ijms-logo

Journal Browser

Journal Browser

Novel Insights into Soft Tissue Sarcoma

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Oncology".

Deadline for manuscript submissions: closed (31 December 2022) | Viewed by 6163

Special Issue Editor


E-Mail Website
Guest Editor
Semmelweis University, Budapest, Hungary

Special Issue Information

Dear Colleagues,

Soft tissue sarcomas (STSs) comprise 1% of adult malignant tumors (7% of childhood malignancies). Sarcomas encompass a heterogeneous group of malignancies of mesenchymal origin and may occur in any site of the body, which represents an additional challenge when it comes to therapy. They are a heterogeneous group of rare tumors, with more than 50 histological subtypes of STSs (WHO classification of tumors) having been identified, of which the most common in adults are malignant fibrous histiocytoma (28%), leiomyosarcoma (12%), liposarcoma (15%), synovial sarcoma (10%), and malignant peripheral nerve sheath tumors (6%). While the prognosis is good for patients diagnosed at an early stage and treated by adequate surgery, unresectable or metastatic diseases shrink the overall survival at 5 years to less than 10%, creating an unmet medical need.

The medical treatment of adult soft tissue sarcomas is more and more dictated by the histological subtype; this applies to both cytotoxic and target therapies. Anthracycline- and ifosfamide-based chemotherapies are the main therapeutic agents in the neoadjuvant, adjuvant, and metastatic adult-type STS disease settings, primarily for high-grade tumors, with the best-established response rates in adult soft tissue sarcomas for several years. In addition to this compound, there is evidence of efficacy of new drugs. Based on our current knowledge, 5–10% of all malignancies are part of hereditary cancer syndromes. Although the increasing diagnostic role of molecular genetic testing make us able to recognize more hereditary cancer patients, the careful exploration of family and clinical history by physicians is still the most important step for the diagnosis. Sarcomas are only 1% of all malignancies, but they often associate with familiar diseases so they can serve as an indicator of these syndromes. The diagnosis of hereditary cancer predisposition syndromes is essential to ensure appropriate therapy and follow-up for our patients. Multidisciplinary approach is mandatory in all cases (involving pathologist, radiologist, surgeons, radiation therapists, medical oncologists), and it should be carried out in reference centers for sarcomas.

Prof. Dr. Zsuzsanna Pápai
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Benefits of Publishing in a Special Issue

  • Ease of navigation: Grouping papers by topic helps scholars navigate broad scope journals more efficiently.
  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
  • External promotion: Articles in Special Issues are often promoted through the journal's social media, increasing their visibility.
  • e-Book format: Special Issues with more than 10 articles can be published as dedicated e-books, ensuring wide and rapid dissemination.

Further information on MDPI's Special Issue polices can be found here.

Published Papers (2 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

18 pages, 2088 KiB  
Article
MicroRNAs as Potential Biomarkers in the Differential Diagnosis of Lipomatous Tumors and Their Mimics
by Hui Min Tan, He Cheng, Yew Chung Tang, Sai Mun Leong, Poh Yin Teo, Chi Kuen Lee, Victor Kwan Min Lee and Susan Swee-Shan Hue
Int. J. Mol. Sci. 2022, 23(14), 7804; https://doi.org/10.3390/ijms23147804 - 15 Jul 2022
Cited by 4 | Viewed by 2020
Abstract
Adipocytic tumors are the most common subtype of soft tissue tumors. In current clinical practice, distinguishing benign lipomas from well-differentiated liposarcomas (WDLPS), as well as dedifferentiated liposarcomas (DDLPS) from their morphologic mimics, remains a significant diagnostic challenge. This is especially so when examining [...] Read more.
Adipocytic tumors are the most common subtype of soft tissue tumors. In current clinical practice, distinguishing benign lipomas from well-differentiated liposarcomas (WDLPS), as well as dedifferentiated liposarcomas (DDLPS) from their morphologic mimics, remains a significant diagnostic challenge. This is especially so when examining small biopsy samples and without the aid of additional ancillary tests. Recognizing the important role that microRNAs (miRNAs) play in tumorigenesis and their potential utility in tumor classification, we analyzed routine clinical tissue samples of benign and malignant lipomatous tumors, as well as other sarcoma mimics, to identify distinguishing miRNA-based signatures that can aid in the differential diagnosis of these entities. We discovered a 6-miRNA signature that separated lipomas from WDLPS with high confidence (AUC of 0.963), as well as a separate 6-miRNA signature that distinguished DDLPS from their more aggressive histologic mimics (AUC of 0.740). Functional enrichment analysis unveiled possible mechanistic involvement of these predictive miRNAs in adipocytic cancer-related biological processes and pathways such as PI3K/AKT/mTOR and MAPK signaling, further supporting the relevance of these miRNAs as biomarkers for adipocytic tumors. Our results demonstrate that miRNA expression profiling may potentially be used as an adjunctive tool for the diagnosis of benign and malignant adipocytic tumors. Further validation studies are warranted. Full article
(This article belongs to the Special Issue Novel Insights into Soft Tissue Sarcoma)
Show Figures

Figure 1

9 pages, 2461 KiB  
Article
A Novel NFIX-STAT6 Gene Fusion in Solitary Fibrous Tumor: A Case Report
by David S. Moura, Juan Díaz-Martín, Silvia Bagué, Ruth Orellana-Fernandez, Ana Sebio, Jose L. Mondaza-Hernandez, Carmen Salguero-Aranda, Federico Rojo, Nadia Hindi, Christopher D. M. Fletcher and Javier Martin-Broto
Int. J. Mol. Sci. 2021, 22(14), 7514; https://doi.org/10.3390/ijms22147514 - 13 Jul 2021
Cited by 4 | Viewed by 3291
Abstract
Solitary fibrous tumor is a rare subtype of soft-tissue sarcoma with a wide spectrum of histopathological features and clinical behaviors, ranging from mildly to highly aggressive tumors. The defining genetic driver alteration is the gene fusion NAB2–STAT6, resulting from a paracentric inversion [...] Read more.
Solitary fibrous tumor is a rare subtype of soft-tissue sarcoma with a wide spectrum of histopathological features and clinical behaviors, ranging from mildly to highly aggressive tumors. The defining genetic driver alteration is the gene fusion NAB2–STAT6, resulting from a paracentric inversion within chromosome 12q, and involving several different exons in each gene. STAT6 (signal transducer and activator of transcription 6) nuclear immunostaining and/or the identification of NAB2–STAT6 gene fusion is required for the diagnostic confirmation of solitary fibrous tumor. In the present study, a new gene fusion consisting of Nuclear Factor I X (NFIX), mapping to 19p13.2 and STAT6, mapping to 12q13.3 was identified by targeted RNA-Seq in a 74-year-old female patient diagnosed with a deep-seated solitary fibrous tumor in the pelvis. Histopathologically, the neoplasm did not display nuclear pleomorphism or tumor necrosis and had a low proliferative index. A total of 378 unique reads spanning the NFIXexon8–STAT6exon2 breakpoint with 55 different start sites were detected in the bioinformatic analysis, which represented 59.5% of the reads intersecting the genomic location on either side of the breakpoint. Targeted RNA-Seq results were validated by RT-PCR/ Sanger sequencing. The identification of a new gene fusion partner for STAT6 in solitary fibrous tumor opens intriguing new hypotheses to refine the role of STAT6 in the sarcomatogenesis of this entity. Full article
(This article belongs to the Special Issue Novel Insights into Soft Tissue Sarcoma)
Show Figures

Figure 1

Back to TopTop