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Discovery Medicine Sep 2014Uterine sarcomas are a heterologous group of rare malignancies accounting for 8-10% of all uterine malignancies, but are significantly more aggressive and have worse... (Review)
Review
Uterine sarcomas are a heterologous group of rare malignancies accounting for 8-10% of all uterine malignancies, but are significantly more aggressive and have worse prognoses. Management of uterine sarcomas including leiomyosarcoma and endometrial stromal sarcoma are reviewed here, with additional discussions regarding high-grade undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are currently staged and treated similar to high-grade epithelial endometrial carcinomas, thus will not be discussed in this review. Gemcitabine/docetaxel with adriamycin holds promise for the treatment of leiomyosarcoma, but currently, limited advancements have been made in discovering targeted therapies to these tumors. Continued translational research in both medical oncology and gynecologic oncology is necessary to forward the development of novel and targeted therapeutic agents in the treatment of sarcoma. Enrollment of these patients in clinical trials is encouraged, and will allow for the development of safer and more effective therapies.
Topics: Adenosarcoma; Chemotherapy, Adjuvant; Clinical Trials as Topic; Combined Modality Therapy; Endometrial Neoplasms; Female; Humans; Leiomyosarcoma; Receptors, Estrogen; Receptors, Progesterone; Sarcoma; Sarcoma, Endometrial Stromal; Translational Research, Biomedical; Uterine Neoplasms
PubMed: 25227754
DOI: No ID Found -
Current Oncology (Toronto, Ont.) Sep 2022Retroperitoneal tumors are extremely rare. More than 70% of primary retroperitoneal soft tissue tumors are malignant. The most common sarcomas in the retroperitoneum... (Review)
Review
Retroperitoneal tumors are extremely rare. More than 70% of primary retroperitoneal soft tissue tumors are malignant. The most common sarcomas in the retroperitoneum include liposarcomas and leiomyosarcoma, however other sarcomas, along with benign mesenchymal tumors, can occur. Sarcomas are a heterogenous group of tumors with overlapping microscopic features, posing a diagnostic challenge for the pathologist. Correct tumor classification has become important for prognostication and the evolving targeted therapies for sarcoma subtypes. In this review, the pathology of retroperitoneal soft tissue sarcomas is discussed, which is important to the surgical oncologist. In addition, less common sarcomas and benign mesenchymal tumors of the retroperitoneum, which may mimic sarcoma clinically and pathologically, are also discussed.
Topics: Humans; Leiomyosarcoma; Liposarcoma; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 36135073
DOI: 10.3390/curroncol29090504 -
Critical Reviews in Oncology/hematology Feb 2018Uterine sarcomas (US) are rare mesenchymal tumours of the uterus and are divided mainly into uterine leiomyosarcoma (uLMS), low grade endometrial stromal sarcoma... (Review)
Review
Uterine sarcomas (US) are rare mesenchymal tumours of the uterus and are divided mainly into uterine leiomyosarcoma (uLMS), low grade endometrial stromal sarcoma (LG-ESS), high grade endometrial stromal sarcoma (HG-ESS), adenosarcomas and high grade undifferentiated sarcoma (HGUS). US are often high-grade tumours with a high local recurrence rate and metastatic risk. We here discuss the current standard of care and knowledge of systemic therapy for adult uterine sarcomas, in particular uLMS, LG-ESS, HG-ESS and HGUS, in both the adjuvant as well as the metastatic setting.
Topics: Adult; Chemotherapy, Adjuvant; Endometrial Neoplasms; Female; Humans; Leiomyosarcoma; Radiotherapy, Adjuvant; Sarcoma; Sarcoma, Endometrial Stromal; Uterine Neoplasms
PubMed: 29458779
DOI: 10.1016/j.critrevonc.2017.12.009 -
Aging Apr 2021Budding Uninhibited By Benzimidazoles are a group of genes encoding proteins that play central roles in spindle checkpoint during mitosis. Improper mitosis may lead to...
Budding Uninhibited By Benzimidazoles are a group of genes encoding proteins that play central roles in spindle checkpoint during mitosis. Improper mitosis may lead to aneuploidy which is found in many types of tumors. As a key mediator in mitosis, the dysregulated expression of BUBs has been proven to be highly associated with various malignancies, such as leukemia, gastric cancer, breast cancer, and liver cancer. However, bioinformatic analysis has not been applied to explore the role of the BUBs in sarcomas. Herein, we investigate the transcriptional and survival data of BUBs in patients with sarcomas using Oncomine, Gene Expression Profiling Interactive Analysis, Cancer Cell Line Encyclopedia, Kaplan-Meier Plotter, LinkedOmics, and the Database for Annotation, Visualization and Integrated Discovery. We found that the expression levels of BUB1, BUB1B and BUB3 were higher in sarcoma samples and cell lines than in normal controls. Survival analysis revealed that the higher expression levels of BUB1, BUB1B and BUB3 were associated with lower overall and disease-free survival in patients with sarcomas. This study implies that BUB1, BUB1B and BUB3 are potential treatment targets for patients with sarcomas and are new biomarkers for the prognosis of sarcomas.
Topics: Cell Cycle Proteins; Gene Expression Regulation, Neoplastic; Humans; Mitosis; Poly-ADP-Ribose Binding Proteins; Prognosis; Protein Serine-Threonine Kinases; Sarcoma; Stomach Neoplasms
PubMed: 33872216
DOI: 10.18632/aging.202944 -
Clinical Radiology Apr 2021Over the last decades, technological developments in the field of radiology have resulted in a widespread use of imaging for personalising medicine in oncology,... (Review)
Review
Over the last decades, technological developments in the field of radiology have resulted in a widespread use of imaging for personalising medicine in oncology, including patients with a sarcoma. New scanner hardware, imaging protocols, image reconstruction algorithms, radiotracers, and contrast media, enabled the assessment of the physical and biological properties of tumours associated with response to treatment. In this context, medical imaging has the potential to select sarcoma patients who do not benefit from (neo-)adjuvant treatment and facilitate treatment adaptation. Due to the biological heterogeneity in sarcomas, the challenge at hand is to acquire a practicable set of imaging features for specific sarcoma subtypes, allowing response assessment. This review provides a comprehensive overview of available clinical data on imaging-based response monitoring in sarcoma patients and future research directions. Eventually, it is expected that imaging-based response monitoring will help to achieve successful modification of (neo)adjuvant treatments and improve clinical care for these patients.
Topics: Biomarkers; Cell Proliferation; Fibroblasts; Glucose; Humans; Hypoxia; Multimodal Imaging; Neoadjuvant Therapy; Neoplasm Metastasis; Patient Care Planning; Precision Medicine; Sarcoma
PubMed: 33483087
DOI: 10.1016/j.crad.2020.12.009 -
Scandinavian Journal of Surgery : SJS :... Sep 2023Primary sarcomas of bone are rare malignant mesenchymal tumors. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The prognosis has... (Review)
Review
Primary sarcomas of bone are rare malignant mesenchymal tumors. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The prognosis has improved over the years, but bone sarcomas are still life-threatening tumors that need a multidisciplinary approach for diagnosis and treatment. Bone sarcomas arising in the pelvis present a unique challenge to orthopedic oncologists due to the absence of natural anatomical barriers, the close proximity of vital neurovascular structures, and the high mechanical demands placed on any pelvic reconstruction following the excision of the tumor. While radiotherapy has an important role especially in Ewing's sarcoma and chemotherapy for both Ewing's sarcoma and osteosarcoma, surgery remains the main choice of treatment for all three entities. While external hemipelvectomy has remained one option, the main aim of surgery is limb salvage. After complete tumor resection, the bone defect needs to be reconstructed. Possibilities to reconstruct the defect include prosthetic or biological reconstruction. The method of reconstruction is dependent on the location of tumor and the surgery required for its removal. The aim of this article is to give an insight into pelvic bone sarcomas, their oncological and surgical outcomes, and the options for treatment based on the authors' experiences.
Topics: Humans; Sarcoma, Ewing; Bone Neoplasms; Prognosis; Sarcoma; Osteosarcoma; Pelvic Bones; Pelvis; Soft Tissue Neoplasms
PubMed: 37438963
DOI: 10.1177/14574969231181504 -
Calcified Tissue International Feb 2018Bone sarcomas are tumours belonging to the family of mesenchymal tumours and constitute a highly heterogeneous tumour group. The three main bone sarcomas are... (Review)
Review
Bone sarcomas are tumours belonging to the family of mesenchymal tumours and constitute a highly heterogeneous tumour group. The three main bone sarcomas are osteosarcoma, Ewing sarcoma and chondrosarcoma each subdivided in diverse histological entities. They are clinically characterised by a relatively high morbidity and mortality, especially in children and adolescents. Although these tumours are histologically, molecularly and genetically heterogeneous, they share a common involvement of the local microenvironment in their pathogenesis. This review gives a brief overview of their specificities and summarises the main therapeutic advances in the field of bone sarcoma.
Topics: Bone Neoplasms; Female; Giant Cell Tumor of Bone; Humans; Male; Sarcoma; Tumor Microenvironment
PubMed: 29238848
DOI: 10.1007/s00223-017-0372-2 -
Journal For Immunotherapy of Cancer Feb 2021Sarcomas are a rare malignancy of mesenchymal tissues, comprizing a plethora of unique subtypes, with more than 60 types. The sheer heterogeneity of disease phenotype... (Review)
Review
Sarcomas are a rare malignancy of mesenchymal tissues, comprizing a plethora of unique subtypes, with more than 60 types. The sheer heterogeneity of disease phenotype makes this a particularly difficult cancer to treat. Radiotherapy, chemotherapy and surgery have been employed for over three decades and, although effective in early disease (stages I-II), in later stages, where metastatic tumors are present, these treatments are less effective. Given the spectacular results obtained by cancer immunotherapy in a variety of solid cancers and leukemias, there is now a great interest in appliying this new realm of therapy for sarcomas. The widespread use of immunotherapy for sarcoma relies on immuno-profiling of subtypes, immunomonitoring for prognosis, preclinical studies and insight into the safety profile of these novel therapies. Herein, we discuss preclinical and clinical data highlighting how immunotherapy is being used in soft tissue sarcoma and bone sarcomas.
Topics: Clinical Trials as Topic; Humans; Immunotherapy; Neoplasm Staging; Osteosarcoma; Prognosis; Sarcoma; Treatment Outcome
PubMed: 33526607
DOI: 10.1136/jitc-2020-001580 -
Annals of the Royal College of Surgeons... May 2011The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumours and malignant neoplasms that can be either primary or metastatic... (Review)
Review
INTRODUCTION
The retroperitoneum can host a wide spectrum of pathologies, including a variety of rare benign tumours and malignant neoplasms that can be either primary or metastatic lesions. Retroperitoneal tumours can cause a diagnostic dilemma and present several therapeutic challenges because of their rarity, relative late presentation and anatomical location, often in close relationship with several vital structures in the retroperitoneal space.
MATERIALS AND METHODS
A comprehensive literature search was conducted using PubMed. Relevant international articles published in the last ten years were assessed. The keywords for search purposes included: retroperitoneum, benign, sarcoma, neoplasm, diagnosis and surgery, radiotherapy, chemotherapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article.
RESULTS
Tumours usually present late and cause symptoms or become palpable once they have reached a significant size. Retroperitoneal tumours are best evaluated with good quality cross-sectional imaging and preoperative histology by core needle biopsy is required when imaging is non-diagnostic. Sarcomas comprise a third of retroperitoneal tumours. Other retroperitoneal neoplasms include lymphomas and epithelial tumours or might represent metastatic disease from known or unknown primary sites. The most common benign pathologies encountered in the retroperitoneum include benign neurogenic tumours, paragangliomas, fibromatosis, renal angiomyolipomas and benign retroperitoneal lipomas.
CONCLUSIONS
Complete surgical resection is the only potential curative treatment modality for retroperitoneal sarcomas and is best performed in high-volume centres by a multidisciplinary sarcoma team. The ability completely to resect a retroperitoneal sarcoma and tumour grade remain the most important predictors of local recurrence and disease-specific survival.
Topics: Chemotherapy, Adjuvant; Humans; Neoplasm Recurrence, Local; Retroperitoneal Neoplasms; Sarcoma; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 21944791
DOI: 10.1308/003588411X571944 -
Human Pathology Apr 2019Many sarcomas contain gene fusions that can be pathogenetic mechanisms and diagnostic markers. In this article we review selected fusion sarcomas and techniques for... (Review)
Review
Many sarcomas contain gene fusions that can be pathogenetic mechanisms and diagnostic markers. In this article we review selected fusion sarcomas and techniques for their detection. CIC-DUX4 fusion sarcoma is a round cell tumor now considered an entity separate from Ewing sarcoma with a more aggressive clinical course, occurrence in older age, and predilection to soft tissues. It is composed of larger cells than Ewing sarcoma and often has prominent necrosis. Nuclear DUX4 expression is a promising immuno histochemical marker. BCOR-CCNB3 fusion sarcoma is cyclin B3-positive, usually occurs in bone or soft tissue of children, and may mimic a poorly differentiated synovial sarcoma. EWSR1-NFATC2 sarcoma may present in bone or soft tissue. It is typically composed of small round cells in a trabecular pattern in a myxoid matrix resembling myoepithelioma. ACTB-GLI1 fusion sarcoma may mimic a skin adnexal carcinoma, showing focal expression of epithelial markers and S100 protein. NTRK-fusion sarcomas include, in addition to infantile fibrosarcoma with ETV6-NTRK3 fusion, LMNA-NTRK1 fusion sarcoma, a low-grade spindle cell sarcoma seen in peripheral soft tissues in children and young adults. Methods to detect gene fusions include next-generation sequencing panels, anchored multiplex polymerase chain reaction systems to detect partner for a known fusion gene, and comprehensive RNA sequencing to detect virtually all gene fusions. In situ hybridization testing using probes for both fusion partners can be used as an alternative confirmation technique, especially in the absence of satisfactory RNA yield. In addition, fusion protein-related and other immunohistochemical markers can have a high specificity for fusion sarcomas.
Topics: Humans; Oncogene Fusion; Oncogene Proteins, Fusion; Sarcoma; Soft Tissue Neoplasms
PubMed: 30633925
DOI: 10.1016/j.humpath.2018.12.006