Presacral Neuroendocrine Neoplasms: A Multi-site Review of Surgical Outcomes.

INTRODUCTION: Presacral neuroendocrine neoplasms (PNENs) are rare tumors, with limited data on management and outcomes. METHODS: A retrospective review of institutional medical records was conducted to identify all patients with PNENs between 2008 and 2022. Data collection included demographics, symptoms, imaging, surgical approaches, pathology, complications, and long-term outcomes. RESULTS: Twelve patients were identified; two-thirds were female, averaging 44.8 years of age, and, for the most part, presenting with back pain, constipation, and abdominal discomfort. Preoperative imaging included computed tomography scans and magnetic resonance images, with somatostatin receptor imaging and biopsies being common. Half of the patients had metastatic disease on presentation. Surgical approach varied, with anterior, posterior, and combined techniques used, often involving muscle transection and coccygectomy. Short-term complications affected one-quarter of patients. Pathologically, PNENs were mainly well-differentiated grade 2 tumors with positive synaptophysin and chromogranin A. Associated anomalies were common, with tail-gut cysts prevalent. Mean tumor diameter was 6.3 cm. Four patients received long-term adjuvant therapy. Disease progression necessitated additional interventions, including surgery and various chemotherapy regimens. Skeletal, liver, thyroid, lung, and pancreatic metastases occurred during follow-up, with no mortality reported. Kaplan-Meier analysis showed a 5-year local recurrence rate of 23.8%, disease progression rate of 14.3%, and de novo metastases rate of 30%. CONCLUSION: The study underscores the complex management of PNENs and emphasizes the need for multicenter research to better understand and manage these tumors. It provides valuable insights into surgical outcomes, recurrence rates, and overall survival, guiding future treatment strategies for PNEN patients.

SIRT1 as a potential therapeutic target in pelvic organ prolapse due to protective effects against oxidative stress and cellular senescence in human uterosacral ligament fibroblasts.

INTRODUCTION: The pathogenesis of pelvic organ prolapse (POP), an age-related disease, has not been fully elucidated. Therapeutic targets of POP are limited. Silencing information regulator 2 related enzyme 1 (SIRT1), a gene considered capable of regulating oxidative stress and cellular senescence, has been widely demonstrated involved in aging and age-related diseases. The present study aimed to explore the role of SIRT1 in POP in vivo and in vitro. METHODS: Expression levels of SIRT1 in uterosacral ligament (USL) tissues from patients with or without POP were measured using immunohistochemical assays. SRT1720, a SIRT1 agonist, was used to upregulate SIRT1, and hydrogen peroxide (H2O2) was used to establish an oxidative stress model in human uterosacral ligament fibroblasts (hUSLFs). The effects of SIRT1 on cell viability, apoptosis, senescence, and reactive oxygen species (ROS) levels were detected, respectively. Western blot assays were used to examine expression levels of apoptosis- and senescence-associated biomarkers. Unpaired Student's t test, Mann-Whitney U test, χ2 test, and one-way ANOVA were performed for determining statistically significant differences. RESULTS: Compared to the control group, expression levels of SIRT1 were downregulated in USL tissues and hUSLFs from patients with POP, and associated with stage (p < 0.05). hUSLFs of patients with POP had lower growth rates (p < 0.0001) than those of the control group, which were improved by upregulating SIRT1 (p < 0.05). The senescent proportion was higher in the POP group than the control group (43.63 ± 10.62% vs. 4.84 ± 5.32%, p < 0.0001), which could be reduced by upregulating SIRT1 (p < 0.0001). High ROS levels in the POP group were also alleviated by SRT1720. H2O2 exposure increased ROS levels, inhibited proliferation, and triggered apoptosis and senescence in hUSLFs of patients without POP in a concentration-dependent manner. Further, these damages were alleviated by pretreatment with SRT1720. CONCLUSIONS: SIRT1 is downregulated in patients with POP, and the development of SIRT1 activators or agonists may have applications in the treatment and prevention of POP through antioxidative stress and antisenescence effects.

Denosumab and sclerotherapy for recurrent spinal aneurysmal bone cyst in a child.

Aneurysmal bone cyst (ABC) is a non-malignant, locally destructive, blood-filled lesion in the bone that tends to grow aggressively. A young girl presented with a rapid recurrence after aggressive surgery of a large symptomatic sacral-spinal ABC. After a multidisciplinary tumour board, she was successfully treated with sclerotherapy and monthly intravenous denosumab. The patient has maintained asymptomatic for over 36 months now and has returned to full activity and strength. She never required surgery and has had radiologic resolution of the lesions. Treatment of recurrent ABC requires a multidisciplinary team approach. We believe this to be the first report to use this combined therapy to provide an alternative to morbid surgery for children with ABCs.

An isolated sacral promyelocytic sarcoma in a child: A rare case report with emphasis on cytomorphology.

Myeloid sarcoma (MS) is an uncommon localized extramedullary tumor composed of immature myeloid precursor cells that can affect any organ. Promyelocytic sarcoma (PS), an extremely rare subtype of MS, is characterized by immature myeloid cells with features of acute promyelocytic leukemia (APL). We describe a case of pediatric PS that presented as a solitary sacral mass without any evidence of systemic or bone marrow involvement. The cytopathologic evaluation using touch imprint demonstrated numerous blasts with bilobed nuclei, cytoplasmic hyper-granularity, and aggregates of Auer rods, which are typical cytomorphologic features of APL. Herein, we report an extremely rare case of isolated PS in a child, emphasizing the importance of cytomorphologic evaluation, which is complemented by the findings from a comprehensive work-up.

Spina bifida, the normal, the pathological and the in-between: first evidence from a forensic osteological collection.

Spina bifida (SB), a rare congenital disorder, is often mentioned as an individualizing factor in Forensic Anthropology. A lack of empirical data regarding SB is noticed in the scientific literature. Moreover, within the scope of anthropological research on SB disparities in terminology, classification systems, and methodological approaches result in incomparable results. The wide range (1,2%-50%) of "spina bifida occulta" reported prevalences is a good example. This research aims to analyze and debate the standard diagnostic criteria of SB on human skeletal remains, and attempts to elaborate on an universal system, premised on the distinction between SB as a pathology, and cleft neural arch (CNA) as an anatomical variant, according to Barnes (1994, p. 360 [1). A study-base of 209 individuals (88 males; 121 females; 44-99 years old) from the 21st Century Identified Skeletal Collection from the University of Coimbra (CEI/XXI) was macroscopically analyzed, focusing on the sacrum and remaining vertebrae. Four individuals presented complete posterior opening of the sacral canal (2,6%[4/156]). The observed bone changes, combined with the analysis of the entire skeleton, indicate that CNA, rather than SB linked to a neural tube defect, is the most reliable explanation for these cases. Overall, CNA was observed on 11 skeletons (7.05% of 156). The viability and applicability of the developed methodology for the identification of SB/CNA in forensic and/or osteological contexts are discussed, as well as the possibility of a lower prevalence of SB occulta, in the general population, than speculated before. HIGHLIGHTS: • Spina bifida has been studied so far under different methodologies, classification systems and nomenclature, leading to unstandardized and incomparable data. • Spina bifida as a pathological manifestation of a neural tube defect, as opposed to cleft neural arch as a simple form of skeletal variation. • Both spina bifida and complete sacral cleft fit the criteria of an individualizing trait in Forensic Anthropology.

A rare case of skin metastasis from a chordoma.

Chordoma is an uncommon, indolent malignant tumor arising from notochordal remnants. The incidence of distant metastasis varies between 30 and 40% in different series. Even though local involvement of the skin by direct invasion of chordoma is common, distant skin metastasis are rare, with less than 30 cases reported in the literature. The present clinical case illustrates the slow-growing natural history of a sacral chordoma, which evolved with lung metastasis, followed three years later by skin metastasis, thus giving us the opportunity to review the diagnostic approach, as well as the clinical and histopathological characteristics of this rare tumor.

Does primary tumor resection improve survival for patients with sarcomas of pelvic bones, sacrum, and coccyx who have metastasis at diagnosis ?

BACKGROUND: Recent studies demonstrated that primary tumor resection (PTR) improves survival of patients with metastatic bone sarcomas. However, it remains quite unclear regarding the role of PTR in the treatment of sarcomas of pelvic bones with synchronous metastasis at diagnosis. METHODS: Using the Surveillance, Epidemiology, and End Results Program, we enrolled a total of 385 patients with sarcomas of pelvic bones, sacrum, and coccyx who have metastasis at initial diagnosis, including 139 patients with osteosarcoma, 176 with Ewing sarcoma, and 70 with chondrosarcoma. Association between PTR and disease-specific survival (DSS) were investigated using the univariable and multivariable Cox regression models. Hazard ratio (HR) and 95% confidence interval (CI) were reported. Representative institutional PTR strategies and clinical outcomes for patients with metastatic pelvic sarcomas from our cancer center were displayed. RESULTS: The usage rate of PTR was 28.1% (39/139) in osteosarcoma, 13.6% (24/176) in Ewing sarcoma, and 41.4% (29/70) in chondrosarcoma with synchronous metastatic lesions. PTR was not associated with an improved DSS for metastatic pelvic osteosarcoma (HR = 0.686, 95% CI = 0.430 ~ 1.094, P = 0.113) and Ewing sarcoma (HR = 0.580, 95% CI = 0.291 ~ 1.154, P = 0.121). The use of PTR was associated with an improved DSS for metastatic pelvic chondrosarcoma (HR = 0.464, 95% CI = 0.225 ~ 0.954, P = 0.037). CONCLUSION: Primary lesion resection may provide a survival benefit for metastatic chondrosarcoma, but not for osteosarcoma and Ewing sarcoma of pelvic bones, sacrum, and coccyx. This population-based study recommends an active surgical intervention for metastatic chondrosarcoma while non-surgical treatment for metastatic osteosarcoma and Ewing sarcoma of the pelvis in terms of survival improvement.

Sacrectomy with Posterior-Only Approach in Sacral Tumors: An Analysis of 26 Cases and Review of Literature.

BACKGROUND: Sacral masses can be removed using anterior, posterior, or combined approaches. Achieving total sacrectomy through a posterior-only approach results in a shorter procedure time, minimal tissue damage, and a reduced risk of complications. In this study, we aimed to share our experience with performing total sacrectomy using a posterior-only approach in 26 patients and to assess their clinical outcomes at our center. MATERIALS AND METHODS: This retrospective study examines the clinical progression, surgical response, and outcomes of 26 patients with various sacral mass pathologies. We accessed patient information from our hospital records. RESULTS: The study included 14 men (54%) and 12 women (46%), with an average age of 49.8 years. Most cases had a normal body mass index, while 6 were overweight. Sacrectomy was performed at a high level in 12 patients and at a middle level in 14 patients. In addition to pain, motor deficits were observed in 9 patients, and sphincter dysfunction was found in 5. Preoperative embolization was conducted for 11 patients. The most prevalent lesions were chordoma (8 patients), malignant peripheral nerve sheath tumor (4 patients), giant cell tumor (3 patients), and solitary plasmacytoma (3 patients). Only 1 patient experienced a temporary partial motor deficit after surgery. There were no instances of cerebrospinal fluid leakage. Five patients experienced local recurrence, and 1 had distant metastasis. CONCLUSIONS: Performing sacrectomy for large or giant sacral tumors through a posterior approach is both feasible and safe, resulting in reduced morbidity and no significant change in overall survival.

Improving Surgical Outcomes in Pelvic Exenteration Surgery: Comparison of Prone Sacrectomy With Anterior Cortical Sacrectomy Techniques.

OBJECTIVE: To assess the effect of changing our sacrectomy approach from prone to anterior on surgical and oncological outcomes. BACKGROUND: In patients with advanced pelvic malignancy involving the sacrum, pelvic exenteration (PE) with en-bloc sacrectomy is the only potential curative option but morbidity is high. Over time sacrectomy techniques have evolved from prone sacrectomy (PS) to abdominolithotomy sacrectomy (ALS, ≤S3) and high anterior cortical sacrectomy (HACS, >S3) to optimize surgical outcomes. METHODS: A retrospective, single institution analysis of prospectively collected data for patients undergoing PE with en-bloc sacrectomy between 1994 and 2021 was performed. RESULTS: A total of 363 patients were identified and divided into PS (n=77, 21.2%), ALS (n=247, 68.0%), and HACS (n=39, 10.7%). Indications were: locally advanced (n=92) or recurrent (n=177) rectal cancer, primary other (n=31), recurrent other (n=60), and benign disease (n=3). PS resulted in longer operating time ( P <0.01) and more blood loss ( P <0.01). Patients with HACS had more major nerve (87.2%) and vascular (25.6%) resections ( P <0.01). Vertical rectus abdominis myocutaneous flap repair was less common following HACS (7.7%) than ALS (25.5%) and PS (27.3%) ( P =0.040). R0 rate was 80.8%, 65.8%, and 76.9% following ALS, PS, and HACS, respectively ( P =0.024). Wound-related complications and re-operations were significantly reduced following ALS and HACS compared with PS. CONCLUSIONS: Changing our practice from PS to an anterior approach with ALS or HAS has been safe and improved overall surgical and perioperative outcomes, while maintaining good oncological outcomes. Given the improved perioperative and surgical outcomes, it would be important for surgeons to learn and adopt the anterior sacrectomy approaches.

Radiotherapy after gross-total resection and subtotal resection of spinal chordoma: a SEER database analysis of overall survival outcomes.

OBJECTIVE: Chordomas are most frequently found in the sacrum, vertebral column, and skull base. Achieving gross-total resection (GTR) has been shown to optimize overall survival (OS); however, the efficacy of radiotherapy (RT) for patients with GTR is currently not well understood. Given that RT may negatively impact patient quality of life, the aim of this study was to evaluate the utility of RT for improving OS in patients who have undergone GTR of spinal chordoma through analysis of the national Surveillance, Epidemiology, and End Results (SEER) database. METHODS: The SEER database (1975-2018) was queried for all adult patients (≥ 21 years) who underwent GTR for spinal chordoma. Bivariate analysis was conducted using chi-square testing for categorical variables, and the log-rank test was performed to find the associations of clinical variables with OS. Cox proportional hazards models were generated for multivariate analyses of the associations among clinical variables and OS. RESULTS: A total of 263 spinal chordomas that underwent GTR were identified. The mean age of all included patients was 58.72 years, and 63.9% of patients were male. In addition, 0.4% had dedifferentiated histology. The mean follow-up was 75.54 months. Of all patients, 152 (57.8%) received no RT and 111 (42.2%) received RT. Patients with sacral tumor location (80.9% vs 51.4%, p < 0.001) were more likely not to undergo RT when compared to patients with vertebral column location. In multivariate analysis, only age ≥ 65 years was associated with poorer OS (HR 3.16, CI 1.54-5.61, p < 0.001). RT did not have a statistically significant association with OS. CONCLUSIONS: RT after GTR of chordoma did not improve OS among SEER chordoma patients to a value that achieved statistical significance. Additional multicenter prospective studies are needed to determine the true efficacy of RT after GTR of spinal chordoma.

Isolated cryptococcal osteomyelitis of the sacrum in an immunocompetent patient: a case report and literature review.

BACKGROUND: Cryptococcus neoformans, an opportunistic fungal pathogen, seldom causes infection in immunocompetent people. Cryptococcal osteomyelitis is an uncommon condition in which Cryptococcus invades the bone. It usually occurs as part of a disseminated infection and rarely in isolation. The spine has been reported as the most common site of cryptococcal osteomyelitis; however, isolated case of sacrum involvement in immunocompetent patients has never been reported. CASE PRESENTATION: We report the case of a 37-year-old man without underlying disease who presented with progressive low back and sacrococcygeal pain. The patient was initially diagnosed with sacral tumour by a local doctor, and subsequently, after admission, was diagnosed with sacral tuberculosis. He was empirically treated with antitubercular drugs. The patient failed to respond to antitubercular drugs and complained of worsening low back pain. Additionally, he developed persistent radiating pain and numbness in his legs. For further diagnosis, we performed a computed tomography-guided puncture biopsy of the sacrum, which revealed granulomatous inflammation with massive macrophage infiltration and special staining revealed a fungal infection. We performed sacral debridement and drainage and obtained purulent specimens for pathological examination and microbial culture. Microbial identification and drug susceptibility tests revealed a Cryptococcus neoformans infection sensitive to fluconazole. Postoperatively, the persistent radiating pain and numbness in the legs resolved. After 12 consecutive weeks of antifungal therapy, all his symptoms resolved. The patient remained without any signs of recurrence at the 8-month follow-up. CONCLUSION: We reported a rare case of isolated sacrum cryptococcal osteomyelitis in an immunocompetent patient. Furthermore, we identified and reviewed 18 published cases of spine cryptococcal osteomyelitis. Immunocompetent individuals are also at risk for cryptococcal osteomyelitis. Clinical manifestation and imaging are insufficient to diagnose cryptococcal osteomyelitis of the spine, and invasive examinations, such as puncture biopsy and fungal examinations, are needed. Antifungal therapy yields satisfactory results for the treatment of cryptococcal osteomyelitis of the spine, however, if the infective lesion is large, especially when it compresses the spinal cord and nerves, a regimen combining aggressive surgery with antifungal therapy is indispensable.

Comparative analysis of histopathological parameters, genome-wide copy number alterations, and variants in genes involved in cell cycle regulation in chordomas of the skull base and sacrum.

Chordomas are rare tumors of the axial skeleton that are refractory to conventional therapy. Few studies have compared the morphological and molecular characteristics of chordomas according to the skull base and sacral locations. Histopathological data and changes revealed by array comparative genomic hybridization (CGH) and next-generation sequencing (NGS) of cell cycle regulation genes were analyzed for 28 skull base (SBCs) and 15 sacral (SC) chordomas. All cases were conventional chordomas. SBCs were significantly more frequent in patients aged <40 years and SCs predominated in patients aged >60 years. Mitotic indices ≥2 mitoses/10 high-power fields were correlated with high degrees of nuclear atypia and Ki67 labeling indices ≥6%. We identified 321 genomic positions, and copy number variation losses were more frequent than gain. Moreover, we report a panel of 85 genetic variants of cell cycle genes and the presence of molecular clusters for chordoma as well in CGH as in NGS. These new data strengthen the view that the chordoma should not be considered as a single molecular entity.

Surgical Strategy and Application of Robotic-Assisted Benign Sacral Neurogenic Tumor Resection.

BACKGROUND: Robotic surgery may be advantageous in neurogenic sacral tumor resection but only a few studies reported robotic-assisted neurogenic sacral tumor resection. OBJECTIVE: To propose a new surgical strategy for robotic-assisted benign sacral neurogenic tumor resection and introduce the ultrasonic osteotomy surgical system in robotic surgery. METHODS: Twelve patients who had robotic-assisted primary benign sacral neurogenic tumor resection between May 2015 and March 2021 were included. Our surgical strategy divides tumors into 4 types. Type I: Presacral tumors with diameter <10 cm. Type II: Narrow-base tumors involving the sacrum with diameter <10 cm. Type III: Broad-base tumors involving the sacrum with diameter <10 cm. Type IV: Tumors involving sacral nerve roots ≥2 levels and/or with diameter ≥10 cm. RESULTS: Five type I, 5 type II, and 1 type III patients underwent tumor resection via an anterior approach, and 1 type IV patient via a combined approach. The median operation time, blood loss, and postoperative hospital stay of type I and II were much less than those of type IV. The ultrasonic osteotomy surgical system facilitated osteotomy in 2 type II and 1 type III patients. Eleven patients had total resections, and 1 type III patient had a partial resection. During the follow-up period of 7.9 to 70.9 months (median: 28.5 months), no local recurrences or deaths were noted. CONCLUSION: With the largest single-center series to our knowledge, this surgical strategy helped to guide robotic-assisted benign sacral neurogenic tumor resection. The ultrasonic osteotomy surgical system was effective for type II and III.

The impact of radiotherapy on survival after surgical resection of chordoma with minimum five-year follow-up.

BACKGROUND CONTEXT: Local control remains a vexing problem in the management of chordoma despite advances in operative techniques and radiotherapy (RT) protocols. Existing studies show satisfactory local control rates with different treatment modalities. However, those studies with minimum follow-up more than 4 years demonstrate increasing rates of local failure. Therefore, mid-term local survival rates may be inadvertently elevated by studies with less than 4 years follow-up. PURPOSE: The purpose of this study is to report the mid-term results of primary spinal chordoma treated with en bloc resection and proton-based RT with minimum 5 years of follow-up. STUDY DESIGN/SETTING: Retrospective, single-center, cohort study. PATIENT SAMPLE: Patients undergoing primary surgical excision of a spine or sacral chordoma tumor between 1990 and 2016 at a single-institution were included. Patients were included if they had a local failure at any time, or they had a minimum of 5 years of follow up with no local failure. Patients were excluded if a prior surgical excision was performed or metastases were present at the time of referral. OUTCOME MEASURES: The outcome measures were local recurrence-free interval (LRFI) and overall survival (OS). METHODS: Demographic, clinical, oncologic and surgical variables, including margin status, as well as radiation doses and schedule (neoadjuvant, adjuvant, or both) were compared using Wilcoxon rank-sum or chi-squared testing. The goal RT dose was 70 Gray (total) and patients were stratified based on completing (C70) or receiving incomplete (I70) dosing. Overall survival (OS) and local-recurrence free interval (LRFI) were calculated using the Kaplan-Meier method. FUNDING STATEMENT: No funding was obtained for this work. RESULTS: Seventy-six patients were included in the final analysis. All patients had a minimum of 5-year follow-up (median 9.3 years, range 5.1-24.7 years). There were no significant clinical differences between the C70 and I70 RT groups. OS was greater for the C70 RT group (5-year OS 82% vs. 63%, p=.001). There was similar OS for the positive margin group (5-year OS 70% vs. 61%, p=.266). LRFI was greater for the C70 RT group (5-year OS 93% vs. 78%, p=.017). There was similar LRFI for the positive margin group (5-year OS 90% versus 87%, p=.810). CONCLUSION: Chordoma outcomes trend towards diminishing LRFI rates in the literature. Here we report the results of the operative management of primary spinal chordoma with minimum five year follow-up, the addition of C70 RT to surgical excision conferred a benefit to OS and local recurrence.

A Rare Case of Melanotic Schwannoma Occurred Intraosseous of Sacrum: A Literature Review.

BACKGROUND: Melanotic schwannoma is a rare tumor when it occurs in the sacrum. Though it is mostly classified as benign, the prognosis is unpredictable due to the possibility of recurrence and metastasis. Here, we reported a case of intraosseous of sacrum with good results and reviewed the literature. CASE PRESENTATION: A 33-year-old male patient complained of low back pain and was discovered to have an obstruction at S2. Following the necessary imaging diagnosis, we treated the patient with piecemeal excision in conjunction with extended curettage, and the frozen biopsy revealed that the tumor was melanotic schwannoma. The intraosseous portion of the lesion was curettaged using high-speed drill to enlarge the edge of curettage, and piecemeal excision for lesion within the sacral canal. After surgery, the patient received total 56Gy radiotherapy and frequent follow-up. After 15 months follow-up, there was no evidence of recurrence, and the nerve function was normal. CONCLUSION: Melanotic schwannoma that occurs intraosseous of the sacrum is extremely rare and lacks typical clinical manifestations; however it can be identified through careful pathological and imaging diagnosis. Intralesional extended curettage combined with radiotherapy can achieve a good local control with a satisfactory clinical effect in this rare disease.

Sacral schwannoma: case report and review of the literature.

Schwannomas are tumors of the macroglia cells, most frequently localized to the brain, in the pontocerebellar angle. We present the case of a 53 year-old female patient who presented multiple times with diffuse abdominal pain and was initially diagnosed as having a complex right adnexal mass. Exploratory laparotomy found a retroperitoneal mass and later on, the presence of a sacral schwannoma was found, suspected initially on contrast magnetic resonance imaging of the abdomen and pelvis, and confirmed by means of lesional biopsy and histopathology. This is a rare and unusual presentation accounting for only 5% of this tumor location and poses a challenge for imaging diagnosis, directly impacting the approach to the patient and any future interventions. There are few reports in the literature about giant sacral schwannomas, but these tumors have been found to extend within the spinal space towards the vertebral space, even occupying part of the abdomen. Hence, the importance of recognizing the presence of this tumor as well as its imaging features.

Cordoma sacral metastático / Metastatic sacral chordoma

Os cordomas sacrais (CS) são tumores ósseos malignos primários da coluna vertebral de ocorrência rara, com incidência entre 0,000005-0,000027%. O objetivo deste estudo é relatar um caso de CS metastático. Homem de 41 anos, sem comorbidades, chega ao serviço de referência apresentando lesão sacral. Ressonância magnética mostrou tratar-se de tumor com 9,3 cm sugestivo de mieloma ou cordoma. Realizou-se biópsia e histopatológico, confirmando o diagnóstico de CS. O paciente submeteu-se à excisão cirúrgica do tumor. Seis meses após a cirurgia, evoluiu com recidiva e implantes metastáticos em coluna vertebral, partes moles da parede torácica, fígado e espa-ço pleural, evoluindo com paraplegia. Não havia indicação de radioterapia e/ou quimioterapia adjuvante. Não havia também possibilidade de liberação de imatinibe pelo Sistema Único de Saúde. Em cerca de 28 meses de seguimento clínico mensal, o paciente foi a óbito. O caso apresentado mostrou um CS sem sucesso cirúrgico, o que é associa-do a pior prognóstico. O paciente apresentou disseminação sistêmica do tumor e paraplegia poucos meses após a cirurgia, indo a óbito em 28 meses de seguimento. (AU)

Sacral chordomas (SC) are rare primary malignant bone tumors of the vertebral column, with an incidence between 0.000005-0.000027%. This study aims to describe a case of metastatic SC. A 42-year-old man without comorbid conditions, arrived at the referral center, presenting with a sacral lesion. MRI showed a tumor measuring 9.3 cm that was suggestive of myeloma or chordoma. A biopsy with histopathology study was performed, confirming the diagnosis of SC. The patient underwent surgical tumor excision. Six months after surgery, the tumor recurred with metastatic vertebral column implants, soft tissues of the chest wall, liver, and pleural space, and the patient developed paraplegia. There was no indication of adjuvant radiotherapy and/or chemotherapy. There was also no possibility that the Unified Health System would approve imatinib. At about 28 months of monthly clinical follow-up, the patient died. The case presented showed unsuccessful SC surgery, which is associated with a worse prognosis. The patient had systemic tumor dissemination and paraplegia a few months after surgery, dying at 28 months of follow-up. (AU)

Augmented Reality Neuronavigation for En Bloc Resection of Spinal Column Lesions.

BACKGROUND: Primary tumors involving the spine are relatively rare but represent surgically challenging procedures with high patient morbidity. En bloc resection of these tumors necessitates large exposures, wide tumor margins, and poses risks to functionally relevant anatomical structures. Augmented reality neuronavigation (ARNV) represents a paradigm shift in neuronavigation, allowing on-demand visualization of 3D navigation data in real-time directly in line with the operative field. METHODS: Here, we describe the first application of ARNV to perform distal sacrococcygectomies for the en bloc removal of sacral and retrorectal lesions involving the coccyx in 2 patients, as well as a thoracic 9-11 laminectomy with costotransversectomy for en bloc removal of a schwannoma in a third patient. RESULTS: In our experience, ARNV allowed our teams to minimize the length of the incision, reduce the extent of bony resection, and enhanced visualization of critical adjacent anatomy. All tumors were resected en bloc, and the patients recovered well postoperatively, with no known complications. Pathologic analysis confirmed the en bloc removal of these lesions with negative margins. CONCLUSIONS: We conclude that ARNV is an effective strategy for the precise, en bloc removal of spinal lesions including both sacrococcygeal tumors involving the retrorectal space and thoracic schwannomas.

Lumbopelvic shortening and local host bone-to-host bone reconstruction: a surgical method for lumbopelvic fusion following total sacrectomy.

PURPOSE: Primary sacral tumors are rare, representing fewer than 7% of spinal neoplasms. Following total sacrectomy, lumbopelvic instrumentation and fusion carries a high risk of non-union with no current consensus on fixation techniques to augment bony defects. We aim to describe the outcome of a reconstruction technique following total sacrectomy whereby lumbopelvic shortening is performed and the posterior pelvic ring is compressed to enable contact with the native L5 vertebra. METHODS: Retrospective chart review of 2 patients with 2 and 7 years post-operative follow-up. A review of hospital records including clinical assessments, complications, pathology and imaging reports. RESULTS: Patient 1 was a 17-years-old male with recurrent sacral chondrosarcoma, who presented with lumbosacral neuropathic pain and radiculopathy after failed intralesional surgery. Patient 2 was a 51-years-old male with chronic low back pain caused by a large low-grade chondroid sacral chordoma. Reconstruction technique involved mobilizing the L5 vertebra into the pelvis and pelvic ring closure to obtain host-bone-to-bone contact, eliminating the need for alternative grafts. Post-operative complications included superficial abdominal wound drainage, lower limb DVT, pulmonary emboli and deep pelvic infection. Serial CT scans demonstrated bony fusion in both patients. Neither patients had evidence of tumor recurrence and were able to ambulate at recent follow-up. Imaging demonstrated changed acetabular version of - 4.6/- 8.1 and - 14.4/- 14.8 (patient 1/2, R/L, respectively). CONCLUSION: Primary lumbopelvic shortening represents an alternative local autograft reconstructive technique for management of large sacral defects following total sacrectomy. This technique obviates the additional morbidity and surgical cost associated with the use of previously described techniques.