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PURPOSE: Intramuscular myxoma (IM) is a rare benign myxoid tumor that may be challenging to differentiate from sarcoma in small amounts of biopsied material. Although IM appears to be well-circumscribed macroscopically, it infiltrates the adjacent edematous muscle microscopically. The recommended treatment is resection, but there is controversy with regard to the appropriate surgical margin. This study aimed to clarify which surgical procedure that should be applied when the preoperative diagnosis is IM and how to manage treatment if the postoperative diagnosis turns out to be a sarcoma. METHODS: We retrospectively examined 55 IM patients treated from January 1982 to December 2014. Patient characteristics, tumor location, tumor size, radiograph, preoperative and postoperative pathological reports, surgical techniques, treatment outcome, and complications were reviewed. The patients were followed up on for at least 5 years. All patients were confirmed not to have Mazabraud syndrome. RESULTS: In the 55 IM patients examined, the mean patient age was 48 years and most were female. The most common tumor locations were in the muscles of the thighs (47%) and buttocks (20%). The mean tumor diameter was 5 cm. Wide resection and marginal resection were performed in 24 and 31 patients, respectively. The mean follow-up duration was 19 years. No local recurrence, malignant transformation, or complications were observed. CONCLUSIONS: Marginal resection is suitable in patients whose preoperative diagnosis is IM, as it is able to prevent local recurrence and allows for the preservation of muscle and muscle fascia. If the postoperative diagnosis turns out to be myxoid sarcoma, minimum surgical contamination makes additional wide resection less invasive.

BACKGROUND Eribulin is widely used for the treatment of breast cancer and soft-tissue sarcoma (STS). Previous studies identified the pre-treatment absolute lymphocyte count, baseline neutrophil-to-lymphocyte ratio (NLR) and C-reactive protein concentration as potential prognostic markers in patients with breast cancer treated with eribulin. However, prognostic factors for eribulin treatment in patients with STS have not been identified. PATIENTS AND METHODS This was a retrospective analysis of data collected prospectively from 53 patients who were treated with eribulin for recurrent or metastatic STS between March 2016 and August 2019. Univariate and multivariate analyses were performed to determine the predictive factors of durable clinical benefit, progression-free survival, and overall survival. RESULTS L-Sarcoma histology [hazard ratio (HR)=28.20, 95% confidence intervaI (CI)=1.67-476.00; p=0.021] and pre-treatment NLR

The overall prognosis of patients with sarcoma-based cancers has changed little in the last 20 years. There is an urgent need to investigate the metastatic potential of these tumors and to develop anti-metastatic drugs. It is becoming increasingly clear that platelets play an important role in the establishment of metastasis of carcinoma cells and could be a useful therapeutic target for patients with carcinoma. However, little is known about the role of platelets in sarcoma progression. Here, we investigated how osteosarcoma progression relates to platelet function to explore the possibility of anti-platelet therapy. We found that, similar to carcinoma cells, podoplanin (also known as Aggrus)-positive osteosarcoma cells induce platelet aggregation and activation. Administration of anti-glycoprotein Ibα (GPIbα, also known as CD42b) antibody reduced the lung metastasis of osteosarcoma. The supernatant from platelets cocultured with osteosarcoma cells contained several growth factors and promoted proliferation, invasiveness, and sphere formation of osteosarcoma cells in vitro. In addition, the development of lung metastasis was highly dependent on direct interaction between osteosarcoma cells and platelets. To explore the therapeutic target, we focused on the interactions between podoplanin on osteosarcoma and C-type lectin-like receptor (CLEC)-2 on platelets. The administration of a depleting antibody against CLEC-2 efficiently suppressed osteosarcoma metastasis into the lung. We also analyzed clinical data from patient samples at primary and metastatic sites. Although GPIbα expression was similar between the two sites, there was a significant increase in podoplanin at the metastatic site compared to that in the primary site, and the level of podoplanin expression in the primary site correlated with patient prognosis. These findings suggest that blockade of interactions between platelets CLEC-2 and osteosarcoma podoplanin represent the most promising therapeutic strategy for preventing the lung metastasis of osteosarcoma. © 2020 American Society for Bone and Mineral Research.

Nodular fasciitis (NF) is a rapid‐growth benign that is misdiagnosed as sarcoma and leads to overtreatment. The spontaneous regression of NF is a possible phenomenon. “Wait and see” ideal is one of the treatment strategies of NF., Nodular fasciitis (NF) is a rapid‐growth benign that is misdiagnosed as sarcoma and lead to overtreatment. The spontaneous regression of NF is a possible phenomenon. “Wait and see” ideal is one of the treatment strategies of NF.

Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft-tissue tumor and often shows extracompartmental tumoral invasion. The aim of our study was to investigate the clinical features, especially extracompartmental tumoral invasion (ETI) of EMC. Patients and methods A total of 35 operative patients diagnosed with EMC were enrolled in this study from January 1980 to March 2018 in the Cancer Institute Hospital of The Japanese Foundation for Cancer Research. The operative procedure was principally wide excision. Univariate analysis assessed how clinicopathological factors (e.g. age, gender, tumor site, tumor size, histopathological grade, surgical margin, metastasis before operation, barrier invasion, local recurrence, metastasis after operation) influenced patient prognosis. We assessed how clinicopathological factors influenced ETI of EMC. Results Among 35 patients, 10 patients showed ETI. The average follow-up was 5.57 (range=0.2-20 years). The 5- and 10-year overall survival was 91.3% and 71.2%, respectively. The 5- and 10-year overall survival of patients with M0 disease was 96.1% and 73.2%, respectively, while both were 75.0% for those with M1 disease, respectively. The patients with distant metastasis at first visit tended to have a poor prognosis (p=0.07). It is notable that all of the 10 patients with ETI had distant metastasis after surgery. Conclusion Patients with distant metastasis at first visit tended to have a poor prognosis. ETI of EMC induced distant metastasis after surgery. Patients with ETI of EMC should, therefore, be carefully monitored over a prolonged period.

Background: Extra-abdominal desmoid tumors often occur in the necks, shoulder, chest wall, back, arm, buttock, thigh and leg. Multicentric extra-abdominal desmoids are rather rare and seem to have other clinical features. The aim of our study was to investigate clinical features, especially multicentric occurrence of extra-abdominal desmoid tumors. Patients and Methods: A total of 135 patients diagnosed with extra-abdominal desmoid were enrolled in this study from January 2005 to December 2019 at the Cancer Institute Hospital of The Japanese Foundation for Cancer Research. The operative procedure was principally wide excision. The clinicopathological factors [e.g., age, gender, pain, restriction of range of motion (ROM), tumor site, tumor size, surgical margin, multicentric occurrence, local recurrence, tumoral regression] were collected and assessed by univariate analysis. We assessed how multicentric occurrence influenced clinicopathological factors of desmoid tumors. Results: The median follow-up was 39.9 months (range=0.29-259 months). Among 135 patients, 20 had multicentric occurrence. Multicentric extra-abdominal desmoids occurred in the neck in six cases, shoulder in four, chest wall in three, back in three, thigh in two and leg in two. In the case of multicentric occurrence on thighs and legs, tumors arose not in the anterior compartment but in the posterior compartment. Univariate analysis showed association of multicentric extra-abdominal desmoids with high local recurrence (p=0.0003), restriction of ROM (p=0.0012) and tumor size larger than 5 cm (p=0.04) but surgical margins were not correlated with local recurrence (p=0.37). Conclusion: Surgery should be performed in those who have severe pain or restriction of ROM. A 'Wait and see' policy is a first-line management, especially for those with multicentric extra-abdominal desmoids.

Background/Aim: Trabectedin and eribulin are widely used for the treatment of soft-tissue sarcoma (STS). Previously it was shown that the baseline neutrophil-to-lymphocyte ratio (NLR) predicts the efficacy of eribulin for STS. However, prognostic factors for trabectedin on STS have not been identified to date. Patients and Methods: We conducted a retrospective study of data collected prospectively from 39 patients treated with trabectedin for recurrent or metastatic STS between October 2012 and December 2019. To determine the predictive factors of overall survival (OS) and progression-free survival (PFS), univariate and multivariate analyses were performed. Results: Age ≥40 (HR=0.33, 95% CI=0.15-0.71; p=0.0050) and changes in NLR (ΔNLR)

Background Recently, the number of osteosarcomas in middle-aged and older patients has demonstrated an increasing trend; moreover, their results are comparatively worse than those of young patients. In Europe and the USA, the prognosis for osteosarcoma in middle-aged and older patients has improved with adjuvant chemotherapy. In Japan, however, the prognosis has remained poor. Materials and Methods We retrospectively analyzed the outcomes of osteosarcoma, especially in regards to preoperative chemotherapy, from January 1980 to July 2014. A total of 29 patients with high-grade osteosarcoma between the age of 40 and 65 years were included. We included patients without distant metastasis and with primary lesions that were deemed resectable. The mean age was 52.8 years (range 41–65 years), and the mean follow-up period was 103.2 months (range 5–314 months). Results Adjuvant chemotherapy was administered to 27 of 29 patients (93%), and 8 of 15 cases (53%) were able to undergo preoperative chemotherapy as planned, including CDDP. A major complication of chemotherapy was acute kidney injury due to CDDP (26%). The 5-year OS and 5-year EFS were 64.9% and 57.1%, respectively. After 2006, a policy to prioritize the resection of the primary lesion was implemented, and if the primary lesion was deemed resectable, preoperative chemotherapy was either not administered or administered for only a short duration. The 5-year OS after 2006 improved to 78.8%. Conclusions This study shows that administration of high-dose intensity preoperative chemotherapy was difficult in middle-aged and older patients due to their high rate of acute kidney injury by CDDP. For cases of osteosarcoma in middle-aged and older patients, if the primary lesion is resectable, preoperative chemotherapy should be minimized to prioritize the resection of the primary lesion. It was considered that, with appropriate measures to prevent complications, adjuvant chemotherapy may lead to improved prognosis. Level of evidence V.

Although the unpredictable malignant behavior of solitary fibrous tumors (SFTs) has been recognized, the clinical features and prognosis of metastatic SFTs have not been well documented due to the extreme rarity of these cases. The aim of this study is to investigate the clinical features, prognostic factors, and optimal management of patients with metastatic SFTs. Sixty patients with metastatic SFT were retrospectively reviewed. Univariate and multivariate analyses were performed to identify the factors associated with survival. Time to next treatment (TNT) was used to evaluate the effects of various chemotherapy regimens. A total of 34 male and 26 female patients (median age 55 years, range, 23–87 years) were included in the study. The median follow-up period after metastasis was 32 months (range 1–126 months). Tumor location and local recurrence were correlated with late metastasis. The 3- and 5-year overall survival rates were 72.7% and 49.2%, respectively. Primary tumor location, number of metastases, and metastasectomy were significantly associated with survival. Metastasectomy was the only significant variable on multivariate analysis. The TNT was significantly different among the various regimens. Patients with metastatic SFTs had relatively longer survival periods compared with those with other metastatic soft-tissue sarcomas. Tumor location and number of metastases was associated with survival. Surgical resection of the metastatic lesions offers the best chance of survival, however further studies are warranted to define patients who would benefit from metastasectomy, and the most effective chemotherapeutic regimen for patients with metastatic SFTs remains unknown.

Clinical evidence regarding eribulin treatment for patients with soft tissue sarcoma (STS) is limited to those with L-sarcoma (leiomyosarcoma and liposarcoma) who have completed at least two chemotherapies. Whether histological subtypes and treatment lines affect the efficacy and safety of eribulin for patients with STS has yet to be elucidated. The current study retrospectively reviewed patients with STS receiving eribulin at the Cancer Institute Hospital of JFCR and evaluated the prognostic factors affecting its efficacy and safety by histological diagnoses and treatment lines. A total of 41 patients with STS, including 26 with L-sarcoma, underwent eribulin treatment. Additionally, a total of and 14 patients, including 12 with L-sarcoma, received eribulin as a second-line treatment. The results revealed that patients with L-sarcoma demonstrated longer progression-free survival (PFS) rates compared with patients without L-sarcoma (4.5 vs. 2.3 months; P=0.005). Furthermore, differences in treatment line significantly affected PFS (4.5 months in second-line treatment vs. 2.4 months in later lines; P=0.037). A high number of patients with L-sarcoma received eribulin as a second-line treatment. Regarding safety, several adverse events were reported, such as neutropenia, which were more frequently observed in patients with L-sarcoma or other patients receiving eribulin as a second-line treatment. However, most adverse events were tolerable. The clinical efficacy of eribulin was increased in patients with L-sarcoma, which was similar to previous clinical trials. However, treatment lines could also affect its efficacy. When evaluating the clinical value of eribulin to STS, it is important to consider treatment lines.

Background Clear cell chondrosarcoma is an extremely rare chondrosarcoma subtype; thus, its treatment outcomes and associated factors have not been widely studied. Knowing more about it is potentially important because clear cell chondrosarcomas are often misdiagnosed as other benign lesions and subsequently treated and followed inappropriately. Questions/purposes (1) What are the patient- and tumor-related characteristics of clear cell chondrosarcoma? (2) What proportion of patients with clear cell chondrosarcoma initially had a misdiagnosis or a misleading initial biopsy result? (3) What is the survivorship of patients with clear cell chondrosarcoma free from death, local recurrence, and distant metastasis, and what factors are associated with greater survivorship or a reduced risk of local recurrence? Methods Between 1985 and 2018, 12 Japanese Musculoskeletal Oncology Group (JMOG) hospitals treated 42 patients with a diagnosis of clear cell chondrosarcoma. All 42 patients had complete medical records at a minimum of 1 year or death, and were included in this multicenter, retrospective, observational study. No patients were lost to follow-up within 5 years of treatment but four were lost to follow-up greater than 5 years after treatment because their physicians thought their follow-up was sufficient. Clinical data were collected by chart review. The median (range) follow-up period was 69 months (2 to 392). In general, when a possibly malignant bone tumor was found on imaging studies, the histological diagnosis was made by biopsy before initiating treatment. Once the diagnosis had been made, the patients were treated by surgery only, complete resection if technically possible, because chondrosarcomas are known to be resistant to chemotherapy and radiotherapy. Unresectable tumors were treated with particle-beam radiation therapy. When patients with chondrosarcoma were referred after unplanned surgical procedures with inadequate surgical margins, immediate additional wide resection was considered before local recurrence developed. This diagnostic and treatment strategy is common to all JMOG hospitals and did not change during the study period. Primary wide resection was performed in 79% (33 of 42) patients, additional wide resection after initial inadequate surgery in 12% (five of 42), curettage and bone grafting in 5% (two of 42) patients, and radiotherapy was administered to 5% (two of 42). Surgical margins among the 40 patients who underwent surgery at JMOG hospitals were no residual tumor in 93% (37 of 42) of patients, microscopic residual tumor in 2% (one of 42), and macroscopic residual tumor or state after curettage or intralesional excision in 5% (two of 42). The oncological endpoints of interest were 5- and 10- year overall survival, disease-free survival, survival free of local recurrence, and survival free of distant metastases; these were calculated using the Kaplan-Meier method and compared using the log-rank test. Risk ratios with their respective 95% confidence intervals (CIs) were estimated in a Cox regression model. The Bonferroni adjustment was used for multiple testing correction. Results The sex distribution was 74% men and 26% women (31 and 11 of 42, respectively), with a mean age of 47 ± 17 years. Eighty one percent (34 of 42) of tumors occurred at the ends of long bones, and the proximal femur was the most common site accounting for 60% (25 of 42). The mean size of the primary tumors was 6.3 ± 2.7 cm. Definite pathologic fractures were present in 26% (10 of 42) and another 26% (10 of 42) had extraskeletal involvement. None had metastases at presentation. Twenty four percent (six of 25) tumors in the proximal femur were misdiagnosed as benign lesions and treated inadequately without biopsy. Twenty nine percent (10 of 35) patients had initial misdiagnoses by biopsy and core needle biopsies had a greater risk of resulting in inaccurate histological diagnoses. The study patients' 5- and 10-year overall survival rates were 89% (95% CI 74 to 96) and 89% (95% CI 74 to 96), respectively; 5- and 10- year disease-free survival rates 77% (95% CI 58 to 89) and 57% (95% CI 36 to 75), respectively; 5- and 10-year local recurrence-free survival rates 86% (95% CI 68 to 95) and 71% (95% CI 49 to 86), respectively; and 5- and 10-year distant metastasis-free survival rates 84% (95% CI 67 to 93) and 74% (95% CI 53 to 88), respectively. Notably, bone metastases (17%, seven of 42) were as common as pulmonary metastases (14%, six of 42); four patients developed both bone and pulmonary metastases. The difference between 10-year overall survival rates and 10-year disease-free survival indicated very late recurrence more than 5 years after the initial treatment. After controlling for multiple comparisons, the only factor we found that was associated with local recurrence-free survival was initial treatment (positive margin versus primary wide resection) (risk ratio 8.83 [95% CI 1.47 to 53.1]; p = 0.022 after the Bonferroni adjustment). Additional wide resection reduced the risk of local recurrence. Conclusions The femoral head was the most common location of clear cell chondrosarcoma and had a high risk of misdiagnosis as common benign lesions that resulted in initial inadequate surgery and a consequent high risk of local recurrence. Immediate additional wide resection should be considered in patients who had initial inadequate surgery to reduce the risk of local recurrence. Because clear cell chondrosarcoma can recur locally or distantly in the bones and lungs in the long term, patients should be informed of the risk of very late recurrence and the necessity of decades-long with surveillance for local recurrence and lung and bone metastases. Level of evidence Level IV, therapeutic study.

Background We aimed to retrospectively investigate patients with multicentric giant cell tumor (MCGCT) who were treated at our hospital and to clarify their clinical features, treatment policy, and follow-up method. Methods Four patients with two or more giant cell tumor (GCT) that occurred in the same patient were treated at our institution between 1978 and 2015. These patients were evaluated for the following: frequency, age of onset, number and site of occurrence, time to occurrence of the next lesion, treatment, recurrence, malignant transformation, metastasis, and oncological outcome. Results The rate of occurrence was 1.7%. The average age was 25.2 (17–44). The total number of lesions was three in two cases and two in two cases. All four cases had only one lesion during the initial visit. The most frequent site of occurrence was the proximal femur, followed by two lesions that occurred in the metaphysis. The interval between confirmation of the initial lesion and occurrence of the second lesion was in average 12.1 years (0.8–27.0). Initial presentations of lesions were treated by en bloc resection in one case and curettage in three cases. Local recurrences occurred in two cases that underwent curettage. The six lesions that occurred after the initial lesion were treated as follows: en bloc resection in four lesions, curettage and radiation therapy in one, and embolization and radiation therapy in one. Pathologically, no lesions presented malignancy. Pulmonary metastasis occurred in one case. The oncological outcome was NED in three cases and AWD in one case. Conclusions No lesions were malignant, and by providing the same treatment as solitary GCT, the oncological outcome was good. It is unnecessary to be concerned of its risks and postoperatively conduct long-term searches for focal lesions across the body.

Objective We analyzed the risk factors for pulmonary metastasis from giant cell tumor of bone and aimed to discuss their therapeutic strategy and appropriate follow-up period. Methods We analyzed 141 patients of giant cell tumor of bone. The variables analyzed included age, gender, primary site, Campanacci grading, surgical treatment on the primary lesion, radiotherapy and local recurrence. Results Pulmonary metastasis occurred in 12 patients. The risk factors were young age, Campanacci Grade III and local recurrence. Median time from initial surgery to metastasis was 1.3 years (0-3.1 years). Among them, eight patients experienced local recurrence of the primary tumor, and the median time from initial surgery to local recurrence was 0.8 years (0.3-2.9 years). Among seven patients who underwent wide resection, three patients showed local recurrence of the soft tissue. Nine patients underwent metastasectomy for pulmonary metastases. Of three patients who did not undergo metastasectomy, one patient died of uncontrollable metastases, and two patients showed no changes in their metastatic lesions. Conclusions Although we found a correlation between local recurrence and pulmonary metastasis, we were still unable to prevent local or metastatic recurrence by wide resection. Local recurrence and metastasis have been found within ~3 years after initial surgery, and routine image examinations of the primary site and chest after initial surgical treatment should be considered for at least 3 years postoperatively.

BACKGROUND The role of neoadjuvant chemotherapy for localized synovial sarcoma is still controversial. This study aimed to explore the influence of neoadjuvant chemotherapy combined with surgery in localized synovial sarcoma through analysis of our hospital's patient records. PATIENTS AND METHODS A total of 122 patients diagnosed with synovial sarcoma were enrolled in this study from January 1980 to December 2016 at the Cancer Institute Hospital of The Japanese Foundation for Cancer Research. The impact of neoadjuvant chemotherapy on overall survival was assessed to show how clinicopathological factors (e.g. age, tumor size, treatment, dose intensity, pathological pattern and histological grading) influenced patient prognosis. RESULTS Among 106 patients, 76 (71.7%) received neoadjuvant chemotherapy and 30 (28.3%) did not. The median follow-up was 39.2 (range=12-286) months. The 5-year and 10-year overall survival rates were 65.4% and 58.4% respectively. The tumor size and histological grade influenced the patient's overall survival (p

Background Although initial trabectedin (1.2 mg/m2 ) is safe and effective for patients with translocation-related sarcoma (TRS) in Japan, its efficacy in other types of soft-tissue sarcomas (STSs) remains unknown. This study retrospectively investigated its efficacy and safety through postmarketing surveillance of trabectedin in patients with unresectable and relapsed STS. Methods One hundred forty patients received intravenous trabectedin (1.2 mg/m2 on day 1 every 21 days) over the course of 24 hours. The primary endpoint was the efficacy and safety of trabectedin. Results Grade 3 or higher adverse events occurred in 100 patients (71%) and included hepatotoxicity (37.8%), neutropenia (32.8%), and rhabdomyolysis (3.6%). Patients at high risk for grade 3 or higher rhabdomyolysis (36%) were classified by height (≥170.3 cm) and age (≤32 years) through a classification and regression tree model (area under the curve, 0.9). The overall median progression-free survival (PFS) was 3.7 months; with respect to the histological type, the median PFS was 17.4 months for myxoid liposarcoma, 4.9 months for leiomyosarcoma, 5.6 months for synovial sarcoma, and 3.7 months for dedifferentiated liposarcoma. Histological type (liposarcoma/leiomyosarcoma [L-sarcoma] and TRS) and grade 3 neutropenia (but not grade 4) were associated with significantly improved PFS after trabectedin treatment (P = .003, P = .04, and P = .001). The median growth modulation index (GMI) was 0.91; 37 patients (36.7%) experienced a GMI > 1.33, and among patients with solitary fibrous tumors and undifferentiated pleomorphic sarcoma, 60% and 42.9%, respectively, had a GMI > 1.33. The median overall survival (OS) was 16.4 months. A GMI > 1.33 was associated with significantly improved OS (P = .0006). Conclusions Initial trabectedin at 1.2 mg/m2 has clinically meaningful benefits for patients with L-sarcoma and certain histological subtypes of TRS.

At times, surgeons face various difficulties in the treatment of patients who require surgical therapy. The patients' general conditions as well as their will and religion can sometimes interfere with the implementation of the best therapeutic option. For instance, the presence of cardiac disease, respiratory disease, or cognitive impairment often can difficult the decision-making process when determining the optimal therapeutic strategies. Pregnancy is not a pathological condition, but it can complicate the decision-making process with respect to choosing the best treatment for pregnant patients who require surgery. Non-obstetric surgery in pregnant patients is relatively common, although it requires a prudent selection of anesthetic drugs, surgical methods and analgesic agents. Additionally, it is a well-known religious practice that Jehovah's Witnesses refuse to receive blood transfusions, and thus, their surgical management can be challenging. Giant cell tumor of bone (GCTB) is classified as a benign bone tumor, but it is locally aggressive. Standard treatment for GCT is

Combined limb salvage surgery and radiation therapy has been gaining traction for the treatment of soft tissue sarcomas of the foot in recent years. However, limb salvage surgery without radiation therapy is more desirable. In this study, we obtained good results from limb salvage surgery without radiation therapy in soft tissue sarcoma of the foot. Twenty patients histologically diagnosed with soft tissue sarcoma who underwent limb salvage surgery, including ray amputation, were evaluated. Their mean age was 39 years. The mean follow-up period was 94.5 months (6–355 months). The histological diagnoses included 9 cases of synovial sarcoma. Treatment, local recurrence rate, survival rate, complication, and ADL were evaluated. Surgical procedures consisted of ray amputation in 5 cases and limb salvage surgery in 15. Of these 15 cases, 14 (93%) required reconstructive surgery for skin defects. One patient (5%) exhibited recurrence. The 5-year overall survival rate was 94.7%, and the 5-year local progression-free survival rate was 92.9%. Early postoperative complications were found in 5 of 20 cases (25%), comprising flap failure in 4 cases and infection in 1 case; however, all patients eventually demonstrated good functional outcomes. In this study, the recurrence rate was 5% for patients who underwent limb salvage surgery without radiation therapy, and we obtained good local control. Since the anatomical structures of the foot act as barriers to tumor progression, the use of a method of evaluating resection margins to determine the extent of resection enabled successful surgery, not combined with radiation, without the recurrence of sarcomas.

The approved standard dose of pazopanib is 800 mg per day, but the appropriate dose of pazopanib to treat soft tissue sarcoma (STS) patients in real-world practice is controversial. Of 124 STS patients treated with pazopanib, we retrospectively analyzed the cases of STS patients who achieved progression-free survival at 12 weeks by pazopanib treatment as pazopanib responders, and we evaluated their relative dose intensity (RDI) in the initial 12 weeks (12W-RDI). We enrolled 78 STS patients in the analyses as pazopanib responders, and 54 patients of the 78 pazopanib responders (69%) were able to maintain 12W-RDI &ge, 80%. In landmark analyses, patients with 12W-RDI of 80% &ge, 80% had significantly longer progression-free survival compared to those with 12W-RDI &lt, 80% (30.7 weeks vs. 22.0 weeks, hazard ratio [HR]: 0.56 [95%CI: 0.33&ndash, 0.94], p = 0.026). The most frequently observed reasons of treatment interruption and/or dose reduction of pazopanib during the initial 12 weeks were anorexia and liver function disorders. Liver toxicity was the adverse event most frequently observed in the 12W-RDI &lt, 80% patients throughout the treatment periods. Based on our results, it appears that maintaining as high a dose intensity as possible that is tolerable&mdash, at least during the initial 12 weeks&mdash, is likely to be the better option in pazopanib treatment for STS patients.

Background Endoprosthetic reconstruction of the proximal humerus is one of the standard procedures after resection of tumors of the proximal humerus and has been considered a reliable method to reconstruct the proximal humerus in recent reports. However, instability of the shoulder joint caused by loss of the rotator cuff and deltoid muscle function is often observed after such an endoprosthetic reconstruction. Methods We performed the endoprosthesis suspension method with polypropylene monofilament knitted mesh. This suspension method, by which the endoprosthesis is suspended from the bone structure, was used after resection of tumors in 9 patients. We assessed postoperative stability of the shoulder joint by comparing these patients with 12 patients who underwent the conventional surgical technique, by which the mesh-wrapped endoprosthesis is attached only to soft tissue. Results In radiographic and physical evaluation, 4 of the 12 patients in the soft tissue reconstruction group showed shoulder joint instability. No patient in the suspension method group showed subluxation of the humeral prosthesis. The mean shoulder flexion was 35° and 65° and the mean shoulder abduction was 40° and 40° for the soft tissue reconstruction group and the suspension method group, respectively. Discussion Shoulder joint subluxation sometimes occurs because of elongation of the attached soft tissue in the conventional reconstruction with mesh, whereas no shoulder joint subluxation occurs after endoprosthetic reconstruction in the suspension method because the bone structure has no leeway for elongation. Excellent stability of our new method enables exercise of the surgical shoulder at an early stage, leading to improved range of shoulder joint motion.

Pelvic Ewing sarcoma is associated with a worse prognosis. Thromboembolic events are relatively common in pediatric patients with cancers including sarcomas. We have presented a case of Ewing sarcoma arising from the left iliac bone with tumor thrombus of inferior vena cava (IVC) which was obtained complete response by both chemotherapy and irradiation. Magnetic resonance imaging (MRI) scan demonstrated that the tumor arising from the left iliac bone extended into the left side of sacral bone, suggesting the difficulty of surgical resection. Computed tomography (CT) revealed the existence of the tumor thrombus of IVC. We performed irradiation (31.2 Gy) and chemotherapy (combination of VCR, Act-D, IFM, and ADR). The tumor was controlled successfully, and the tumor thrombus of IVC has completely vanished. Four years after the treatment, coin lesion in the left upper lung appeared. Suspected of metastasis, segmental resection of the left upper lung was performed. Fourteen years after the surgery, the patient has been remained free of recurrence. It is clinically significant for surgeons to treat pelvic Ewing sarcoma with tumor thrombus.

Osteosarcomas are primary malignant tumors that are characterized by the production of osteoid or immature bone. However they are the most common primary malignant tumor of bone in adolescents and young adults, the diagnosis of osteosarcoma is often delayed. It is because of the rarity of population, the variety of findings, nonspecific symptoms and so on. A 46-year-old male patient had left knee pain for 3 months without any symptoms in his right knee. At a nearby clinic, X-ray of his healthy side was taken by chance. The irregular bone formation was however seen in the metaphysis of the distal femur with spreading into the soft tissues. No abnormal findings were pointed out at his left side knee. A diagnosis of pleomorphic sarcoma was made by incisional biopsy. Pulmonary CT showed multiple nodules which were considered as multiple lung metastases. He was diagnosed as right femoral sarcoma, clinical stage IV (T1N0M1a) by TNM classification. He underwent preoperative chemotherapy, followed by limb-saving operation. No preoperative complaints were found in his left knee. Final diagnosis of surgically resected specimen was osteoblastic osteosarcoma which showed neoplastic osteoid formation within pleomorphic malignant cells. However, osteosarcomas occurred in adult populations are sometimes occurred as a secondary neoplasms, he had a history of diabetes but no other medical history, such as radiation therapy for a solid cancer, Paget’s disease, other benign bone lesions or inherited conditions. As far as our knowledge, we believe that this is a first case report of asymptomatic incidental osteosarcoma.

Giant cell tumor (GCT) is classified as a benign bone tumor, but it is locally aggressive, and sometimes metastasizes in a benign state. In addition, malignant transformation occurs once in a while. Most of the secondary malignancies in GCT occur after treatment of benign GCT that has included radiation therapy [1, 2]. As a cytogenetic characteristic of GCT, telomeric associations (tas) were reported [3, 4]. Tas may generate dicentric chromosomes (dic) and chromatoid breakagefusion-bridges, which lead to chromosomal instability and tumorigenesis [5, 6]. Recently, the relationship between cytogenetic abnormalities and clinical behavior in GCT has begun to be elucidated. For example, the DNA ploidy pattern may predict the recurrence potential of GCT, chromosomal abnormalities superimposed on tas are responsible for an aggressive clinical course [7], and centrosome amplification may be useful in predicting the clinical behavior of GCT [8]. Here we report a case of secondary malignancy in GCT. Malignant transformation occurred in a relatively early period, and any radiation therapy was not administered to the primary lesion. Malignant transformation was demonstrated not only by histopathological study but also by cytogenetic analysis. The recurrent tumor, which was a secondary malignancy in GCT, had a near-triploid karyotype with multiple structural abnormalities as observed in pleomorphic sarcoma, while the primary benign GCT had a near-diploid karyotype with tas and dic.

Background Polymethymethacrylate (PMMA) is often used to reconstruct defects after curettage of Giant Cell Tumors (GCT). While GCTs usually originate in the epiphysis, the use of PMMA in distal femoral lesions may induce the risk of degenerative osteoarthritis (OA). We investigated the limb function of patients after curettage with PMMA beyond 20 years of follow-up. Methods Patients with more than 20 years of follow-up who underwent curettage with PMMA for distal femoral GCTs were observed. We retrospectively investigated the radiographic assessment of OA and functional assessment of the limb. The Kellgren and Lawrence (KL) grading system was used for radiographic evaluation. Results Five patients were included in this study. The mean age was 33 years, and the mean period from application of PMMA to final follow-up observation was 28.1 years. Four lesions were primary, and one lesion was recurrent. There were no patients with postoperative recurrence. There were no OA changes in preoperative radiographs. The shortest mean distance from PMMA to the articular cartilage was 4.6 mm on radiographs immediately after surgery. On radiographs at final follow-up observation, the KL grading were as follows: grade 1, 2 patients; grade 2, 1 patient; grade 3, 2 patients. All patients were able to independently ambulate without a crutch, and there was not enough pain to require nonsteroidal anti-inflammatory drugs. The mean flexion of the knee joint was 116°. Conclusions Although PMMA used for distal femoral GCTs exhibited OA changes beyond a 20 year follow-up period, there were no cases requiring artificial joints, and the affected limbs demonstrated good function.

Atypical femoral fractures (AFFs) occur in both osteoporosis patients and cancer patients who receive long-term bisphosphonate treatment. Denosumab offers an alternative approach for the treatment of bone metastases. We describe a 59-year-old woman with a history of breast carcinoma and bone metastasis who was prescribed denosumab for 4 years. The patient had no history of any prior bisphosphonate use. Bone scintigraphy showed an abnormal uptake in the right femur, which was confirmed as an impending AFF or atypical femoral stress reaction. In oncological patients receiving long-term denosumab, AFF should be included as a differential diagnosis for focal femoral findings.

Winged deformity of the scapula is defined as prominence of the medial border of the scapula. Osteochondroma arising from the ventral side of the scapula has been demonstrated to cause the mechanical symptoms of winged deformation. We present a 22-year-old man with winged deformity of the left scapula due to osteochondroma. Complete removal of the tumor relieved the symptoms, and histopathologic examination confirmed benign osteochondroma. There were no signs of recurrence on radiological images at the last follow-up of 10 months.

The acetabular component orientation of total hip arthroplasty is of critical importance to the clinical results. Although navigation systems have recently been introduced, acetabular component alignment guides are still used in most of ordinary hospitals. However, the accuracy of alignment guides themselves has not been evaluated. Fifteen types of alignment guide were examined. In all the alignment guides, the angles actually indicated and those stated by manufacturers were different. Our results showed that usage of modern alignment guides inherently misleads anteversion into decrease by a mean of 6 degrees (maximum, 12 degrees ) and inclination into increase by a mean of 2 degrees (maximum: 4 degrees ). Such setting of alignment guides could be one of the factors of error in acetabular component orientation.

This study focused on the evaluation of data concerning the clinical features of patients who were initially diagnosed with bone metastases of carcinoma from unknown primary sites that could not be detected, even using state of-the-art diagnostic modalities. The oncologic outcome of these patients is discussed. The clinical records of seven patients who had presented with bone metastases of carcinoma from unknown primary sites were retrospectively reviewed. Clinical features, treatment and outcome were analyzed. Extraskeletal metastases were located in the lymph nodes, liver, skeletal muscle, kidney, adrenal gland, and pleura. Six cases were observed in the pelvis, three in the femur, three in the skull, two in the rib, two in the cervical spine, two in the thoracic spine, two in the lumbar spine, one in the humerus, one in the radius, one in the clavicle, one in the scapula and one in the sternum. Four patients received systemic chemotherapy including platinum. At the last follow-up time of average 272 days, six patients were dead of disease and one patient was alive with disease. Although considerable progress has been made in the development of diagnostic modalities, including more recently FDG-PET, the primary tumor site cannot always be identified. Multiple bone and visceral organ metastases are often present in patients whose primary tumor was not detected. In the present study, it was found that systemic chemotherapy can appreciably increase the survival time of the patients with carcinoma metastases from unknown primary sites.

Recently, use of high-flexion design was introduced in cruciate-retaining (CR) total knee prostheses. The purpose of this study was to prospectively compare the ranges of motion (ROMs) of 89 knees with standard and 87 knees with high-flexion CR total knee prostheses. Differences in age, gender, diagnosis, preoperative ROM of the knee, and Knee Society Score between the 2 groups were not statistically significant. At 12-month follow-up, average ROM was 112.0 degrees +/- 12.6 degrees for standard, and 115.3 degrees +/- 13.4 degrees for high-flexion CR prosthesis (P = .101). To our knowledge, this is the first report on the ROM with the high-flexion CR total knee prosthesis. Using the technique of anterior referencing for femoral component sizing and using a fixed 7 degrees slope for the tibial component, we found no significant differences between groups with regard to ROM, clinical, or radiographic parameters.

In total hip arthroplasty (THA), acetabular component orientation has critically important effects on dislocation, range of motion, polyethylene wear, pelvic osteolysis, and component migration. The differences in the pelvic orientation in the intraoperative lateral position for insertion of acetabular component during operation and that in the postoperative supine position for evaluation of acetabular component orientation will be one of the factors, which make outliers in acetabular component orientation. We compared acetabular component orientation between intraoperative lateral position and postoperative supine position in 100 consecutive primary THAs.A total of 100 consecutive primary THAs (between October 2004 and December 2005) in 100 patients performed by a single surgical team were investigated. Intraoperative anteroposterior radiographs of pelvis in the lateral position and postoperative anteroposterior radiographs of pelvis in the supine position were taken. Acetabular component orientation (vertical tilt and anteversion) were measured using computer software.The absolute values of difference between measurements in the two positions were 5.3 degrees +/- 4.5 degrees (mean +/- SD) for vertical tilt and 5.1 degrees +/- 3.7 degrees for anteversion. The difference in the vertical tilt between the two positions was significant (P0.0001).The difference in the acetabular component orientation between the two positions, which might be caused by the difference between intra- and postoperative pelvic orientation, should be considered during THA.

e22541Background: The correlation between relative dose intensity (RDI) and clinical outcome of pazopanib therapy to STS patients in real-world clinical practice has not been established. Methods: ...

BackgroundSoft tissue metastases, in particular intraneural metastasis, from any carcinomas seldom occur. To our knowledge, no case of sciatic nerve palsy due to intraneural metastasis of gastric carcinoma is reported in the literature.Case presentationA case is reported of a 82-year old woman with sciatic nerve palsy with intraneural metastasis of gastric carcinoma. Although she had undergone partial gastrectomy with T2b, N0, M0 two years ago and primary site was cured, she developed sciatic nerve palsy from the carcinoma metastasis directly to the nerve. Operative resection and Histological examination revealed poorly differentiated adenocarcinoma, the same as her primary site adenocarcinoma.ConclusionsSciatica is usually caused by a herniated disc or spinal canal stenosis. Sciatic nerve palsy may be caused by nondiscogenic etiologies that may be either intrapelvic or extrapelvic. It is important to image the entire course of the nerve to distinguish these etiologies quickly. The longer the nerve compression the less likely a palsy will recover. Surgery is a good intervention that simultaneously obtains a tissue diagnosis and decompresses the nerve.

Background Dermatofibrosarcoma protuberans (DFSP) may recur locally but rarely metastasizes. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans (FS-DFSP) is said to have worse prognosis compared with ordinary DFSP (O-DFSP). Since DFSP rarely metastasizes, there have been few reports summarizing data on distant metastasis cases at single institution. The aim of this retrospective study is to review DFSP cases in order to analyze risk factors for metastasis. Patients and methods This retrospective study involved 67 patients. We analyzed O-DFSP and FS-DFSP metastasis rates, metastasis sites, time to metastasis, the relationship between frequency of local recurrence and metastasis, and the relationship between primary tumor size and metastasis. Results Distant metastasis was found in 5 (7.4 %) of 67 cases with DFSP. Of the five cases, the histopathological diagnosis was FS-DFSP in four cases and O-DFSP in one case. Out of five cases with metastasis, three had not recurred and two had recurred twice. No clear correlation was identified (Fisher’s exact test: p = 0.216). The primary tumor diameters in the metastatic cases were 15.0, 12.6, 20.5, 13.0, and 5.0 cm, respectively. The tumor diameters in metastatic cases were significantly larger (Fisher’s exact test: p