scholarly journals Translating Molecular Profiling of Soft Tissue Sarcomas into Daily Clinical Practice

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 512
Author(s):  
Celine Jacobs ◽  
Lore Lapeire
Keyword(s):  
Soft Tissue ◽  
Unmet Need ◽  
Soft Tissue Sarcomas ◽  
Molecular Profiling ◽  
Point Of View ◽  
Molecular Techniques ◽  
Mesenchymal Tumors ◽  
The Past ◽  
Cancer Types ◽  
Sarcoma Treatment

Soft tissue sarcomas are a group of rare mesenchymal tumors with more than 70 subtypes described. Treatment of these subtypes in an advanced setting is mainly according to a one-size-fits-all strategy indicating a high unmet need of new and more targeted therapeutic options in order to optimize survival. The introduction of advanced molecular techniques in cancer has led to better diagnostics and identification of new therapeutic targets, leading to more personalized treatment and improved prognosis for several cancer types. In sarcoma, a likewise evolution is seen, albeit at a slower pace. This manuscript describes how in the past years advanced molecular profiling in soft tissue sarcomas was able to identify specific and often pathognomonic aberrations, deferring standard sarcoma treatment in favor of more targeted treatment from an oncologist’s point of view.

scholarly journals Molecular Determinants of Soft Tissue Sarcoma Immunity: Targets for Immune Intervention

2021 ◽  
Vol 22 (14) ◽  
pp. 7518
Author(s):  
Marcella Tazzari ◽  
Laura Bergamaschi ◽  
Alessandro De Vita ◽  
Paola Collini ◽  
Marta Barisella ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Malignant Tumors ◽  
Soft Tissue Sarcomas ◽  
Point Of View ◽  
Molecular Features ◽  
Molecular Determinants ◽  
Genetic Landscape ◽  
Current Therapies ◽  
Cumulative Evidence

Soft tissue sarcomas (STSs) are a family of rare malignant tumors encompassing more than 80 histologies. Current therapies for metastatic STS, a condition that affects roughly half of patients, have limited efficacy, making innovative therapeutic strategies urgently needed. From a molecular point of view, STSs can be classified as translocation-related and those with a heavily rearranged genotype. Although only the latter display an increased mutational burden, molecular profiles suggestive of an “immune hot” tumor microenvironment are observed across STS histologies, and response to immunotherapy has been reported in both translocation-related and genetic complex STSs. These data reinforce the notion that immunity in STSs is multifaceted and influenced by both genetic and epigenetic determinants. Cumulative evidence indicates that a fine characterization of STSs at different levels is required to identify biomarkers predictive of immunotherapy response and to discover targetable pathways to switch on the immune sensitivity of “immune cold” tumors. In this review, we will summarize recent findings on the interplay between genetic landscape, molecular profiling and immunity in STSs. Immunological and molecular features will be discussed for their prognostic value in selected STS histologies. Finally, the local and systemic immunomodulatory effects of the targeted drugs imatinib and sunitinib will be discussed.

scholarly journals DNA Methylation Profiling for Diagnosing Undifferentiated Sarcoma with Capicua Transcriptional Receptor (CIC) Alterations

2020 ◽  
Vol 21 (5) ◽  
pp. 1818 ◽  
Author(s):  
Evelina Miele ◽  
Rita De Vito ◽  
Andrea Ciolfi ◽  
Lucia Pedace ◽  
Ida Russo ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Soft Tissue ◽  
Abdominal Wall ◽  
Current Knowledge ◽  
Soft Tissue Sarcomas ◽  
Molecular Techniques ◽  
Round Cell ◽  
Undifferentiated Sarcoma ◽  
Dna Methylation Profiling ◽  
Methylation Profiling

Undifferentiated soft tissue sarcomas are a group of diagnostically challenging tumors in the pediatric population. Molecular techniques are instrumental for the categorization and differential diagnosis of these tumors. A subgroup of recently identified soft tissue sarcomas with undifferentiated round cell morphology was characterized by Capicua transcriptional receptor (CIC) rearrangements. Recently, an array-based DNA methylation analysis of undifferentiated tumors with small blue round cell histology was shown to provide a highly robust and reproducible approach for precisely classifying this diagnostically challenging group of tumors. We describe the case of an undifferentiated sarcoma of the abdominal wall in a 12-year-old girl. The patient presented with a voluminous mass of the abdominal wall, and multiple micro-nodules in the right lung. The tumor was unclassifiable with current immunohistochemical and molecular approaches. However, DNA methylation profiling allowed us to classify this neoplasia as small blue round cell tumor with CIC alterations. The patient was treated with neoadjuvant chemotherapy followed by complete surgical resection and adjuvant chemotherapy. After 22 months, the patient is disease-free and in good clinical condition. To put our experience in context, we conducted a literature review, analyzing current knowledge and state-of-the-art diagnosis, prognosis, and clinical management of CIC rearranged sarcomas. Our findings further support the use of DNA methylation profiling as an important tool to improve diagnosis of non-Ewing small round cell tumors.

Current status of biomarker testing in historically rare, high-unmet-need tumors: soft tissue sarcomas and thyroid cancers

2019 ◽  
Vol 19 (11) ◽  
pp. 929-938
Author(s):  
Bridgette Schroader ◽  
Sheldon Kong ◽  
Sibyl Anderson ◽  
Todd Williamson ◽  
Anthony Sireci ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Unmet Need ◽  
Soft Tissue Sarcomas ◽  
Current Status ◽  
Thyroid Cancers ◽  
Biomarker Testing

scholarly journals Primary Paratesticular Leiomyosarcoma: A Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Zeineb Naimi ◽  
Semia Zarraa ◽  
Salma Kammoun ◽  
Safia Yahyaoui ◽  
Maha Driss ◽  
...  
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Conformal Radiotherapy ◽  
Pelvic Irradiation ◽  
Mesenchymal Tumors ◽  
Scrotal Mass ◽  
Surgical Scar ◽  
Tumor Bed ◽  
Prescription Dose

Paratesticular soft tissue sarcomas are very rare malignant mesenchymal tumors. With only few cases reported in the literature, data regarding diagnostic and management of these tumors are limited. We reported a case of primary paratesticular leiomyosarcoma in a 72-year-old man complaining of a progressively growing painless right scrotal mass. The patient underwent radical inguinal right orchiectomy and adjuvant 3D conformal radiotherapy to the tumor bed including the surgical scar. The prescription dose was 54 Gy, and no pelvic irradiation was performed. He remained free of recurrence for the last 16 months.

scholarly journals Sinonasal Hemangiopericytomas: Clinicopathologic and Imaging Findings

2005 ◽  
Vol 84 (2) ◽  
pp. 99-102 ◽  
Author(s):  
Enrique Palacios ◽  
Santiago Restrepo ◽  
Luciano Mastrogiovanni ◽  
Giovanni D. Lorusso ◽  
Rafael Rojas
Keyword(s):  
Soft Tissue ◽  
Retrospective Review ◽  
Soft Tissue Sarcomas ◽  
The Body ◽  
Vascular Tumors ◽  
Imaging Findings ◽  
Mesenchymal Tumors ◽  
Imaging Characteristics ◽  
Neoplastic Lesions ◽  
Pathologic Findings

Hemangiopericytomas are rare soft-tissue neoplastic lesions that can arise in any part of the body. They are mesenchymal tumors that account for 3 to 5% of all soft-tissue sarcomas and 1% of all vascular tumors. They originate in extravascular cells (pericytes). Some 15 to 30% of all hemangiopericytomas occur in the head and neck; of these, approximately 5% occur in the sinonasal area. We describe our brief retrospective review of 7 histologically proven cases of sinonasal hemangiopericytoma, and we discuss the imaging characteristics and clinical and pathologic findings in these patients.

Adjuvant and Neoadjuvant Chemotherapy for Soft Tissue Sarcomas: A Personal Point of View

2017 ◽  
Vol 103 (3) ◽  
pp. 213-216 ◽  
Author(s):  
Robert S. Benjamin
Keyword(s):  
Breast Cancer ◽  
Adjuvant Chemotherapy ◽  
Soft Tissue ◽  
Neoadjuvant Chemotherapy ◽  
Chemotherapeutic Agent ◽  
Soft Tissue Sarcomas ◽  
Point Of View ◽  
Medical Oncologists

Dr. Gianni Bonadonna is internationally recognized as one of the foremost medical oncologists of the 20th century. He is best known for his pioneering work in the development of adjuvant chemotherapy for breast cancer, but he was also the father of sarcoma chemotherapy. The first investigator to study the new chemotherapeutic agent adriamycin in the late 1960s, he noted activity against sarcomas. This article, focusing on adjuvant chemotherapy, adriamycin, and sarcomas, memorializes his achievements and their progeny.

The present state of the art in chemotherapy for soft tissue sarcomas in adults: the EORTC point of view

1995 ◽  
Vol 20 (3) ◽  
pp. 193-201 ◽  
Author(s):  
J. Verweij ◽  
H.T. Mouridsen ◽  
O.S. Nielssen ◽  
P.J. Woll ◽  
R. Somers ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Present State ◽  
State Of The Art ◽  
Soft Tissue Sarcomas ◽  
Point Of View

Molecular profiling of soft tissue sarcomas using next-generation sequencing: a pilot study toward precision therapeutics

2014 ◽  
Vol 45 (8) ◽  
pp. 1563-1571 ◽  
Author(s):  
George Jour ◽  
John D. Scarborough ◽  
Robin L. Jones ◽  
Elizabeth Loggers ◽  
Seth M. Pollack ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Pilot Study ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Molecular Profiling ◽  
Next Generation ◽  
Generation Sequencing ◽  
Precision Therapeutics

scholarly journals Immune-Based Therapies for Sarcoma

Sarcoma ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Seth M. Pollack ◽  
Elizabeth T. Loggers ◽  
Eve T. Rodler ◽  
Cassian Yee ◽  
Robin L. Jones
Keyword(s):  
Clinical Practice ◽  
Soft Tissue ◽  
Systemic Therapy ◽  
Unmet Need ◽  
Soft Tissue Sarcomas ◽  
Exact Role ◽  
Cancer Testis Antigens ◽  
Standard Clinical Practice ◽  
Tumor Types ◽  
Cancer Testis

Immunotherapy has shown promise in a number of tumor types, but its exact role in sarcoma remains to be defined. Advanced bone and soft tissue sarcomas are challenging diseases to treat with an unmet need for effective systemic therapy. Previous reports have suggested that immune-based treatments may be effective in sarcoma, but such approaches have not yet become part of standard clinical practice. A number of sarcoma subtypes express targets known as cancer testis antigens and hence may be excellent targets for immunotherapy. This paper will focus on the recent advances and understanding of cancer testis antigens in sarcoma and also clinical data of immunotherapeutic approaches in these diseases.

scholarly journals MicroRNAs in Soft Tissue Sarcomas: Overview of the Accumulating Evidence and Importance as Novel Biomarkers

2014 ◽  
Vol 2014 ◽  
pp. 1-15 ◽  
Author(s):  
Tomohiro Fujiwara ◽  
Toshiyuki Kunisada ◽  
Ken Takeda ◽  
Koji Uotani ◽  
Aki Yoshida ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Current Knowledge ◽  
Soft Tissue Sarcomas ◽  
Chromosomal Translocation ◽  
Clinical Applications ◽  
Healthy Individuals ◽  
Circulating Mirnas ◽  
The Past ◽  
Novel Biomarkers ◽  
Mirna Deregulation

Sarcomas are distinctly heterogeneous tumors and a variety of subtypes have been described. Although several diagnostic explorations in the past three decades, such as identification of chromosomal translocation, have greatly improved the diagnosis of soft tissue sarcomas, the unsolved issues, including the limited useful biomarkers, remain. Emerging reports on miRNAs in soft tissue sarcomas have provided clues to solving these problems. Evidence of circulating miRNAs in patients with soft tissue sarcomas and healthy individuals has been accumulated and is accelerating their potential to develop into clinical applications. Moreover, miRNAs that function as novel prognostic factors have been identified, thereby facilitating their use in miRNA-targeted therapy. In this review, we provide an overview of the current knowledge on miRNA deregulation in soft tissue sarcomas, and discuss their potential as novel biomarkers and therapeutics.

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