A case of pelvic EWSR1-PATZ1 fusion sarcoma treated with carbon ion radiotherapy.

EWSR1-PATZ1 fusion sarcoma is a type of round-cell sarcoma with EWSR1-non-EST fusion that was newly categorized in the 2020 World Health Organization classification of soft tissue and bone tumors. In general, local disease is managed via surgical resection; however, at present, there is no standard therapy for locally advanced or metastatic disease. Here, we report our experience with a middle-aged male patient with pelvic EWSR1-PATZ1 fusion sarcoma who was treated with carbon ion radiotherapy and maintained stable disease for 13 months. The patient's clinical course suggests that carbon ion radiotherapy may be effective in patients with locally advanced EWSR1-PATZ1 fusion sarcoma.

The Combined Use of Inflammation Markers, Modified Glasgow Prognostic Score, and Sarculator Nomogram in Extremity Soft Tissue Sarcoma: A Multicenter Observational Study.

BACKGROUND: Sarculator is a validated nomogram designed to predict overall survival (OS) in extremity soft tissue sarcoma (STS). Inflammation plays a critical role in cancer development and progression. There were no reports which investigated the relationship between Sarculator and inflammation. METHODS: A total of 217 patients with extremity STS were included. The Sarculator-predicted 10-year probability of OS (pr-OS) was stratified into two subgroups: lower risk (10-year pr-OS ≥ 60%) and higher risk (10-year pr-OS < 60%). The modified Glasgow prognostic score (mGPS) varied from 0 to 2. RESULTS: Out of the 217 patients, 67 were classified as higher risk, while 150 were lower risk. A total of 181 patients had an mGPS of 0, and 36 had a score of 1 or 2. The 5-year OS was 83.3%. When patients were divided into two groups according to the 10-year pr-OS, those with a higher risk had poorer OS than those with a lower risk. Among the patients with a higher risk, those with an mGPS of 1 or 2 had poorer OS compared to those with a score of 0. CONCLUSIONS: The mGPS could potentially play an important role in identifying patients who are at high risk of death and metastasis in the higher-risk group on the Sarculator.

Prognostic nutrition index as a predictive factor for overall survival in trabectedin-treated advanced soft tissue sarcoma.

BACKGROUND: Trabectedin binds covalently to the DNA minor groove and causes DNA to bend toward the main groove, then trabectedin regulates the transcription of the involved genes in cell proliferation or acts on the mononuclear phagocyte system in tumors, which contributes to its antitumor effects. Several clinical trials confirmed the efficacy of trabectedin for patients with advanced soft tissue sarcoma (STS) although clinically useful biomarkers remained unidentified. This study aimed to identify prognostic factors of trabectedin treatment, especially focusing on the systemic inflammatory, immune response, and nutritional status. METHODS: This study included 44 patients with advanced STS treated with trabectedin from January 2018 to August 2022. We evaluated the associations of clinical factors that influence the efficacy of trabectedin treatment with progression-free survival (PFS) and overall survival (OS), focusing on systemic inflammatory, immune response, and nutritional status represented by the absolute lymphocyte count (ALC), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic inflammation response index (SIRI), prognostic nutrition index (PNI), and C-reactive protein (CRP) using the Kaplan-Meier method and the log-rank test. RESULTS: ALC, LMR, PNI, NLR, PLR, and SIRI demonstrated no association with PFS. Patients with CRP of ≥0.3 had a significantly shorter PFS than those with CRP of <0.3 (median PFS: 863 vs. 105 days, P = 0.045). PNI of ≥44 (median: 757 days vs. 232 days, P = 0.021) and CRP of <0.3 (median: 877 days vs. 297 days, P = 0.043) were significantly good prognostic factors in terms of OS. CONCLUSIONS: The study results indicate pretreatment PNI and CRP levels as prognostic factors for trabectedin treatment in advanced STS.

Intra- and inter-rater reliabilities of skin mechanical properties measured in healthy individuals using skin elasticity meter.

PURPOSE: The aim of this study is to establish a standardized measurement method and to examine the intra- and inter-reliabilities and absolute reliability of measuring skin mechanical properties using a skin elasticity meter (Cutometer®). METHODS: Ten healthy participated in the study. Skin mechanical properties were measured at four sites: upper arm, lower arm, upper leg and lower leg on both sides in supine position using a non-invasive skin elasticity meter by two trained different raters. The measurements include quantitative indices of the maximal distensibility (R0), elasticity (R2, R5, R7), and viscoelasticity (R6). Intra- and inter- relative reliabilities were determined using the intraclass correlation coefficient (ICC) (1,1) and ICC (2,1) methods, respectively. The absolute reliability was assessed via the Bland-Altman analysis. Moreover, we evaluated the minimal detectable change at a 95% confidence level (MDC95). RESULTS: At each site, the ICC (1,1) values were >0.90, and the ICC (2,1) values were >0.50. The Bland-Altman analysis did not reveal any fixed errors, and several sites and parameters have proportional errors. CONCLUSIONS: In this study, intra- and inter-reliabilities were measured at "excellent" and more than "moderate" levels, respectively. However, because some proportional errors were observed, the limits of reliability agreement should be considered when using the proposed methods. We believe that the results of this study can be applied to clinical research in field of rehabilitation treatment.

Relationship between muscle thickness measured by ultrasound and physical functions: A 2-year follow-up study of allogeneic hematopoietic stem cell transplantation recipients.

BACKGROUND: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a potentially curative treatment for hematological malignancies. Several complications following allo-HSCT, such as graft-versus-host disease, infection, and malnutrition, often cause physical dysfunction, and the assessment of physical function and evaluation of muscle mass are incompletely performed. Use of ultrasound (US) allows muscle mass measurement in patients with poor general conditions. In allo-HSCT recipients, the correlation between physical function and muscle thickness, as measured by US, remains unclear. OBJECTIVE: To clarify whether muscle thickness measured by US correlated with physical function in allo-HSCT recipients. DESIGN: A single-center prospective cohort study. SETTING: Hospital. PATIENTS: Ninety-two patients underwent allo-HSCT at our hospital from April 2017 to March 2019. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE(S): Biceps and quadriceps muscle thickness measured by US, grip strength, isometric muscle strength (elbow flexion and knee extension), and 6-minute walking test (6MWT) before allo-HSCT and on days 30, 90, 180, 1 year, and 2 years after allo-HSCT. The implementation rates of these assessments were also investigated. RESULTS: Correlations were observed between biceps thickness and elbow flexion muscle strength/grip strength before allo-HSCT, on days 30, 90, 180, 1 year, and 2 years after allo-HSCT (r = 0.71/0.74, 0.73/0.72, 0.70/0.79, 0.67/0.75, 0.72/0.75, and 0.85/0.79, respectively, all p < .001). At the same time points, quadriceps thickness moderately correlated with knee extensor strength (r = 0.49, 0.50, 0.45, 0.64, 0.61, and 0.58, all p < .001). However, biceps and quadriceps thicknesses did not correlate with the 6MWT. The percentages of patients measured with US and 6MWT were 93.4% and 82.4% (p = .01) on day 30 and 97.5% and 87.8% (p = .02) on day 90, respectively. CONCLUSIONS: US assessment may be a useful alternative method for estimating muscle strength in fragile allo-HSCT recipients, particularly when physical function assessment is difficult to quantify.

Secondary leiomyosarcoma of the nasal cavity in a treated patient with possible hereditary retinoblastoma with germline reciprocal translocation of RB1 and DMXL1 and somatic TP53 mutation: A case report.

Retinoblastoma is a common primary intraocular malignant tumor that affects infants and young children. Radiation therapy for hereditary retinoblastoma increases the risk of secondary malignancy. The present report discusses the case of a retinoblastoma survivor who developed secondary leiomyosarcoma 42 years after receiving radiation therapy. The retinoblastoma of the patient was unilateral, and the patient had no family history of the disease. RNA and DNA panel sequencing of the leiomyosarcoma tissue was performed to elucidate the molecular mechanism of this secondary malignancy. The RNA panel sequencing detected a germline reciprocal translocation of RB1 and DMXL1, leading to a diagnosis of possible hereditary retinoblastoma. Furthermore, it detected a somatic fusion gene (RAD51-KNL1). The DNA panel sequencing identified various germline or somatic variants, including a somatic splice acceptor site mutation of TP53. We hypothesized that the molecular mechanism of the secondary malignancy of this patient was the combination of a germline reciprocal translocation of RB1 and DMXL1 and the accumulation of various somatic mutations containing the splice acceptor site mutation of TP53, which ultimately led to the development of a secondary leiomyosarcoma. Further prospective investigations are necessary to fully understand the role of reciprocal translocation of RB1 and DMXL1 or other mutations in the tumorigenesis of second malignancies in patients with hereditary retinoblastoma.

SHARPIN Enhances Ferroptosis in Synovial Sarcoma Cells via NF-κB- and PRMT5-Mediated PGC1α Reduction.

Sarcoma is a rare type of cancer for which new therapeutic agents are required. Ferroptosis is a nonapoptotic cell death triggered by iron-mediated lipid peroxidation. We found that TFRC, an iron uptake protein, was expressed at higher levels in sarcoma cell lines than in noncancer and carcinoma cell lines. Glutathione peroxidase 4 (GPX4) protects cells against ferroptosis, and its inhibition using RAS-selective lethal 3 (RSL3) had an antitumor effect that was more pronounced in sarcoma cell lines, particularly synovial sarcoma cells, compared to non-sarcoma cells. Because NF-κB can provoke ferroptosis, we examined the role of SHARPIN, an activator of NF-κB, in sarcoma. We found that SHARPIN expression was significantly associated with reduced survival in cohorts of patients with cancer, including sarcoma. In addition, SHARPIN promoted the sensitivity of sarcoma cells to ferroptosis. Further analyses revealed that the PGC1α/NRF2/SLC7A11 axis and BNIP3L/NIX-mediated mitophagy are regulated through NF-κB and PRMT5 downstream of SHARPIN. Our findings suggest that ferroptosis could have a therapeutic effect in sarcoma, particularly in subpopulations with high TFRC and SHARPIN expression.

Genetic characterization of a novel organoid from human malignant giant-cell tumor.

Malignant giant-cell tumors are extremely rare bone sarcomas that transform from conventional giant-cell tumors during long periods of treatment. Owing to their rarity, no further analysis of their molecular pathogenesis exists, and thus, no standard treatment has been established. Recently, organoid culture methods have been highlighted for recapturing the tumor microenvironment, and we have applied the culture methods and succeeded in establishing patient-derived organoids (PDO) of rare sarcomas. This study aimed to investigate the genomic characteristics of our established novel organoids from human malignant giant-cell tumors. At our institute, we treated a patient with malignant giant-cell tumor. The remaining sarcoma specimens after surgical resection were cultured according to the air-liquid interface organoid-culture method. Organoids were xenografted into NOD-scid IL2Rgnull mice. The developed tumors were histologically and genomically analyzed to compare their characteristics with those of the original tumors. Genetic changes over time throughout treatment were analyzed, and the genomic status of the established organoid was confirmed. Organoids from malignant giant-cell tumors could be serially maintained using air-liquid interface organoid-culture methods. The tumors developed in xenografted NOD-scid IL2Rgnull mice. After several repetitions of the process, a patient-derived organoid line from the malignant giant-cell tumor was established. Immunohistochemical analyses and next-generation sequencing revealed that the established organoids lacked the H3-3A G34W mutation. The xenografted organoids of the malignant giant-cell tumor had phenotypes histologically and genetically similar to those of the original tumor. The established organoids were confirmed to be derived from human malignant giant-cell tumors. In summary, the present study demonstrated a novel organoid model of a malignant giant-cell tumor that was genetically confirmed to be a malignant transformed tumor. Our organoid model could be used to elucidate the molecular pathogenesis of a malignant giant-cell tumor and develop novel treatment modalities.

Local surgery feasibility and safety after carbon ion radiotherapy for primary bone sarcomas.

BACKGROUND: It is known that several complications are caused by local surgery after radiotherapy. Clinical reports that describe the postoperative complications associated with surgery after carbon ion radiotherapy are sparse. This study aimed to elucidate local surgery feasibility after carbon ion radiotherapy specifically for primary bone sarcomas. METHODS: The medical, surgical, and irradiation records of patients who had local surgery at the area irradiated with carbon ion beams between 2004 and 2018 were reviewed retrospectively to evaluate the feasibility and indication of local surgery after CIRT. RESULTS: There were eight patients who had 10 local surgeries at the irradiated sites among the 42 carbon ion radiotherapy patients. There were seven males and one female with a median age of 50 years (range 26-73 years). The reasons for surgery were three for skin toxicity and associated infection, five for bone collapse, and associated implant failure, and two for tumor regrowth. All surgical fields included the area of more than 60 Gy (RBE) irradiated dose. All three surgical cases caused by skin toxicity and associated infection had Grade I wound complication after surgery according to the Clavien-Dindo Classification. CONCLUSION: Local surgery after CIRT appeared feasible in selected patients with primary bone sarcoma, especially for the patients with bone collapse and associated implant failure. However, infection and prescribed irradiation dose at the incision site must be carefully evaluated.

Hemi-Resection of Carpal and Extensor Resection Reconstructed With Wrist Joint Arthrodesis and Palmaris Longus Tendon Graft for Synovial Sarcoma on the Dorsal Side of the Wrist: A Case Report.

Bone and soft-tissue sarcomas infrequently develop in the hand and wrist. Given the complex anatomy of this area, wide resection with adequate margins often impairs hand function because of the resection of essential structures, including tendons, bones, and tissues adjacent to the sarcoma. Here, we present a case of primary synovial sarcoma adjacent to the dorsal side of the carpal bones, which were resected with hemi-resection of the carpus and resection of extensor tendons, followed by wrist joint arthrodesis and palmaris longus tendon grafting. Hand function was satisfactory despite some disability, and no evidence of local recurrence was observed at the 24-month postoperative follow-up. This method may be effective for not only achieving tumor resection with a negative margin but also preserving hand function.

Does oral intake of thin fluids increase aspiration pneumonia for dysphagia after head and neck cancer surgery? A retrospective study for a total of 654 cases.

OBJECTIVE: Head and neck cancer (HNC) treatment causes dysphagia, which may lead to aspiration pneumonia (AP). Thickened fluids are widely used to prevent aspiration in patients with dysphagia; however, there is little evidence that they can prevent AP. This study aimed to clarify the differences between restriction of oral intake of fluids (R), only thickened fluids (TF), and no restriction of fluids (NR) for AP in patients with dysphagia after HNC treatment. METHODS: We retrospectively studied 654 patients with dysphagia after HNC surgery between 2012 and 2021. Of these, 255 had some restriction of fluids. The development of possible AP and administration of antibacterial drugs were used as outcomes. Multivariate linear regression and propensity score matching analyses were performed. RESULTS: The mean patient age was 64 ± 13, 67 ± 11, and 68 ± 10 years, while the Dynamic Imaging Grade of Swallowing Toxicity score 3-4 was 2.8%, 27.5, and 53.3%% water in NR, TF, and R groups, respectively. AP was diagnosed or suspected after starting oral intake in 37 (9.3%), 11 patients (15.9%), and 45 (17.6%) and antibacterial drugs were administered in 11 (2.8%), 7 patients (10.1%), and 25 (9.8%) in NR, TF, and R groups, respectively. R and TF had significant negative impacts on AP. CONCLUSIONS: Fluid restrictions may not reduce the risk of AP or affect the administration of antibacterial drugs. Medical staff should bear in mind that fluid restrictions do not necessarily prevent AP in patients with HNC.

Short-term clinical outcomes of Kyocera Modular Limb Salvage System designed cementless stems for the endoprosthetic reconstruction of lower extremities: a Japanese Musculoskeletal Oncology Group multi-institutional study.

BACKGROUND: The high rate of aseptic loosening of cemented stems has led to their frequent use in endoprosthetic reconstruction. However, problems, such as stem breakage and stress shielding at the insertion site, remain. The Japanese Musculoskeletal Oncology Group (JMOG) has developed Kyocera Modular Limb Salvage System (KMLS) cementless stems with a unique tapered press-fit and short fixation design. This study aimed to clarify the short-term postoperative outcomes of this prosthesis and validate the stem design. METHODS: One hundred cases of KMLS cementless stems (51 male patients; median age, 49 years; mean follow-up period, 35 months), with a minimum follow-up of 2 years, for the proximal femur (PF), distal femur (DF), and proximal tibia were prospectively registered for use. Prosthesis survival, complication rates, postoperative functional, and radiographical evaluation were analyzed. Complications or failures after insertion of the KMLS endoprostheses were classified into five types and functional results were analyzed according to the MSTS scoring system at postoperative 1 year. The diaphyseal interface and anchorage were graded by the ISOLS system at postoperative 2 years. RESULTS: The overall prosthesis survival rates at 2 and 4 years were 88.2 and 79.6%, respectively. The prosthesis-specific survival rate excluding infection and tumor recurrence was 90.2 and 87.9%, respectively. Younger age (p = 0.045) and primary tumor (p = 0.057) were associated with poor prognosis of prosthesis-specific survival excluding infection and tumor recurrence. Complications were observed in 31 patients, 13 patients underwent revision surgery. The mean MSTS functional score at 1 year postoperatively was 68%. Early implant loosening was significantly more common in the DF (p = 0.006) and PF/DF straight stem (p = 0.038). The ISOLS radiographic evaluation at 2 years after surgery revealed good bone remodeling and anchorage in most cases (bone remodeling: 90% / excellent and good, anchorage: 97% / excellent and good). CONCLUSIONS: Tumor endoprosthesis long-term fixation to the diaphysis of the lower extremity remains challenging. The KMLS cementless stem with a unique tapered press fit design showed good short-term results in maintaining bone stock. To prevent early loosening, a curved stem should be used in PF and DF, but long-term follow-up is necessary.

Establishment of Organoids From Human Epithelioid Sarcoma With the Air-Liquid Interface Organoid Cultures.

Background: Although biological resources are essential for basic and preclinical research in the oncological field, those of sarcoma are not sufficient for rapid development of the treatment. So far, some sarcoma cell lines have been established, however, the success rate was low and the established sarcoma types were frequently biased. Therefore, an efficient culture method is needed to determine the various types of sarcomas. Organoid culture is a 3-dimentional culture method that enables the recapitulation of the tumor microenvironment and the success rate reported is higher than the 2-dimentional culture. The purpose of this study was to report our newly established organoids from human epithelioid sarcoma using the air-liquid interface organoid culture method. Methods: We treated 2 patients with epithelioid sarcoma in our institute. The remaining sarcoma specimens after surgical resection were embedded in collagen type 1 gels according to the air-liquid interface organoid culture method. After serial passages, we xenografted the organoids to NOD-scid IL2Rgnull (NSG) mice. Using the developed tumors, we performed histological and genomic analyses to compare the similarities and differences with the original epithelioid sarcoma from the patient. Results: Organoids from the epithelioid sarcoma could be serially cultured and maintained in collagen type 1 gels for more than 3 passages. Developed orthotopic tumor xenografts were detected in the NSG mice. After the process was repeated severally, the patient derived organoid lines from the epithelioid sarcoma were established. The established organoids showed loss of integrase interactor 1 expression with polymerase chain reaction and immunohistochemical analyses. The xenografted organoids of the epithelioid sarcoma had histologically similar phenotypes with the original tumor and genetically resembled it to some degree. Conclusions: The present study demonstrated 2 novel established organoid models of epithelioid sarcoma, and our organoid models could be used to investigate the molecular pathogenesis and develop a novel treatment.

Impact of Surgery and Chemotherapy on Metastatic Extrauterine Leiomyosarcoma.

Background: Few studies have described the characteristics and prognostic factors of patients with metastatic extrauterine leiomyosarcoma (euLMS). Therefore, we retrospectively investigated the clinicopathological features, clinical outcomes, and prognostic factors of patients with euLMS. Methods: We recruited 61 patients with metastatic euLMS treated from 2006 to 2020 and collected and statistically analyzed information on patient-, tumor-, and treatment-related factors. The median follow-up period was 21.1 months. Results: Sixty-one patients with euLMS and a median age of 59 years were included. Furthermore, their five-year overall survival (OS) rate was 38.3%. Univariate analysis revealed that primary tumor size >10 cm, synchronous metastasis, initial metastatic sites >1, and no metastasectomy with curative intent were significantly associated with poor OS rate. Multivariate analysis identified primary tumor size >10 cm as an independent prognostic factor for poor OS. Among 24 patients who received metastasectomy with curative intent, the interval from the initial diagnosis to development of metastasis ≤6 months was significantly correlated with unfavorable OS. Among 37 patients who did not receive metastasectomy, chemotherapy after metastasis development was significantly related to better OS. Conclusions: Complete metastasectomy should be considered for metastatic euLMS treatment. Moreover, chemotherapy could prolong survival in patients with metastasis who are ineligible for metastasectomy.

Comorbidity, body mass index, and performance status as prognostic factors in older patients with soft-tissue sarcoma.

INTRODUCTION: Surgery, chemotherapy, and radiotherapy are the treatments for soft tissue sarcoma (STS) similar to those for other cancers. However, treatment is even more difficult in older patients because of aging-related weakness. The influence of aging-associated factors on prognosis in the patients with STS has not been fully elucidated. This study aimed to clarify the factors that impact prognosis in older patients with STS. MATERIALS AND METHODS: The present study retrospectively analyzed the data of 262 (≥60 years old) patients with STS. Moreover, this study investigated (1) tumor-associated parameters (e.g., tumor site, tumor size, depth, histological grade [Federation Nationale des Centres de Lutte le Cancer], American Joint Committee on Cancer [AJCC, eighth edition] stage, and histotype); (2) treatment-related factors (i.e., presence of surgery, neoadjuvant/adjuvant chemotherapy, adjuvant radiotherapy, and surgical margin); and (3) patient-dependent counterparts (e.g., age, gender, performance status [PS], body mass index [BMI], and Charlson comorbidity index [CCI]). Statistical analysis was conducted to evaluate the impact of each factor on overall survival (OS). RESULTS: Among the 262 patients, 217 and 45 were 60-79 and ≥80 years old, respectively. A comparison of the sociodemographic background showed no significant difference other than surgical margin and PS between those groups. According to univariate analysis, the significant factors to impact OS were age, stage, tumor size, depth, surgical margin, PS, BMI, and CCI. Multivariate analysis indicated that advanced stage, poor PS, and low BMI were negative prognostic factors for OS. Intriguingly, multivariate analysis demonstrated that CCI was a not significant factor for OS in this study. DISCUSSION: While AJCC stage is a well-known determinant, this study suggests that physicians should be alerted to poor PS and low BMI in older patients with STS.

A novel EWSR1-HOXB13 rearrangement in a fibroblastic tumor from the abdomen of a young woman.

We describe a novel EWSR1-HOXB13-fusion in a fibroblastic tumor from the abdominal wall of a 29-year-old woman. This tumor caused intermittent intense pain and had grown to approximately 5 cm in size over two years. The tumor was located beneath subfascial section of the abdominal wall and was invading the abdominal cavity and pressing on the liver. The tumor was well-circumscribed and consisted of intersected fascicles of monomorphic spindle-shaped cells with uniform ovoid nuclei lacking nuclear pleomorphism or mitotic activity. This tumor was immunohistochemically negative for pan-cytokeratin AE1/AE3, desmin, SMA, S100, myogenin, MyoD1, CD34, melanosome, SOX10, STAT6, SS18-SSX, and ERG. H3K27me3 was retained. RNA sequencing revealed a unique EWSR1-HOXB13-fusion, and strong, diffuse nuclear immunostaining for HOXB13 was observed. No local recurrence or evident distant metastasis were observed over eight months without chemotherapy, implying that the behavior of this tumor is not yet known.

Reliability and validity of ultrasound to measure of muscle mass following allogeneic hematopoietic stem cell transplantation.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) recipients frequently show physical dysfunction due to loss of muscle mass. This study aimed to clarify the reliability and validity of ultrasound in evaluating muscle mass and to analyze the patterns of change in muscle mass before and after allo-HSCT. We conducted a prospective observational study using data from 68 patients who had undergone their first allo-HSCT. We evaluated the thickness of the quadriceps, biceps, and suprahyoid muscle. Three individual evaluators underwent this examination for each muscle before transplantation and on days 30, 90, and 180 after allo-HSCT. Inter-rater reliability was calculated using the interclass correlation (ICC), and the level of correlation between muscle mass measured by ultrasound and psoas muscle mass assessed using computed tomography (CT) was assessed using Pearson correlation. ICC values ranged from 0.897 to 0.977 in the measurement. The correlation scores were 0.730, 0.546 and 0.579 between psoas muscle and the biceps, quadriceps, and suprahyoid muscle. The thickness of the biceps and quadriceps muscle were both significantly decreased after allo-HSCT from baseline. These results showed that the ultrasound technique was a reliable tool for evaluating muscle mass and detecting changes in muscle mass following allo-HSCT.

Clinical Outcomes and Prognostic Factors for Patients with Malignant Peripheral Nerve Sheath Tumour.

INTRODUCTION: Few studies have described the characteristics and prognostic factors of patients with malignant peripheral nerve sheath tumour (MPNST). In this study, we retrospectively investigated the clinicopathological features, clinical outcomes, and prognostic factors of these patients. Patients and Methods. We recruited patients with MPNST who were treated at our institutions from 1991 to 2020. We collected and statistically analysed information on patient-, tumour-, and treatment-related factors. The median follow-up period was 61 months (range, 1-335.8 months). RESULTS: A total of 60 patients (31 males, 29 females) with a median age of 55 years (range, 8-84 years) at initial diagnosis were included. The median tumour size was 7 cm (range, 1.6-30 cm) in the greatest dimension. The 5-year overall survival (OS) rate of all patients was 69.5%. Univariate analysis revealed that large-sized tumour, metastasis at diagnosis, and no surgery of the primary tumour were significantly associated with patients with worse OS. Multivariate analysis identified surgery of the primary tumour as an independent prognostic factor for improved OS. Among patients with localised disease at diagnosis who underwent surgery of the primary tumour at our institutions, the 5-year OS, local recurrence-free survival (LRFS), and metastasis-free survival (MFS) rates were 81.1%, 78.2%, and 70.3%, respectively. Univariate analysis revealed that positive surgical margin was significantly correlated with unfavourable OS and LRFS, and high grade was a poor prognostic indicator for MFS. CONCLUSION: Complete surgical resection with negative surgical margins is necessary for a successful MPNST treatment. Multidisciplinary management of MPNST with aggressive features is important for optimising patient outcomes.

18F-Fluorodeoxyglucose Positron Emission Tomography Is Useful in the Evaluation of Prognosis in Retroperitoneal Sarcoma.

Background: Retroperitoneal sarcomas are rare neoplasms that occur in the retroperitoneum. Complete surgical resection is the only effective treatment option. The prediction of prognosis by histological diagnosis has not yet been established. The purpose of this study was to identify the usefulness of [18-F] fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging for validating the prognosis of retroperitoneal sarcoma (RPS) established by histological diagnosis. Methods: We retrospectively reviewed 201 patients with RPS treated at the Osaka International Cancer Institute between 2010 and 2021. We extracted the clinical data, including standardized uptake values (SUVs), evaluated with FDG-PET, and statistically analyzed the data. Results: The median age of patients was 64 years (range, 31-85 years). A total of 101 (50.2%) patients were men, and 100 (49.8%) were women. Surgical resection was performed in 155 (77.1%) patients. On histological analysis, 75 (37.3%), 52 (25.9%), and 29 (14.4%) patients were diagnosed with dedifferentiated liposarcoma, well-differentiated liposarcoma, and leiomyosarcoma, respectively. The median survival time for patients with high maximum SUV (SUVmax) (≥4) or low SUVmax (<4) was 275.8 months and 79.5 months, respectively. Furthermore, among the patients with dedifferentiated liposarcoma, the overall survival rate for patients with high SUVmax (≥4) was significantly lower than that of those with low SUVmax (<4). Conclusions: The present study demonstrated that SUVmax calculated with FDG-PET was useful as a prognostic factor in RPS, especially in dedifferentiated liposarcoma and Grade2 RPS. To devise a treatment strategy for RPS, SUVmax during FDG-PET scan may be considered for clinical assessment.