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Pathogenesis and Novel Therapeutic Approaches for Sarcomas
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Pathogenesis and Novel Therapeutic Approaches for Sarcomas

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 20 January 2025 | Viewed by 2151

Special Issue Editor

Dr. Francisco Giner

E-Mail Website
Guest Editor
Department of Pathology, University Hospital “La Fe”, 46009 Valencia, Spain
Interests: soft tissue and bone tumors; colon cancer; inflammatory bowel disease

Special Issue Information

Dear Colleagues,

Malignant bone and soft tissue tumors (sarcomas) are relatively common neoplasms. Some of them are typical of the pediatric age, with all the social, medical, and family stress that this entails. In general, low-grade tumors show a tendency of local recurrence and are often difficult to resect surgically depending on the location. In addition, low-grade sarcomas do not usually show a good response to adjuvant cancer treatments. On the other hand, high-grade sarcomas are very aggressive and have a tendency to metastasize at a distance, and although they show a better response to cancer treatment they usually create resistance to them over time, and the disease ends up progressing. For all of these reasons, it is important to find new therapeutic targets that help us control the spread and progression of the disease. For this, it is important to study the structure, immunophenotype, and molecular alterations of each tumor that help us to better understand the biology of this characteristic group of neoplasms.

Dr. Francisco Giner
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.

Keywords

  • soft tissue tumors
  • bone tumors
  • molecular biology
  • target therapies

Published Papers (2 papers)

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13 pages, 4229 KiB  
Article
The Combined Immunohistochemical Expression of GLI1 and BCOR in Synovial Sarcomas for the Identification of Three Risk Groups and Their Prognostic Outcomes: A Study of 52 Patients
by Francisco Giner, Emilio Medina-Ceballos, Raquel López-Reig, Isidro Machado, José Antonio López-Guerrero, Samuel Navarro, Luis Alberto Rubio-Martínez, Mónica Espino, Empar Mayordomo-Aranda and Antonio Llombart-Bosch
Int. J. Mol. Sci. 2024, 25(14), 7615; https://doi.org/10.3390/ijms25147615 - 11 Jul 2024
Viewed by 367
Abstract
Synovial sarcoma (SS) is a rare soft-tissue tumor characterized by a monomorphic blue spindle cell histology and variable epithelial differentiation. Morphologically, SSs may be confused with other sarcomas. Systemic treatment is more effective for patients with high-risk SSs, patients with advanced disease, and [...] Read more.
Synovial sarcoma (SS) is a rare soft-tissue tumor characterized by a monomorphic blue spindle cell histology and variable epithelial differentiation. Morphologically, SSs may be confused with other sarcomas. Systemic treatment is more effective for patients with high-risk SSs, patients with advanced disease, and younger patients. However, further studies are required to find new prognostic biomarkers. Herein, we describe the morphological, molecular, and clinical findings, using a wide immunohistochemical panel, of a series of SS cases. We studied 52 cases confirmed as SSs by morphological diagnosis and/or molecular studies. Clinical data (gender, age, tumor size, tumor location, resection margins, adjuvant treatment, recurrences, metastasis, and survival) were also retrieved for each patient. All the available H&E slides were examined by four pathologists. Three tissue microarrays (TMAs) were constructed for each of the tumors, and a wide immunohistochemical panel was performed. For time-to-event variables, survival analysis was performed using Kaplan–Meier curves and log-rank testing, or Cox regression. Statistical significance was considered at p < 0.05. The mean age of our patients was 40.33, and the median was 40.5 years. We found a predominance of males versus females (1.7:1). The most frequent morphological subtype was monophasic. TRPS1, SS18-SSX, and SSX-C-terminus were positive in 96% of cases. GLI1 expression was strong in six and focal (cytoplasmic) in twenty patients. Moreover, BCOR was expressed in more than half of SSs. Positive expression of both proteins, BCOR and GLI1, was correlated with a worse prognosis. Multivariate analysis was also performed, but only BCOR expression appeared to be significant. The combination of GLI1 and BCOR antibodies can be used to group SSs into three risk groups (low, intermediate, and high risk). We hypothesize that these findings could identify which patients would benefit from receiving adjuvant treatment and which would not. Moreover, these markers could represent therapeutic targets in advanced stages. However, further, larger series of SSs and molecular studies are necessary to corroborate our present findings. Full article
(This article belongs to the Special Issue Pathogenesis and Novel Therapeutic Approaches for Sarcomas)
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11 pages, 264 KiB  
Review
Recent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma
by Jun Nishio, Shizuhide Nakayama and Mikiko Aoki
Int. J. Mol. Sci. 2024, 25(2), 1127; https://doi.org/10.3390/ijms25021127 - 17 Jan 2024
Cited by 1 | Viewed by 1195
Abstract
Myxoinflammatory fibroblastic sarcoma (MIFS) is an infiltrative, locally aggressive fibroblastic neoplasm of intermediate malignancy that typically arises in the distal extremities of middle-aged adults. It can histologically be confused with a number of benign and malignant conditions. Recently, high-grade examples of MIFS have [...] Read more.
Myxoinflammatory fibroblastic sarcoma (MIFS) is an infiltrative, locally aggressive fibroblastic neoplasm of intermediate malignancy that typically arises in the distal extremities of middle-aged adults. It can histologically be confused with a number of benign and malignant conditions. Recently, high-grade examples of MIFS have been described. Immunohistochemistry plays a very limited role in the diagnosis of MIFS. Several genetic alterations have been identified in MIFS, including a t(1;10)(p22;q24) translocation with TGFBR3 and/or OGA rearrangements, BRAF rearrangement, and VGLL3 amplification. Although it appears that VGLL3 amplification is the most consistent alteration, the molecular pathogenesis of MIFS remains poorly understood. A wide resection is considered the standard treatment for MIFS. Radiotherapy may be a viable option in cases with inadequate surgical margins or cases where surgery is likely to cause significant functional impairment. The systemic treatment options for advanced or metastatic disease are very limited. This review provides an updated overview of the clinicoradiological features, pathogenesis, histopathology, and treatment of MIFS. Full article
(This article belongs to the Special Issue Pathogenesis and Novel Therapeutic Approaches for Sarcomas)
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