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Cancers
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Management of Soft Tissue Sarcomas and GIST
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Management of Soft Tissue Sarcomas and GIST

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Immunology and Immunotherapy".

Deadline for manuscript submissions: closed (31 December 2020) | Viewed by 34201

Special Issue Editors

Prof. Dr. Giuseppe Badalamenti

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Guest Editor
Department of Surgical, Oncological, and Oral Sciences, Section of Medical Oncology, University of Palermo, Via Del Vespro 127, Palermo, Italy
Interests: soft tissue sarcomas; gastrointestinal stromal tumors; neuroendocrine tumors
Special Issues, Collections and Topics in MDPI journals
Dr. Lorena Incorvaia

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Guest Editor
Department of Biomedicine, Neuroscience and Advanced Diagnostics, University of Palermo, 90127 Palermo, Italy
Interests: soft tissue sarcomas; gastrointestinal stromal tumors; gynecological and genitourinary tumors; cancer genetics; hereditary breast and ovarian cancers; prognostic and predictive biomarkers; molecular mechanisms of targeted therapy and immunotherapy resistance
Special Issues, Collections and Topics in MDPI journals
Dr. Daniele Fanale

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Guest Editor
Section of Medical Oncology, University Hospital Policlinico “Paolo Giaccone” - Palermo. 90127 Palermo, Italy
Interests: Molecular biology and translational research of rare tumors; heredo-familial tumors; Gene expression; Molecular pathway network in solid tumors; Cancer tumor microenvironment; Tumor immunology; Predictive and prognostic biomarkers in oncology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Soft tissue sarcomas and GIST (gastrointestinal stromal tumors) are rare cancers and very heterogeneous neoplasms. For this reason, histological diagnosis is often very difficult, and the correct therapeutic approach is very complex. Soft tissue sarcomas and GISTs must always be referred to reference centers.
First-line treatment of soft tissue sarcomas is well standardized, but second-line treatment can vary depending on the histotype (histolog-driven treatment). In recent years, many new drugs (target therapy) have been tested in the treatment of sarcomas with promising results. Immunotherapy has reported disappointing results, showing some activity only against some particular histologies.

The treatment of metastatic GISTs is less complex and involves the use of tyrosine kinase inhibitors. At this time, four drugs are indicated and registered for metastatic GIST treatment: imatinib in the first line, sunitinib in the second line, regorafenib in the third line, and avapritinib for metastatic GIST with PDGFRA D842V mutation. The third line study comparing avapritinib versus regorafenib was recently closed, and the second line study ripretinib versus sunitinb is still open.

In this Special Issue, experts in this field will review the current approaches to the management of patients with sarcomas and GIST and they will also focus on the molecular and immunological aspects of this heterogeneous group of neoplasms.

Dr. Giuseppe Badalamenti
Dr. Lorena Incorvaia
Dr. Daniele Fanale
Guest Editors

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. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). 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

  • sarcomas
  • GIST
  • target therapy
  • immunotherapy

Published Papers (10 papers)

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Research

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14 pages, 1907 KiB  
Article
Prognostic Role of Plasma PD-1, PD-L1, pan-BTN3As and BTN3A1 in Patients Affected by Metastatic Gastrointestinal Stromal Tumors: Can Immune Checkpoints Act as a Sentinel for Short-Term Survival?
by Daniele Fanale, Lorena Incorvaia, Giuseppe Badalamenti, Ida De Luca, Laura Algeri, Annalisa Bonasera, Lidia Rita Corsini, Chiara Brando, Antonio Russo, Juan Lucio Iovanna and Viviana Bazan
Cancers 2021, 13(9), 2118; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers13092118 - 27 Apr 2021
Cited by 22 | Viewed by 2217
Abstract
Gastrointestinal stromal tumors (GISTs) represent 1% of all primary gastrointestinal tumors. Immune surveillance is often overcome by cancer cells due to the activation of immunoregulatory molecules such as programmed death protein (PD-1) and its ligand PD-L1, and butyrophilin sub-family 3A/CD277 receptors (BTN3A). Because [...] Read more.
Gastrointestinal stromal tumors (GISTs) represent 1% of all primary gastrointestinal tumors. Immune surveillance is often overcome by cancer cells due to the activation of immunoregulatory molecules such as programmed death protein (PD-1) and its ligand PD-L1, and butyrophilin sub-family 3A/CD277 receptors (BTN3A). Because several studies demonstrated that tumor PD-1 and PD-L1 expression may have a prominent prognostic function, this investigation aimed to discover if soluble forms of these molecules may be useful in predicting survival of metastatic GIST (mGIST) patients. Through specific ad hoc developed ELISA assays not yet available on the market, the circulating PD-1, PD-L1, BTN3A1, and pan-BTN3As levels were examined in 30 c-KIT exon 11-mutated mGIST patients, prior to imatinib therapy. Using specific thresholds derived by ROC analysis, we found that high baseline levels of sPD-1 (>8.1 ng/mL), sPD-L1 (>0.7 ng/mL), sBTN3A1 (>7.0 ng/mL), and pan-BTN3As (>5.0 ng/mL) were correlated with shorter progression-free survival (PFS) and poor prognosis. Contrariwise, subjects with lower plasma concentrations exhibited a median PFS about 20 months longer than to the earlier. Finally, an additional multivariate analysis revealed that circulating levels of sPD-L1 ≤ 0.7 ng/mL and pan-sBTN3As ≤ 5.0 ng/mL, and the absence of KIT exon 11 deletions or delins at codons 557 and/or 558 were associated with a longer PFS in mGIST patients. Our investigation, for the first time, revealed that evaluating the plasma concentration of some immune checkpoints may help prognosticate survival in mGIST patients, suggesting their potential use as prognostic biomarkers beyond the presence of KIT exon 11 Del or Delins at codons 557/558. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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15 pages, 2351 KiB  
Article
Type and Gene Location of KIT Mutations Predict Progression-Free Survival to First-Line Imatinib in Gastrointestinal Stromal Tumors: A Look into the Exon
by Lorena Incorvaia, Daniele Fanale, Bruno Vincenzi, Ida De Luca, Tommaso Vincenzo Bartolotta, Roberto Cannella, Gianni Pantuso, Daniela Cabibi, Antonio Russo, Viviana Bazan and Giuseppe Badalamenti
Cancers 2021, 13(5), 993; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers13050993 - 27 Feb 2021
Cited by 11 | Viewed by 3732
Abstract
In previous studies on localized GISTs, KIT exon 11 deletions and mutations involving codons 557/558 showed an adverse prognostic influence on recurrence-free survival. In the metastatic setting, there are limited data on how mutation type and codon location might contribute to progression-free survival [...] Read more.
In previous studies on localized GISTs, KIT exon 11 deletions and mutations involving codons 557/558 showed an adverse prognostic influence on recurrence-free survival. In the metastatic setting, there are limited data on how mutation type and codon location might contribute to progression-free survival (PFS) variability to first-line imatinib treatment. We analyzed the type and gene location of KIT and PDGFRA mutations for 206 patients from a GIST System database prospectively collected at an Italian reference center between January 2005 and September 2020. By describing the mutational landscape, we focused on clinicopathological characteristics according to the critical mutations and investigated the predictive role of type and gene location of the KIT exon 11 mutations in metastatic patients treated with first-line imatinib. Our data showed a predictive impact of KIT exon 11 pathogenic variant on PFS to imatinib treatment: patients with deletion or insertion/deletion (delins) in 557/558 codons had a shorter PFS (median PFS: 24 months) compared to the patients with a deletion in other codons, or duplication/insertion/SNV (median PFS: 43 and 49 months, respectively) (p < 0.001). These results reached an independent value in the multivariate model, which showed that the absence of exon 11 deletions or delins 557/558, the female gender, primitive tumor diameter (≤5 cm) and polymorphonuclear leucocytosis (>7.5 109/L) were significant prognostic factors for longer PFS. Analysis of the predictive role of PDGFRA PVs showed no significant results. Our results also confirm the aggressive biology of 557/558 deletions/delins in the metastatic setting and allow for prediction at the baseline which GIST patients would develop resistance to first-line imatinib treatment earlier. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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14 pages, 3334 KiB  
Article
MDM2-Dependent Rewiring of Metabolomic and Lipidomic Profiles in Dedifferentiated Liposarcoma Models
by Andrew Patt, Bryce Demoret, Colin Stets, Kate-Lynn Bill, Philip Smith, Anitha Vijay, Andrew Patterson, John Hays, Mindy Hoang, James L. Chen and Ewy A. Mathé
Cancers 2020, 12(8), 2157; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12082157 - 04 Aug 2020
Cited by 9 | Viewed by 3944
Abstract
Dedifferentiated liposarcoma (DDLPS) is an aggressive mesenchymal cancer marked by amplification of MDM2, an inhibitor of the tumor suppressor TP53. DDLPS patients with higher MDM2 amplification have lower chemotherapy sensitivity and worse outcome than patients with lower MDM2 amplification. We hypothesized that MDM2 [...] Read more.
Dedifferentiated liposarcoma (DDLPS) is an aggressive mesenchymal cancer marked by amplification of MDM2, an inhibitor of the tumor suppressor TP53. DDLPS patients with higher MDM2 amplification have lower chemotherapy sensitivity and worse outcome than patients with lower MDM2 amplification. We hypothesized that MDM2 amplification levels may be associated with changes in DDLPS metabolism. Six patient-derived DDLPS cell line models were subject to comprehensive metabolomic (Metabolon) and lipidomic (SCIEX 5600 TripleTOF-MS) profiling to assess associations with MDM2 amplification and their responses to metabolic perturbations. Comparing metabolomic profiles between MDM2 higher and lower amplification cells yielded a total of 17 differentially abundant metabolites across both panels (FDR < 0.05, log2 fold change < 0.75), including ceramides, glycosylated ceramides, and sphingomyelins. Disruption of lipid metabolism through statin administration resulted in a chemo-sensitive phenotype in MDM2 lower cell lines only, suggesting that lipid metabolism may be a large contributor to the more aggressive nature of MDM2 higher DDLPS tumors. This study is the first to provide comprehensive metabolomic and lipidomic characterization of DDLPS cell lines and provides evidence for MDM2-dependent differential molecular mechanisms that are critical factors in chemoresistance and could thus affect patient outcome. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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10 pages, 1665 KiB  
Article
Genomic Database Analysis of Uterine Leiomyosarcoma Mutational Profile
by Annalisa Astolfi, Margherita Nannini, Valentina Indio, Angela Schipani, Alessandro Rizzo, Anna Myriam Perrone, Pierandrea De Iaco, Maria Giulia Pirini, Antonio De Leo, Milena Urbini, Paola Secchiero and Maria Abbondanza Pantaleo
Cancers 2020, 12(8), 2126; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12082126 - 31 Jul 2020
Cited by 48 | Viewed by 3479
Abstract
Uterine Leiomyosarcoma (uLMS) is by far the most common type of uterine sarcoma, characterized by an aggressive clinical course, a heterogeneous genetic profile and a very scarce response to cytotoxic chemotherapy. The genetic make-up of uLMS is an area of active study that [...] Read more.
Uterine Leiomyosarcoma (uLMS) is by far the most common type of uterine sarcoma, characterized by an aggressive clinical course, a heterogeneous genetic profile and a very scarce response to cytotoxic chemotherapy. The genetic make-up of uLMS is an area of active study that could provide essential cues for the development of new therapeutic approaches. A total of 216 patients with uLMS from cBioPortal and AACR-GENIE databases were included in the study. The vast majority of patients (81%) carried at least one mutation in either TP53, RB1, ATRX or PTEN. The most frequently mutated gene was TP53, with 61% of the patients harboring at least one mutation, followed by RB1 at 48%. PTEN alteration was more frequent in metastases than in primary lesions, consistent with a later acquisition during tumor progression. There was a significant trend for TP53 and RB1 mutations to occur together, while both TP53 and RB1 were mutually exclusive with respect to CDKN2A/B inactivation. Overall survival did not show significant correlation with the mutational status, even if RB1 mutation emerged as a favorable prognostic factor in the TP53-mutant subgroup. This comprehensive analysis shows that uLMS is driven almost exclusively by the inactivation of tumor suppressor genes and suggests that future therapeutic strategies should be directed at targeting the main genetic drivers of uLMS oncogenesis. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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12 pages, 1540 KiB  
Article
Impact of Chemotherapy in the Adjuvant Setting of Early Stage Uterine Leiomyosarcoma: A Systematic Review and Updated Meta-Analysis
by Alessandro Rizzo, Margherita Nannini, Annalisa Astolfi, Valentina Indio, Pierandrea De Iaco, Anna Myriam Perrone, Antonio De Leo, Lorena Incorvaia, Valerio Di Scioscio and Maria Abbondanza Pantaleo
Cancers 2020, 12(7), 1899; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12071899 - 14 Jul 2020
Cited by 26 | Viewed by 2564
Abstract
Background: Although the use of adjuvant chemotherapy (AC) appears to be increasing over the past few years, several clinical trials and previous meta-analyses failed to determine whether AC could improve clinical outcomes in uterine leiomyosarcoma (uLMS). The aim of this systematic review and [...] Read more.
Background: Although the use of adjuvant chemotherapy (AC) appears to be increasing over the past few years, several clinical trials and previous meta-analyses failed to determine whether AC could improve clinical outcomes in uterine leiomyosarcoma (uLMS). The aim of this systematic review and meta-analysis was to compare AC (with or without radiotherapy) versus observation (obs) after primary surgery in early stage uLMS. Materials and Methods: Randomized controlled (RCTs) and non-randomized studies (NRSs) were retrieved. Outcomes of interest were as follows: distant recurrence rate, locoregional recurrence rate and overall recurrence rate. Results about distant recurrence rate, locoregional recurrence rate and overall recurrence rate were compared by calculating odds ratios (ORs) with 95% confidence intervals (CIs); ORs were combined with Mantel–Haenszel method. Results: Nine studies were included in the analysis, involving 545 patients (AC: 252, obs: 293). Compared with obs, AC did not reduce locoregional and distant recurrence rate, with a pooled OR of 1.36 and 0.63, respectively. Similarly, administration of AC did not decrease overall recurrence rate in comparison to obs. Conclusion: According to our results, AC (with or without radiotherapy) did not decrease recurrence rate in early stage uLMS; thus, the role of AC in this setting remains unclear. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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24 pages, 4842 KiB  
Article
Inhibition of FGF2-Mediated Signaling in GIST—Promising Approach for Overcoming Resistance to Imatinib
by Sergei Boichuk, Aigul Galembikova, Ekaterina Mikheeva, Firuza Bikinieva, Aida Aukhadieva, Pavel Dunaev, Dinar Khalikov, Semen Petrov, Refat Kurtasanov, Elena Valeeva, Igor Kireev, Vera Dugina, Anna Lushnikova, Maria Novikova and Pavel Kopnin
Cancers 2020, 12(6), 1674; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12061674 - 24 Jun 2020
Cited by 10 | Viewed by 3408
Abstract
Inhibition of KIT-signaling is a major molecular target for gastrointestinal stromal tumor (GIST) therapy, and imatinib mesylate (IM) is known as the most effective first-line treatment option for patients with advanced, unresectable, and/or metastatic GISTs. We show here for the first time that [...] Read more.
Inhibition of KIT-signaling is a major molecular target for gastrointestinal stromal tumor (GIST) therapy, and imatinib mesylate (IM) is known as the most effective first-line treatment option for patients with advanced, unresectable, and/or metastatic GISTs. We show here for the first time that the inhibition of KIT-signaling in GISTs induces profound changes in the cellular secretome, leading to the release of multiple chemokines, including FGF-2. IM increased migration, invasion, and colony formation of IM-resistant GISTs in an FGF2-dependent manner, whereas the use of blocking anti-FGF2 antibodies or BGJ398, a selective FGFR inhibitor, abolished these effects, thus suggesting that the activation of FGF2-mediated signaling could serve as a compensatory mechanism of KIT-signaling inhibited in GISTs. Conversely, FGF-2 rescued the growth of IM-naive GISTs treated by IM and protected them from IM-induced apoptosis, consistent with the possible involvement of FGF-2 in tumor response to IM-based therapy. Indeed, increased FGF-2 levels in serum and tumor specimens were found in IM-treated mice bearing IM-resistant GIST xenografts, whereas BGJ398 used in combination with IM effectively inhibited their growth. Similarly, increased FGF-2 expression in tumor specimens from IM-treated patients revealed the activation of FGF2-signaling in GISTs in vivo. Collectively, the continuation of IM-based therapy for IM-resistant GISTs might facilitate disease progression by promoting the malignant behavior of tumors in an FGF2-dependent manner. This provides a rationale to evaluate the effectiveness of the inhibitors of FGF-signaling for IM-resistant GISTs. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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Review

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19 pages, 318 KiB  
Review
Immune Checkpoint Inhibitory Therapy in Sarcomas: Is There Light at the End of the Tunnel?
by Vasiliki Siozopoulou, Andreas Domen, Karen Zwaenepoel, Annelies Van Beeck, Evelien Smits, Patrick Pauwels and Elly Marcq
Cancers 2021, 13(2), 360; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers13020360 - 19 Jan 2021
Cited by 23 | Viewed by 2884
Abstract
Soft tissue and bone sarcomas are a very heterogeneous group of tumors with many subtypes for which diagnosis and treatment remains a very challenging task. On top of that, the treatment choices are limited, and the prognosis of aggressive sarcomas remains poor. Immune [...] Read more.
Soft tissue and bone sarcomas are a very heterogeneous group of tumors with many subtypes for which diagnosis and treatment remains a very challenging task. On top of that, the treatment choices are limited, and the prognosis of aggressive sarcomas remains poor. Immune checkpoint inhibitors (ICIs) have drawn a lot of attention last years because of their promising response rates and their durable effects. ICIs are currently widely used in the daily routine practice for the treatment of a different malignancies, such as melanoma, Hodgkin lymphoma, and non-small cell lung carcinoma. Still, ICIs are not included in the standard treatment protocols of the different sarcoma types. However, a plethora of clinical trials investigates the clinical benefit of ICIs in sarcomas. There is clear need to develop predictive biomarkers to determine which sarcoma patients are most likely to benefit from immune checkpoint blockade. This review will focus on (i) the clinical trial results on the use of ICIs in different sarcoma types; and on (ii) possible biomarkers predictive for the effectiveness of these drugs in sarcomas. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
22 pages, 319 KiB  
Review
Optimal Clinical Management and the Molecular Biology of Angiosarcomas
by Tom Wei-Wu Chen, Jessica Burns, Robin L. Jones and Paul H. Huang
Cancers 2020, 12(11), 3321; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12113321 - 10 Nov 2020
Cited by 16 | Viewed by 4093
Abstract
Angiosarcomas comprise less than 3% of all soft tissue sarcomas but have a poor prognosis. Most angiosarcomas occur without obvious risk factors but secondary angiosarcoma could arise after radiotherapy or chronic lymphedema. Surgery remains the standard treatment for localized angiosarcoma but neoadjuvant systemic [...] Read more.
Angiosarcomas comprise less than 3% of all soft tissue sarcomas but have a poor prognosis. Most angiosarcomas occur without obvious risk factors but secondary angiosarcoma could arise after radiotherapy or chronic lymphedema. Surgery remains the standard treatment for localized angiosarcoma but neoadjuvant systemic treatment may improve the curability. For advanced angiosarcoma, anthracyclines and taxanes are the main chemotherapy options. Anti-angiogenic agents have a substantial role but the failure of a randomized phase 3 trial of pazopanib with or without an anti-endoglin antibody brings a challenge to future trials in angiosarcomas. Immune checkpoint inhibitors as single agents or in combination with oncolytic virus may play an important role but the optimal duration remains to be investigated. We also report the current understanding of the molecular pathways involved in angiosarcoma pathogenesis including MYC amplification, activation of angiogenic pathways and different molecular alterations that are associated with angiosarcomas of different aetiology. The success of the patient-partnered Angiosarcoma Project (ASCProject) has provided not only detailed insights into the molecular features of angiosarcomas of different origins but also offers a template for future fruitful collaborations between patients, physicians, and researchers. Lastly, we provide our perspective of future developments in optimizing the clinical management of angiosarcomas. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
24 pages, 530 KiB  
Review
Radiation Therapy in Adult Soft Tissue Sarcoma—Current Knowledge and Future Directions: A Review and Expert Opinion
by Falk Roeder
Cancers 2020, 12(11), 3242; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12113242 - 03 Nov 2020
Cited by 14 | Viewed by 4048
Abstract
Radiation therapy (RT) is an integral part of the treatment of adult soft-tissue sarcomas (STS). Although mainly used as perioperative therapy to increase local control in resectable STS with high risk features, it also plays an increasing role in the treatment of non-resectable [...] Read more.
Radiation therapy (RT) is an integral part of the treatment of adult soft-tissue sarcomas (STS). Although mainly used as perioperative therapy to increase local control in resectable STS with high risk features, it also plays an increasing role in the treatment of non-resectable primary tumors, oligometastatic situations, or for palliation. Modern radiation techniques, like intensity-modulated, image-guided, or stereotactic body RT, as well as special applications like intraoperative RT, brachytherapy, or particle therapy, have widened the therapeutic window allowing either dose escalation with improved efficacy or reduction of side effects with improved functional outcome. This review summarizes the current evidence for RT in adult STS including typical indications, outcomes, side effects, dose and fractionation regimens, and target volume definitions based on tumor localization and risk factors. It covers the different overall treatment approaches including RT either as part of a multimodal treatment strategy or as a sole treatment, namely its use as an adjunct to surgery in resectable STS (perioperative RT), as a primary treatment in non-resectable tumors (definitive RT), as a local treatment modality in oligometastatic disease or as palliative therapy. Due to the known differences in clinical course, general treatment options and, consequently, outcome depending on lesion localization, the main part of perioperative RT is divided into three sections according to body site (extremity/trunk wall, retroperitoneal, and head and neck STS) including the discussion of special applications of radiation techniques specifically amenable to this region. The review of the current evidence is accompanied by a summary on ongoing clinical research pointing at future directions of RT in STS. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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15 pages, 707 KiB  
Review
Neoadjuvant Systemic Treatment of Primary Angiosarcoma
by Kimberley M. Heinhuis, Nikki S. IJzerman, Winette T. A. van der Graaf, Jan Martijn Kerst, Yvonne Schrage, Jos H. Beijnen, Neeltje Steeghs and Winan J. van Houdt
Cancers 2020, 12(8), 2251; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers12082251 - 12 Aug 2020
Cited by 19 | Viewed by 2904
Abstract
Angiosarcoma is an extremely rare and aggressive malignancy. Standard of care of localized tumors includes surgery ± radiation. Despite this multimodal treatment, >50% of the angiosarcoma patients develop local or distant recurrent disease. The role of neoadjuvant systemic therapy is still controversial and [...] Read more.
Angiosarcoma is an extremely rare and aggressive malignancy. Standard of care of localized tumors includes surgery ± radiation. Despite this multimodal treatment, >50% of the angiosarcoma patients develop local or distant recurrent disease. The role of neoadjuvant systemic therapy is still controversial and we therefore performed a systematic review of the literature to define the role of neoadjuvant systemic therapy based on available evidence. We focused on the effects of neoadjuvant systemic therapy on: 1. The success of surgical resection and 2. the long-term survival. All articles published before October 2019 on Ovid Medline, Ovid Embase, Cochrane library and Scopus were evaluated. Eighteen case reports and six retrospective cohort studies were included. There were no randomized controlled trials. This literature showed a beneficial role of neoadjuvant chemotherapy on downsizing of the tumor resulting in an improvement of the resection margins, especially in patients with cardiac or cutaneous angiosarcoma. However, no definitive conclusions on survival can be drawn based on the available literature lacking any prospective randomized studies in this setting. We advise that neoadjuvant chemotherapy should be considered, since this could lead to less mutilating resections and a higher rate of free resection margins. An international angiosarcoma registry could help to develop guidelines for this rare disease. Full article
(This article belongs to the Special Issue Management of Soft Tissue Sarcomas and GIST)
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