Favorable clinical efficacy of cytotoxic chemotherapy in patients with progressive desmoid tumors: a retrospective real-world study.

BACKGROUND: The real-world evidence about the efficacy of cytotoxic chemotherapy in desmoid tumors is still limited. We investigated the efficacy of chemotherapy in the treatment of recurrent or progressive desmoid tumors. METHODS: The patients with desmoid tumors who had received cytotoxic chemotherapy between November 2007 and June 2020 in two tertiary hospitals in Korea were reviewed. RESULTS: A total of 25 patients were included in the analysis. The most common primary tumor site was the intra-abdominal or pelvic cavity (56%), followed by the trunk and abdominal wall (24%), extremities (16%), and head and neck (4%). Sixty percent of the patients had familial adenomatous polyposis and 76% received doxorubicin plus dacarbazine. The objective response rate and disease control rate was 64% (95% confidence interval [CI]: 40.7-82.8) and 96% (95% CI: 77.2-99.9), respectively. With the median follow-up time of 55 months (95% CI: 41.0-68.2), the 3-year PFS rate was 65% (95% CI: 41.1-80.5), and the 3-year OS rate was 89% (95% CI: 63.8-97.3). Grade 3 or 4 hematologic adverse events were reported in 14 patients, all of which were manageable. CONCLUSION: Our real-world evidence suggests that doxorubicin-based cytotoxic chemotherapy can be an effective treatment option for recurrent and progressive desmoid tumors with respect to favorable clinical outcomes.

High-dose chemotherapy followed by autologous stem cell transplantation in pediatric patients with relapsed osteosarcoma.

BACKGROUND: Patients with relapsed osteosarcoma have poor treatment outcomes. High-dose chemotherapy with autologous stem cell transplantation (HDCT/ASCT) has been used in several high-risk malignant solid tumors; however, few studies have evaluated their role in treating osteosarcoma. We evaluated the effectiveness of HDCT/ASCT in relapsed pediatric osteosarcoma cases. PROCEDURE: We retrospectively reviewed the medical records of 40 patients diagnosed with and treated for relapsed osteosarcoma at Asan Medical Center and Samsung Medical Center from January 1996 to July 2019. RESULTS: The median age of this cohort was 13.4 years (range: 6.1-18.2). The cohort's 5-year overall survival (OS) was 51.0% ± 0.1% during a median follow-up period of 67.5 months. Twenty-five patients (62.5%) achieved complete remission (CR) with salvage treatment, and the 5-year OS was 82.4% ± 0.1%, whereas none of the remaining 15 patients who did not achieve CR survived (p < .0001). Of the 25 CR cases, 15 underwent subsequent HDCT/ASCT. We compared the effect of HDCT/ASCT among patients who achieved CR. There were no significant differences in the 5-year OS outcomes between patients who did and did not receive HDCT/ASCT (83.9% ± 0.1%, 13/15 vs. 80.0% ± 0.1%, 8/10, respectively; p = .923). CONCLUSION: To our knowledge, we report the first comparative cohort study that proved HDCT/ASCT does not significantly improve survival outcomes in relapsed osteosarcoma. Achievement of CR remains the most crucial factor for good survival outcomes.

Pathological fractures of the femur after radiation therapy for soft tissue tumor: a case series of seven patients treated with repeated internal fixation.

INTRODUCTION: Pathological femoral fracture (PFF) after radiation therapy (RT) for soft tissue tumor is a debilitating complication with a high rate of nonunion that requires multiple subsequent procedures and hindrance of functional activity. We aimed to evaluate healing rate after repeated internal fixation in a case series of PFF after RT. MATERIALS AND METHODS: We retrospectively reviewed the records of patients who had PFF after RT and were treated at our center between 2007 and 2018. We analyzed our surgical protocols and fracture healing rate. INTERPRETATION: We identified and analyzed a total of seven patients (six females and one male) whose mean age at fracture was 58 years. Primary tumors consisted of soft tissue sarcoma (n = 5), melanoma (n = 1), and desmoid tumor (n = 1). All primary tumors were treated with surgical excision and adjuvant RT. The mean follow-up duration after fracture was 33 months (range, 16-58). At the last follow-up, five out of seven fractures had completely healed, and two lesions showed delayed union at 15 and 16 months, respectively. Among the five complete unions, one fracture was healed after the first fixation. The remaining four lesions were treated with second internal fixation due to nonunion/metal failure or delayed union. The mean time to radiologic healing after the last fixation was 6.8 months (range, 5-8). CONCLUSIONS: Our results suggest that bones within the RT field retain healing capacities, and that repeated internal fixation may be a viable option for the treatment of PFF after RT.

Oncologic safety of propeller flap and free flap in reconstruction after soft tissue sarcoma resection.

BACKGROUND AND OBJECTIVES: The propeller flap is a reliable option for reconstruction after soft tissue sarcoma resection. However, some parts of the resection margin may move away from its original position during flap rotation and thus can be excluded from the clinical target volume of adjuvant radiotherapy. This study aimed to evaluate local recurrence after soft tissue sarcoma resection with propeller flap or free flap reconstruction. METHODS: Patients who underwent resection of soft tissue sarcoma followed by a free flap or propeller flap reconstruction and adjuvant radiotherapy at a single institution were retrospectively reviewed. RESULTS: The 1- and 3-year local control rates were 94.6% and 88.6% in the free flap group vs 90.6% and 87.5% in the propeller flap group, without statistical significance. There were no statistically significant differences in 5-year local recurrence-free survival (88.6% vs 87.5%) and disease-free survival (82.5% vs 74.8%) between the groups. CONCLUSIONS: Although there was no significant difference in local control and disease-free survival rates between propeller flap and free flap reconstruction after soft tissue sarcoma resection, a multidisciplinary approach is needed to obtain surgical information for determining the accurate clinical target volume of adjuvant radiotherapy and the area for meticulous follow-up postoperatively.

Bone oxaloma-a localized manifestation of bone oxalosis.

Oxalosis is a metabolic disorder characterized by the accumulation of calcium oxalate deposits in various organ systems. Bone oxalosis is a systemic manifestation of oxalosis, in which calcium oxalate deposits accumulate in the skeletal system. This report describes a 69-year-old female patient who presented with right hip pain and was later found to have an osseous mass-like lesion in the right proximal femur, with radiographic findings resembling those of a benign bone tumor. CT-guided biopsy and surgical biopsy showed that the mass had pathologic findings typical of bone oxalosis. Because the patient had no predisposing factors for systemic oxalosis and had no hyperoxalemia or hyperoxaluria, she could be diagnosed with localized bone oxalate deposition disease or bone oxaloma rather than bone oxalosis. This is the first report of this clinical entity to present as a benign-appearing bone mass and without any predisposing systemic causes.

Added value of diffusion-weighted imaging to conventional MRI for predicting fascial involvement of soft tissue sarcomas.

OBJECTIVES: This study was conducted to evaluate the added value of diffusion-weighted imaging (DWI) to conventional magnetic resonance imaging (MRI) for predicting fascial involvement of soft tissue sarcomas located in close proximity to fascial boundaries. METHODS: This retrospective study included 29 patients with surgically resected soft tissue sarcomas located in proximity to deep fascia and with a curvilinear tail-like hyperintensity in the adjacent fascia on T2-weighted images. All patients underwent conventional MRI and DWI at 3.0 T and had detailed histologic reports on involvement of fascia. Two musculoskeletal radiologists with 21 and 1 year of experience independently reviewed conventional MRI and conventional imaging with added DWI. Readers scored their confidence for tumor involvement of fascia using a three-point scale. Diagnostic performance (area under the curve [Az]) of the two MRI sets was assessed with receiver-operating characteristic curve analysis. RESULT: Fascial involvement was present in 22/29 patients (75.9%). Both readers showed improvement in diagnostic performance with the addition of DWI (Az, from 0.545 to 0.792 and from 0.646 to 0.792 for reader 1 and reader 2, respectively). Adding DWI did not improve sensitivity or specificity for either reader (p > 0.05). Interobserver agreement for the confidence scores improved from fair to moderate with the addition of DWI (κ, from 0.390 to 0.560). CONCLUSIONS: Adding DWI to conventional MRI improved diagnostic performance on prediction of fascial involvement of soft tissue sarcomas located in proximity to fascia, without significant improvement in sensitivity or specificity. KEY POINTS: • Adding DWI to conventional MRI improved readers' confidence level for the prediction of fascial involvement of soft tissue sarcomas that are close to the deep fascia. • Addition of DWI also improved interobserver agreement. • Conversely, compared with conventional MRI, adding DWI did not significantly improve the sensitivity or specificity for the detection of fascial involvement.

Postmetastasis survival in high-grade extremity osteosarcoma: A retrospective analysis of prognostic factors in 126 patients.

BACKGROUND AND OBJECTIVES: Prognostic factors predictive of postmetastasis survival (PMS) in metastatic osteosarcoma are poorly understood. Our aims were to evaluate PMS in patients with high-grade osteosarcoma in extremities, and to identify prognostic factors related to PMS. METHODS: A retrospective review of data for 126 patients with metastatic osteosarcoma was conducted. The study population consisted of 70 men and 56 women, with a mean age of 21 years (range: 4-75 years). The mean postmetastasis follow-up period was 37 months (range: 1-245 months). RESULTS: The 5-year PMS rate was 31% and median PMS duration was 22 months. In the multivariate analyses, no metastasectomy (P < 0.001), local recurrence prior to metastasis (P = 0.016), extrapulmonary metastasis (P = 0.006), and poor histologic response to preoperative chemotherapy (P = 0.047) were significant poor prognostic factors. The 5-year PMS without any negative prognostic factor was 60.2%; with one factor, 31.6%; and with more than two factors, 3.6%. CONCLUSIONS: PMS in osteosarcoma patients was influenced by primary tumor-related factors such as histologic response to chemotherapy, as well as metastasis-related factors such as complete metastasectomy and metastasis site. A certain group of patients without such poor prognostic factors could be cured even after the development of metastasis.

Cortical Atrophy Related to Tumor Prosthesis in Skeletally Immature Osteosarcoma Patients.

BACKGROUND: Cortical atrophy is commonly observed after prosthetic reconstruction for bone sarcomas. However, relevant literature regarding this phenomenon in skeletally immature patients is limited. Therefore, in this study, we evaluated the incidence and patterns of cortical atrophy. We then assessed its predisposing factors. Finally, we analyzed whether cortical atrophy was associated with poor prosthesis survival. METHODS: We retrospectively reviewed 31 stems in 19 skeletally immature osteosarcoma patients who were treated with resection and tumor prosthesis surgery. We measured the cortical thickness using plain radiographs annually. The mean patient age was 11 years (range, 6 to 14 y) and the mean follow-up period was 10 years (range, 2 to 14 y). RESULTS: Cortical atrophy developed in 13 stems, all within 3 years. There were 3 gross types of cortical atrophy: hourglass in 5, sharpening in 4, and shortening atrophy in 4. On multivariate analysis, stem-cortex diameter ratio of ≥0.5 and age of less than 10 years were significantly associated with cortical atrophy (P=0.002 and P=0.019, respectively). Cortical atrophy was significantly associated with eventual prosthesis failure (9/13 in the cortical atrophy group vs. 4/18 in the nonatrophy group, P=0.035). CONCLUSIONS: Cortical atrophy is common in the skeletally immature patients with tumor prosthesis and is associated with prosthesis failure. Patients with cortical atrophy should be closely monitored for prosthesis failure and appropriately managed to prevent failures. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Combined Autograft and Bone Cement for Painful Chondroblastoma: A Case Report.

Chondroblastomas of the talus can lead to joint collapse and are often treated using curettage and bone grafting. In the present report, we describe the case of a 19-year-old female with a large chondroblastoma of the talus associated with a secondary aneurysmal cyst. We treated the large cartilage lesion, which involved most of the talus, with an iliac bone graft combined with bone cement to fill the large bone defect and preserve the subchondral bone of the articular surface of the dome of the talus.

Periprosthetic fractures of the distal femur following total knee arthroplasty: even very distal fractures can be successfully treated using internal fixation.

PURPOSE: We treat periprosthetic fracture of the distal femur above total knee arthroplasty using single or double plating and the minimally invasive plate osteosynthesis (MIPO) technique. Here, we report the results of using this operative treatment and our analysis of whether very distally extended fractures can also be successfully treated using internal fixation. METHODS: We retrospectively reviewed 32 periprosthetic distal femoral fractures. Mean patient age was 73 years and mean follow-up period 25 months. There were 11 (34.4 %) Su type I/II and 21 (65.6 %) type III fractures. All Su type I/II fractures were treated by single plating, whereas 14 of 21 (66.7 %) type III fractures were treated using double plating and the MIPO technique. We defined bony union as the primary endpoint of this study based on callus formation across the fracture site at two or more cortices (1 medial and 1 other) on 3D computed tomography (CT). RESULTS: Bony union was confirmed in 30 cases (93.8 %) after a mean time to union of 3.7 (range, 3-7) months. Two patients showed nonunion (6.2 %), and one demonstrated delayed union (3.1 %), none of whom complied with our rehabilitation protocol. There was one instance of nonunion among both Su types I/II and III fractures; however, this difference was not significant (p = 0.577). CONCLUSIONS: Periprosthetic fractures of the distal femur can be successfully healed using internal fixation, either the single- or double-plate MIPO technique, even for very distally extended fractures.

Non-spine bone metastasis as an initial manifestation of cancer in Korea.

Non-spine bone metastasis accounts for approximately 20% of all skeletal metastases, but little data have been published that focused on bone metastasis to the pelvis and extremities as an initial manifestation of cancer. We determined 1) clinicopathologic characteristics of patients who presented with non-spine bone metastasis of unknown primary malignancy, and 2) process by which the diagnosis of primary cancer was made. We retrospectively reviewed 84 patients with bone metastasis of unknown primary cancer site at the time of presentation. The study population consisted of 56 men and 28 women, with a mean age of 59.1 yr (17.5-85.6 yr). The average follow-up period was 20.8 months (1-120 mo). Primary cancer site was identified in 79 patients (94.0%), and was determined to be the lung (46.4%), kidney (13.1%), liver (9.5%), thyroid (8.3%), and prostate (4.8%). Five-year overall survival rate was 28.0%. Multiple bone metastases, distant organ metastasis, and multiple bone with organ metastases were the significant prognostic factors in univariate analysis. Multiple bone metastases remained significant after multivariate analysis (P = 0.008). Lung cancer is the most common site of primary cancer, and patients with multiple bone metastases have a poor prognosis, possibly due to disseminated cancer and a greater tumor burden.

Double tension band wiring for treatment of olecranon fractures.

PURPOSE: Although tension band wiring (TBW) is generally accepted as standard treatment for olecranon fractures, it has several shortcomings such as loss of reduction, skin irritation, and migration of the K-wires. To overcome these problems and increase fixation stability, we used a rigid fixation technique with double tension band wiring (DTBW). Here, we describe the technique and outcomes of the treatment. METHODS: We retrospectively reviewed 21patients with olecranon fractures who were treated by DTBW. We evaluated clinical and radiological outcomes by checking the range of motion, loss of reduction, functional scoring, skin complications, and pin migration. There were 15 cases of Mayo type IIA fractures (71%) and 6 of type IIB fractures (29%). The mean follow-up period was 37 months (range, 12-58 mo). We also compared the mechanical stability of DTBW and TBW in a sawbone model using a single cycle load to failure protocol. RESULTS: All fractures united without displacement, and no migration of the K-wires was observed during the period of follow-up. Mean loss of elbow extension was 2° (range, 0°-15°) and mean elbow flexion was 134° (range, 125°-140°). The mean Mayo Elbow Performance Score was 94 (range, 70-100). Biomechanical testing revealed greater mechanical strength in the DTBW technique than in the TBW when measured by mean maximum failure load and mean bending moment at failure. CONCLUSIONS: DTBW produced good clinical and radiological outcomes and could be an effective option for the treatment of olecranon fractures by providing additional stability through a second TBW. Biomechanical comparison with a control group (TBW) supported the mechanical benefits of DTBW. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Characterization of sacral chordoma and differential diagnosis from other sacral malignancy using [18F]FDG PET/CT.

2-Deoxy-2-[18F]fluoro-D-glucose ([18F]FDG) positron emission tomography (PET)/computed tomography (CT) is known to be a helpful imaging modality for sacral chordoma, but its detailed characteristics have not been fully described. The purpose of our study was to identify the [18F]FDG PET/CT imaging characteristics of sacral chordoma and compare them with other sacral malignancy. This retrospective study included patients who underwent [18F]FDG PET/CT because of a mass involving the sacrum. Investigated visual findings included visual score and distribution, and semiquantitative parameters measured included standardized uptake values (SUVmax, SUVpeak, SUVmean), tumor-to-liver ratio (TLR), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and tumor size. Comparison studies and receiver operating characteristics (ROC) curve analysis were performed to differentiate between sacral chordoma and other sacral malignancy. Ten patients with sacral chordoma were finally included (M:F = 6:4, median age = 67 yr). On [18F]FDG PET/CT, sacral chordomas presented as a mass with minimal-moderate uptake with a usually heterogenous distribution. Compared with 12 patients with other sacral malignancies (M:F = 4:8, median age 42 yr), sacral chordoma showed a significantly lower TLR (median value 2.1 vs 6.3, P = .021). In ROC curve analysis, TLR showed the largest area under the curve (AUC) of 0.79 (cutoff ≤ 4.0; sensitivity 100.0%, specificity 58.3%; P = .004), and SUVmax showed the second largest AUC of 0.73 (cutoff ≤ 6.9; sensitivity 80.0%, specificity 66.7%; P = .034). [18F]FDG PET/CT of sacral chordoma showed minimal-moderate uptake. The TLR of [18F]FDG PET/CT was significantly lower than that of other sacral malignancy and was the most useful parameter for differentiating sacral chordoma, with the largest AUC. SUVmax could be another helpful semiquantitative parameter.

Enhancing local recurrence detection in patients with high-grade soft tissue sarcoma: value of short-term Ultrasonography added to post-operative MRI surveillance.

BACKGROUND: Limited data exist on the optimal postoperative surveillance protocol for high-grade soft tissue sarcoma, particularly regarding the optimal imaging modality and imaging interval for detecting local recurrence. This study aimed to assess the benefit of short-term postoperative ultrasonography (USG) for detecting local recurrence in patients with high-grade soft tissue sarcoma. METHODS: Patients with newly diagnosed high-grade soft tissue sarcoma who underwent surgical resection between January 2010 and June 2020 were included. Short-term USG was added to the follow-up protocol as a surveillance tool alongside routine magnetic resonance imaging (MRI). The primary outcome was the additional detection rate of short-term USG compared with routine MRI surveillance for early local recurrence detection. Subgroup analysis was performed to evaluate factors influencing USG detection rate. The additional detection rate of short-term USG for detection of metastatic lymph nodes was also evaluated. The secondary outcome was the false referral rate of short-term USG. RESULTS: In total, 198 patients (mean age ± standard deviation: 52.1 ± 15.8 years; 94 women) were included. Local recurrence occurred in 20 patients (10.1%; 20/198). Short-term USG detected local recurrence in advance of routine MRI visits in 7 out of 198 patients, resulting in an additional detection rate of 3.5% (95% CI: 1.7-7.1%). Subgroup analysis showed no significant difference in the short-term USG detection rate based on initial tumor characteristics, and receipt of radiotherapy or chemotherapy. Short-term USG additionally detected five of seven patients with metastatic lymph nodes [2.5% (95% CI, 1.1-5.8%, 5/198)]. The false referral rate of short-term USG was 3.5% (95% CI: 1.7-7.1%; 7/198). CONCLUSIONS: Short-term USG as part of postoperative surveillance for high-grade soft tissue sarcoma can enhance early detection of local tumor recurrence and metastatic lymphadenopathy. Early detection of local tumor recurrence could lead to a prompt surgical resection and aid in local disease control.

What are the resection accuracy and guide-fitting errors associated with 3D-printed, patient-specific resection guides for bone tumour resections?

AIMS: This study aimed to analyze the accuracy and errors associated with 3D-printed, patient-specific resection guides (3DP-PSRGs) used for bone tumour resection. METHODS: We retrospectively reviewed 29 bone tumour resections that used 3DP-PSRGs based on 3D CT and 3D MRI. We evaluated the resection amount errors and resection margin errors relative to the preoperative plans. Guide-fitting errors and guide distortion were evaluated intraoperatively and one month postoperatively, respectively. We categorized each of these error types into three grades (grade 1, < 1 mm; grade 2, 1 to 3 mm; and grade 3, > 3 mm) to evaluate the overall accuracy. RESULTS: The maximum resection amount error was 2 mm. Out of 29 resection amount errors, 15 (51.7%) were grade 1 errors and 14 (38.3%) were grade 2 errors. Complex resections were associated with higher-grade resection amount errors (p < 0.001). The actual resection margins correlated significantly with the planned margins; however, there were some discrepancies. The maximum guide-fitting error was 3 mm. There were 22 (75.9%), five (17.2%), and two (6.9%) grade 1, 2, and 3 guide-fitting errors, respectively. There was no significant association between complex resection and fitting error grades. The guide distortion after one month in all patients was rated as grade 1. CONCLUSION: In terms of the accurate resection amount according to the preoperative planning, 3DP-PSRGs can be a viable option for bone tumour resection. However, 3DP-PSRG use may be associated with resection margin length discrepancies relative to the planned margins. Such discrepancies should be considered when determining surgical margins. Therefore, a thorough evaluation of the preoperative imaging and surgical planning is still required, even if 3DP-PSRGs are to be used.Cite this article: Bone Joint J 2023;105-B(2):190-197.

Detecting residual soft tissue sarcoma after unplanned excision; model-free analysis of dynamic contrast-enhanced MRI at short-term follow-up.

OBJECTIVES: To evaluate diagnostic utility of additional DCE-MRI for detecting residual soft tissue sarcomas (STS) after unplanned excision (UPE). METHODS: We retrospectively evaluated 32 patients with UPE of STS, followed by conventional MRI with DCE-MRI and wide excision (WE), between November 2019 and January 2022. Residual tumors on conventional MRI were categorized into three groups: Lesion-type-0, no abnormal enhancement, Lesion-type-1, an indeterminate lesion, and Lesion-type-2, a definite enhancing nodule. On DCE-MRI, ROIs were manually placed on enhancing areas of suspected residual tumor. The mean and 95th percentile values of AUC of time-intensity curve were calculated at 60, 90, and 120 s of Enhancement-cycle-1 and -2. Optimal DCE parameters were identified by ROC analysis. Diagnostic performance of conventional MRI and DCE-MRI was compared using McNemar's test. RESULTS: On WE, residual tumor was present in 23 (71.9%) of 32 patients. On MRI, Lesion-type-1 was found in 16/32 (50%) patients and Lesion-type-2 in 16/32 (50%). The optimal DCE parameter was the 95th percentile value of AUC at 120s of Enhancement-cycle-2. The sensitivity, specificity, and AUC were as follows: 65.2% (95% CI, 45.8-85.7%), 88.9% (CI, 68.4-100%), and 0.77 (CI, 0.62-0.92) for conventional MRI, and 100%, 55.6% (CI, 23.1-88.0%), and 0.78 (CI, 0.61-0.95) for combined conventional and DCE-MRI. CONCLUSIONS: Additional DCE-MRI aided in detecting residual STS after UPE, particularly in cases without definite soft tissue nodular enhancement. ADVANCES IN KNOWLEDGE: Close follow up may be suggested for patients showing abnormality in DCE-MRI, with more suspicion of residual tumor.

Management of talar lesions with cement augmentation and autologous bone graft.

BACKGROUND: Treatment of bone lesions involved with the articular cartilage at the talus is challenging. We report the management of talus lesions, particularly tumors and avascular necrosis (AVN), at the articular surface through treatment with cement augmentation and autologous bone graft. METHODS: Eight benign bone tumors and three cases of AVN were reviewed retrospectively at a mean follow-up of 56 months (range, 12-162). The mean age of all patients was 36.1 years old (range, 15-73) when assessed between February 2005 and November 2021. Curettage of tumorous and necrotic lesions resulted in significant bone defects filled with bone cement augmentation. Cartilage defects of the talar dome were supported with autologous cancellous bone graft. Tolerable weight-bearing ambulation was permitted immediately after surgery. Radiological and functional evaluations were recorded. RESULTS: We observed an increase in the average The American Orthopaedic Foot and Ankle Score (AOFAS) (p = .003) and a decrease in the average Visual Analogue Scale pain score (p = .003). There was no statistically significant decrease in ROM before or after surgery (p = .114). Additionally, no talus collapse of the ankle joint occurred. Talar dome status did not aggravate before or after surgery, except for one patient. Despite no radiographic osteoarthritis exacerbation before or after surgery in six patients, five patients had osteoarthritic change. CONCLUSION: Cement implantation and autologous bone graft performed simultaneously for benign bone tumors with joint cartilage damage and AVN are technically simple, have good outcomes, and may be a suitable alternative to standard treatments.

NY-ESO-1 as a diagnostic and prognostic marker for myxoid liposarcoma.

INTRODUCTION: Myxoid liposarcoma (MLS) is a common lipogenic sarcoma, which is difficult to diagnose in small specimens. New York oesophageal squamous cell carcinoma 1 (NY-ESO-1) is a cancer-testis antigen expressed in neoplastic tissue. In this study, NY-ESO-1 expression was assessed in various soft tissue tumors (STTs), and we also evaluated its diagnostic utility. METHODS: We included 434 cases of STTs for collection of clinicopathological data. Tissue microarrays were designed, and immunostaining for NY-ESO-1 was examined. We investigated the correlation between NY-ESO-1 expression and various clinicopathological parameters. We also evaluated the role of NY-ESO-1 as a diagnostic marker for MLS and its possible use in prognostication. RESULTS: Sixty-four of the 434 STTs (14.75%) were immunoreactive for NY-ESO-1, and the most frequent type of tumor in the NY-ESO-1 positive group was MLS (70.3%, 45/64), followed by synovial sarcoma (17.2%, 11/64). MLS showed 72.6% (45/62) immunopositivity for NY-ESO-1. The sensitivity and specificity of NY-ESO-1 expression for the diagnosis of MLS were 84.4% and 100%, respectively, compared to DDIT3 fluorescence in situ hybridization. When restricting analysis to the MLS (n=62), the NY-ESO-1 positive group had a poor overall survival (OS) rate (P=0.039). CONCLUSION: NY-ESO-1 was substantially and widely expressed in the majority of MLS cases. NY-ESO-1 positivity by IHC staining was also a predictor of a poor OS in patients with MLS. It is possible to use NY-ESO-1 for diagnosis and for predicting a prognosis in patients with MLS, and it may be used as a therapeutic target.

Distinct histologic and genetic characteristics of round cell sarcoma with CIC-DUX4 fusion and comparison with ewing sarcoma.

CIC-DUX4 fusion gene associated sarcoma is a new emerging subgroup of round cell sarcoma with Ewing sarcoma-like morphology. Distinguishing these tumors from Ewing sarcoma family tumors (ESFT) is critical because of the clinical impact but is still challenging due to the overlapped histological and immunohistochemical phenotypes of each subtype. The present study investigated small round cell sarcoma to identify CIC-DUX4 fusion positive sarcoma, examined clinical, histopathologic and immunohistochemical characteristics of CIC-DUX4 sarcoma, and evaluated parameters to differentiate Ewing sarcoma family tumors. Seventy patients with undifferentiated round cell sarcoma or Ewing-like sarcoma were retrieved. Molecular tests including EWSR1, CIC break apart FISH, and RT-PCR for CIC-DUX4 gene fusion were performed and immunohistochemistry was performed. Six cases (8.6%) of CIC-DUX4 sarcomas were detected. Histologically, CIC-DUX4 sarcomas composed of heterogeneous round, plasmacytoid, and spindle cells and more commonly showed cytologic pleomorphism with bizarre nuclei and multinucleated cells and myxoid stoma unlike ESFT. CIC-DUX4 sarcomas didn't show overall survival differences (p = 0.325) compared to ESFT but they demonstrated short disease-free survival (p = 0.034) and poor response to treatment (p = 0.007). Therefore, molecular analysis to detect the distinctive genetic alteration is mandatory in tumors with atypical histologic, immunohistochemical and/or clinical presentation for accurate diagnosis and treatment.

Clinicopathologic Characteristics and Clinical Outcome of Localized Liposarcoma: A Single-Center Experience over 25 Years and Evaluation of PD-L1 Expression.

PURPOSE: For liposarcoma (LPS), clinical course and proper treatment strategies have not been well-established. Recently, immune-checkpoint inhibitors have shown potential efficacy in LPS. We aimed to describe the clinical course of LPS and evaluate the clinical impact of programmed death-ligand 1 (PD-L1). MATERIALS AND METHODS: We reviewed all consecutive patients (n=332) who underwent curative-intent surgery for localized LPS at Asan Medical Center between 1989 and 2017. PD-L1 testing was performed in well-differentiated and dedifferentiated LPS. RESULTS: The median age was 56 years with males comprising 60.8%. Abdomen-pelvis (47.6%) and well-differentiated (37.7%) were the most frequent primary site and histologic subtype, respectively. During a median follow-up of 81.2 months, recurrence was observed in 135 (40.7%), and 86.7% (117/135) were loco-regional. Well-differentiated subtype (hazard ratio [HR], 0.38), abdomen-pelvis origin (HR, 2.43), tumor size larger than 5 cm (HR, 1.83), positive resection margin (HR, 2.58), and postoperative radiotherapy (HR, 0.36) were significantly related with recurrence-free survival as well as visceral involvement (HR, 1.84) and multifocality (HR, 3.79) in abdomen-pelvis LPS. PD-L1 was positive in 31.5% (23/73) and 51.3% (39/76) of well-differentiated and dedifferentiated LPS, respectively, but had no impact on survival outcomes. CONCLUSION: Clinical course of LPS was heterogeneous according to histology and anatomic location. Clear resection margin was important to lower recurrence and postoperative radiotherapy might have additional benefit. A decent portion of well-differentiated and dedifferentiated LPS were positive for PD-L1, but its prognostic role was unclear. Further research is needed to determine clinical implications of PD-L1, especially for advanced-stage LPS with unmet needs for effective systemic treatment.