How to Prevent Local Recurrence of Sacral Chordoma Treated with Carbon-Ion Radiotherapy: An Analysis of the Risk Factors of Local Failure and an Adequate Disease Margin.

INTRODUCTION: Recent reports have described the usefulness of carbon ion radiotherapy (CIRT) for inoperable sacral chordomas. However, its long-term local control rate needs to be improved. The present study identified the risk factors that affect the local relapse of sacral chordomas and the appropriate margins from the tumors. METHODS: Forty-nine patients with sacral chordoma treated with CIRT between 2011 and 2022 were retrospectively analyzed. Factors predicting the risk of local recurrence were evaluated, including age, sex, tumor size, muscle invaded with tumor, and surgery before CIRT. To determine the appropriate margin, the distance between the clinical target volume (CTV) and the out-field recurrent lesions was analyzed. RESULTS: The patients included 37 males and 12 females with a mean age of 67.1 years. A multivariate analysis showed that a tumor size >8 cm and invasion into the gluteus maximus muscle were significant risk factors with hazard ratios of 5.56 and 15.20 (p = 0.02 and 0.01), respectively. Out-field recurrence occurred in 13 cases, with 6, 3, and 4 relapses occurring in the muscle, bone, and both, respectively. The tumor occurred within 20 mm from the CTV in 60% of relapses in the muscles. CONCLUSION: The current study presented novel findings on CIRT for sacral chordomas, although there were several limitations, such as a short follow-up period to investigate slow-growth tumors and a small number of tumor specimens owing to inoperative cases. A tumor size >8 cm and invasion into the gluteus maximus muscle were shown to be risk factors for recurrence in the treatment of sacral chordoma with CIRT. Our findings further suggest that an additional 2-cm margin from the CTV in the muscle fiber direction is recommended during CIRT.

Dermoscopy of hypopigmented macules unveiling genetic diagnosis of tuberous sclerosis complex type 2 in an infant presenting with sacral chordoma.

A 2-month-old male with surgically resected sacral chordoma presented with multiple hypopigmented macules showing characteristic patchy, sharply demarcated areas of pigment network on dermoscopy. These dermoscopic findings were suggestive of the ash-leaf macules of tuberous sclerosis over other common hypopigmented macules in neonates. Chordomas presenting in early childhood in the sacral location have been reported as a rare manifestation of tuberous sclerosis complex. The combination of these findings led to a diagnosis of tuberous sclerosis, confirmed with the finding of a heterozygous TSC2 gene deletion; treatment with sirolimus resulted in regression of cardiac rhabdomyomas and hypopigmented macules.

Chordoma: Genetics and Contemporary Management.

Chordomas, arising from notochord remnants, are rare neoplasms with aggressive growth patterns despite their histologically low-grade nature. This review explores their embryological origins, molecular markers like brachyury, and genetic alterations driving pathogenesis. Diagnosis relies on advanced imaging and biopsy confirmation due to overlapping features with chondrosarcoma. The WHO classification distinguishes conventional, dedifferentiated, and poorly differentiated chordomas, each with distinct prognostic implications. Recent genomic analyses uncovered recurrent mutations in PI3K signaling pathways and chromatin remodeling genes, informing prognostic models. Surgery remains the cornerstone of treatment, though adjuvant radiation complements surgical resection. Although chordomas are generally considered refractory to medical therapy, emerging targeted molecular strategies show potential promise in ongoing trials. This review aims to provide a concise yet comprehensive overview of chordomas, guiding clinicians in diagnosis, treatment, and prognostication for improved patient outcomes.

Challenges of enbloc mid-sacrectomy as redo surgery for sacral chordoma: a case report.

Enbloc Sacrectomy is the procedure of choice for aggressive sacral lesions but not widely practiced in Pakistan, both by Neurosurgeons and Orthopaedic surgeons. Only one case has been mentioned in indexed local literature so far and that too not operated in Pakistan. The case of a 27 year old neurologically intact male is presented. He had a huge residual mass and midline non-healing wound after two attempts at intralesional debulking and one full course of local irradiation. He presented to the Mayo Hospital, Lahore on 29th December 2021 for a redo surgery of sacral chordoma. A marginal excision was achieved utilizing posterior only approach. This case will help to understand the key steps in enbloc mid-Sacrectomy and importance of involving multidisciplinary team for ensuring adequate wound closure.

Two-stage surgery for large sacrococcygeal chordomas: How I do it.

Sacrococcygeal chordoma is a malignant, slow-growing, and locally aggressive bone tumor. A wide surgical margin is recommended to prevent local recurrence and metastasis. This disease tends to cause massive defects when rectal resection and sacrectomy are required. Therefore, soft tissue reconstruction is required and a pedicled vertical rectus abdominis muscle flap (VRAM) is a viable option. Important anatomical landmarks, advantages and limitations are discussed and the procedure is described step by step. This case report presents a two-stage operation with an anterior rectal resection and VRAM flap harvest followed by a complementary posterior approach with sacrectomy and soft tissue reconstruction: approach and results. The wound completely healed in six weeks. Three years after surgery, no local recurrence or distal metastasis was detected. This two-stage strategy presents a viable and safe option for large sacrococcygeal chordomas.

High-dose proton therapy and tomotherapy for the treatment of sacral chordoma: a retrospective monocentric study.

OBJECTIVE: Radiation therapy (RT) is used for the treatment of sacral chordoma, in combination with surgery or alone for unresected tumours, to improve local control (LC) and potentially overall survival (OS). The purpose of the present study was to evaluate efficacy and toxicity of proton therapy (PT), and/or intensity modulated radiation therapy (IMRT), particularly Tomotherapy, for sacral chordoma treatment. Material: Between November 2005 and June 2018, 41 consecutive patients who were not included in clinical trials, received sacral chordoma radiation treatment in Institut Curie with Tomotherapy alone in 13 patients, and combined PT and Tomotherapy boost (Proton - Tomo) in 28 patients. RT was delivered as the exclusive local treatment in 11 patients, and as a post-operative complementary treatment in 30 patients. RESULTS: After a median follow-up of 46 months (range, 0-125 months), eight local relapses were observed, and seven patients developed distant metastasis (particularly bone and lung). The 2- and 5- year local relapse rates were 11.4% CI (0.65-22.2%) and 29% (10.5-47.4%), respectively. Over the follow-up period, ten patients died (24.4%). The estimated 2- and 5-year OS rates were 91.4% CI (82.5-100%) and 74.5% (59.4-93.5%), respectively. Fibrosis, cauda equina syndrome, and pain were the most common late toxicities. The comparison between Tomotherapy alone and Proton - Tomo revealed that acute and late cystitis were significantly more frequent in the Tomotherapy group: SHR = 0.12 IC95% (0.01-0.90 [p = .04]), as well as late proctitis. A dosimetric comparison confirmed the interest of PT to spare rectum and bladder in this context. CONCLUSION: RT remains essential to improve local control in sacral chordoma. The combination of proton and photon seems to improve organ at risk sparing, resulting in a decreased rate of reported late toxicities.

Association between patient age and the risk of mortality following local recurrence of a sacral chordoma.

BACKGROUND: Local recurrence (LR) of sacral chordoma is a difficult problem and the mortality risk associated with LR remains poorly described. The purpose of this study was to evaluate the risk of mortality in patients with LR and determine if patient age is associated with mortality. METHODS: A total of 218 patients (144 male, 69 female; mean age 59 ± 15 years) with sacrococcygeal chordomas were reviewed. Cumulative incidence functions and competing risks for death due to disease and nondisease mortality were employed to analyze mortality trends following LR. RESULTS: The 10-year overall survival (OS) was 55%. Patients with LR had 44% 10-year OS, similar to patients without (59%; P = .38). The 10-year OS between those less than 55 compared with ≥55 years were similar (69% vs 48%; P = .52). The 10-year death due to disease was worse in patients with LR compared with those without (44% vs 84%; P < .001). In patients without LR, patients ≥55 years were 1.6-fold more likely to experience death due to other causes. CONCLUSIONS: Patients with an LR are more likely to die due to disease. Advanced patient age was associated with higher all-cause mortality following resection of sacral chordoma. LR of chordoma was associated with increased disease-specific mortality, regardless of age.

Carbon Ion Radiotherapy for sacral chordoma: a systematic review of the literature.

The aim of the present study is to describe the clinical outcomes and the incidence of complications related to Carbon Ion Radiotherapy (CIRT) in the treatment of sacral chordoma. Through a systematic review of published investigations on CIRT, we collected the local control rates (LC), the overall survival rates (OS) and the post-CIRT adverse effects. Afterwards, we calculated their weighted average, to have a broader perspective. PubMed/Medline and Google Scholar databases were searched to identify studies on Carbon Ion Radiotherapy as a treatment for sacral chordoma. We used Medical Subject Heading (MeSh) terms and keywords. We based our systematic review on the PRISMA guidelines. No data limitations were applied in the search on Pubmed/ Medline database; data limitation (from 2000 to 2019) was applied in the search on Google Scholar. Six studies were included in our review. Local control proportions reported in individual studies ranged between 77% and 96% (95% confidence interval), with respect to a 5-years follow-up. Overall survival rates ranged from 52% to 86% (95% confidence interval), with respect to a 5-years follow-up. Adverse CIRT-related events involving bone occurred in 7% of patients. Neurological and skin toxicities affected 20% and 5% of patients, respectively. Nowadays the gold standard of treatment for sacral chordoma is the surgical resection with wide margins. Whenever adequate oncological margins could not be achieved or could be achieved only by sacrificing neurological structures with consequent functional impairment, CIRT is an effective alternative which has been demonstrated to reach optimal local control and overall survival rate. The caregiver, anyway, should be aware of the potential adverse events and complications related to this kind of treatment.

Sacral chordoma: clinical experience of a series of 11 patients over 18 years.

Sacral chordoma are rare low-to-intermediate grade malignant tumours that occur most commonly within the sacrum. Sacrectomy with wide resection margins seems to offer the best long-term prognosis.  This study aims to review the management of sacral chordomas including the duration of symptoms, features, treatment, complications and local recurrence rate following surgery at a tertiary centre. We retrospectively reviewed 11 patients treated at our institution between years 1999 and 2015. Patient data included age, sex, history, radiographs, surgical details, onset of recurrence, subsequent treatment, disease-free survival and overall survival were analyzed. Nine patients underwent surgical management with 1 through a sacral approach and eight patients through a combined abdominosacral approach. Despite wide resection in our series, sacral chordoma poses a major problem with approximately 60% of patients having local recurrence in their follow-up.

[Complications of surgical treatment of sacral tumors]. / Oslozhneniia khirurgicheskogo lecheniia opukholei kresttsovoi lokalizatsii.

Sacral tumors are a group of neoplasms heterogeneous in the histological type, malignancy, and growth pattern, but with common localization. Surgical treatment of these tumors is associated with the risk of major and minor complications, both during and after surgery. Usually, any surgery aimed at removing tumors in the sacrum or sacral region is associated with the need for reconstruction of bones and/or soft tissues to provide conditions for normal wound healing and the possibility of activating the patient. AIM: The study aim was to analyze complications associated with surgery for sacral tumors. MATERIAL AND METHODS: The study is based on the experience of surgical treatment of 57 patients with the diagnosis of sacral tumor. The patients underwent 60 surgical interventions related to treatment of the underlying pathology and complications of surgical treatment. Major and minor complications developed in 37 (68%) operated patients. A total of 47 different complications were recorded. In 6 patients, complications in the early postoperative period required additional surgery. CONCLUSION: Surgical treatment of sacral tumors is associated with a high risk of complications. Despite the potential risk of major complications, sacrectomy for primary, or potentially aggressive, or malignant neurogenic tumors of the sacrum is necessary to improve local control and survival of patients.