Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Preoperative Interventional Therapy for Childhood Undifferentiated Embryonal Liver Sarcoma: Two Retrospective Cases from a Single Center.

ABSTRACT: Background Undifferentiated embryonal liver sarcoma (UELS) accounts for only 9 to 15% of all malignant liver tumors in children. Typically, UELS occurs in older children and presents as an abdominal mass. Most UELS are unresectable because of the later diagnosis. The outcome of UELS is very poor, with a 5-year overall survival of < 37.5%. Transarterial chemoembolization (TACE) has been reported to be an effective modality for unresectable liver tumors. To investigate the effects of TACE on UELS in children, we present two cases of children with UELS who underwent TACE and surgical resection in our center within the past 10 years. Methods In this study, two children with UELS were treated using TACE with cisplatin, doxorubicin, and iodized oil. The size of the tumors was measured before and after TACE using ultrasonography. Routine was also given before and after surgical resection. Side effects were recorded. Both patients had follow-up. Results After interventional therapy, both patients presented with vomiting, fever, and transient liver dysfunction without cardiac or renal dysfunction. One patient had bone marrow depression. The size of the tumors was reduced by 23% to 31% after TACE. The tumors were completely removed by surgical procedures after 4 weeks of TACE in both patients. One patient survived free of disease for 1 year, and the other survived free of disease for 9 years. Conclusion TACE yielded satisfactory results for unresectable UELS in children, with lower dosage of chemotherapy and fewer side effects. It may be applied as a preoperative therapy for children with unresectable UELS.

SUBMITTER: Zhao X

PROVIDER: S-EPMC4712051 | biostudies-other | 2015 Dec

REPOSITORIES: biostudies-other

ACCESS DATA

Json Xml

Similar Datasets

Undifferentiated embryonal sarcoma of the liver mimicking venolymphatic malformation.

Project description:Undifferentiated embryonal sarcoma of the liver (UESL) is a primitive hepatic neoplasm that presents in a variety of forms on ultrasonography, computed tomography, and magnetic resonance imaging. In this case report, we present an UESL with fluid-fluid cysts mimicking a radiographic presentation commonly seen in venolymphatic malformation on magnetic resonance imaging. This is the first described case of UESL, with this radiographic presentation and outlines, the importance of considering this malignant lesion when evaluating liver tumors in children.

| S-EPMC6462802 | biostudies-literature

Undifferentiated embryonal sarcoma of the liver in adults: Retrospective analysis of a case series and systematic review

Project description:Adult undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive malignant tumor. As UESL is rare, the literature predominantly includes case reports, with a limited number of small case series. The aim of the present study was to investigate the presentation, treatment modalities and outcomes of this rare tumor. The present study includes a case series of adult UESL and a systematic review. A single-institution case series of adult UESL were retrospectively analyzed, and a systematic review of adult UESL was performed by searching MEDLINE, Web of Science, EMBASE, the Google Scholar database and the Cochrane Library. For all identified adult UESL cases, the demographic variables, treatments and survival were analyzed. Three female adult patients with UESL (median age, 21 years) were successfully treated by complete tumor resection, with or without adjuvant chemotherapy, at Beijing Tsinghua Changgung Hospital between 2015 and 2018. Of these patients, two are currently alive (follow-up, 9 and 41 months), and one died after pulmonary recurrence 17 months post-diagnosis. The present systematic review identified 108 cases of adult UESL. Among all 111 analyzed cases, the median overall and disease-free survival rates were as follows: 1-year, 72 and 67%; 3-year, 56 and 40%; and 5-year, 47 and 35%, respectively. Treatment strategies combining complete tumor resection and chemotherapy promoted improved overall and disease free survival time compared with radical tumor resection alone. The present analysis included one of the largest case series of UESL in adults, and is the first such study to present survival rates. The results of the present study confirmed that survival was improved by treatment strategies combining complete tumor resection and chemotherapy.

| S-EPMC7439129 | biostudies-literature

Evidence for an unanticipated relationship between undifferentiated pleomorphic sarcoma and embryonal rhabdomyosarcoma.

Project description:Embryonal rhabdomyosarcoma (eRMS) shows the most myodifferentiation among sarcomas, yet the precise cell of origin remains undefined. Using Ptch1, p53 and/or Rb1 conditional mouse models and controlling prenatal or postnatal myogenic cell of origin, we demonstrate that eRMS and undifferentiated pleomorphic sarcoma (UPS) lie in a continuum, with satellite cells predisposed to giving rise to UPS. Conversely, p53 loss in maturing myoblasts gives rise to eRMS, which have the highest myodifferentiation potential. Regardless of origin, Rb1 loss modifies tumor phenotype to mimic UPS. In human sarcomas that lack pathognomic chromosomal translocations, p53 loss of function is prevalent, whereas Shh or Rb1 alterations likely act primarily as modifiers. Thus, sarcoma phenotype is strongly influenced by cell of origin and mutational profile.

| S-EPMC3040414 | biostudies-literature

Radiomics Models for the Preoperative Prediction of Pelvic and Sacral Tumor Types: A Single-Center Retrospective Study of 795 Cases.

Project description:PurposeTo assess the performance of random forest (RF)-based radiomics approaches based on 3D computed tomography (CT) and clinical features to predict the types of pelvic and sacral tumors.Materials and methodsA total of 795 patients with pathologically confirmed pelvic and sacral tumors were analyzed, including metastatic tumors (n = 181), chordomas (n = 85), giant cell tumors (n =120), chondrosarcoma (n = 127), osteosarcoma (n = 106), neurogenic tumors (n = 95), and Ewing's sarcoma (n = 81). After semi-automatic segmentation, 1316 hand-crafted radiomics features of each patient were extracted. Four radiomics models (RMs) and four clinical-RMs were built to identify these seven types of tumors. The area under the receiver operating characteristic curve (AUC) and accuracy (ACC) were used to evaluate different models.ResultsIn total, 795 patients (432 males, 363 females; mean age of 42.1 ± 17.8 years) were consisted of 215 benign tumors and 580 malignant tumors. The sex, age, history of malignancy and tumor location had significant differences between benign and malignant tumors (P < 0.05). For the two-class models, clinical-RM2 (AUC = 0.928, ACC = 0.877) performed better than clinical-RM1 (AUC = 0.899, ACC = 0.854). For the three-class models, the proposed clinical-RM3 achieved AUCs between 0.923 (for chordoma) and 0.964 (for sarcoma), while the AUCs of the clinical-RM4 ranged from 0.799 (for osteosarcoma) to 0.869 (for chondrosarcoma) in the validation set.ConclusionsThe RF-based clinical-radiomics models provided high discriminatory performance in predicting pelvic and sacral tumor types, which could be used for clinical decision-making.

| S-EPMC8459744 | biostudies-literature

Molecular cytogenetic characterization of undifferentiated embryonal sarcoma of the liver: a case report and literature review.

Project description:Undifferentiated embryonal sarcoma of the liver (UESL) represents a heterogeneous group of tumors derived from mesenchymal tissues. Earlier cytogenetic studies in limited cases demonstrated that UESL is associated with a recurrent translocation t(11;19)(q11;q13.3-q13.4) or add(19)(q13.4). In this report, we present our array comparative genomic hybridization (aCGH), fluorescence in situ hybridization (FISH) findings, and a missense mutation of TP53 gene by DNA sequencing in a 19-year-old patient with UESL. The data were compared to laboratory findings reported by previous studies.

| S-EPMC3478990 | biostudies-literature

Secondary hematological malignancies in patients with sarcoma: A single‑center retrospective study.

Project description:The present retrospective study investigated the clinical features and prognosis of secondary hematological malignancies (SHMs) in patients with sarcoma at Korea Cancer Center Hospital (Seoul, South Korea). Patients who had been diagnosed with SHMs after having received treatment for sarcoma between January 2000 and May 2023 were enrolled. Clinical data were collected from the patients' medical records. Clinical characteristics were analyzed, including SHM incidence, type and prognosis. Of 2,953 patients with sarcoma, 18 (0.6%) were diagnosed with SHMs. Their median age at the time of sarcoma diagnosis was 39.5 (range, 9-72) years, and 74% (n=14) of these patients were male. The histological features of sarcoma varied, with osteosarcoma diagnosed in nine patients (50%). All patients with sarcoma underwent surgical treatment, and 16 (88.8%) received chemotherapy. The most common type of SHMs was acute myeloid leukemia (n=6; 33.3%), followed by myelodysplastic syndrome (n=5; 27.7%). The median latency period between the sarcoma diagnosis and SHM identification was 30 (range, 11-121) months. A total of 13 (72.2%) patients received treatment for the SHM. The median overall survival after SHM diagnosis was 15.7 (range, 0.4-154.9) months. The incidence of SHMs in sarcoma in the present study was consistent with that reported previously. The presence of SHMs was associated with a poor patient prognosis, especially if treatment for SHMs was not administered.

| S-EPMC10988193 | biostudies-literature

Hedgehog pathway activity in pediatric embryonal rhabdomyosarcoma and undifferentiated sarcoma: a report from the Children's Oncology Group.

Project description:BackgroundAberrant activation of the hedgehog (Hh) signaling pathway is implicated widely in both pediatric and adult malignancies. Inactivation of the Hh regulator PTCH is responsible for the Gorlin cancer predisposition syndrome. The spectrum of tumors found in Gorlin Syndrome includes basal cell carcinoma, medulloblastoma, and rarely, rhabdomyosarcoma (RMS). A previous report utilizing in situ hybridization has provided initial evidence for the expression of Hh targets GLI1 and PTCH in RMS tumors.ProcedureTo investigate the role of Hh pathway signaling in pediatric RMS and undifferentiated sarcoma (US) tumors, the expression of Hh pathway targets GLI1 and PTCH was measured. RNA was extracted from archival human tumor specimens collected from pediatric patients enrolled on Intergroup Rhabdomyosarcoma Study III and IV, and subjected to quantitative reverse transcriptase-polymerase chain reaction.ResultsExpression of GLI1 with or without PTCH was detected in substantial subsets of embryonal RMS (ERMS) and US tumors but only rarely in alveolar RMS tumors. Neither PTCH mutations nor activating SMO mutations were detected in ERMS tumors with high GLI1 expression. Microarray analysis demonstrated relative overexpression of downstream Hh targets in ERMS tumors with high or intermediate GLI1 expression. Unlike a recent report, Hh pathway activity in ERMS tumors did not correlate with a unique clinical phenotype.ConclusionsOur findings support a role for Hh pathway activation in the genesis of a subset of ERMS and US tumors. Hh signaling may represent a novel therapeutic target in affected tumors.

| S-EPMC3164386 | biostudies-literature

Lymphopenia in Covid-19: A single center retrospective study of 589 cases.

Project description:BackgroundLymphopenia is one of features that helps identify patients with severe Covid-19. This retrospectively study analyzed the association of lymphopenia with the severity of COVID-19 infection, determinate the predictive factors of lymphopenia and the significance of mortality in patient with lymphopenia.MethodsThis retrospective study included patients diagnosed with Covid-19 and admitted to intensive care unit of our university hospital center From Mars 1st 2020, to December 31st, 2020.ResultsIn this study, 589 patients were included, a group had lymphopenia with 357 cases (60.06%) and the non-lymphopenia group with 232 cases (39.4%). The median age of our patients having lymphopenia was 65 years (56-76). Hypertension and diabetes were noted in the majority of patients with lymphopenia than in the non-lymphopenia group. Lymphopenia was strongly correlated to the inflammatory biomarkers of COVID-19 and were significant. A significant correlation was found between lymphopenia group and CT scan. Lymphopenia was observed as an indicator of prolonged duration of hospitalization but was not significant.ConclusionAnalytical data from this retrospective study shows the importance in the association between lymphopenia and the severity of COVID-19 infection, hence the need for dynamic monitoring of the number of lymphocytes on admission and during hospitalization of these patients.

| S-EPMC8423773 | biostudies-literature

Activity of PD1 inhibitor therapy in advanced sarcoma: a single-center retrospective analysis.

Project description:BACKGROUND:Sarcomas constitute a heterogeneous group of tumors with different clinical behaviors and variable responses to systemic therapies. Recent immunotherapy studies with PD1 inhibitors (PD1i) show promising results with use in certain soft-tissue sarcomas; however, the clinical and molecular features that best predict response to PD1i remain unclear. METHODS:Demographic, imaging, histologic, and genetic sequencing data was collected for sarcoma patients who received nivolumab or pembrolizumab (PD1i) treatment at our institution between January 1st 2015 and April 23rd 2018. The primary objective was to determine progression-free survival (PFS) in patients with advanced sarcomas receiving PD1i. Secondary objectives included determining overall survival (OS) and assessment of characteristics associated with response to PD1i. Fifty-six patients who were treated with PD1i therapy met inclusion criteria for this study. RESULTS:Partial response towards PD1i treatment was seen in 3 in 26 evaluable patients, but no complete responses were observed (overall response rate 11.5%). Within this group of patients, the 90?day PFS was found to be 48.8%. In patients in whom PD1 expression was known, there was a statistically significant positive correlation between expression of PD1 and longer PFS and OS rates. Patients that were treated with more than four cycles of PD1i therapy were also more likely to have a greater OS. CONCLUSIONS:This study suggests activity of PD1i in a pretreated cohort of advanced sarcoma patients, particularly for the subset of patients with PD1 positive tumors. Our results highlight the importance of further research to better target the optimal patient population and markers of response.

| S-EPMC7275332 | biostudies-literature

Intravenous Leiomyomatosis of the Uterus: A Retrospective Single-Center Study in 14 Cases.

Project description:PurposeThis study aimed to retrospectively review the diagnosis and surgical treatment of uterine intravenous leiomyomatosis (IVL).MethodsThe clinical data of 14 patients with uterine IVL admitted to our hospital between 2013 and 2018 were retrospectively analyzed, including their demographics, imaging results, surgical procedures, perioperative complications, and follow-up results.ResultsThe tumors were confined to the pelvic cavity in 7 patients, 1 into the inferior vena cava, 4 into the right atrium, and 2 into the pulmonary artery (including 1 into the superior vena cava). Only one case was misdiagnosed as right atrial myxoma before the operation, which was found during the surgery and was treated by staging surgery; all the other patients underwent one-stage surgical resection. Three patients underwent complete resection of the right atrial tumor through the abdominal incision, and one patient died of heart failure in the process of resection of heart tumor without abdominal surgery. During the 6-60 months of follow-up, 4 patients developed deep venous thrombosis of the lower extremity, and 1 patient developed ovarian vein thrombosis and pulmonary embolism. After anticoagulation treatment, the symptoms disappeared. One patient refused hysterectomy and the uterine fibroids recurred 4 years after the operation.ConclusionSpecific surgical plans for uterine IVL can be formulated according to cardiac ultrasound and computed tomography (CT). For the first type of tumor involving the right atrium, the right atrium tumor can be completely removed through the abdominal incision alone to avoid thoracotomy. The disease is at high risk of thrombosis and perioperative routine anticoagulation is required.

| S-EPMC7155762 | biostudies-literature

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data