Inflammatory Rhabdomyoblastic Tumor: Clinicopathologic and Molecular Analysis of 13 Cases.

Inflammatory rhabdomyoblastic tumors (IRMTs) are newly recognized skeletal muscle tumors with uncertain malignant potential. We investigated 13 IRMTs using clinicopathologic, genetic, and epigenetic methods. The cohort included 7 men and 6 women, aged 23 to 80 years (median, 50 years), of whom 2 had neurofibromatosis type 1. Most tumors occurred in the deep soft tissues of the lower limbs, head/neck, trunk wall, and retroperitoneum/pelvis. Two tumors involved the hypopharyngeal submucosa as polypoid masses. Eight tumors showed conventional histology of predominantly spindled cells with nuclear atypia, low mitotic activity, and massive inflammatory infiltrates. Three tumors showed atypical histology, including uniform epithelioid or plump cells and mitotically active histiocytes. The remaining 2 tumors demonstrated malignant progression to rhabdomyosarcoma; one had additional IRMT histology and the other was a pure sarcoma. All 11 IRMTs without malignant progression exhibited indolent behavior at a median follow-up of 43 months. One of the 2 patients with IRMTs with malignant progression died of lung metastases. All IRMTs were positive for desmin and PAX7, whereas myogenin and MyoD1 were expressed in a subset of cases. Targeted next-generation sequencing identified pathogenic mutations in NF1 (5/8) and TP53 (4/8). All TP53 mutations co-occurred with NF1 mutations. TP53 variant allele frequency was much lower than that of NF1 in 2 cases. These tumors showed geographic (subclonal) strong p53 immunoreactivity, suggesting the secondary emergence of a TP53-mutant clone. DNA methylation-based copy number analysis conducted in 11 tumors revealed characteristic flat patterns with relative gains, including chromosomes 5, 18, 20, 21, and/or 22 in most cases. Widespread loss of heterozygosity with retained biparental copies of these chromosomes was confirmed in 4 tumors analyzed via allele-specific profiling. Based on unsupervised DNA methylation analysis, none of the 11 tumors tested clustered with existing reference entities but formed a coherent group, although its specificity warrants further study.

Image-guided core needle biopsy for musculoskeletal lesions.

BACKGROUND: Image-guided percutaneous core needle biopsy (CNB) has been an important diagnostic procedure for musculoskeletal lesions. Here we surveyed the variety of diagnostic strategies available and assessed the clinical usefulness and limitations of image-guided CNB carried out by a multidisciplinary team comprising specialists in various fields. METHODS: We conducted a retrospective study of 284 image-guided CNBs among 1899 consecutive biopsy procedures carried out at our institution for musculoskeletal tumorous conditions, focusing on their effectiveness including diagnostic accuracy and utility for classification of specimens according to malignant potential and histological subtype as well as their correlation with biopsy routes. RESULTS: Among the 284 studied biopsies, 252 (88.7%) were considered clinically "effective". The sensitivity for detection of malignancy was 94.0% (110/117) and the specificity was 95.3% (41/43). The diagnostic accuracy for detection of malignancy was 94.4% (151/160) and that for histological subtype was 92.3% (48/52). The clinical effectiveness of the procedure was correlated with the complexity of the biopsy route (P = 0.015); the trans-pedicular, trans-retroperitoneal and trans-sciatic foramen approaches tended to yield ineffective results. Repeat biopsy did not have a significant impact on the effectiveness of image-guided CNB (P = 0.536). CONCLUSIONS: The diagnostic accuracy rates of image-guided CNB performed at multidisciplinary sarcoma units were usable even for patients who have variety of diagnostic biopsy procedures. It is important to establish and implement diagnostic strategies based on an understanding that complicated routes, especially for spine and pelvic lesions, may be associated with ineffectiveness and/or complications.

Prognostic impact of IDH mutations in chondrosarcoma.

BACKGROUND: Mutant isocitrate dehydrogenase (IDH) in chondrosarcoma produces the oncometabolite 2-hydroxyglutarate (2-HG) and contributes to malignant progression, and is therefore a potential therapeutic target for chondrosarcoma. Robust historical control data are important in clinical trials of rare cancers such as chondrosarcoma in order to show a clear benefit of new drugs. However, it remains controversial whether IDH mutation status is associated with the clinical outcome of chondrosarcoma, and this hinders the development of mutant IDH inhibitors in clinical trials.background METHODS: We investigated the relationship between IDH gene status and clinicopathological data in 38 chondrosarcoma patients from whom frozen tumor samples were obtained at the time of biopsy or surgery. Targeted next-generation sequencing was also performed to compare genetic alterations between patients with and without IDH mutations. METHODS RESULTS: The results revealed 15 cases (40%) of heterozygous IDH1 mutations and five cases (13%) of IDH2 mutations. IDH-mutant chondrosarcoma was associated with worse overall survival than IDH-wild-type chondrosarcoma (IDH1/2 Mut vs. IDH Wt, P = 0.006; IDH1 Mut vs. IDH Wt, P = 0.030; IDH2 Mut vs. IDH Wt, P < 0.0001). IDH mutation was also a significant poor prognostic factor both in univariate (P = 0.026) and multivariate (P = 0.048) analyses. Targeted next-generation sequencing revealed that characteristic mutations in chondrosarcoma, including TP53 and COL2A1, were more common in the IDH-mutant group than in the IDH-wild-type group.results CONCLUSION: This study is the first to report in detail the characteristics and clinical courses of IDH-mutant chondrosarcoma patients in Japan. Our data suggested that IDH-mutant chondrosarcomas might have a worse prognosis than that of IDH-wild-type chondrosarcoma, possibly through the more aggressive characters after metastasis. This information will be useful for designing clinical trials of mutant IDH inhibitors for treatment of advanced chondrosarcoma.

Efficacy and safety of TAS-115, a novel oral multi-kinase inhibitor, in osteosarcoma: an expansion cohort of a phase I study.

Background osteosarcoma is a rare, primary malignant bone tumour with limited available treatments for advanced or recurrent disease, resulting in a poor prognosis for patients. TAS-115 is a novel tyrosine kinase inhibitor under investigation in a phase I study in patients with solid tumours. We report data of osteosarcoma patients in the expansion cohort of this ongoing study. Patients and methods an analysis of this multicentre, open-label study was performed 6 months after the final patient was enrolled, and included patients aged ≥15 years, with unresectable or recurrent osteosarcoma, and who had refractory to standard therapy or for whom no standard therapy was available. TAS-115 650 mg/day was orally administered in a 5 days on/2 days off schedule. Results a total of 20 patients with osteosarcoma were enrolled. The most common adverse drug reactions (ADRs) were neutrophil count decreased (75%), aspartate aminotransferase increased (50%), and platelet count decreased (50%); 85% of patients had grade ≥ 3 ADRs. Long-term disease control (>1 year) with TAS-115 was achieved in three patients. The best overall response was stable disease (50%); no patient achieved a complete or partial response. Median progression-free survival was 3 months; 4-month and 12-month progression-free rates were 42% and 31%, respectively. Conclusion the safety and tolerability of TAS-115 and long-term disease stability for patients with unresectable or recurrent osteosarcoma were confirmed in this study, suggesting that TAS-115 is a promising novel therapy for advanced osteosarcoma patients. Trial registration number: JapicCTI-132333 (registered on November 8, 2013).

Establishment and characterization of patient-derived cancer models of malignant peripheral nerve sheath tumors.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of soft-tissue sarcoma, derived from a peripheral branch or the sheath of the sciatic nerve, brachial plexus, or sacral plexus. The clinical outcomes for MPNST patients with unresectable or metastatic tumors are dismal, and novel therapeutic strategies are required. Although patient-derived cancer cell lines are vital for basic research and preclinical studies, few MPNST cell lines are available from public cell banks. Therefore, the aim of this study was to establish cancer cell lines derived from MPNST patients. METHODS: We used tumor tissues from five patients with MPNSTs, including one derived from a rare bone tissue MPNST. The tumor tissues were obtained at the time of surgery and were immediately processed to establish cell lines. A patient-derived xenograft was also established when a sufficient amount of tumor tissue was available. The characterization of established cells was performed with respect to cell proliferation, spheroid formation, and invasion. The mutation status of actionable genes was monitored by NCC Oncopanel, by which the mutation of 114 genes was assessed by next-generation sequencing. The response to anti-cancer agents, including anti-cancer drugs approved for the treatment of other malignancies was investigated in the established cell lines. RESULTS: We established five cell lines (NCC-MPNST1-C1, NCC-MPNST2-C1, NCC-MPNST3-C1, NCC-MPNST4-C1, and NCC-MPNST5-C1) from the original tumors, and also established patient-derived xenografts (PDXs) from which one cell line (NCC-MPNST3-X2-C1) was produced. The established MPNST cell lines proliferated continuously and formed spheroids while exhibiting distinct invasion abilities. The cell lines had typical mutations in the actionable genes, and the mutation profiles differed among the cell lines. The responsiveness to examined anti-cancer agents differed among cell lines; while the presence of an actionable gene mutation did not correspond with the response to the anticipated anti-cancer agents. CONCLUSION: The established cell lines exhibit various characteristics, including proliferation and invasion potential. In addition, they had different mutation profiles and response to the anti-cancer agents. These observations suggest that the established cell lines will be useful for future research on MPNSTs.

Minimal clinically important differences in Toronto Extremity Salvage Score for patients with lower extremity sarcoma.

BACKGROUND: The Toronto Extremity Salvage Score (TESS) is the most widely used patient-reported outcome measure for orthopaedic oncology patients. However, minimal clinically important differences (MCIDs) in the TESS have not been analyzed. The aim of this study was to define the MCIDs of TESS in patients with lower extremity sarcoma. METHODS: A total of 85 patients were investigated to calculate the MCIDs for TESS. Three different methods were used: 1) distribution-based methods based on one-half of the standard deviation and standard error of measurement (SEM) at the baseline, 2) anchor-based and receiver operating characteristic (ROC) analysis, and 3) anchor-based using Akaike's Information Criterion (AIC) analysis. RESULTS: The MCIDs at 6 months were 4.9-7.8 by distribution-based methods and 4.3-4.4 by anchor-based methods. The MCIDs at 12 months were 4.0-6.9 by distribution-based methods and 10.6-11.6 by anchor-based methods. CONCLUSIONS: We calculated MCID values for the TESS based on distribution- and anchor-based approaches. Our results seem reasonable since MCIDs calculated by the different approaches had similar values. This knowledge will enable clinicians to identify meaningful functional improvements in sarcoma patients.

Free latissimus dorsi musculocutaneous flap for external hemipelvectomy reconstruction.

INTRODUCTION: External hemipelvectomy is one of the most extensive surgical procedures for locally advanced pelvic tumors. Stump coverage with the local tissues can be difficult in recurrent cases. Herein, we report our experience with immediate stump coverage using a free latissimus dorsi musculocutaneous (LDMC) flap after external hemipelvectomy for recurrent pelvic malignancies. METHODS: Six patients underwent external hemipelvectomy and immediate reconstruction using a free LDMC flap between November 2012 and June 2017. The mean age of the patients was 65 years (range: 63-69 years). The primary tumors were myxoid liposarcoma, chondrosarcoma, osteosarcoma, squamous cell carcinoma, and pleomorphic liposarcoma. A free LDMC flap was harvested from the ipsilateral back and transferred to the defect. When an intercostal nerve was found at the recipient site, the thoracodorsal nerve was coaptated with the intercostal nerve to reinnervate the muscle. RESULTS: The mean flap size was 23 × 10 cm and the range was 20 × 8-27 × 13.5 cm. The contralateral deep inferior epigastric vessels were used as recipient vessels in all patients. Thoracodorsal-intercostal nerve coaptation was performed in 2 patients. The flap survived in all patients. Three patients had complications of abscess formation. No patient developed postoperative hernia. CONCLUSION: Although it is challenging to do reconstruction after external hemipelvectomy, a free LDMC flap has several advantages, including a large coverage area, stability of circulation, ease of elevation, and preservation of the strength of the remaining abdominal wall. Technical tips for selecting anastomosis vessels are important and nerve coaptation could be effective.

Development of a patient-oriented disease specific outcome measure of health-related quality of life (HRQOL) for musculoskeletal oncology patients.

BACKGROUND: According to improved functional outcome and life expectancy in orthopaedic oncology patients, there has been a growing interest in not only oncologic and functional outcomes but also health-related quality of life (HRQOL), including body image, mental status, or social activities, after surgery. However, there has been a lack of disease-specific measures focusing on the ability of orthopaedic oncology patients to evaluate their HRQOL comprehensively. Therefore, our aims in the present study were 1) to develop a patient-oriented disease-specific outcome measure of HRQOL for musculoskeletal oncology patients (COMMON-LE), and 2) to examine the practical applicability, reliability and validity of the COMMON-LE for patients with musculoskeletal tumors in the lower extremity. METHODS: The COMMON-LE was developed by expert committee of orthopaedic oncology and rehabilitation. A total of 101 patients were surveyed using the COMMON-LE, as well as the TESS, the MSTS score, and the SF-36, to assess their psychometric characteristics, including reliability, validity, and responsiveness. RESULTS: The COMMON-LE showed no marked floor and ceiling effects. Test-retest reliability and internal consistency determined using the intraclass correlation coefficient (0.928) and Cronbach's alpha (0.948-0.968), respectively, were excellent. Each domain of the COMMON-LE (pain, ADL, socioemotional condition and general health) was well correlated with the scores of the standard measures (SF-36, TESS, MSTS score). Factor analysis and the AIC network showed the questionnaire items of the COMMON-LE were clearly separable into three clusters according to their content, corresponding to each domain of the questionnaire. CONCLUSIONS: We have successfully developed and validated a disease-specific measure, the COMMON-LE, to evaluate not only physical function, but also various aspects of HRQOL in patients with musculoskeletal tumors. The COMMON-LE has sufficient reliability and internal consistency, and good validity, and appears to be practically applicable to this group of patients.

Reconstruction of Complex Groin Defects After Sarcoma Resection.

BACKGROUND: Complex groin defects after sarcoma resection require reconstruction of multiple vital structures that can include the major vessels, the overlying skin, the abdominal wall, and the hip joint. We investigated the feasibility of limb preservation after complex groin reconstruction. METHODS: The subjects were 12 consecutive patients who underwent complex groin reconstruction after sarcoma resection. In all patients, the defect included a major artery (external iliac or femoral) and overlying skin. The defect included the abdominal wall in 5 patients and the hip joint in 2. Arterial reconstruction was performed with an autologous vein graft in 9 patients and with an expanded polytetrafluoroethylene graft in 3. Soft-tissue coverage was performed with a pedicled rectus abdominis musculocutaneous flap in 7 patients, a free latissimus dorsi musculocutaneous flap in three, and a free anterolateral thigh flap in 2. RESULTS: Postoperative complications occurred in 7 patients (58.3%). Limb salvage was possible in 8 patients (66.7%) with 39 months' median follow-up. Two patients required amputation because of wound problems, and 2 required amputation because of local recurrence of the tumor. CONCLUSIONS: Limb salvage is possible even after extensive resection of groin sarcoma, although the rate of postoperative wound complications is substantial. Proper selection of the arterial conduit and the soft-tissue flap, depending on the components of the defect, are mandatory for successful reconstruction.

Cross-cultural adaptation and validation of the Japanese version of the Toronto Extremity Salvage Score (TESS) for patients with malignant musculoskeletal tumors in the upper extremities.

BACKGROUND: The Toronto Extremity Salvage Score (TESS) is a widely used disease-specific patient-completed questionnaire for the assessment of physical function in patients with musculoskeletal tumors; however, there had not been the validated Japanese version of the TESS. The aim of this study was to validate the Japanese version of the TESS in patients with musculoskeletal tumors in the upper extremity. METHODS: After developing a Japanese version of the TESS, the questionnaire was administered to 53 patients to examine its reliability and validity in comparison with the Musculoskeletal Tumor Society (MSTS) scoring system and Short Form-36 (SF-36). RESULTS: Test-retest reliability with intraclass correlation coefficient (0.93) and internal consistency with Cronbach's alpha (0.90) were excellent. Factor analysis showed that the construct structure consisted of 3-item clusters, and the Akaike Information Criterion network also demonstrated that the items could be divided into 3 domains according to their content. The TESS strongly correlated with the MSTS rating scale (r = 0.750; P < 0.001) and the SF-36 physical functioning scale (r = 0.684; P < 0.001). However, as expected, the TESS had low correlations with the SF-36 mental health and role-emotional subscales and the MSTS scoring system manual dexterity domain. CONCLUSIONS: Our study suggests that the TESS is a reliable and valid instrument to measure patient-reported physical functioning in patients with upper extremity sarcoma.

Successful Treatment of Extra-Renal Noncerebral Rhabdoid Tumors with VIDE.

Extra-renal noncerebral rhabdoid tumors (ERRTs) are highly aggressive and often lethal. An optimal chemotherapy regimen for ERRT remains undetermined. We report on three pediatric patients successfully treated with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE). Two of our patients who had metastatic or refractory disease have survived more than 2 years, one disease free without myeloablative megatherapy. The treatment with high-dose alkylator therapy is reported to have a beneficial effect on survival. A VIDE regimen containing high-dose ifosfamide is feasible and appears to prolong the survival of patients with ERRT. This regimen may be a promising option for ERRT treatment without myeloablative megatherapy.

[A Case of Severe Hematological Toxicity in Response to Pazopanib].

The patient(woman, approximately 46 years old)began pazopanib (PAZ) treatment (800 mg/day)f ollowing the recurrence of retroperitoneal leiomyosarcoma. Prior to treatment, the patient's platelet count was 18.6×10(4)/µl and her neutrophil count was 1.61×10(3)/µl . The platelet count decreased to 9.2×10(4)/µl on day 7 and to 5.4×10(4)/µl on day 21 after commencement of treatment. The neutrophil count was 0.97×10(3)/µl on day 28 and 0.68×10(3)/µl on day 35 after commencement of treatment. Thus, PAZ treatment was stopped on day 35. The blood sampling results on day 42 after commencement of treatment showed that the platelet count was 13.0×10(4)/µl and that the neutrophil count had recovered to 1.28×10(3)/µl . At that time, PAZ treatment was resumed at a reduced dose of 600 mg/day. By day 84 after commencement of treatment, the platelet count had increased from 12.7 to 13.8×10(4)/µl and the neutrophil count had increased from 1.02 to 1.34×10(3)/µl ; treatment was subsequently continued. The main adverse effects that have been reported for PAZ are hypertension and frequent liver dysfunction; these reports also indicate that the incidence of severe cytopenia(thrombocytopenia, neutropenia)is quite low. However, our patient exhibited cytopenia after commencement of PAZ treatment and her blood cell counts recovered once treatment was ceased, independent of other possible medications. Our findings suggest that cytopenia should be considered as an adverse effect of PAZ.

Pediatric myositis ossificans mimicking osteosarcoma.

Myositis ossificans (MO) is a rare benign cause of heterotopic bone formation in soft tissue that most commonly affects young adults, typically following trauma. We report the case of an 11-year-old girl who developed MO mimicking osteosarcoma in her right shoulder. Plain radiography and computed tomography showed poorly defined flocculated densities in the soft tissue and a periosteal reaction along the proximal humerus. On magnetic resonance imaging, the mass displayed an ill-defined margin and inhomogeneous signal change. Histologically, the mass had a pseudosarcomatous appearance. Based on these findings, the patient was initially misdiagnosed with osteosarcoma at another hospital. The diagnosis was difficult because the patient was 11 years old and had no trauma history, with atypical radiographic changes and a predilection for the site of origin for osteosarcomas. We finally made the correct diagnosis of MO by carefully reviewing and reflecting on the pathological differences between stages.

Free flow-through anterolateral thigh flap for complex knee defect including the popliteal artery.

Reconstruction of complex knee defects including the popliteal artery presents a challenging problem in reconstructive microsurgery. Reconstruction of the popliteal artery and soft-tissue coverage should be performed simultaneously for limb salvage. We present the one-stage reconstruction of a complex knee defect including the popliteal artery using a free flow-through anterolateral thigh (ALT) flap as a bypass flap in two patients with sarcomas. In both patients, the popliteal artery and vein were reconstructed using branches of the lateral circumflex femoral arterial system. The flaps survived without vascular compromise and the limbs were preserved successfully. Free flow-through ALT flap enables simultaneous leg revascularization and soft-tissue coverage with a single free flap. The lateral circumflex femoral arterial system has many branches with large diameters, making it suitable for reconstruction of multiple infrapopliteal arteries.

Pedicled superficial femoral artery perforator flaps for reconstruction of large groin defects.

Soft-tissue defects after wide resection of groin sarcomas have been reconstructed with well-characterized flaps, such as rectus abdominis, gracilis, and anterolateral thigh flaps. To our knowledge, the use of superficial femoral artery perforator (S-FAP) flaps for this purpose has not been reported. We report on three female patients in whom groin defects after sarcoma resection were reconstructed with pedicled S-FAP flaps. The dimensions of the skin defects ranged from 13.5 × 11 to 16 × 14.5 cm. Sizable perforators from the superficial femoral arteries were identified preoperatively around the apex of the femoral triangle with computed tomographic angiography or color Doppler ultrasonography. The lengths of the flaps ranged from 17 to 19 cm. The main perforator penetrated the sartorius muscle in two patients and emerged between the sartorius and the adductor longus muscles in the other patient. The postoperative course was uneventful, and results were satisfactory in all patients. The main advantages of the S-FAP flap over more commonly used flaps are that it is easier to harvest and is associated with less donor-site morbidity. We believe that the S-FAP flap may be a versatile option for the coverage of groin defects.

miR-34a predicts survival of Ewing's sarcoma patients and directly influences cell chemo-sensitivity and malignancy.

Identification of factors to detect chemotherapy-resistant tumours at diagnosis is a first priority for risk-adapted therapy in the oncology of children and young adults, where more individualized, effective, and less toxic treatments are highly desirable. In this study, we analysed the miRNAs discriminating Ewing's sarcoma (EWS) patients with different clinical outcomes in order to identify new indicators of prognosis. miRNA expression was investigated in 49 primary EWSs by using the Agilent human miRNA microarray v.2 and/or qRT-PCR. Statistical power of the samples studied for miRNA expression was verified, indicating adequate sample size. Microarray analysis defined a signature of five miRNAs (miR-34a, miR-23a, miR-92a, miR-490-3p, and miR-130b) as an independent predictor of risk for disease progression and survival. Validation analysis in the extended sample set indicated that both miR-34a and miR-490-3p achieved sufficient statistical power to predict prognosis. Results were particularly robust for miR-34a, which appeared associated with either event-free or overall survival and emerged as a significant predictor also after multivariate analysis. Patients with the highest expression of miR-34a did not experience adverse events in 5 years; in contrast, patients with the lowest expression recurred within 2 years. High expression of miR34a can be detected also in paraffin-embedded tissues by in situ hybridization, thus contributing to an easy routine evaluation of this miRNA. Functional analysis of miR-34a in EWS cell lines indicated that when miR-34a expression was enforced, cells were less proliferative, less malignant, and sensitized to doxorubicin and vincristine. Expression of miR-34a could be increased in p53wt cells by treatment with nutlin-3a. Accordingly, nutlin-3a synergizes with doxorubicin. Overall, our data indicate that miR-34a expression is a strong predictor of outcome in EWS. Restoration of miR-34a activity may be useful to decrease malignancy and increase tumour sensitivity to current drugs, so sparing excessive long-term toxicity to EWS patients.

An analysis of factors related to recurrence of myxofibrosarcoma.

OBJECTIVE: Myxofibrosarcoma is clinically characterized by a high frequency of local recurrence after surgery. To improve the clinical outcome of patients with myxofibrosarcoma, it is imperative to control any postsurgical local recurrence. METHODS: In this study, we performed a retrospective clinicopathologic analysis of 100 consecutive patients with myxofibrosarcoma to identify factors related to poor prognosis. All of the patients had been diagnosed, and had undergone surgery at the National Cancer Center Hospital between 1999 and 2008. RESULTS: At the initial visit to our hospital, 64 patients had primary myxofibrosarcoma, whereas 36 had undergone primary unplanned resection at other facilities. Of the 36 patients, 11 consulted our hospital before recurrence and 25 did so after recurrence. A histologically positive margin after surgery was evident in 28% of the cases overall. The estimated 5-year recurrence-free survival rate was 74.8%. Univariate analysis showed that primary unplanned resection at another facility (P = 0.0001) and a histologically positive margin (P = 0.0224) were significant predictors of local recurrence. When these two factors were subjected to multivariate analysis, only primary unplanned resection at another facility was significantly correlated with the estimated recurrence-free survival rate (P = 0.0011). Primary unplanned resection was also significantly related to the 5-year disease-free survival rate (P = 0.0401). CONCLUSIONS: Our findings indicate that primary unplanned resection at a non-referral hospital is the most important risk factor related to poor prognosis of myxofibrosarcoma. Accurate diagnosis and adequate initial surgery are most important factors for improving the clinical outcomes of myxofibrosarcoma.

Salvage high-dose rate brachytherapy for myxofibrosarcoma of the brachium: a technical report.

An 80-year-old male presented with T1N0M0 myxofibrosarcoma in or next to the humeral canal, which is located between the biceps and triceps of the right upper arm. Because the tumor was close to critical anatomical structures such as the brachial artery, median nerve and ulnar nerve, it was deemed impossible to perform limb-sparing surgery with an adequate resection margin. Therefore, preoperative external beam radiation therapy (EBRT) followed by limb-sparing surgery was offered. Magnetic resonance imaging taken after 40 Gy/20 fractions of EBRT showed an inadequate response, and limb-sparing surgery was not deemed possible at this point. Amputation of the right arm was offered, but the patient refused. Therefore, salvage high-dose-rate interstitial brachytherapy (HDR-ISBT) was offered. Under local anesthesia and sedation, 14 plastic needles were inserted, and 36 Gy in 6 fractions of HDR-ISBT was performed. Although radiation-induced incomplete paralysis of the median nerve was noted, no local progression or distant metastasis was found on the CT that was taken 2 years after the treatment.

Prediction of treatment outcomes in patients with chest wall sarcoma: evaluation with PET/CT.

OBJECTIVE: The aim of this study was to investigate the prognostic implications of (18)F-2-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography in patients with chest wall sarcoma. METHODS: Positron emission tomography/computed tomography scans of 42 patients (mean age: 46 years) with chest wall sarcomas were analyzed. Pathologic confirmation was obtained by surgical specimens in all patients. Tumor grade assessed by Ki-67 (MIB-1) immunohistochemical analysis and expression of glucose transporter protein 1 were compared with a maximum standardized uptake value. Univariate and multivariate analyses were conducted for estimates of overall and event-free survivals. RESULTS: The median maximum standardized uptake value of the tumor was 10.2 and the median MIB-1 index of the tumor was 32.5%. Glucose transporter protein 1 expression was found in 29 patients (69%). Univariate analyses revealed that surgery, chemotherapy, MIB-1 labeling index (cut-off 32.5%), MIB-1 grade, glucose transporter protein 1 expression and maximum standardized uptake value were possible predictors for overall and event-free survival. Multivariate analysis revealed that surgery (hazard ratio, 4.852; P = 0.017), maximum standardized uptake value (hazard ratio, 3.077; P = 0.037) and MIB-1 labeling index (hazard ratio, 6.549; P = 0.003) were independent predictors of event-free survival. In addition, surgery (hazard ratio, 4.092; P = 0.021) and maximum standardized uptake value (hazard ratio, 2.968; P = 0.027) were independent predictors of overall survival. CONCLUSIONS: (18)F-2-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography allows the prediction of prognosis after treatment in patients with chest wall sarcoma and may be useful in selecting high-risk patients for more risk-adapted treatments.