bluuebunny/crossref_metadata_2025_split huggingface dataset:
Diverse genomic characterization of Chinese patients with osteosarcoma and soft tissue sarcoma.
Authors: Sun Mengxiong , Quan Fei , Yi Huan , Lu Zhongyu , Yu Yangyang , Chen Siqi , Ding Ran , Deng Wanglong , Bu Fanfeng 
Published: June 2023

e23528 Background: Osteosarcoma(OST) differs from soft tissue sarcoma(STS) in terms of pathology and chemotherapy, but there is partial overlap in targeted therapy. Few articles have described the differences in genetic profiles of OST and STS in Chinese patients based on extensive sample data, at this point, identifying the difference genomic characterization between them may provide new drug targets for clinical diagnosis and treatment. Methods: A 551-gene targeted panel of DNA-based Next Generation Sequencing (NGS) assay was performed on tumor tissue(n = 613) and plasma(n = 136) obtained from STS(n = 586) and OST(n = 163) patients, the majority were male(n = 430, 57.4%), median age was 49(range 1-87), 101 patients also measured additional 67 gene fusions based on RNA-seq in tumor samples. All variants (SNV, INDEL, CNV and fusion) were entered into the result analysis. PD-L1expression was tested by IHC(SP263). Results: The top 5 mutant genes were TP53(28%), CDK4(20%), MDM2(18%), CDKN2A(15%), CDKN2B(15%) in STS, and TP53(26%), MYC(19%), CDK4(18%), MUC16(13%), CDKN2B(13%) in OST respectively. MDM2(18% vs 10%, p = 0.030) and PTEN(11% vs 4%, p = 0.007) were significantly higher in STS, while GNAS(7% vs 3%, p = 0.045) and IDH1(5% vs 1%, p = 0.002) were more common in OST. The most frequently affected pathways were Cell cycle(42%), Genome integrity(34%), RTK signaling(27%) in STS and Cell cycle(36%), Genome integrity(33%), transcription factor(28%) in OST. Additional significantly co-altered genes were RB1/PTEN, LRP1B/FAT3, FAT2/SPTA1 in STS, and MUC16/LRP1B, LRP1B/POLE, MED12/STAG2 in OST. More rare fusions were identified in STS. Wefound 15 PTPRB fusions, 14 of which were from STS. MDM2 fusions only detected in STS, 8 of which were from liposarcoma. Almost all patients were defined as MSS(625/629), with only 4 MSI-L and no MSI-H. 2.9%(17/589) STS and 3.1%(5/163) OST scored as TMB-H(≥10.00 Muts/Mb). TP53(p＜0.001), MUC16(p＜0.001), LRP1B(p＜0.001), FAT3(p＜0.001)were more likely to appear in the TMB-H group of STS, while MUC16(p＜0.001) was more likely to appear in TMB-H group of OST. 32.3%(83/257) STS and 29.6%(8/27) OST were PD-L1-positive(PD-L1≥1%). In STS, PDCD1LG2(p＜0.001), CD274(p＜0.001) and JAK2 (p = 0.001)were more common in the PD-L1-positive group, while MDM4 (p = 0.018) and PARP1(p = 0.003)were more common in the PD-L1-negtive group. There were no statistically significant differences due to limited PD-L1 data in OST. Conclusions: The analysis revealed that there are some differences in genetic profiles between Chinese OST and STS, which may provid more precise basis for personalized targeted therapy and immunotherapy.
Comorbidities in patients with sarcoma: Insights from the National In-Patient Database from 2006 to 2014.
Authors: Cuartas Mesa Maria Cristina , Quelal Analuisa Karol , Litvin Rafaella , Deenadayalan Vaishali , Olafimihan Ayobami Gbenga , Ibrahim Muhammad Bilal , Bittar Gianfranco , Martinez Trevino Enrique Francisco , Nieto Alejandro 
Published: June 2023

e23534 Background: Sarcoma represents a rare solid malignancy in adults. Given the likelihood of metastatic disease, the prognosis is usually poor. Comorbidities are independent determinants of prognosis, and cardiovascular and respiratory diseases are the most common. We aimed to study the prevalence, trends, and outcomes of CVD and pulmonary circulation disorders in patients admitted with sarcoma over eight years. Methods: We present a retrospective analysis of hospital admissions for soft tissue malignancies between 2006 and 2014 from the NIS database using its respective ICD-9-CM code. We obtained the prevalence of cardiovascular diseases (HF, CAD, HTN) and pulmonary circulatory disorders (PE, PHTN). We assessed yearly trends with the Cochran-Armitage trend test. Using multivariate binomial logistic regression (adjusting for sex, race, age, and Elixhauser comorbidities) we calculated adjusted odds ratios (aOR) to identify the association of these comorbidities with in-hospital mortality. Results: Between 2006-2014 there were 352320 admissions with a primary diagnosis of soft tissue malignancies. Of them, 55.8% were males, 60% were white, and 57.5% were 50 years old and older. In 39% of the population, CVD was present: HTN 35.5%, CAD 8.5%, HF 4.5%. The rate of pulmonary circulatory disorders was 2.5%: PE 1.7%, and pulmonary hypertension 0.9%. In-hospital mortality accounted for 3.7% of admissions. The CVD yearly prevalence increased from 34.6% in 2006 to 41.7% in 2014 (p < 0.001). Pulmonary circulatory disorders rose from 1.7% in 2006 to 3.3% in 2014 (p < 0.001). Pulmonary circulatory diseases were associated with high in-hospital mortality aOR 3.02 (CI 95% 2.44-3.73, p = 0.00). Cardiovascular disease was not statistically significantly associated with an increased rate of in-hospital mortality aOR 1.04 (CI 95% 0.93-1.15, p = 0.53). Conclusions: In sarcoma patients, it is essential to quantify their comorbidities as these often influence their chosen therapy. Cardiovascular disease affected one-third of the patients. However, despite the low prevalence of pulmonary circulatory disorders, they were associated with high in-hospital mortality. Further studies are needed to understand the causality and underlying physiopathology.
Epidemiology, survival, and treatment of liposarcoma subtypes: An analysis of the national cancer database.
Authors: Kennard Jared , Silberstein Peter T. , Braaten Marco 
Published: June 2023

e23535 Background: Liposarcomas are a soft tissue sarcoma which grow in adipose tissue, mainly in the subcutaneous fat of the limbs, and are the most common type of soft tissue sarcoma. Relatively little is known about the epidemiology, prevalence, and survival of various histological subtypes of liposarcoma, therefore, in the largest study to date, we analyzed this patient population using the NCDB. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with Liposarcoma from 2004 to 2019 using the histology codes 8850, 8851, 8852, 8853, 8855, and 8858 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, Chi-Square, and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 27 and statistical significance was set at α = 0.05. Results: We identified 19,351 patients with liposarcoma. Mean age at diagnosis was 59 years for all subtypes and 83.3% of patients were white. Well-differentiated tumors were the most common histological subtype (34.8%), conferred the longest overall survival, and were the largest tumors on average. Round-cell tumors were the rarest (2.4%) and had smaller average tumor size than other subtypes. Patients with the de-differentiated subtype (20.5%) experienced the lowest median survival (70.5 months; p < 0.05) and an increased hazard ratio (HR = 2.8; p < 0.05) after controlling for other subtypes, age, and stage at diagnosis. Surgery was the mainstay of therapy with 90% of patients receiving surgery. 11.2% received additional adjuvant chemotherapy and 2.8% received adjuvant radiotherapy. Round cell and Myxoid tumors were most amenable to resection (89.8% and 88.9% of patients having no residual tumor) compared to only 64.4% of dedifferentiated tumors with complete surgical resection. Conclusions: Liposarcomas are a heterogeneous group of tumors which carry unique patterns of presentation, treatment, and prognosis. Well differentiated tumors are common and amenable to treatment; de-differentiated tumors are more aggressive and remain difficult to fully treat.
Factors associated with overall and cancer specific mortality in primary cardiac sarcoma.
Authors: Bangolo Ayrton , Fwelo Pierre , Nagesh Vignesh , Jarri Amer , Chacko Angel Ann , Dey Shraboni , Proverbs-Singh Tracy Ann , Atoot Adam 
Published: June 2023

e23539 Background: Primary cardiac sarcomas (PCS) are extremely rare malignant tumors involving the heart. Only isolated case reports have been described. There is a paucity of data on the epidemiological characteristics of PCS. Virtually all types of sarcomas have been reported in the heart. For most types, only isolated case reports have been described. We used a nationally representative database to evaluate the independent prognostic factors amongst patients with PCS. This study has the objective of investigating the epidemiologic characteristics, survival outcomes and independent prognostic factors of PCS. Methods: We enrolled a total of 362 patients with PCS, between 2000 and 2017, by retrieving the Surveillance, Epidemiology, and End Results (SEER) database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of PCS. Variables with a p value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio (HR) of greater than 1 representing adverse prognostic factors.All statistical tests were performed by using Software STATA 16.1. The SEER database is a publicly available dataset for which there was no need for IRB approval. Results: The male gender (50.83%), and non-Hispanic whites (58.84%) were the most represented groups in our cohort. The most encountered histologic subtype was angiosarcoma (42.51%), which represented nearly half the cases. Multivariate cox proportional hazard regression analyses of factors affecting all-cause mortality and PCS related mortality among US patients between 2000 and 2017 demonstrated Higher overall mortality in age 80+ (HR = 13.261, 95% CI 5.839-30.119, p = 0), followed by age 60-79 (HR = 1.916, 95% CI 1.213-3.025, p = 0.005); and advanced disease with distant metastasis (HR = 2.013, 95% CI 1.355-2.99, p = 0.001), followed by regional involvement (HR = 1.518, 95% CI 1.041-2.214, p = 0.03). Lower OM rate was observed in patients with rhabdomyosarcoma (HR = 0.364, 95% CI 0.154-0.86, p = 0.021) and widowed (HR = 0.506, 95% CI 0.263-0.977, p = 0.042). Age 80+ (HR = 11.177, 95% CI 4.449-28.08, p = 0) and advanced disease with distant metastases (HR = 2.117, 95% CI 1.37-3.271, p = 0.001) have the highest cancer specific mortality. Patients with rhabdomyosarcoma (HR = 0.344, 95% CI 0.128-0.929, p = 0.035) have a lower CSM. Conclusions: In this United States population-based retrospective cohort study using the SEER database, we found that age at diagnosis and advanced disease at diagnosis were independent factors predicting poor prognosis. Furthermore, patients with rhabdomyosarcoma had a lower mortality compared to other histologic subtypes. Surgical resection of the primary tumor did not significantly impact the overall mortality or the cancer-specific mortality.
Italian report of expanded access for nab-sirolimus in patients with malignant perivascular epithelioid cell tumor (PEComa).
Authors: Sanfilippo Roberta , Fabbroni Chiara , Fumagalli Elena , Franza Andrea , Provenzano Salvatore , Collini Paola , Greco Francesca Gabriella , Duca Matteo , Casali Paolo Giovanni 
Published: June 2023

e23541 Background: Malignant perivascular epithelioid cell tumor (PEComa) is a rare aggressive sarcoma, with no approved treatment in Europe, while the mechanistic target of rapamycin (mTOR) inhibitor nab-sirolimus has been approved in US. In Europe, an expanded access program is available for patients with advanced or metastatic PEComa. Methods: We retrospectively collected data of patients with advanced/metastatic PEComa treated at our institution from December 2020 through 3rd February 2023 with nab-sirolimus. Nab-sirolimus was administered as an intravenous infusion over 30 minutes at a dosage of 100 mg/m2 on days 1 and 8 of each 21-day cycle. Results: At the time of this analysis, 10 patients were treated with nab-sirolimus and 8 are evaluable for response. Of them, 4 patients received nab-sirolimus as their first mTOR inhibitor therapy, while 4 had already received another mTOR inhibitor, being discontinued for PD. Amongst patients not pretreated with mTOR inhibitors, 2/4 had a PR as their best response, 1/4 obtained SD and 1/4 obtained PD. Amongst patients pretreated with another mTOR inhibitor, all but 1 obtained PD as their best response and 1/4 had SD. The majority of nab-sirolimus-related adverse events were grade 1 or 2 and were manageable for long-term treatment. No grade 4 treatment-related adverse events were observed. Amongst the 10 patients treated with nab-sirolimus, tumor profiling is available for 6 patients, being ongoing for the other 4. Conclusions: nab-Sirolimus is active in patients with malignant PEComa. Activity is mainly confined to those not already treated with other mTOR inhibitors.
Clinical outcomes of soft tissue sarcoma in the Philippines: A single-center experience.
Authors: Maaño Omar Paningbatan , Gorospe Agnes Evangelista , Pascual-Panganiban Anna Marie 
Published: June 2023

e23549 Background: Soft-tissue sarcomas (STS) are a heterogeneous group of uncommon tumors that account for less than 1 percent of all adult malignant tumors. Data are scarce in the Asia-Pacific region, especially in the Philippines. This study aims to evaluate the epidemiology and clinical outcomes of STS from a private tertiary hospital in the Philippines. Methods: All adult patients treated at St. Luke’s Medical Center (SLMC), Quezon City and Global City, between January 2010 and December 2020 were reviewed. Continuous variables are described in median and categorical variables are described in count and percentage. Kaplan-Meier curves for overall survival (OS) and recurrence-free survival (RFS) were compared by the Log-Rank test. Results: One hundred eighty-seven patients diagnosed with STS were eligible for the study. The median age at diagnosis was 53 years old; the majority were male (54.55%). The most common histologic subtypes were liposarcoma (19.79%), pleomorphic sarcoma, undifferentiated (16.58%), and leiomyosarcoma (11.76%). Most were diagnosed with localized disease (90.9%), with the lower extremities (44.39%) as the most common primary site. The median tumor size was 10.3 cm, primarily high grade (46.52%). Nodal involvement on diagnosis was 4.28%. Twelve patients (6.42%) presented with de novo metastasis; lung (45%) was the most common site. The majority had surgery (81.82%) on the primary tumor, with 56.86% achieving R0. Eighty patients (42.78%) had radiotherapy; the majority were given post-operatively (N = 60). Sixty-three patients (33.68%) received systemic therapy. Out of 170 patients with localized disease, 70 (40%) had disease recurrence, mostly local recurrence (57.14%). The 3-year OS and RFS were 58.67% and 55.20%, respectively, for the entire cohort; for localized disease was 95.06%. There was a statistical difference in OS between distant vs local recurrence (23.28% vs 49.11%)(p < 0.001), nodal involvement (p = 0.041), surgery (p < 0.001), and radiotherapy (p = 0.001). Conclusions: Most STS seen and diagnosed in SLMC is mostly localized disease but with lower rates of resection than in other countries in the Asia-Pacific region. The clinicopathologic features, treatment modalities received, histological subtype, and primary anatomical site are similar to other data. The low survival rate may be attributed to the site of recurrence, nodal involvement, and treatment pattern received.
Clear cell sarcoma: The OSU experience.
Authors: Tinoco Gabriel , Husain Marium , Kim Hangil , Xu Menglin , Liebner David A. 
Published: June 2023

e23554 Background: Clear cell sarcoma (CCS) is an ultra-rare translocation-driven sarcoma. Often affects young adults with no sex preponderance, and whites have a higher occurrence. Frequently present as a slow-growing mass in the extremities or trunk. The EWSR1–ATF1/CREB1 mutation is pathognomonic. The optimal treatment has not been outlined, but it is extrapolated from soft tissue sarcomas. The role of systemic therapies still needs to be fully elucidated, largely due to limited data. This is one of the largest single-institution cohorts reported. Methods: Patients evaluated at The Ohio State University James Comprehensive Cancer Center between January 1, 2010, and April 1, 2022, were included. Data collected included baseline demographic variables, pathologic and molecular features, treatment regimen, and outcomes. Due to a small cohort size, descriptive statistics were performed instead of formal power calculations. Results: A total of five patients were identified. 3 of 5 were white (60%), and 2 of 5 patients were black (40%). The mean age at diagnosis was 46 years, median at 43 years, ranging from 29 to 66 years. 3 of the 5 patients were female (60%), and 2 were male (40%). The mean size of the tumor at presentation is 5.62 cm. 2 of the 5 patients had stage 1 disease at presentation (40%), one had stage 3 (20%), and 2 had Stage 4 (40%). 3 out of 5 patients received treatment with curative intent. 4 patients had primary in the abdomen/lower back or lower extremities (80%). 4 patients received chemotherapy, and these patients received 3 to > 5 cycles. 3 of the 4 on chemotherapy progressed (50%). A patient who received Sunitinib had the shortest time to progression, at 30 days (or 4.3 weeks). The patient who experienced the longest time to progression received Pazopanib, with progression occurring after 339 days (or 48 weeks). Within the early-stage patients, one of the three underwent surgery progressed (33.3%). None of the two patients that received radiation progressed (0%). 3 of 5 participants (60%) eventually expired during the study dates (2 with metastatic disease at diagnosis and 1 with localized disease). All patients received next-generation sequencing. One of these patients demonstrated positive expression levels for PD-L1. The median tumor mutation burden (TMB) value was 11.86 ranging from 2.06 to 28.16. Conclusions: We found that CCS has a high rate of disease progression despite the multimodality approach, and there is a high mortality associated with the disease. Due to the sample size of this single institution study, we cannot identify significant associations between clinical and demographic factors and treatment outcomes in clear cell sarcoma. However, these results stress the importance of multi-institutional (national and international) collaboration around ultra-rare subtypes of sarcoma to provide more significant patient data, improve outcomes, and design clinical trials for these patients.
Differential overall survival and treatment for non-dedifferentiated liposarcoma in academic and non-academic facilities: An NCDB analysis.
Authors: Czaplicki Alec , Bine Christopher , Nielson Eric , Braaten Marco , Silberstein Peter T. 
Published: June 2023

e23556 Background: Liposarcoma is a malignant soft tissue tumor sarcoma which most commonly arises in subcutaneous fat of the limbs and abdomen. Previous studies found overall survival and treatments to vary between patients treated at academic and non-academic facilities. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with non-dedifferentiated liposarcoma from 2004 to 2019 using the histology codes 8850, 8851, 8852, 8853, 8855, and 8857 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA, Chi-Square, and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 27 and statistical significance was set at α = 0.05. Results: Of the 15,256 patients queried, 72% were treated at an academic/research facility (AF). Patients treated at an AF had an improved median survival compared to a non-academic facility (NA) (184 months versus 154 months; p < 0.001). After controlling for stage, income, and therapy type, treatment at an AF was associated with an independent decrease in hazard (HR = .824; p < 0.001). AF patients disproportionately came from a higher income bracket and were more likely to carry private insurance. Additionally, AF patients were more likely to receive surgery, have margins with complete tumor resection, and receive adjuvant chemotherapy (p < 0.001). There were no significant differences in stage or metastasis at presentation between the two groups. Conclusions: Patients with non-dedifferentiated liposarcoma treated at an academic/research facility had improved overall survival, higher rates of surgery, higher rates of chemotherapy, and better surgical margins compared to patients treated at a non-academic facility.
Affecting burden and cost of care for the caregiver of dependent patients with advanced stage of cancer who had an arrangement for expected death at home.
Authors: Palanupap Kanokrat 
Published: June 2023

e24000 Background: The terminal stage cancer patients suffer from disease and related therapeutic processes, lead to increasing need for caring from their caregivers. This affects the quality of life(QOL) of caregivers. Currently, there is no data on burden of caregivers who caring for cancer patients who are choose to die at their home. Objective: This study examined burden of caregivers, impact on QOL and satisfaction of caregivers for patients with terminal cancer, comparing in the group of patients who die at home versus at hospital, comparing in regular care group(RC) versus palliative care group(PC). Methods: The semi-qualitative-quantitative study enrolled caregivers of advance cancer patients who had PPS < 60 at Ramathibodi Hospital from Dec 2021-Nov 2022. We assessed participants on 3-time-points (enrolment, 1, 2 months) for QOL and burden via telephone interviews, questionnaires by EQ5D5L and Zarit Burden. If the patients passed away, we interviewed caregivers in qualitative study. Analysis responses were recorded, transcribed and organized thematically analyzed. Analysis stratified on care with palliative care or not, and place of death. Results: We enrolled 108 participants, child (56%) and spouses (29%). They spent carring time average of 16.56 hr/day. 52 patients have passed away: home death(34) and hospital death(18). No significant difference in burden score and QOL between RC and PC. Burden score significantly increasing when time of death approach in both groups. However, significantly higher satisfaction score in caregivers of patients who died at home(P < 0.001). In patient who died in hospital, early integrated palliative showed significantly higher satisfaction score(P = 0.046). Qualitative analysis revealed 3 primary themes, 1) caregiver burden is increasing and chronic stress condition until the death of their loves and still continue post their death 2) need more help in coping and nursing strategies, more early palliative care can relieve their distresses 3) opinion of the caregivers in group of hospital death, If they could turn back time, they would have promised to look after their lovers better in changing the pass away at their home. Conclusions: Caregivers for terminal cancer patients have a high burden. However, caregiver of patients who died at their home rather have more satisfaction. Early integrated palliative care improves satisfaction in caregiver of patients who died at hospital. [Table: see text]