The efficacy and safety of vincristine, irinotecan and anlotinib in Epithelioid Sarcoma.

BACKGROUND: Epithelioid sarcoma is a rare soft tissue sarcoma characterized by SMARCB1/INI1 deficiency. Much attention has been paid to the selective EZH2 inhibitor tazemetostat, where other systemic treatments are generally ignored. To explore alternative treatment options, we studied the effects of irinotecan-based chemotherapy in a series of epithelioid sarcoma patients. METHODS: We retrospectively reviewed data from patients with metastatic or unresectable epithelioid sarcoma at the Peking University People's Hospital treated with irinotecan (50 mg/m2/d d1-5 Q3W) in combination with Anlotinib (12 mg Qd, 2 weeks on and 1 week off) from July 2015 to November 2021. RESULTS: A total of 54 courses were administered. With a median follow up of 21.2 months (95% CI, 12.2, 68.1), the 5-year overall survival rate was 83.3%. Five of eight (62.5%) patients presented with unresectable localized lesions, including local tumor thrombosis and lymphatic metastasis. The other patients had unresectable pulmonary metastases. Six of eight (75%) patients had progressed following two lines of systemic therapy. The objective response rate reached 37.5% (three of eight patients) while stabilized disease was observed in 62.5% (five of eight) of patients. No patient had progressed at initial evaluation. At the last follow up, two patients were still using the combination and three patients had ceased the therapy due to toxicities such as diarrhea, nausea, and emesis. One patient changed to tazemetostat for maintenance and one patient stopped treatment due to coronavirus disease 2019 (COVID-19). Another patient stopped therapy as residual lesions had been radiated. CONCLUSIONS: The combination of irinotecan and Anlotinib as a salvage regimen may be considered another effective treatment option for refractory epithelioid sarcoma. TRIAL REGISTRATION: This study was approved in the Medical Ethics Committee of Peking University People's Hospital on October 28, 2022 (No.: 2022PHD015-002). The study was registered in Clinicaltrials.gov with identifier no. NCT05656222.

The clinical effectiveness and safety of Zone III REBOA for resection of sacropelvic tumors in patients older than 70 years.

BACKGROUND: REBOA is a method used to manage bleeding during surgery involving sacropelvic tumors. Nevertheless, studies on the use of REBOA among elderly people are lacking. The aim of this research was to investigate the efficacy and safety of Zone III REBOA in patients aged more than 70 years. METHODS: A comparative study was conducted using case-control methods. A group of patients, referred to as Group A, who were younger than 70 years was identified and paired with a comparable group of patients, known as Group B, who were older than 70 years. Continuous monitoring of physiological parameters was conducted, and blood samples were collected at consistent intervals. RESULTS: Totally, 188 participants were enrolled and received REBOA. Among the 188 patients, seventeen were aged more than 70 years. By implementing REBOA, the average amount of blood loss was only 1427 ml. Experiments were also conducted to compare Group A and Group B. No notable differences were observed in terms of demographic variables, systolic blood pressure (SBP), arterial pH, lactate levels, blood creatinine levels, potassium levels, or calcium levels at baseline. Additionally, after the deflation of the REBOA, laboratory test results, which included arterial pH, lactate, potassium concentration, calcium concentration, and blood creatinine concentration, were not significantly different (P > 0.05). CONCLUSION: This study indicated that in selected patients aged more than 70 years can achieve satisfactory hemodynamic and metabolic stability with Zone III REBOA. LEVEL OF EVIDENCE: Therapeutic study, Level III.

Renal hemodynamic evaluation protocol based on the pathophysiological mechanism of acute kidney injury: Critical Care UltraSound Guided-A(KI)BCDE.

The multiple etiological characteristics of acute kidney injury (AKI) have brought great challenges to its clinical diagnosis and treatment. Renal injury in critically ill patients always indicates hemodynamic injury. The Critical Care UltraSound Guided (CCUSG)-A(KI)BCDE protocol developed by the Chinese Critical Ultrasound Study Group (CCUSG), respectively, includes A(KI) diagnosis and risk assessment and uses B-mode ultrasound, Color doppler ultrasound, spectral Doppler ultrasound, and contrast Enhanced ultrasound to obtain the hemodynamic characteristics of the kidney so that the pathophysiological mechanism of the occurrence and progression of AKI can be captured and the prognosis of AKI can be predicted combined with other clinical information; therefore, the corresponding intervention and treatment strategies can be formulated to achieve targeted, protocolized, and individualized therapy.

Effect of Blood Flow Rate on the Accuracy of Central Venous Pressure Measurement during Continuous Renal Replacement Therapy.

BACKGROUND: The objective of this study was to study the influence of extracorporeal blood flow rate (BFR) on the accuracy of central venous pressure (CVP) measurement during continuous renal replacement therapy (CRRT). METHODS: Eligible patients were randomly divided into 3 groups based on the location of catheters used for their CRRT and CVP measurement. CVP levels measured at increased extracorporeal BFR (from 0 to 300 mL/min) in the normal and reverse positions of inlet and outlet lines connected to the CV catheter (CVC) in the course of the CRRT session were collected. RESULTS: CVP levels measured at different extracorporeal BFRs did not significantly differ between and among the 3 groups. Inversion of inlet and outlet lines connected to the catheters did not affect the accuracy of CVP measurement. BFR had a negative correlation with inflow/access pressure but a positive correlation with outflow/return pressure. Neither inflow pressure nor outflow pressure was correlated with CVP. CONCLUSIONS: Extracorporeal BFR has no influence on the accuracy of CVP measurement during CRRT with the net machine balance adjusted to zero regardless of the location of the catheter and the connection method between catheters and CRRT lines. Thus, CRRT does not need to be discontinued to obtain an accurate CVP measurement.

Efficacy and safety of a 3D-printed arthrodesis prosthesis for reconstruction after resection of the proximal humerus: preliminary outcomes with a minimum 2-year follow-up.

BACKGROUND: The purpose of this study was to investigate the feasibility of using a three-dimensional (3D)-printed arthrodesis prosthesis for reconstruction of the proximal humeral defect after tumor resection. METHODS: A novel proximal humeral prosthesis was designed to restore bone continuity and shoulder arthrodesis and was fabricated via 3D printing technology. Ten patients with primary malignancies in the proximal humerus underwent intra-articular resection and replacement with this prosthesis from 2017 to 2019. Baseline and operative data, oncological and prosthetic survival, and functional status were summarized. RESULTS: This cohort consisted of 9 males and 1 female with a mean age of 32.1 ± 16.1 years. Diagnoses included 5 cases of osteosarcoma, 3 cases of chondrosarcoma and 1 each case of undifferentiated pleomorphic sarcoma and malignant myoepithelioma. The mean operative duration, intraoperative hemorrhage and postoperative length of hospitalization were 151.5 ± 61.0 min, 410.0 ± 353.4 ml and 5.3 ± 1.9 d, respectively. The mean follow-up duration was 29.3 ± 6.4 months, with a minimum of 24 months for the surviving patients. Two patients experienced local recurrence, and four patients developed distant metastases. Detachment of the taper occurred in two patients. One was managed conservatively, and the other received amputation due to concurrent tumor recurrence. The mean MSTS-93 and ASES scores and ranges of forwards flexion and abduction were 24.9 ± 3.1, 79.4 ± 8.3, 71.3 ± 19.4°, and 61.3 ± 16.4°, respectively. The functional outcomes were independent of the preservation of the axillary nerve. Histological study of the glenoid component showed evidence of bone ingrowth at the bone-prosthesis porous interface. CONCLUSION: Application of the 3D-printed arthrodesis prosthesis might be a safe and efficacious method for functional reconstruction in patients who underwent resection of the proximal humerus, especially for those without preservation of the axillary nerve.

A clinical study of the hemodynamic and metabolic effects of Zone 3 REBOA for sacral and pelvic tumor resections.

BACKGROUND: Resuscitative endovascular balloon occlusion of the aorta (REBOA) is a key procedure in sacral and pelvic tumor resection that provides hemorrhage control. However, few studies have been performed to capture the effects of REBOA in a nonshock condition and provide a detailed description of the changes occurring with prolonged occlusion time. This study aimed to examine the hemodynamic and metabolic effects of Zone 3 REBOA for sacral and pelvic tumor resections following different periods of REBOA. METHODS: In total, 121 patients who underwent surgical tumor resections of the pelvis and/or the sacrum with the use of aortic balloon occlusion were prospectively enrolled from October 2020 to December 2021. All cases were divided into Group A (occlusion time ≤ 60 min, n = 57) and Group B (occlusion time ≥ 90 min, n = 64). Physiologic parameters were continuously recorded, and laboratory specimens were obtained at regular intervals. RESULTS: Balloon inflation resulted in a significant increase in SBP from 106 to 120 mmHg and decreased to 96 mmHg immediately following balloon deflation. With the application of REBOA, the median blood loss was only 1200 ml (range, 400-7900). When deflating the REBOA, the arterial pH was lower than baseline (7.36 vs. 7.41, p < 0.01), the arterial lactate concentration increased from 0.9 to 1.4 mmol/L (p < 0.01), serum potassium measurements increased from 3.99 to 4.12 mmol/L, serum calcium measurements decreased from 2.31 to 2.04 mmol/L, and blood creatinine decreased from 64 to 60 µmol/L. The operating time of Group B was longer than that of patients in Group A, and the patients in Group B needed more blood units to be transfused. Although laboratory measurements, including pH, potassium, calcium, and blood creatinine, were at the same level in two groups comparison, the lactate was significantly higher in Group B after deflation (p = 0.01). CONCLUSIONS: The results of this study showed that acceptable hemodynamic and metabolic stability can be attained when the occlusion time of REBOA is more than 90 min, although the long duration of occlusion caused relatively higher lactate levels.

Analysis for clinical feature and outcome of chondroblastoma after surgical treatment: A single center experience of 92 cases.

BACKGROUND: Chondroblastoma is a rare, benign, cartilaginous bone tumor derived from epiphyseal chondrocytes. Although the clinical characteristics and experience of surgical treatment of the smaller number of patients has been reported in the literature, it is difficult to draw conclusions about the clinical and radiographic features and the outcome for surgical treatment of this disease due to the rarity of chondroblastoma. This study was aiming to review the epidemiologic characteristics and outcome of surgical management for patients with chondroblastoma. METHODS: We performed a retrospective analysis of 92 patients with chondroblastoma. Clinical data, radiographic images, surgical treatment and outcome were analyzed. Eighty-two patients received the extensive intralesional curettage and ten cases had the En-block resection. RESULTS: The most common site of disease was proximal femur (20.7%, 19/92), followed by distal femur (18.5%, 17/92) and proximal tibia (16.3%, 15/92). The secondary aneurysmal bone cyst component was most common for chondroblastoma of the small irregular bones, such as patella and foot. Four (4.3%, 4/92) cases receiving the extensive intralesional curettage developed the local recurrence, respectively two at the proximal tibia, one at the pelvis and one at the calcaneus. Time to local recurrence were respectively 14.5, 8.8, 27.0 and 5.6 months, with the average 14 months. Kaplan-Meier estimated survivorship curve of local recurrence-free survival rates of one, two and five years were respectively 97.7%, 96.2% and 93.9%. The mean Musculoskeletal Tumor Society (MSTS) score was 29.4. At the last follow-up, no one had the pulmonary metastasis and death associated with the disease. CONCLUSION: Intralesional curettage plus local adjuvants can obtain satisfactory outcome for chondroblastoma. LEVEL OF EVIDENCE: Level IV.

How to confront the high prevalence of pulmonary micro nodules (PMNs) in osteosarcoma patients?

PURPOSE: Pulmonary metastasis was a negative factor of osteosarcoma prognosis. However, there is no universal criteria to confirm pulmonary metastasis at pulmonary micro nodule (PMN, Dmax ≤ 5 mm) stage other than pathology. We aimed to identify prevalence of PMNs, determine prognosis of osteosarcoma with PMNs, and analyze risk factors related to PMN progression. METHODS: We retrospectively reviewed 425 consecutive osteosarcoma patients. According to dynamic change in size and number of PMNs, patients were divided into PMN progression and non-progression group. Demographic data, initial laboratory data, radiological features, and oncological evaluations were analyzed. Cox regression was used to identify risk factors for PMN progression. Overall survival rate was measured and analyzed with Kaplan-Meier method. Differences with p < 0.05 were considered significant. RESULTS: PMNs were found in 74% (315/425) osteosarcoma patients, half of whom (157/315) suffering PMN progression. Overall survival rate was 70.2%, while survival rates for PMN progression group and non-progression group were 53.40% and 87.40%, respectively. Clinical risk factors for PMN progression in certain patients included blood vessel invasion, extrapulmonary metastases, low tumour cell necrosis rate, and large tumour size. Radiologic risk factors included greatest diameter, distance to pleura, CT value, solid components, and smooth border. CONCLUSION: PMN is quite common in osteosarcoma patients. PMN progression is related to both certain clinical and radiological factors, which could assist surgeons to determine its possibility to progress at an early stage.

Anlotinib, Vincristine, and Irinotecan for Advanced Ewing Sarcoma After Failure of Standard Multimodal Therapy: A Two-Cohort, Phase Ib/II Trial.

BACKGROUND: Both protracted irinotecan and antiangiogenesis therapy have shown promising efficacy against Ewing sarcoma (EWS). METHODS: Patients diagnosed with recurrent or refractory EWS were enrolled and further categorized into cohort A (≥16 years) or cohort B (<16 years). In the dose-defining phase Ib portion, anlotinib was given daily at a fixed dose, while a 3+3 design with dose de-escalation was used to determine the dose of irinotecan. The next dose-expanding phase II portion employed a conventional two-stage study design model. The primary endpoint was objective response rate at 12 weeks (ORR12w ). RESULTS: A total of 41 patients finally received the treatment regimen, including 29 in cohort A and 12 in cohort B. For cohort A, the first five patients were treated at the initial level of 20 mg/m2 /d d × 5 × 2, and two of them subsequently a dose-limiting toxicity (DLT). An additional six patients were then treated at 15 mg/m2 without any DLT, and the RP2D was determined. Notably, 23 out of 24 patients in cohort A were available for response evaluation at 12 weeks. ORR12w was determined to be 62.5%. For cohort B, no DLT was observed in the first six patients at the initial dose level. At last, 12 patients were included in cohort B. The ORR12w was 83.3%. The most frequently observed grade 3/4 adverse events were leukopenia (28.5%), neutropenia (24.4%), anemia (8.7%), and diarrhea (3.7%). CONCLUSION: The combination of vincristine, irinotecan, and anlotinib demonstrated an acceptable toxicity profile and promising clinical efficacy in patients with advanced EWS. IMPLICATIONS FOR PRACTICE: This is the first trial to evaluate an irinotecan-based regimen in combination with antiangiogenesis tyrosine kinase inhibitors in Ewing sarcoma (EWS). A 3+3 design with dose de-escalation was used to determine the most appropriate dose of irinotecan in each cohort. The next dose-expanding phase II portion employed a conventional two-stage study design model. The objective response rate was 62.5% for adults and 83.3% for children. Median overall survival was not matured. This study shows that the combination of vincristine, irinotecan, and anlotinib demonstrates an acceptable toxicity profile and promising clinical efficacy in patients with advanced EWS.

Iliosacral Bone Tumor Resection Using Cannulated Screw-Guided Gigli Saw - A Novel Technique.

BACKGROUND: Adequate margins are technically difficult to achieve for malignant tumors involving the sacroiliac joint due to limited accessibility and viewing window. In order to address the technical difficulties faced in iliosacral tumor resection, we proposed a technique for precise osteotomy, which involved the use of canulated screws and Gigli saw (CSGS) that facilitated directional control, anteroposterior linkage of resection points and adequate surgical margins. The purpose of the current study was to evaluate whether CSGS technique facilitated sagittal osteotomy at sacral side, and were adequate surgical margins achieved? Also functional and oncological outcomes was determined along with the noteworthy complications. METHODS: From April 2018 to November 2019, we retrospectively reviewed 15 patients who underwent resections for primary tumors of pelvis or sacrum necessitating iliosacral joint removal using the proposed CSGS technique. Chondrosarcoma was the most common diagnosis. The osteotomy site within sacrum was at ipsilateral ventral sacral foramina in 8 cases, midline of sacrum in 5 cases, and contralateral ventral sacral foramina and sacral ala with 1 case each. The average intraoperative blood loss was 3640 mL (range, 1200 and 6000 mL) with a mean operation duration of 7.4 hours (range, 5 to 12 hours). The mean follow-up was 23.0 months (range, 18 and 39 months) for alive patients. RESULTS: Surgical margins were wide in 12 patients (80%), wide-contaminated in 1 patient (6.7%), and marginal in 2 patients (13.3%). R0 resection was achieved in 12 (80%) patients and R1 resection in 3 patients. There were three local recurrences (20%) occurred at a mean time of 11 months postoperatively. No local recurrence was observed at sacral osteotomy. The overall one-year and three-year survival rate was 86.7% and 72.7% respectively.Complications occurred in three patients. CONCLUSIONS: The current study demonstrated that CSGS technique for tumor resection within the sacrum and pelvis was feasible and can achieve ideal resection accuracies. The use of CSGS was associated with high likelihood of negative margin resections in the current series. Intraoperative use of CSGS appeared to be technically straightforward and allowed achievement of planned surgical margins. It is worthwhile to consider the use of CSGS technique in resection of pelvic tumors with sacral invasion and iliosacral tumors, however further follow-up at mid to long-term is warranted to observe local recurrence rate.

Intercalary prosthetic replacement is a reliable solution for metastatic humeral shaft fractures: retrospective, observational study of a single center series.

BACKGROUND: Treatments for metastatic fracture of the humeral shaft continue to evolve as advances are made in both oncological and operative management. The purposes of this study were to critically evaluate the effectiveness of intercalary endoprostheses in treating metastatic humeral shaft fractures and to clarify the surgical indications for this technique. METHODS: Sixty-three patients treated surgically for 66 metastatic fractures of the humerus shaft were retrospectively reviewed. Intramedullary nailing (IMN) was performed in 16 lesions, plate fixation (PF) in 33 lesions, and prosthetic replacement in 17 lesions. The operative time, intraoperative blood loss, and postoperative complications were noted. The function of the upper extremities was assessed by the Musculoskeletal Tumor Society (MSTS) score and American Shoulder and Elbow Surgeons (ASES) score. All included patients were followed until reconstructive failure or death. RESULTS: The operative time was relatively shorter in the prosthesis group than in either the IMN group (p = 0.169) or PF group (p = 0.002). Notably, intraoperative blood loss was significantly less in the prosthesis group than in either the IMN group (p = 0.03) or PF group (p = 0.012). The average follow-up time was 20.3 (range, 3-75) months, and the overall survival rate was 59.7% at 12 months and 46.7% at 24 months. One rotator cuff injury, 3 cases of iatrogenic radial nerve palsy, 5 cases of local tumor progression, and 1 mechanical failure occurred in the osteosynthesis group, whereas one case of aseptic loosening of the distal stem and one case of local relapse were observed in the prosthesis group. There were no significant differences in functional scores among the three groups. CONCLUSIONS: Intercalary prosthetic replacement of the humeral shaft may be a reliable solution for pathologic fractures patients; it is indicated for lesions with substantial bone loss, or accompanied soft tissue mass, or for those patients with better prognosis.

Surgical treatment of benign osteolytic lesions in the femoral head and neck: a systematic review.

BACKGROUND AND OBJECTIVES: Treatment of benign osteolytic lesions in the femoral head and neck can be extremely challenging, particularly in children with open physis or for aggressive tumors with pathological fracture. There remains the difficult management decision as to whether to perform complete excision of the involved area or only curettage. Moreover, there is no agreed consensus on the optimal approach to lesion access when performing curettage, which included the transcervical, open and direct approach. The current systematic review aims to provide guidance for selection of surgical methods in clinical practice by comparing the advantages and drawbacks of different procedures. METHODS: A comprehensive literature search of PubMed, Embase and Web of Science databases were executed for human studies restricted to the English language. The search was filtered to include studies published from January 1980 to January 2020. RESULTS: A total of 33 articles including 274 patients were enrolled in the final analysis. The most common diagnosis was chondroblastoma (CBT) (104, 38.0%), followed by giant cell tumor (GCT) (56, 20.4%). There were 57 (20.8%) patients with pathological fracture. Intralesional curettage was performed in 257 (93.8%) patients with the local recurrence of 12.5% at the mean follow-up of 51.5 months. The patients who were presented with open physis or curetted via transcervical approach developed higher local recurrence in patients with CBT (P < 0.001). The local recurrence rate of GCT is 33.3% after curettage, while 8 of 9 (88.9%) patients with fracture were treated successfully with joint preservation. Two of 45 (4.4%) patients developed avascular necrosis (AVN) of femoral head after surgical hip dislocation. The reported Musculoskeletal Tumor Society (MSTS) Score was comparable among patients with different approaches to curettage. CONCLUSION: The majority of benign osteolytic lesions in the femoral head and neck can be treated with intralesional curettage with acceptable local tumor control and satisfactory function. The incidence of local recurrence might be decreased dramatically for lesion access under direct visualization. The native joint maintenance could be achieved even in patients with aggressive lesions presenting pathological fracture.

Assessment of the Viability and Union Feature of Diaphysis Reconstruction Using Pasteurized Tumor Bone and Intramedullary Free Fibular After Tumor Resection.

BACKGROUND: Reconstruction using pasteurized tumor bone (PTM) and intramedullary-placed vascularized free fibular (VFG) has the combined advantages of mechanical strength and biological activity. The aims of this study were to investigate scintigraphic viability and radiographic union patterns of composite grafts after surgery, union rates, functional outcomes, and complication rates. METHODS: Seventeen patients underwent intercalary composite reconstruction using PTM and VFG (12 male, 2 female, mean age: 10.9 y). Retrospective evaluation of radiography and bone scintigraphy was performed. The most common diagnosis was osteosarcoma (12/14, 85.7%). All radiography and bone scintigraphy was reviewed for bone healing and tracer uptake semiquantitatively using the ratio of the reconstructed portion to the contralateral side. Complications and oncological and functional outcomes were recorded. RESULTS: At a mean follow-up of 33.1 months, primary union was achieved in all 28 host-graft junctions in all 14 patients. The respective mean times to proximal and distal junction bone union were 6.9 and 6.5 months. Metaphyseal junctions healed faster than diaphysis junctions (5.2 vs. 8.0 mo, P=0.02). Free fibular was integrated with surrounding bone after a mean of 10.1 months. The mean tracer uptake ratio was 1.1 after a mean of 4.8 months postoperatively. Half patients (50%) had higher uptake on bone scan than contralateral side. There was no significant correlation between uptake value and bone healing time. Five complications occurred in 4 patients (28.6%), and 1 patient (7.1%) contracted an infection. No fractures or breakages were observed. The mean Musculoskeletal Tumor Society 93 score was 87.6%. CONCLUSIONS: Reconstruction using PTM combined with VFG is associated with good short-term biological activity as indicated by bone scintigraphy, a high union rate, and an acceptable complication rate. The technique is a useful reconstruction option for large segmental bone defects after tumor resection in lower extremities. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Surgical Strategy of Pediatric Benign Sacral Tumors.

BACKGROUND: Primary benign osseous tumors and tumor-like lesions at the sacrum are rare in the pediatric population and exact surgical strategy is still unclear. In this study, we evaluate the outcome for pediatric patients with benign tumors and tumor-like lesions at the sacrum who were receiving surgical treatment according to our proposed surgical strategy and classification. METHODS: We analyzed 49 pediatric patients with sacral benign tumors or tumor-like lesions aged 18 years and below from 2005 to 2018. There were 23 men and 26 women with a mean age of 14.0±3.8 years. Nineteen patients had giant cell tumors (GCTs), 9 aneurysmal bone cysts, 5 osteoblastomas, 5 neurogenic tumors, 3 hemangiomas, 3 teratomas, 2 Langerhans cell histiocytosis, 1 chondroblastoma, 1 fibrous dysplasia, and 1 GCT of tendon sheath. We proposed our surgical plan and surgical classification for pediatric patients with sacral benign tumors or tumor-like lesions. RESULTS: The mean follow-up duration was 6.2 years (range, 1.0 to 18.9 y). GCTs (39%, 19/49) and primary aneurysmal bone cysts (18%, 9/49) are the top 2 common histologic types. Preoperative selective arterial embolization (SAE) was performed in 12 cases and 24 patients received intraoperative aortic balloon occlusion (ABO) as the preoperative surgical plan. Furthermore, according to tumor location at the sacrum, we classified surgical excision of sacral benign tumors and tumor-like lesions into 3 types. Fourteen cases were classified as type I, 27 as type II, 3 as type III, and 5 patients with neurogenic tumors cannot be classified into this surgical classification. Ten patients had wound complications. Two had femoral artery thrombosis because of ABO application. One had mechanical failure. Rate of local recurrence was 16%. Seven patients with GCTs and 1 with neurogenic tumor had local recurrence. No patient died of disease at the last follow-up. For the assessment of neurological function, the rate of neurological dysfunction was 12% (6/49). Four cases had urinary incontinence, 3 fecal incontinence, and 3 had bowel obstruction. Next, univariate analysis for influence of preoperative SAE and intraoperative ABO on complications demonstrated that both of them exerted no significant influence on the occurrence of oncological and nononcological complications. CONCLUSIONS: The proposed surgical strategy can provide an excellent therapeutic effect for pediatric benign tumors and tumor-like lesions at the sacrum. Preoperative SAE and intraoperative ABO can safeguard pediatric patients with high vascularity of benign tumor at the sacrum during the operation. LEVEL OF EVIDENCE: Level IV.

Pulmonary metastasis of giant cell tumour: a retrospective study of three hundred and ten cases.

BACKGROUND: Giant cell tumour (GCT) is an invasive benign bone tumour, and the incidence of pulmonary metastasis is rare. We are aiming to analyze risk factors of pulmonary metastasis and clinical prognosis for giant cell tumour patients with pulmonary metastasis. METHOD: We performed a retrospective study of 310 patients with GCT between December 2004 and December 2016. Risk factors of pulmonary metastasis were analyzed by univariate and multivariate logistic regression analysis. Then, the influence of risk factors of overall LR (local recurrence), recurrent tumor at presentation, LR after our therapy, and with soft tissue mass on the pulmonary metastasis-free survival rates was analyzed. RESULTS: The mean follow-up of the present cohort was 45.6 ± 35.3 months (median, 36.6 months; range, 6.1-193.4 months). Eighteen (5.8%) of 310 patients developed pulmonary metastasis. The average interval from surgery of primary tumour to detection of pulmonary metastasis was 15 months. Multivariate logistic regression analysis showed overall local recurrence was the independent risk factor of developing pulmonary metastasis. Among 18 patients with pulmonary metastasis, sixteen cases had history of local recurrence (88.9%, 16/18), including eleven (68.8%, 11/16) with local recurrence at presentation before receiving our therapy and seven (43.8%, 7/16) with local recurrence after receiving treatment in our hospital. Time to local recurrence had obvious difference between patients with and without pulmonary metastasis. Patients with pulmonary metastasis were prone to recur earlier. Furthermore, overall local recurrence, local recurrence after our therapy, recurrent tumor at presentation, and tumour with a soft tissue mass showed statistical differences in the pulmonary metastasis-free survival rates. CONCLUSIONS: Giant cell tumour patients with soft tissue mass and overall local recurrence are prone to develop pulmonary metastasis. Although giant cell tumour is a benign tumor, more attention should be paid to the problem of pulmonary metastatic lesions, and chest CT scan should be recommended during follow-up.

Differences in clinical characteristics and tumor prognosis between primary and secondary conventional pelvic chondrosarcoma.

BACKGROUND: Chondrosarcoma (CS) most commonly involves the pelvis. This study aimed to analyze differences in clinical characteristics and prognostic factors between primary and secondary conventional pelvic CS, and provide reference for clinical diagnosis and treatment. METHODS: Eighty patients (54 primary cases and 26 secondary cases) with pelvic CS were included in this retrospective study. The tumor site, Enneking stage, soft tissue mass, margin, initial tumor grade, incidence of local recurrence and distant metastasis were evaluated. Kaplan-Meier method was used to calculate the overall survival rate. X2 test and log-rank test were used for univariate analysis, and Cox test was used in multivariate analysis. RESULTS: The average age of patients with secondary CS was significantly younger than that of patients with primary CS (P < 0.001). The soft tissue mass of patients with secondary CS was significantly larger than that of patients with primary CS (P = 0.002). There was a significant difference in initial tumor pathologic grade between the two groups (P = 0.002). No statistically significant difference was observed in the local recurrence rate between the two groups. The median recurrence time of patients with primary CS after the first treatment was significantly shorter than that of patients with secondary CS (P < 0.001). The overall survival rate of patients with secondary CS was much higher than that of patients with primary CS (P = 0.003). Cox regression analysis showed that the initial tumor grade was an independent factor in the overall survival rate of patients with CS. CONCLUSION: There were significant differences in age, soft tissue mass, initial tumor grade, and overall survival rate between the two groups. The overall survival rate of pelvic CS was related to the initial tumor grade of CS.

Prognosis and risk factors for malignant peripheral nerve sheath tumor: a systematic review and meta-analysis.

BACKGROUND: No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate, and LR rate for MPNST, and to assess potential risk factors for prognosis. METHODS: Electronic articles published between January 1, 1966 and February 29, 2020 were searched and critically evaluated. The authors independently reviewed the abstracts and extracted data for 5-year OS rate, 5-year EFS rate, LR rate, and potential risk factors for prognosis. RESULTS: Twenty-eight literatures were finally included for meta-analysis. The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 38%, respectively. The significant prognostic factors for survival were NF1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival. CONCLUSION: Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1, large size, deep to fascia, high grade, metastases, and location (trunk and head and neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies.

Epidemiological characteristics of 1385 primary sacral tumors in one institution in China.

BACKGROUND: Sacral tumors and tumor-like lesions are a rare group of lesions that can affect children and adults of all ages. Little is known about clinical characteristics of age, gender, histologic type, and anatomic site in China. METHODS: A total of 1385 patients with sacral tumors and tumor-like lesions, which had the clinical record at our bone tumor center from January 2000 to November 2018 were analyzed. The metastatic cancers were not included in the present study. RESULTS: A total of 51.7% (716 cases) were malignant and 48.3% (669 cases) were benign tumors or tumor-like lesions. Of malignant tumors, chordoma was the most common malignant tumor (316 cases, 22.8% of all tumors), followed by chondrosarcoma, myeloma, and other histologic types. The most common histological type of benign tumors was a giant cell tumor accounting for 14.8% (205 cases) of all tumors, followed by neurofibroma, schwannoma, and other types. The most common age group affected by malignant bone tumors was the 51- to 60-year-old group, followed by the 41- to 50-year-old group. The most commonly affected age group for benign tumors and tumor-like lesions was the 31- to 50-year-old group, followed by the 21- to 30-year old group. Furthermore, the following histologic types had gender predilection. Chordoma, chondrosarcoma, myeloma, and osteosarcoma affected more frequently males than females. Malignant peripheral nerve sheath tumor, lymphoma, giant cell tumor, neurofibroma, tuberculosis, teratoma, and epidermoid cyst more frequently affected females than males. CONCLUSIONS: The large cohort of sacral tumors and tumor-like lesions in our database may reveal their clinical characteristics of age, gender, histologic type, and anatomic site in China and features of sacral tumors and tumor-like lesions are fairly distinct from the mobile spine and extremities.

Outcome of surgical treatment of children and adolescents with primary malignant sacral tumours.

BACKGROUND: Little is known about the clinical outcome of children and adolescent patients with primary malignant sacral tumours. METHOD: We retrospectively reviewed 40 patients with malignant sacral tumours aged ≤ 18 years, receiving surgery based on the previous proposed surgical resection classification at our centre from 2003 to 2018. The following data were collected in the present study: age, gender, radiological images, detailed information of the surgical procedure, pulmonary and other metastasis at presentation, complications, local recurrence, metastasis, and death during the follow-up were recorded from the clinical and follow-up files. RESULTS: The mean follow-up was 30.7 months (range, 5.2-146.7 months). The incidence of local recurrence was 27.5% (11/40). Seven cases had surgical site infection and there were 12 cases of wound dehiscence. One had a deep venous thrombosis and one had femoral artery thrombosis. Three had fixation breakage and then received a revision. Tenty-two patients (22/40, 55%) were free of disease. A total of 13 deaths (13/40, 32.5%) were observed and the mean overall survival period was 17.1 months (range, 6.3-34.2 months), and a pulmonary metastasis occurred in 18 patients (45%, 18/45) at the 12.0 ± 10.3 months (range, 2.2-35.3 months) after initial surgery. The overall survival rates at one, two and five years were respectively 88.3%, 62.5%, and 51.9%. In the stratification analysis of young patients with primary malignant tumours at the sacrum after surgery, it revealed the influence of pathological grade, location, and age on the oncological outcomes. Kaplan-Meier estimated a survivorship curve of patients with high and low-grade malignant tumours and showed statistical differences in the overall survival and distant relapse-free survival rates between two groups. Afterwards, the result demonstrated that paediatric patients aged ≤ 14 years had worse prognosis than those aged > 14 years. CONCLUSIONS: It is satisfactory for the outcome of surgical treatment of children and adolescent patients with primary malignant sacral tumours based on proposed surgical classification. Furthermore, paediatric patients aged ≤ 14 years have the tendency of poor prognosis compared to adolescent aged > 14 years.

Lateral malleolus en bloc resection for the distal fibula osteosarcoma based on a new classification and proposed reconstruction choice: Analysis of 6 cases prognosis and literature review.

BACKGROUND: Amputation has been regarded as the standard surgical treatment for distal fibula osteosarcoma. With the advances in surgery and adjuvant chemotherapy, it have made limb salvage possible. However, the choice of a specific reconstruction procedure is frequently based on the surgeon's preference and it lacks of guidelines and high quality studies with the objective result on the subject. MATERIALS AND METHODS: Six patients with the distal fibular osteosarcoma which were received biological reconstruction were retrospectively reviewed at our bone tumor center from November 2003 to November 2015. There were 6 male with a mean age of 24.2 years (range, 12-47 years). The minimum follow-up duration was 53.3 months (median, 96.3 months; average, 108.4 months; range, 53.3-204.1 months). No patient was lost at the last follow-up. All data were obtained from the clinical and radiograph records. Furthermore, the literature review was based on the Google Scholar, Medline, EMBASE and Pubmed databases. The search was performed using the terms "distal fibula", "lower limb tumour", "sarcoma", "fibular metastasis" and "limb-salvage surgery" for the literature review from 1979 to 2017. RESULTS: Of the six patients with the final follow-up in the present study, four cases (83.3%, 4/6) achieved excellent prognosis without oncologic complications. A second surgical procedure was performed in two patients, one to treat local recurrence and one to receive metastasectomy due to the pulmonary metastasis. Case 6 received the below knee amputation due to recurrence. All the cases available for functional evaluation at the final follow-up had a mean functional MSTS score of 29.6 (range, 28-30) except case 6 receiving the amputation below the knee. Meanwhile, the VAS evaluation had a mean functional score of 0.2 points (range, 0-1 points). The results of our cohort and literature review illustrated that the patient with the malignant tumor of lateral malleolus could obtain excellent oncological prognosis and ankle function. CONCLUSIONS: We have provided treatment recommendations depending on the tumor volume and associated extent and proposed the primary ankle arthrodesis was performed after en bloc resection of Type II and III lateral malleolus osteosarcoma, based on the proposed classification. Furthermore, the patient with the malignant tumor of lateral malleolus could obtain excellent oncological prognosis.