Bipolar Head Perforation With Rhabdomyosarcoma of the Thigh: A Case Report With Literature Review.

Background/Aim: High complication rates during the perioperative management of sarcomas around the pelvis have been reported; however, few include the detailed clinical course or complications in the late postoperative period. Radiotherapy is a multidisciplinary strategy for treating sarcomas. However, irradiated bone and soft tissues show a permanent loss of repair and immunocompetence. We present a case of pleomorphic rhabdomyosarcoma of the thigh that resulted in acetabular collapse induced by radiation and intestinal perforation during long-term follow-up. Additionally, we discuss the risk factors for late complications and pelvic reconstruction methods. Case Report: A 75-year-old man presented with a 1-month history of a recurring fever. Ten years prior, he was diagnosed with pleomorphic rhabdomyosarcoma of the right thigh and underwent a wide resection and bipolar hip arthroplasty, followed by chemotherapy and radiotherapy. Radiographs showed central dislocation of the bipolar head. Computed tomography revealed free air in the hip joint and thickening of the colon wall. Colonoscopy revealed displacement of the bipolar head into the colon wall. Colon resection and hip disarticulation were performed, as the bipolar head was contaminated with intestinal contents. Currently, he is able to walk stably with a walker, and there is no evidence of recurrence or metastasis of the tumor. Conclusion: Irradiation of the periacetabular bone may induce resorptive destruction, resulting in future structural failure. Hardware should not be used for periacetabular bone reconstruction; the risk of pelvic organ damage should be considered when the acetabular collapse becomes deteriorated. Therefore, other reliable and permanent reconstruction options are required.

Bone metastases with "false negative" findings on 18F-FDG PET/CT in patients with angiosarcoma: A case series with literature review.

RATIONALE: Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) is considered a reliable and indispensable imaging method when evaluating distant metastases and clinical staging of angiosarcomas. Here, we report 2 cases of angiosarcoma with bone metastases with "false negative" findings on 18F-FDG PET/CT. PATIENT CONCERNS: Case 1, a 39-year-old woman, who had undergone mastectomy for primary angiosarcoma 2 years prior, presented with a 5-month history of right coxalgia. Case 2 was a 37-year-old woman, who had undergone mastectomy for primary angiosarcoma 4 months prior. During postoperative follow-up, multiple bone lesions were detected on magnetic resonance imaging (MRI). DIAGNOSES: Based on the histopathological findings, both cases were diagnosed with bone metastases of angiosarcoma. Although MRI showed multiple bone metastatic lesions, 18F-FDG PET/CT showed no uptake or osteolytic destruction in both cases. INTERVENTIONS: Weekly paclitaxel was initiated as a salvage chemotherapy in both cases. OUTCOMES: No uptake or osteolytic lesions were observed on 18F-FDG PET/CT, despite multiple bone metastases detected on MRI. LESSONS: False-negative findings on 18F-FDG PET/CT should be considered when evaluating bone metastases of angiosarcoma. Even with negative findings on 18F-FDG PET/CT, open biopsy should be performed if MRI indicates bone metastases.

Clinical characteristics of sarcoma cases in which long-term disease control was achieved with trabectedin treatment: A retrospective study.

Trabectedin is a therapeutic option for patients with advanced sarcoma. While a randomized trial demonstrated its prolonged progression-free survival (PFS), the reported PFS was <6 months. Some patients can achieve long-term disease control with this treatment. However, the reference information is insufficient. Herein, we retrospectively reviewed 51 sarcoma patients who received trabectedin. We analyzed the clinicopathological features, trabectedin dose, administration schedule, and clinical outcomes, including the overall response rate (ORR) and PFS. Among them, we assessed the detailed data of patients who achieved long-term disease control (PFS >1 year). The ORR in the 49 evaluable patients was 8%, and the median PFS in 51 patients was 7.5 months. Six patients (12%) achieved PFS of >1 year. Five of the six patients had metastatic lesions at trabectedin initiation. The pathological subtypes were myxoid liposarcoma (n = 2), leiomyosarcoma (n = 2), synovial sarcoma (n = 1), and Ewing sarcoma (n = 1). The final administration dose was the minimum dose (0.8 mg/m2) in two patients who continued the treatment over 20 cycles. The best radiological response was partial response (PR) in two myxoid liposarcoma patients and stable disease in four. The durations from trabectedin initiation to the first response in the two PR cases were 163 and 176 days, respectively. Our results support the validity of continuing trabectedin at a sustainable dose and interval in patients who can tolerate it. These results may be useful when considering the clinical application of trabectedin.

Post-Systemic Chemotherapy Prognoses of Recurrent/Metastatic Soft Tissue Sarcoma Patients with Retroperitoneal/Intra-Abdominal Origin versus Those with Extremities/Trunk Origin.

BACKGROUND: The clinical benefit of systemic chemotherapy for recurrent/metastatic retroperitoneal/intra-abdominal soft tissue sarcoma (STS) compared to its benefits for other primary lesions has not been known or sufficiently evaluated. METHODS AND PATIENTS: We retrospectively reviewed the cases of the STS patients who consulted a department of medical oncology in Tokyo between June 2011 and March 2018, and we extracted the cases of patients with primary sites at the retroperitoneum/intra-abdomen (cohort R) or extremities/trunk (cohort E) who received systemic chemotherapy in a recurrent/metastatic setting, comparing the cohorts' characteristics, chemotherapy details, and prognoses. RESULTS: Of all 337 STS patients, we enrolled 49 patients in cohort R and 75 patients in cohort E. Liposarcoma was more frequently observed in cohort R (51.0%) than cohort E (22.7%). The median chemotherapy treatment line was two lines (range: 1-6) in cohort R and three lines (range: 1-9) in cohort E. The doxorubicin usage rates differed in recurrent/metastatic settings (90.0% in cohort R and 55.0% in cohort E), due mainly to the higher rate of a perioperative chemotherapy treatment history in cohort E (52.0% vs. 6.1% in cohort R). The median overall survival from the start of salvage chemotherapy was 31.9 months (cohort R; 95% CI: 20.9-42.8) and 27.1 months (cohort E; 95% CI: 21.6-32.5) (p = 0.549). CONCLUSION: There were differences in the distributions of pathology and antitumor drugs used in a salvage setting between retroperitoneal/intra-abdominal and extremities/trunk STS patients in recurrent/metastatic settings, but the prognoses with salvage chemotherapy were similar in the two cohorts.

Pre-Treatment Neutrophil-to-Lymphocyte Ratio (NLR) as a Predictive Marker of Pazopanib Treatment for Soft-Tissue Sarcoma.

Pazopanib with trabectedin and eribulin is widely used to treat soft-tissue sarcoma (STS). We have shown that baseline neutrophil-to-lymphocyte ratio (NLR) may predict the efficacy and patient prognosis of eribulin. Changes in NLR, but not baseline NLR, can predict patient prognosis of trabectedin. However, prognostic factors of pazopanib for STS have not been identified. We present a retrospective analysis of 141 patients treated with pazopanib for recurrent or metastatic non-round cell STS. Univariate and multivariate analyses were performed to determine the predictive factors of durable clinical benefit (DCB), overall survival (OS), and progression-free survival. L-sarcoma histology (odds ratio [OR] = 0.31, 95% CI = 0.12-0.79; p = 0.014) and pre-treatment NLR < 3.0 (OR = 2.03, 95% CI = 1.02-6.67; p = 0.045) were independent predictive factors of DCB. Pre-treatment NLR < 3.0 (hazard ratio [HR] = 0.55, 95% CI = 0.36-0.84; p = 0.0057), liposarcoma histology (HR = 1.78, 95% CI = 1.09-2.91; p = 0.022), primary extremity site (HR = 0.48, 95% CI = 0.31-0.75; p = 0.0010), ECOG PS ≥ 1 (HR = 1.62, 95% CI = 1.08-2.42; p = 0.019), and CRP < 0.3 (HR = 0.52, 95% CI = 0.33-0.82; p = 0.0050) were independent predictive factors of OS. These findings indicate that baseline NLR predicts the efficacy and patient prognosis of pazopanib for STS.

Surgical Treatment of Intramuscular Myxoma.

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.

Pre-treatment Neutrophil-to-Lymphocyte Ratio Predicts Efficacy of Eribulin for Soft-tissue Sarcoma.

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 <3.0 (HR=9.96, 95% CI=1.28-77.7; p=0.028) were independent factors predictive of durable clinical benefit. In addition, pre-treatment NLR <3.0 (HR=0.34, 95% CI=0.16-0.74; p=0.0059) and male sex (HR=0.23, 95% CI=0.10-0.52; p<0.001) were independent factors predictive of better progression-free survival. CONCLUSION: This retrospective study found that baseline NLR predicts the efficacy of eribulin for STS.

Differences in the efficacy and safety of eribulin in patients with soft tissue sarcoma by histological subtype and treatment line.

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.

Changes in Neutrophil-to-lymphocyte Ratio Predict Efficacy of Trabectedin for Soft-tissue Sarcoma.

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) <0.5 (HR=2.40, 95% CI-1.01-5.72; p=0.048) were independent factors predictive of longer OS. In addition, age ≥40 (HR=0.23, 95% CI=0.10-0.52; p<0.001) was an independent predictor of longer PFS. Conclusion: Changes in NLR and age ≥40 years were able to predict the efficacy of trabectedin for STS.

The Clinical Features of Multicentric Extra-abdominal Desmoid Tumors.

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.

Osteosarcoma arising from acetabulum extended to femoral head through round ligament: a case report.

A skip metastasis was defined as a solitary separate focus of osteosarcoma occurring synchronously with a primary osteosarcoma in the absence of anatomic extension. The progression of skip metastasis is considered less likely because the articular cartilage acts as a barrier, so there have been few reports on progression of the extremity bone tumor across a joint. In our case report, the acetabular osteosarcoma progressed to the femoral head through the ligament of the femoral head. From the findings of magnetic resonance imaging and resected specimen and tissue specimen, we considered that the tumor progressed between ligament and synovial tissue covering the ligament, and not passing through the inside of the ligament. This case suggested a possibility that the tumor might progress through the synovium around the ligament of femoral head in the cases of osteosarcoma arising from the proximal femur and acetabulum.

Primary intramucosal synovial sarcoma of the sigmoid colon in a patient with a germline mutation in the MSH2 gene: A case report.

Synovial sarcoma is a high-grade soft tissue sarcoma that occurs primarily in the deep soft tissue of extremities, and primary colorectal synovial sarcoma is extremely rare. In this report, we present a synovial sarcoma mostly located within the mucosa of the sigmoid colon. The patient was a man in his forties with a germline deletion in the MSH2 gene. He had experienced undifferentiated pleomorphic sarcoma of the left forearm 7 years before and adenocarcinoma of the transverse colon 6 years before, both of which were successfully treated and exhibited no recurrence to date. A surveillance colonoscopy for Lynch syndrome revealed the tumor which had a submucosal tumor-like appearance with central erosion and endoscopic resection was performed. Histologically, it was composed of monotonous proliferation of spindle cells arranged in cellular fascicles; these findings were compatible with monophasic fibrous synovial sarcoma. In the tumor cells, the presence of the SS18-SSX1 fusion gene was confirmed. Protein expression of mismatch repair genes was intact in the tumor cells, indicating the association between microsatellite instability and synovial sarcoma was weak. The present case highlights a rare primary site of synovial sarcoma in a patient with Lynch syndrome.

Definitive surgery of primary lesion should be prioritized over preoperative chemotherapy to treat high-grade osteosarcoma in patients aged 41-65 years.

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.

Simultaneous bifocal and asymptomatic intramuscular nodular fasciitis of the thigh: A case report.

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.

The Role of Neoadjuvant Chemotherapy in Resectable Primary Synovial Sarcoma.

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<0.05). Among the patients with grade 2 tumor, tumor size did not influence prognosis. Neoadjuvant chemotherapy improved the overall survival of patients who had grade 3 tumors. CONCLUSION: Treatment with neoadjuvant chemotherapy proved beneficial for high-risk patients with grade 3 synovial sarcoma but was not effective for those with low-risk and grade 2 tumor. Tumor size and histological grade were important factors in patient prognosis but had no connection with pathological patterns.

The Extracompartmental Tumoral Invasion of Extraskeletal Myxoid Chondrosarcoma Induces Distant Metastasis.

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.

Relative Dose Intensity of Induction-Phase Pazopanib Treatment of Soft Tissue Sarcoma: Its Relationship with Prognoses of Pazopanib Responders.

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 ≥80%. In landmark analyses, patients with 12W-RDI of 80% ≥80% had significantly longer progression-free survival compared to those with 12W-RDI <80% (30.7 weeks vs. 22.0 weeks, hazard ratio [HR]: 0.56 [95%CI: 0.33⁻0.94], 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 <80% patients throughout the treatment periods. Based on our results, it appears that maintaining as high a dose intensity as possible that is tolerable-at least during the initial 12 weeks-is likely to be the better option in pazopanib treatment for STS patients.

Giant cell tumor of the distal femur: Outcome beyond 20 years of follow-up after curettage with polymethylmethacrylate.

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.

Successful Complete Response of Tumor Thrombus after Combined with Chemotherapy and Irradiation for Ewing Sarcoma.

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.

Multicentric giant cell tumor of bone: Case series of 4 patients.

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.