particles such as protons or carbon ions to achieve local tumor control in radioresistant tumors like sacral chordomas. This approach has demonstrated high local control rates of 88 % at 5 years in unresectable chordomas.7 CIRT delivers linear energy transfer to tumors while minimizing scatter to surrounding tissues, thus creating favorable toxicity profiles compared to conventional photon-based radiotherapy. Other nonoperative treatment options include chemotherapy, molecular therapies, or no treatment, with some studies reporting patients living as long as 7.5 years with no treatment after diagnosis.29

When the risks of surgery outweigh the potential oncologic benefit, nonoperative approaches such as high-dose radiotherapy and image-guided cryoablation must be considered as therapeutic alternatives for disease control with lower morbidity.30 An illustrative example is provided by Fig. 2, involving an 81-year-old female Jehovah's Witness patient who elected proton beam radiotherapy to avoid surgical blood loss, as her beliefs prohibited blood transfusions, and surgery would have posed an unacceptable risk of violating her faith. This case underscores the benefits associated with particle radiation in medically or ethically complex patients.

Patient is an 81-year-old female with sacral chordoma with anterior extension (arrow). (a) T1 Sagittal MRI without contrast of an S3-S5 sacral chordoma. (b) Fat-suppressed T1 sagittal MRI with contrast of S3-S5 sacral chordoma; These scans were taken shortly after diagnosis of the chordoma. T2 (c) and T1 (d) sagittal MRI of the S3-S5 sacral chordoma after 1 year of proton beam radiation. The radiographic scan denotes a marked reduction in size after radiotherapy; included arrows delineate boundaries of the chordoma for comparison.
Fig. 2 Patient is an 81-year-old female with sacral chordoma with anterior extension (arrow). (a) T1 Sagittal MRI without contrast of an S3-S5 sacral chordoma. (b) Fat-suppressed T1 sagittal MRI with contrast of S3-S5 sacral chordoma; These scans were taken shortly after diagnosis of the chordoma. T2 (c) and T1 (d) sagittal MRI of the S3-S5 sacral chordoma after 1 year of proton beam radiation. The radiographic scan denotes a marked reduction in size after radiotherapy; included arrows delineate boundaries of the chordoma for comparison.
4.3

4.3 Diagnosis and imaging

The diagnosis of chordomas relies on a combination of imaging and biopsy of the tumor. Magnetic resonance imaging (MRI) is generally performed to characterize the tumor, while computed tomography (CT) scans defines its bony extent.31 Following imaging studies, a biopsy is typically performed to distinguish chordoma from morphologically similar tumors such as chondrosarcomas and benign notochordal cell tumors. Chordomas contain large vacuolated cells that correspond to the characteristic hyperintense signal seen on T2-weighted MRI (Supplementary Fig. 1).32 A preoperative CT-guided biopsy is recommended posteriorly and along the midline to accommodate surgical resection of the biopsy tract during en-bloc resection.23 These tumors show a characteristic lytic lesion on CT scans, often demonstrating well-defined, sometimes lobulated margins corresponding histologically to a pseudocapsule (Supplementary Fig. 2).33

MRI provides superior visualization of bone marrow and soft tissue invasion, with low T1 and high T2 signals reflecting the myxoid component. Soft tissue masses are consistently larger anteriorly and presacral space, with hemorrhage or proteinaceous content occasionally appearing as T1 hyperintense foci (66–77 % of cases).33 Both CT and MRI can detect extension into neural foramina, the spinal canal, and sacroiliac joints (Supplementary Fig. 1).

While en bloc surgical resection with wide margins is the gold standard for treatment of sacral chordomas, a significant number of patients are not surgical candidates due to advanced age, medical comorbidities, or tumor invasion into adjacent neurovascular structures. Surgical management protocols for sacral chordomas are well established, but the literature is limited regarding the indications, efficacy, and outcomes of nonoperative management. Our study addresses this gap by reviewing all available clinical studies on the nonoperative management of sacral chordomas from 1998 to 2023.

4.4

4.4 Systematic review

In the reviewed literature, the majority of patients with sacral chordomas were male, with a mean age in the early 60s. The most common presenting symptoms included pain and neurologic deficits, often with a prolonged duration prior to diagnosis. Diagnosis was typically established through a combination of imaging and biopsy. Nonoperative management primarily consisted of high-dose particle radiotherapy, including carbon ion and proton beam therapy, with some cases receiving image-guided cryoablation or supportive care alone.

The most frequently involved sacral level was S2 (25 %). Although tumor involvement spanned S1 to S5, lower sacral levels (S3 and below) were less frequently managed nonoperatively, reflecting the more surgically permissive anatomy in this region. Resections at these levels are technically less challenging due to the relative distance from critical neurovascular structures, and excision of sacral roots distal to S3 is generally associated with minimal motor, sensory, or sphincter deficits.34

Furthermore, the prevalence of CIRT and proton beam therapy in our dataset shows a trend toward high dose particle radiation modalities in lieu of surgery for certain patients. Both modalities deliver high-dose particle radiation – providing favorable functional outcomes and survival rates in the reviewed cases9,10,12,15 – pointing to a shared advantage over conventional photon-based therapies. For example, Imai et al. reported 5-year overall and cause-specific survival rates of 52 and 94 % respectively, with an overall local control rate of 96 %.16 While CIRT is an emerging treatment of choice from studies in Asia and Europe, it remains limited to a few specialized centers worldwide, whereas proton beam therapy exhibits greater accessibility in the U.S. setting.

Additionally, some reviewed cases such as those by Kennamer et al. and D'Amore et al. pursued supportive or pharmacologic management alone due to advanced age, comorbidities, or patient preference – prolonging over seven years of life in both studies (Table 1).10,11 Non-medical factors, particularly personal or religious convictions such as in the case of the Jehovah's Witness patient (Fig. 2), can also play a role in treatment decisions despite otherwise resectable tumors. These findings point to not only the clinical benefit, but also the psychosocial utility of nonoperative management when patient preferences are involved in treatment planning.

Our findings show that patients experience acceptable survival outcomes and functional preservation from nonoperative management of sacral chordomas, supporting the accumulating data on this approach. Low recurrence and mortality rates among patients with shorter follow-up durations may reflect favorable early responses to nonoperative management. However, the notably shorter median follow-up among patients alive with no evidence of disease (21 months) suggests that these outcomes may partly result from limited observation rather than true differences in tumor biology or treatment efficacy. Despite this limitation, the findings support nonoperative management as a reasonable option for select patients with sacral chordoma, particularly those with upper sacral involvement, contraindications, or preferences against surgery.

5

5 Limitations

The rarity of chordomas poses a limitation due to the small sample size of patients, as most available studies are small, retrospective case reports or series, resulting in a limited evidence base for nonoperative management of sacral chordomas. Treatment regimens, radiation doses, and imaging protocols varied between institutions, making direct comparisons and pooled analyses difficult. The systematic analysis of quality of life and functional outcomes were also complicated by inconsistent documentation, particularly in the reporting of presenting symptoms and symptom duration before initial treatment. Additionally, follow-up durations were highly variable, ranging from 6 to 121 months across studies, which limits the interpretation of absolute counts of deaths or recurrences, as patients with shorter follow-up may not yet have experienced late events. While our own institutional cases provide nuanced insights into potential risks such as radiation-induced sacral fracture and management in patients with surgical contraindications, the small sample size and retrospective nature of the data preclude definitive recommendations. These limitations highlight the need for larger, prospective studies with standardized protocols to better evaluate the safety, efficacy, and functional outcomes of nonoperative treatment for sacral chordomas.

6

6 Conclusion

Our literature review suggests that nonoperative treatment of sacral chordomas shows promise as a management approach, demonstrating low morbidity in select patients. Future studies should prioritize long-term follow-up and standardize data collection including associated symptoms, resolution of symptoms, and comorbidities. The exploration of biomarkers such as brachyury and INI1 that may predict responsiveness to radiotherapy is also warranted. As the need for nonsurgical treatment modalities for sacral chordomas increases globally, prospective studies will be imperative in establishing effective treatment.

Ethical approval and informed consent

No informed consent was required. Waived from IRB approval due to the nature of the study.

Data availability statement

Not applicable.

Ethical approval

This study was exempt from Institutional Review Board (IRB) review due to the nature of the study and the use of de-identified data. The study was conducted in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments.

Credit author statement

Sean Bae: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Mark Ehioghae: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Jonathan P. Japa: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Ala Alshomali: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Aaron Phung: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Linus Lee: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Justin Hyde: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Jamie Lee: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Kevin Yoon: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration. Addisu Mesfin: Conceptualization, Methodology, Validation, Formal Analysis, Investigation, Resources, Data Curation, Writing - Original Draft, Writing - Review & Editing, Visualization, Supervision, Project Administration.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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