Cancers

Editorial

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4 pages, 201 KiB

Open AccessEditorial

Treatment Strategies for Metastatic Soft Tissue Sarcomas

by Lisette M. Wiltink, Rick L. M. Haas, Hans Gelderblom and Michiel A. J. van de Sande

Cancers 2021, 13(7), 1722; https://doi.org/10.3390/cancers13071722 - 6 Apr 2021

Cited by 7 | Viewed by 1884

Abstract

Soft tissue sarcomas (STS) are a diverse group of rare tumors of mesenchymal origin with different clinical, histologic and molecular characteristics [...] Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

Research

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12 pages, 665 KiB

Open AccessArticle

Primary High-Grade Myxoid Liposarcoma of the Extremities: Prognostic Factors and Metastatic Pattern

by Gianmarco Tuzzato, Roberta Laranga, Federico Ostetto, Elisa Bubbico, Giulio Vara and Giuseppe Bianchi

Cancers 2022, 14(11), 2657; https://doi.org/10.3390/cancers14112657 - 27 May 2022

Cited by 4 | Viewed by 2411

Abstract

(1) Background: This retrospective study aimed to analyze the history and treatment outcomes of localized, high-grade MLS of the extremities. (2) Methods: We retrospectively reviewed 82 patients with primary high-grade MLS of the extremities. OS, LRFS, MFS, PRS, and DFS were analyzed. (3) [...] Read more.

(1) Background: This retrospective study aimed to analyze the history and treatment outcomes of localized, high-grade MLS of the extremities. (2) Methods: We retrospectively reviewed 82 patients with primary high-grade MLS of the extremities. OS, LRFS, MFS, PRS, and DFS were analyzed. (3) Results: Five-year OS and LRS were 96% (95% CI: 86–98) and 94% (95% CI: 85–98), respectively. Statistical analysis indicated no risk factors for OS and LFRS. MFS was 77% (65–85) at 5-year follow-up. Size (p = 0.0337) was the only risk factor statistically significant for MFS (HR = 0.248, 95% CI: 0.07–0.84). Median PRS after distant metastasis was 34 months (range: 1–127 months). Five-year PRS was 79% (48–93). Overall, the 5-year DFS was 76% (65–85). (4) Conclusions: Patients with MLS were found to have a good prognosis. In high-grade deep-seated tumors, common risk factors for MLS do not correlate with survival. Tumor size appears to be the only predictor of long-term DSF and MSF. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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13 pages, 1213 KiB

Open AccessArticle

The Association of Metastasis Pattern and Management of Metastatic Disease with Oncological Outcomes in Patients with Malignant Peripheral Nerve Sheath Tumors: A Multicenter Cohort Study

by Ibtissam Acem, Enrico Martin, Winan J. van Houdt, Michiel A. J. van de Sande, Dirk J. Grünhagen, Cornelis Verhoef and MONACO Collaborators

Cancers 2021, 13(20), 5115; https://doi.org/10.3390/cancers13205115 - 12 Oct 2021

Cited by 6 | Viewed by 1494

Abstract

Purpose: This multicenter cohort study aimed to identify clinicopathologic and treatment-related factors associated with the development of distant metastasis (DM) and with overall survival (OS) after DM diagnosis in patients with malignant peripheral nerve sheath tumors (MPNST). Methods: All patients diagnosed with primary [...] Read more.

Purpose: This multicenter cohort study aimed to identify clinicopathologic and treatment-related factors associated with the development of distant metastasis (DM) and with overall survival (OS) after DM diagnosis in patients with malignant peripheral nerve sheath tumors (MPNST). Methods: All patients diagnosed with primary MPNST from 1988 to 2019 who were surgically treated for the primary tumor were included. Multivariable Cox regression analyses were performed to identify factors associated with DM and OS after DM diagnosis. Results: A total of 383 patients were included in this analysis, of which 150 developed metastatic disease. No differences in clinicopathologic characteristics and clinical outcome were found between patients with synchronous and metachronous DM. Neurofibromatosis type 1 (NF1), high grade, tumor size, triton and R2 resections were independent risk factors for the development of DM. NF1 and more than two metastasis sites were independently associated with worse OS after DM diagnosis. Metastasectomy, chemotherapy and the metastatic site category ‘other’ were associated with prolonged survival after DM diagnosis. Conclusions: This analysis provides important insights into clinicopathologic and treatment factors associated with outcomes in metastatic MPNST. Moreover, NF1-status is associated with a higher risk of DM; it is also independently associated with worse survival in metastatic MPNST. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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11 pages, 876 KiB

Open AccessArticle

Pathologic Response Rates after Neoadjuvant Therapy for Sarcoma: A Single Institution Study

by Crystal Seldon, Gautam Shrivastava, Melanie Fernandez, John Jarboe, Sheila Conway, Juan Pretell, Laura Freedman, Aaron Wolfson, Wei Zhao, Deukwoo Kwon, Andrew Rosenberg, Ty Subhawong, Jonathan Trent and Raphael Yechieli

Cancers 2021, 13(5), 1074; https://doi.org/10.3390/cancers13051074 - 3 Mar 2021

Cited by 5 | Viewed by 2926

Abstract

(1) Background: Pathologic necrosis of soft tissue sarcomas (STS) has been used to determine treatment response, but its relationship to neoadjuvant treatments remains indeterminate. In this retrospective, single institution study, we hypothesized that neoadjuvant chemoradiation (NA-CRT) yields higher rates of pathologic complete response [...] Read more.

(1) Background: Pathologic necrosis of soft tissue sarcomas (STS) has been used to determine treatment response, but its relationship to neoadjuvant treatments remains indeterminate. In this retrospective, single institution study, we hypothesized that neoadjuvant chemoradiation (NA-CRT) yields higher rates of pathologic complete response (pCR) than neoadjuvant radiation (NA-XRT) or chemotherapy (NA-CT) alone. (2) Methods: Patients with extremity STS between 2011–2020 who received neoadjuvant treatment were included. pCR was defined as percent necrosis of the surgical specimen greater than or equal to 90%. (3) Results: 79 patients were analyzed. 51.9% of the population were male with a mean age of 58.4 years. 49.4% identified as Non-Hispanic White. Twenty-six (32.9%) patients achieved pCR while 53 (67.1%) did not. NA-CT (OR 15.82, 95% CI = 2.58–96.9, p = 0.003 in univariate (UVA) and OR 24.7, 95% CI = 2.88–211.2, p = 0.003 in multivariate (MVA), respectively) and NA-XRT (OR 5.73, 95% CI = 1.51–21.8, p = 0.010 in UVA and OR 7.95, 95% CI = 1.87–33.7, p = 0.005 in MVA, respectively) was significantly associated with non- pCR when compared to NA-CRT. The analysis also demonstrated that grade 3 tumors, when using grade 2 as reference, also had significantly higher odds of achieving pCR (OR 0.23, 95% CI = 0.06–0.80, p = 0.022 in UVA and OR 0.16, 95% CI = 0.04–0.70, p = 0.015 in MVA, respectively). (4) Conclusion: NA-CRT yields superior pCR compared to other neoadjuvant regimens. This extends to higher grade tumors. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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15 pages, 942 KiB

Open AccessArticle

A Growth Modulation Index-Based GEISTRA Score as a New Prognostic Tool for Trabectedin Efficacy in Patients with Advanced Soft Tissue Sarcomas: A Spanish Group for Sarcoma Research (GEIS) Retrospective Study

by Javier Martínez-Trufero, Luis Miguel De Sande-González, Pablo Luna, Javier Martin-Broto, Rosa Álvarez, Gloria Marquina, Roberto Diaz-Beveridge, Andrés Poveda, Juana María Cano, Josefina Cruz-Jurado, Antonio López Pousa, María Angeles Vaz Salgado, Claudia M. Valverde-Morales, Isabel Sevilla, Jerónimo Martínez-García, Jordi Rubio-Casadevall, Ana De Juan, Juan Antonio Carrasco, David S Moura, Ibon Gurruchaga-Sotes and Antonio Gutiérrez Show full author list Hide full author list

Cancers 2021, 13(4), 792; https://doi.org/10.3390/cancers13040792 - 14 Feb 2021

Cited by 7 | Viewed by 3484

Abstract

The aim of this study was to identify an easily reliable prognostic score that selects the subset of advanced soft tissue sarcoma (ASTS) patients with a higher benefit with trabectedin in terms of time to progression and overall survival. A retrospective series of [...] Read more.

The aim of this study was to identify an easily reliable prognostic score that selects the subset of advanced soft tissue sarcoma (ASTS) patients with a higher benefit with trabectedin in terms of time to progression and overall survival. A retrospective series of 357 patients with ASTS treated with trabectedin as second- or further-line in 19 centers across Spain was analyzed. First, it was confirmed that patients with high growth modulation index (GMI > 1.33) were associated with the better clinical outcome. Univariate and multivariate analyses were performed to identify factors associated with a GMI > 1.33. Thus, GEISTRA score was based on metastasis free-interval (MFI ≤ 9.7 months), Karnofsky < 80%, Non L-sarcomas and better response in the previous systemic line. The median GMI was 0.82 (0–69), with 198 patients (55%) with a GMI < 1, 41 (11.5%) with a GMI 1–1.33 and 118 (33.1%) with a GMI > 1.33. The lowest GEISTRA score showed a median of time-to-progression (TTP) and overall survival (OS) of 5.7 and 19.5 months, respectively, whereas it was 1.8 and 3.1 months for TTP and OS, respectively, for the GEISTRA 4 score. This prognostic tool can contribute to better selecting candidates for trabectedin treatment in ASTS. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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Review

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11 pages, 255 KiB

Open AccessReview

Radiation Therapy in Metastatic Soft Tissue Sarcoma: From Palliation to Ablation

by Nishant K. Shah, Nikhil Yegya-Raman, Joshua A. Jones and Jacob E. Shabason

Cancers 2021, 13(19), 4775; https://doi.org/10.3390/cancers13194775 - 24 Sep 2021

Cited by 14 | Viewed by 2546

Abstract

The management of patients with metastatic cancer is rapidly changing. Historically, radiotherapy was utilized for the treatment of localized disease or for palliation. While systemic therapy remains the mainstay of management for patients with metastatic cancer, radiotherapy is becoming increasingly important not only [...] Read more.

The management of patients with metastatic cancer is rapidly changing. Historically, radiotherapy was utilized for the treatment of localized disease or for palliation. While systemic therapy remains the mainstay of management for patients with metastatic cancer, radiotherapy is becoming increasingly important not only to palliate symptoms, but also to ablate oligometastatic or oligoprogressive disease and improve local control in the primary site. There is emerging evidence in multiple solid malignancies that patients with low volume metastatic disease that undergo local ablative therapy to metastatic sites may have improved progression free survival and potentially overall survival. In addition, there is increasing evidence that select patients with metastatic disease may benefit from aggressive treatment of the primary site. Patients with metastatic soft tissue sarcoma have a poor overall prognosis. However, there may be opportunities in patients with low volume metastatic soft tissue sarcoma to improve outcomes with local therapy including surgery, ablation, embolization, and radiation therapy. Stereotactic body radiation therapy (SBRT) offers a safe, convenient, precise, and non-invasive option for ablation of sites of metastases. In this review article, we explore the limited yet evolving role of radiotherapy to metastatic and primary sites for local control and palliation, particularly in the oligometastatic setting. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

10 pages, 669 KiB

Open AccessReview

Immunotherapy Strategies for Gastrointestinal Stromal Tumor

by Junaid Arshad, Philippos A. Costa, Priscila Barreto-Coelho, Brianna Nicole Valdes and Jonathan C. Trent

Cancers 2021, 13(14), 3525; https://doi.org/10.3390/cancers13143525 - 14 Jul 2021

Cited by 17 | Viewed by 2982

Abstract

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. The management of locally advanced or metastatic unresectable GIST involves detecting KIT, PDGFR, or other molecular alterations targeted by imatinib and other tyrosine kinase inhibitors. The role [...] Read more.

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. The management of locally advanced or metastatic unresectable GIST involves detecting KIT, PDGFR, or other molecular alterations targeted by imatinib and other tyrosine kinase inhibitors. The role of immunotherapy in soft tissue sarcomas is growing fast due to multiple clinical and pre-clinical studies with no current standard of care. The potential therapies include cytokine-based therapy, immune checkpoint inhibitors, anti-KIT monoclonal antibodies, bi-specific monoclonal antibodies, and cell-based therapies. Here we provide a comprehensive review of the immunotherapeutic strategies for GIST. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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Other

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24 pages, 3066 KiB

Open AccessSystematic Review

Natural History and Treatment Strategies of Advanced PEComas: A Systematic Review

by Agathe Bourgmayer, Simon Nannini, Paul Bonjean, Jean-Emmanuel Kurtz, Gabriel G. Malouf and Justine Gantzer

Cancers 2021, 13(20), 5227; https://doi.org/10.3390/cancers13205227 - 18 Oct 2021

Cited by 12 | Viewed by 2821

Abstract

PEComas is a family of rare mesenchymal tumors. This systematic review aims to better understand the natural history of advanced PEComas. After a search on the PubMed database and main oncology meeting libraries according to the PRISMA guidelines, 88 articles reported in the [...] Read more.

PEComas is a family of rare mesenchymal tumors. This systematic review aims to better understand the natural history of advanced PEComas. After a search on the PubMed database and main oncology meeting libraries according to the PRISMA guidelines, 88 articles reported in the English literature were included. Data on clinical and histological features, treatments and outcomes were collected. To identify risk factors, univariate and multivariate analyses were performed. Seven cohorts of patients and 124 individual patients were identified. Focusing on case reports, most patients were metastatic, and the median overall survival (OS) of the entire cohort was 60 months (95%CI 33; NA). Risk factors significantly associated with OS in the multivariate analysis were the presence of metastasis at diagnosis (HR: 2.59, 95%CI 1.06; 6.33, p = 0.036) and the grouped-Bleeker’s risk category (HR: 4.66; 95%CI 1.07; 20.19; p = 0.039). In the metastatic population, only the presence of lymph node metastasis was associated with OS (HR: 3.11; 95%CI 1.13; 8.60, p < 0.05). Due to a lack of events, it was not possible to conclude on other factors. This review of the literature highlights the heterogeneity of literature data and shows the great diversity of clinical management strategies. Full article

(This article belongs to the Special Issue Metastatic Soft-Tissue Sarcomas)

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