Chondrosarcoma of the Proximal Humerus: Does the Margin Affect Survival?

Chondrosarcoma (CS) is the second most common primary malignant bone tumour and, in the absence of reliable chemotherapy and radiotherapy, is effectively a surgical disease. Overall disease specific survival (DSS) is affected by tumour grade, whilst resection margin contributes to local recurrence free survival (LRFS). The aim of this study was to investigate factors that affect the local and systemic prognoses for conventional central CSs arising from the proximal humerus. A multi-centre, retrospective study from three international collaborative sarcoma centres identified 110 patients between 1995 and 2020 undergoing treatment for a conventional central CS of the proximal humerus; 58 patients (53%) had a grade 1 tumour, 36 (33%) had a grade 2 tumour, and 16 patients (13%) had a grade 3 CS. The mean age of patients was 50 years (range 10-85). The incidence of local recurrence (LR) was 9/110 (8.2%), and the disease specific mortality was 6/110 (5.5%). The grade was a statistically significant factor for LRFS (p < 0.001). None of the grade 1 tumours developed LR. The DSS was affected by the grade (p < 0.001) but not by the LR (p = 0.4). Only one patient with a grade 2 tumour died from the disease. The proximal humeral grade 1 CS behaved as a benign tumour, having no cases of LR nor death due to disease. Grade 2 CSs of the proximal humerus behaved in a more indolent way when compared with comparable grade tumours elsewhere in the appendicular skeleton, being locally aggressive with a higher LR rate than grade 1 CSs but still having very low mortality and a high rate of DSS. The LR in grade 2 CSs did not affect the DSS; therefore, surgical management in proximal humeral grade 2 CSs should have a greater emphasis on preserving function whilst maintaining an adequate margin for resection. The proximal humeral grade 3 CS was, as elsewhere in the skeleton, an aggressive, high-grade tumour. Therefore, surgical management should include en bloc resection with clear margins to avoid LR.

Chondrosarcoma of the Chest Wall: A Single Institution Review of 50 Cases.

INTRODUCTION: Chondrosarcoma (CS) is most common primary osseous tumor of the chest wall. The aim of this study was to report results from surgical procedures and evaluate clinical factors predicting survival of patients with chest wall CSs treated in a single tertiary sarcoma center. MATERIALS AND METHODS: Fifty patients with primary CS located in the ribs and sternum were included. Details of the clinical data and oncological outcomes, including local recurrence (LR) and disease-specific survival (DSS), were collected. RESULTS: The tumor was primarily originated in the sternum in 6 patients (12.5%) and in ribs 2 to 11 in the remaining patients. Specimens were histologically graded 1 in 13 patients (26%), 2 in 28 (56%), 3 in 8 (16%), and 1 (2%) as mesenchymal grade 3 CS. R0 margins were obtained in all cases. Reconstruction was warranted in 47 (94%) cases. Local recurrence developed in 3 (6%) patients, and the median time to LR was 17 (range, 16-68) months. Eight (16%) patients developed metastasis. Increasing tumor volume was a statistically significant factor for reduction of DSS. CONCLUSIONS: Chondrosarcoma of the chest wall can be treated effectively with clear margins, resulting in lower LR rate and higher DSS than CS of the extremities and pelvis. Metastasis of the chest wall mostly occurs in high-grade tumors, and the locations of the metastases differ greatly from those observed in CS of the extremities and pelvis. Metastases are commonly extrapulmonary, indicating the need for postoperative follow-up with multiple imaging modalities to monitor recurrence and metastases.

Preoperative coagulation biomarkers associate with survival and pulmonary embolism after surgical treatment of non-spinal skeletal metastases.

BACKGROUND: Thrombotic complications are synergistic and associated with orthopedic procedures, trauma, and malignancy. Because cancer enhances coagulation activity and vice versa, we assessed preoperative biomarkers for survival and complications after treatment of pathologic fractures in non-spinal skeletal metastases. PATIENTS/METHODS: Our study population comprised 113 actual or impending pathologic fractures in 100 patients admitted to two referral centers. Laboratory variables were collected retrospectively from patient records and analyzed related to incidence of pulmonary embolism (PE) and mortality (Kaplan-Meier and Cox regression analyses and biomarker quartiles). RESULTS: Preoperative coagulation variables were high without exceptions. PE occurred in 12 patients at 36 post-operative days at incidence of 11% in the lower and 13% in the upper extremity fractures. Patients with fibrinogen exceeding 5 g/l (log-rank 0.022) developed PE earlier (5 to 15 days postoperatively) than others. Also, mean patient survival with normal fibrinogen range (2-4 g/l) was 34 months, whereas it halved upon elevated fibrinogen (log-rank p = 0.009). Survival in patients with FVIII levels under 326 IU/dl (Q3) was 22 months, but only 7 months if FVIII exceeded 326 IU/dl (log-rank p = 0.002). Combined elevated fibrinogen and FVIII predicted survival: for patients with levels below threshold limits was 22 months versus only 7 months when both variables exceeded the ranges (log-rank p < 0.001). Multivariate analysis to control confounders supported an independent role of fibrinogen and FVIII for survival. CONCLUSIONS: Our study has established fibrinogen and FVIII as potential preoperative contributors of survival and complications after treatment of metastatic fractures. These results highlight the need for novel anticoagulation and thromboprophylaxis strategies among these patients.

Measuring functional outcome in upper extremity soft-tissue sarcoma: Validation of the Toronto extremity salvage score and the QuickDASH patient-reported outcome instruments.

Interest in functional outcome (FO) and health-related quality of life (HRQL) in extremity soft-tissue sarcoma (STS) patients has increased. The aim of this study was to validate two FO questionnaires for upper extremity STS patients: the Toronto Extremity Salvage Score (TESS) and short version of the Disability of Arm, Shoulder and Hand (QuickDASH), based on Finnish population data. A multi-center study was conducted at two academic sarcoma centers. Surgically treated upper extremity STS patients were invited to participate. Patients completed the TESS and the QuickDASH with HRQL questionnaires the 15D and the QLQ-C30. The scores were analyzed and compared. Fifty-five patients with a mean follow-up period of 4.7 years were included. Mean age was 63 years (standard deviation [SD] 14.6). The mean score for TESS was 88.5 (SD 15.1) and for QuickDASH 17.8 (SD 19.6). The QuickDASH had a statistically significantly better score coverage. A ceiling effect was noted, 27% and 20% for TESS and QuickDASH, respectively. The TESS and QuickDASH scores were strongly correlated (r= -0.89). The TESS score strongly correlated with the QLQ-C30 (r = 0.79) and the 15D score (r = 0.70). The QuickDASH score correlated strongly with the QLQ-C30 score (r=-0.71) and moderately with the 15D score (r= -0.56). The TESS score had a statistically significantly stronger correlation with the 15D score than QuickDASH (p<0.005). Both the TESS and the QuickDASH provide reliable scores for assessing FO in upper extremity STS patients. The QuickDASH has a better coverage, whereas TESS showed a stronger correlation to HRQL scores.

Clinical differences between central and peripheral chondrosarcomas.

AIMS: Chondrosarcoma is the second most common primary sarcoma of bone: conventional chondrosarcoma accounts for 85% of all cases. Conventional chondrosarcoma may be central or peripheral. Most studies group central and peripheral chondrosarcomas together, although there is growing evidence that their clinical behaviour and prognosis differ. The aims of this study were to analyze any differences in characteristics between central and peripheral chondrosarcomas and to investigate the incidence and role of different syndromes. METHODS: Data from two international tertiary referral sarcoma centres between January 1995 and December 2018 were retrospectively reviewed. The study population consisted of 714 patients with surgically treated conventional chondrosarcoma of the pelvis and limbs. RESULTS: In patients with Ollier's disease and Mafucci's syndrome, 12/20 (60%) and 2/5 (60%) of malignancies, respectively, were in the limbs, most frequently in the proximal humerus, proximal tibia, and in the hands and feet. In patients with hereditary multiple exostosis (HME), 20/29 (69.0%) of chondrosarcomas were in the pelvis and scapula, specifically in the ilium in 13/29 (44.8%) and the scapula in 3/29 (10.3%). In central chondrosarcoma, survival of patients with Ollier's disease and non-syndromic patients was the same (p = 0.805). In peripheral chondrosarcoma, survival among HME patients was similar (p = 0.676) in patients with tumours of the pelvis and limbs. CONCLUSION: Both central and peripheral chondrosarcoma have specific characteristics. HME is frequently seen in patients with a peripheral chondrosarcoma, in whom tumours are commonly located in the ilium and scapula. The incidence of Ollier's disease is uncommon in patients with a central chondrosarcoma. Disease-specific survival is equal in different subtypes after adjustment for histological grade. The local recurrence-free survival is the same for different locations and subtypes after adjustment for surgical margin. Cite this article: Bone Joint J 2021;103-B(5):984-990.

The risk of osteochondral fracture after patellar dislocation is related to patellofemoral anatomy.

PURPOSE: Despite the comprehensive literature on the anatomical risk factors for patellar dislocation, knowledge on the risk factors for subsequent osteochondral fracture (OCF) remains limited. METHODS: Magnetic resonance imaging was used to compare measures of patellofemoral anatomy in patients with OCF after patellar dislocation and propensity score matched patients without OCF. For differing measures, limit values showing a 50% probability for the occurrence of OCF were calculated using predictive logistic regression modelling. Proportions of abnormal measures in the groups were compared using Chi-square test. The association of anatomical measures with OCF location was examined by comparing subgroup mean values in the different OCF locations. RESULTS: Propensity score matching provided a total of 111 matched pairs of patients with OCF and patients without OCF. The patients with and without OCF differed in patellotrochlear index (PTI; 0.54 [95% CI 0.52-0.57] vs. 0.47 [95% CI 0.45-0.49]; p < 0.001), tibial tubercle-posterior cruciate ligament distance (TT-PCL; 21.6 mm [95% CI 21.0-22.3 mm] vs. 20.5 mm [95% CI 20.0-21.1 mm]; p = 0.013), trochlear depth (2.5 mm [95% CI 2.3-2.7 mm] vs. 3.0 mm [95% CI 2.8-3.2 mm]; p < 0.001) trochlear facet asymmetry ratio (0.54 [95% CI 0.51-0.57] vs. 0.43 [95% CI 0.42-0.45]; p < 0.001) and trochlear condyle asymmetry ratio (1.04 [95% CI 1.03-1.04] vs. 1.05 [95% CI 1.04-1.05]; 0.013. Thresholds for increased OCF risk were > 0.51 for PTI > 21.1 mm for TT-PCL < 2.8 mm for trochlear depth > 0.48 for trochlear facet asymmetry ratio and < 1.04 for trochlear condyle asymmetry ratio. CONCLUSION: In patients with OCF after patellar dislocation, trochlear configuration and patella vertical location were closer to normal anatomy, whereas patella lateralization was more severe when compared to patients without OCF. These anatomical factors contribute to the risk of OCF during patellar dislocation. LEVEL OF EVIDENCE: III.

Chondrosarcoma of the hands and feet.

METHODS: A multicentre retrospective study was carried out at two tertiary sarcoma centres. A database search identified all patients with a CS treated between January 1995 and January 2018. There were 810 CSs of which 76 (9.4%) were located in the fingers, toes, metacarpals, and metatarsal bones. RESULTS: The median age of the study population was 55 years (36 to 68) with a median follow-up of 52 months (22 to 87) months. Overall, 70% of the tumours were in the hand (n = 54) and 30% in the foot (n = 22). Predictors for LR were margin (p = 0.011), anatomical location (p = 0.017), and method of surgical management (p = 0.003). Anatomical location (p = 0.026), histological grade between 1 and 3 (p = 0.004) or 2 and 3 (p = 0.016), and surgical management (p = 0.001) were significant factors for LR-free survival. Disease-specific survival was affected by histological grade (p < 0.001), but not by LR (p = 0.397). CONCLUSION: Intralesional curettage of a low-grade CS is associated with an increased risk of LR, but LR does not affect disease-specific survival. Therefore, for low-grade CSs of the hands and feet, surgical management should aim to preserve function. In grade 2 CS, our study did not show any decreased disease-specific survival after recurrence; however, we suggest a more aggressive surgical approach to these tumours to prevent local recurrence, especially in the metacarpal and metatarsal bones. In high-grade tumours, the incidence of progressive disease is high and, therefore, the treatment of the primary tumour should be aggressive where possible, and patients observed closely for the development of metastatic disease. Cite this article: Bone Joint J 2021;103-B(3):562-568.

Soft Tissue Sarcoma of Lower Extremity: Functional Outcome and Quality of Life.

BACKGROUND: Few studies have focused on patient-related factors in analyzing long-term functional outcome and health-related quality of life (HRQoL) in patients with postoperative lower extremity soft tissue sarcoma (STS). OBJECTIVE: The purpose of this study was to investigate factors associated with postoperative functional outcome and HRQoL in patients with lower extremity STS. METHODS: This cross-sectional study was performed in a tertiary referral center using the Toronto Extremity Salvage Score (TESS), Quality-of-Life Questionnaire (QLQ)-C30 and 15 Dimension (15D) measures. Functional outcome and HRQoL data were collected prospectively. All patients were treated by a multidisciplinary team according to a written treatment protocol. RESULTS: A total of 141 patients who had undergone limb-salvage surgery were included. Depending on the outcome measure used, 19-51% of patients were completely asymptomatic and 13-14% of patients had an unimpaired HRQoL. The mean score for TESS, 15D mobility score, and QLQ-C30 Physical Functioning scale were 86, 0.83, and 75, respectively, while the mean score for 15D was 0.88, and 73 for QLQ-C30 QoL. Lower functional outcome was statistically significantly associated with higher age, higher body mass index (BMI), and the need for reconstructive surgery and radiotherapy, while lower HRQoL was statistically significantly associated with higher age, higher BMI, and reconstructive surgery. CONCLUSION: Functional outcome and HRQoL were generally high in this cross-sectional study of patients with STS in the lower extremity. Both tumor- and treatment-related factors had an impact but patient-related factors such as age and BMI were the major determinants of both functional outcome and HRQoL.

Surgical treatment of skeletal metastases in proximal tibia: a multicenter case series of 74 patients.

Background and purpose - The proximal tibia is a rare site for metastatic bone disease and is a challenging anatomical site to manage due to the proximity to the knee joint and poor soft tissue envelope. We investigated implant survival and complications of different surgical strategies in the treatment of proximal tibia pathological fractures.Patients and methods - The study comprised a 4 medical center, retrospective analysis of 74 patients surgically treated for metastases of the proximal tibia. Patient records were reviewed to identify outcome, incidence, and type of complications as well as contributing factors.Results - Reconstruction techniques comprised cement-augmented osteosynthesis (n = 33), tumor prosthesis (n = 31), and total knee arthroplasty with long cemented stems (n = 10). Overall implant survival was 88% at 6 months and 1 year, and 67% at 3 years. After stratification by technique, the implant survival was 82% and 71% at 1 and 3 years with tumor prosthesis, 100% at 1 and 3 years with total knee arthroplasty, and 91% at 1 year and 47% at 3 years with osteosynthesis. Preoperative radiotherapy decreased implant survival. Complications were observed in 19/74 patients. Treatment complications led to amputation in 5 patients.Interpretation - In this study, the best results were seen with both types of prothesis reconstructions, with good implant survival, when compared with treatment with osteosynthesis. However, patients treated with tumor prosthesis showed an increased incidence of postoperative infection, which resulted in poor implant survival. Osteosynthesis with cement is a good alternative for patients with short expected survival whereas endoprosthetic replacement achieved good medium-term results.

Further validation of the Toronto extremity salvage score for lower extremity soft tissue sarcoma based on Finnish patients.

The most widely used patient-reported outcome (PRO) measure for soft tissue sarcoma (STS) patients is the Toronto Extremity Salvage Score (TESS). The aim of the study was to validate and test the reliability of the TESS for patients with lower extremity STS based on Finnish population data. Patients were assessed using the TESS, the QLQ-C30 Function and Quality of life (QoL) modules, the 15D and the Musculoskeletal tumour Society (MSTS) score. The TESS was completed twice with a 2- to 4-week interval. The intraclass correlation coefficient (ICC) was used for test-retest reliability. Construct validity was tested for structural validity and convergent validity. Altogether 136 patients completed the TESS. A ceiling effect was noted as 21% of the patients scored maximum points. The ICC between first and second administration of the TESS was 0.96. The results of exploratory factor analysis together with high Cronbach's alpha (0.98) supported a unidimensional structure. The TESS correlated moderately with the MSTS score (rho = 0.59, p< 0.001) and strongly with the mobility dimension in the 15D HRQL instrument (rho = 0.76, p < 0.001) and the physical function in QLQ-C30 (rho = 0.83, p< 0.001). The TESS instrument is a comprehensive and reliable PRO measure. The TESS may be used as a validated single index score, for lower extremity STS patients for the measurement of a functional outcome. The TESS seems to reflect patients' HRQoL well after the treatment of lower extremity soft tissue sarcomas.

Cost-Effectiveness of Operative Versus Non-Operative Treatment for Clavicle Fracture: a Systematic Literature Review.

PURPOSE OF REVIEW: Operative and non-operative treatment of midshaft clavicle fractures seems to yield comparative functional results. Furthermore, it has been suggested that surgery is more expensive compared with non-operative treatment of clavicle fracture. Cost-effectiveness seems to be more important in trends of treatment decisions. The purpose of this study is to investigate the cost-effectiveness of clavicle fracture treatment. RECENT FINDINGS: Seven publications were selected, and 5 studies showed that operative treatment is more expensive than non-operative treatment. The mean overall cost per person in discounted prices was 10,230 USD for operative and 7923 USD for non-operative treatment. The mean absence from work ranged 8-193 and 24-69 days for operative and non-operative treatment, respectively. Studies varied in methods of assessing the cost-effectiveness of treatment modalities. Based on this literature review, routine operative treatment seems to be more expensive. In some cases, operative treatment might be more cost-effective. In all studies, direct and indirect costs of health care were calculated, but a great heterogeneity exists in the sources of cost data between countries. The cost-effectiveness of the treatment of clavicle fracture depends strongly on the cost of operative treatment and length of absence from work. Cost-effectiveness analysis could be a routine in RCT studies in the future.

Inter- and intraobserver reliability of non-weight-bearing foot radiographs compared with CT in Lisfranc injuries.

BACKGROUND: Injury of the tarsometatarsal (TMT) joint complex, known as Lisfranc injury, covers a wide range of injuries from subtle ligamentous injuries to severely displaced crush injuries. Although it is known that these injuries are commonly missed, the literature on the accuracy of the diagnostics is limited. The diagnostic accuracy of non-weight-bearing radiography (inter- or intraobserver reliability), however, has not previously been assessed among patients with Lisfranc injury. METHODS: One hundred sets of foot radiographs acquired due to acute foot injury were collected and anonymised. The diagnosis of these patients was confirmed with a CT scan. In one-third of the radiographs, there was no Lisfranc injury; in one-third, a nondisplaced (< 2 mm) injury; and in one-third, a displaced injury. The radiographs were assessed independently by three senior orthopaedic surgeons and three orthopaedic surgery residents. RESULTS: Fleiss kappa (κ) coefficient for interobserver reliability resulted in moderate correlation κ = 0.50 (95% CI: 0.45- 0.55) (first evaluation) and κ = 0.58 (95% CI: 0.52-0.63) (second evaluation). After three months, the evaluation was repeated and the Cohen's kappa (κ) coefficient for intraobserver reliability showed substantial correlation κ = 0.71 (from 0.64 to 0.85). The mean (range) sensitivity was 76.1% (60.6-92.4) and specificity was 85.3% (52.9-100). The sensitivity of subtle injuries was lower than severe injuries (65.4% vs 87.1% p = 0.003). CONCLUSIONS: Diagnosis of Lisfranc injury based on non-weight-bearing radiographs has moderate agreement between observers and substantial agreement between the same observer in different moments. A substantial number (24%) of injuries are missed if only non-weight-bearing radiographs are used. Nondisplaced injuries were more commonly missed than displaced injuries, and therefore, special caution should be used when the clinical signs are subtle. LEVEL OF EVIDENCE: III.

Modified Harrington's procedure for periacetabular metastases in 89 cases: a reliable method for cancer patients with good functional outcome, especially with long expected survival.

Background and purpose - The pelvis is the 3rd most common site of skeletal metastases. In some cases, periacetabular lesions require palliative surgical management. We investigated functional outcome, complications, and implant and patient survival after a modified Harrington's procedure.Patients and methods - This retrospective cohort study included 89 cases of surgically treated periacetabular metastases. All patients were treated with the modified Harrington's procedure including a restoration ring. Lesions were classified according to Harrington. Functional outcome was assessed by Harris Hip Score (HHS) and Oxford Hip Score (OHS). Postoperative complications, and implant and patient survival are reported.Results - The overall postoperative functional outcome was good to fair (OHS 37 and HHS 76). Sex, age, survival > 6 and 12 months, and diagnosis of the primary tumor affected functional outcome. Overall implant survival was 96% (95% Cl 88-100) at 1 year, 2 years, and 5 years; only 1 acetabular implant required revision. Median patient survival was 8 months (0-125). 10/89 patients had postoperative complications: 6 major complications, leading to revision surgery, and 4 minor complications.Interpretation - Our modified Harrington's procedure with a restoration ring to achieve stable fixation, constrained acetabular cup to prevent dislocation, and antegrade iliac screws to prevent cranial protrusion is a reliable reconstruction for periacetabular metastases and results in a good functional outcome in patients with prolonged survival. A standardized procedure and low complication rate encourage the use of this method for all Harrington class defects.

Functional Outcome Measurement in Patients with Lower-Extremity Soft Tissue Sarcoma: A Systematic Literature Review.

BACKGROUND: The importance of functional outcome (FO) in the treatment of patients with extremity soft tissue sarcoma (STS) has been increasingly recognized in the last three decades. This systematic review aimed to investigate how FO is measured in surgically treated lower-extremity STS patients. METHODS: A systematic search of PubMed, Web of Science, and Scopus was performed based on the PRISMA guidelines. The methodologic quality of the publications was measured using the MINORS tool. The results from the included studies examining measurement types, measures, and time of FO measurement were compiled. The FO pooled mean and standard deviation were calculated as a weighted average for the groups. The validity of the applied measures is reported. RESULTS: The literature search found 3461 publications, 37 of which met the inclusion criteria. The measurement types used were clinician-reported outcomes (n = 27), patient-reported outcomes (n = 20), and observer-reported outcomes (n = 2). The most frequently used measures were the Toronto Extremity Salvage Score (TESS) (n = 16) and the Musculoskeletal Tumor Society (MSTS) score 1993 (n = 12). The postoperative FO was relatively good. The pooled mean TESS and MSTS 1993 scores were respectively 83.3 and 86.2 (out of 100). Of the 10 previously reported measures, 3 provide validated FO scores. The methodologic quality of publications was generally low. CONCLUSIONS: Based on this systematic review, several different methods exist for assessing FO in patients with lower-extremity sarcoma. The most frequently used measure is a validated TESS. The postoperative FO of patients with lower-extremity STS seems to increase to the preoperative baseline level during long-term follow-up evaluation.

Revision rate of reconstructions in surgically treated diaphyseal metastases of bone.

INTRODUCTION: Skeletal metastases can weaken the bone, necessitating surgery, and surgical treatment options vary. The aim of this study was to investigate the revision rate of reconstructions in surgically treated diaphyseal skeletal metastases. MATERIALS AND METHODS: Between 2000 and 2018 at Helsinki and Tampere university hospitals in Finland, a total of 164 cases with diaphyseal skeletal metastases were identified from a prospectively maintained database. Tumor location was humerus, femur, and tibia in 106 (65%), 53 (32%), and 5 (3.0%) cases, respectively. A total of 82 (50%) cases were treated with intramedullary nailing (IMN), 73 (45%) with IMN and cementation, and 9 (5%) with another technique. RESULTS: In the upper extremity, implant survival (IS) was 96.4% at 1, 2, and 5 years; in the lower extremity, it was 83.8%, 69.1%, and 57.6% at 1, 2, and 5 years, respectively. Lower extremity IS for impending lesions was 100% at 1, 2, and 5 years, and in cases operated for true pathologic fracture, it was 71.6%, 42.9%, and 21.5% at 1, 2, and 5 years, respectively. In IMN cases without cement, the complication rate was 16% (13/82) when compared to 6% (4/73) in IMN cases with cementation. DISCUSSION: We would advocate for early intervention in patients with metastatic bone disease affecting the femur rather that watchful waiting with the risk for fracture and the need for urgent intervention. However, this choice must be balanced against the underlying risk of surgical intervention in a potentially fragile population with often limited prognoses.

Tibia Adamantinoma Resection and Reconstruction with a Custom-Made Total Tibia Endoprosthesis: A Case Report with 8-Year Follow-Up.

This case study describes a total tibia resection and reconstruction with a custom-made endoprosthetic replacement (EPR) and a long-term, 8-year follow-up. The patient underwent a total tibia adamantinoma resection in 2009. Reconstruction was performed with a custom-made total tibia EPR, where both the knee joint and ankle joint were reconstructed. Two muscle flaps, latissimus dorsi free flap and a pedicled medial gastrocnemius flap, were used for soft tissue reconstruction. The patient returned to normal life as a kindergarten teacher, without complications for eight years. This case demonstrated the importance of successful multidisciplinary teamwork in close collaboration with industry. In our best knowledge, no over 2 years of follow-up of total tibia replacement reports have been published.