Special Issue "Soft Tissue and Bone Sarcoma"

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A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: closed (31 January 2013)

Special Issue Editor

Guest Editor
Dr. Edwin Choy

Division of Hematology and Oncology, Massachusetts General Hospital, P.O. Box 226, Boston, MA 02114, USA
Website | E-Mail
Phone: 617-643-0230
Fax: +1 617 643 1915
Interests: sarcoma; osteosarcoma; Ewing's sarcoma; giant cell tumor; chordoma

Special Issue Information

Dear Colleagues,

Soft Tissue and Bone Sarcomas disproportionately affect children, adolescents, and young adults. Although considered a rare disease, sarcomas continue to have a devastating effect on these patients and their loved ones, and their impact on our society far exceed their relatively low prevalence. Because of its rarity and heterogeneity, clinical decision making on management of sarcomas are often individualized and without a consensus treatment strategy. Furthermore, research to improve clinical outcomes are particularly challenging as sizeable studies are hard to produce. By improving our knowledge about the molecular biology of sarcomas, we hope to identify new treatment strategies and targets for further drug development. We would like to invite manuscripts that aim to elucidate the pathophysiology and carcinogenesis of soft tissue and bone sarcomas and identify new arenas for sarcoma research. We are also particularly interested in manuscripts deciphering the mechanisms of putative targets for sarcoma treatments. By devoting a special issue just on soft tissue and bone sarcomas, we hope to display and bridge a critical mass of new and exciting research that can lead the way to improving cancer care for this cohort of patients.

Dr. Edwin Choy
Guest Editor

Submission

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. Papers will be published continuously (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as 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 refereed through a 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 monthly 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 800 CHF (Swiss Francs).

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Keywords

  • sarcoma
  • osteosarcoma
  • bone tumor
  • biomarker
  • drug development
  • connective tissue oncology

Published Papers (9 papers)

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Research

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Open AccessArticle Head and Neck Sarcomas: A Comprehensive Cancer Center Experience
Cancers 2013, 5(3), 890-900; doi:10.3390/cancers5030890
Received: 20 May 2013 / Revised: 20 May 2013 / Accepted: 18 June 2013 / Published: 15 July 2013
Cited by 6 | PDF Full-text (274 KB) | HTML Full-text | XML Full-text
Abstract
Head/neck sarcomas are rare, accounting for about 1% of head/neck malignancies and 5% of sarcomas. Outcomes have historically been worse in this group, due to anatomic constraints leading to difficulty in completely excising tumors, with high rates of local recurrence. We retrospectively analyzed
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Head/neck sarcomas are rare, accounting for about 1% of head/neck malignancies and 5% of sarcomas. Outcomes have historically been worse in this group, due to anatomic constraints leading to difficulty in completely excising tumors, with high rates of local recurrence. We retrospectively analyzed cases of head/neck soft tissue sarcomas (STS) and osteogenic sarcomas managed in a multi-disciplinary setting at Fox Chase Cancer Center from 1999–2009 to describe clinicopathologic characteristics, treatment, outcomes, and prognostic factors for disease control and survival. Thirty patients with STS and seven patients with osteogenic sarcoma were identified. Most STS were high grade (23) and almost all were localized at presentation (28). Common histologies were synovial cell (6), rhabdomyosarcoma (5), angiosarcoma (4), liposarcoma (4) and leiomyosarcoma (3). The type of primary therapy and disease outcomes were analyzed. Cox proportional hazards regression analysis was performed to identify predictors of disease-free survival (DFS) and overall survival (OS). The HR and 95% CI for Cox model and median DFS/OS analyzed by Kaplan-Meier curves were calculated. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessArticle Ex Vivo Behaviour of Human Bone Tumor Endothelial Cells
Cancers 2013, 5(2), 404-417; doi:10.3390/cancers5020404
Received: 19 February 2013 / Revised: 19 March 2013 / Accepted: 3 April 2013 / Published: 11 April 2013
Cited by 1 | PDF Full-text (907 KB) | HTML Full-text | XML Full-text
Abstract
Cooperation between endothelial cells and bone in bone remodelling is well established. In contrast, bone microvasculature supporting the growth of primary tumors and metastasis is poorly understood. Several antiangiogenic agents have recently been undergoing trials, although an extensive body of clinical data and
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Cooperation between endothelial cells and bone in bone remodelling is well established. In contrast, bone microvasculature supporting the growth of primary tumors and metastasis is poorly understood. Several antiangiogenic agents have recently been undergoing trials, although an extensive body of clinical data and experimental research have proved that angiogenic pathways differ in each tumor type and stage. Here, for the first time, we characterize at the molecular and functional level tumor endothelial cells from human bone sarcomas at different stages of disease and with different histotypes. We selected a CD31+ subpopulation from biopsies that displayed the capability to grow as adherent cell lines without vascular endothelial growth factor (VEGF). Our findings show the existence in human primary bone sarcomas of highly proliferative endothelial cells expressing CD31, CD44, CD105, CD146 and CD90 markers. These cells are committed to develop capillary-like structures and colony formation units, and to produce nitric oxide. We believe that a better understanding of tumor vasculature could be a valid tool for the design of an efficacious antiangiogenic therapy as adjuvant treatment of sarcomas. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessArticle A Phase I Study of the Combination of Temsirolimus with Irinotecan for Metastatic Sarcoma
Cancers 2013, 5(2), 418-429; doi:10.3390/cancers5020418
Received: 16 February 2013 / Revised: 25 March 2013 / Accepted: 6 April 2013 / Published: 11 April 2013
Cited by 1 | PDF Full-text (521 KB) | HTML Full-text | XML Full-text
Abstract
mTOR inhibitors are emerging as important anti-neoplastic agents with a wide range of clinical applications. The topoisomerase I inhibitor irinotecan is a potent DNA damaging drug, with a broad spectrum of anticancer activities. mTOR appears to enhance cancer cell survival following DNA damage,
[...] Read more.
mTOR inhibitors are emerging as important anti-neoplastic agents with a wide range of clinical applications. The topoisomerase I inhibitor irinotecan is a potent DNA damaging drug, with a broad spectrum of anticancer activities. mTOR appears to enhance cancer cell survival following DNA damage, thus the inhibition of mTOR after irinotecan could theoretically show synergistic activities in patients. Both mTOR inhibitors and irinotecan have been used as single agents in soft tissue sarcomas with limited efficacy. We completed a phase I trial of the combination of the mTOR inhibitor, temsirolimus, and irinotecan in patients with advanced soft tissue sarcoma. Seventeen patients were recruited. The Phase II recommended dose is 20 mg of temsirolimus and 80 mg/m2 of irinotecan administered on weekly basis for three out of four weeks. Most frequently encountered toxicities include cytopenias, fatigue, and gastrointestinal toxicities. Two patients (one with leiomyosarcoma and one with high grade undifferentiated sarcoma) had stable disease for more than 12 months. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessArticle Protein Kinase C Epsilon and Genetic Networks in Osteosarcoma Metastasis
Cancers 2013, 5(2), 372-403; doi:10.3390/cancers5020372
Received: 16 February 2013 / Revised: 22 March 2013 / Accepted: 26 March 2013 / Published: 8 April 2013
Cited by 2 | PDF Full-text (1254 KB) | HTML Full-text | XML Full-text
Abstract
Osteosarcoma (OS) is the most common primary malignant tumor of the bone, and pulmonary metastasis is the most frequent cause of OS mortality. The aim of this study was to discover and characterize genetic networks differentially expressed in metastatic OS. Expression profiling of
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Osteosarcoma (OS) is the most common primary malignant tumor of the bone, and pulmonary metastasis is the most frequent cause of OS mortality. The aim of this study was to discover and characterize genetic networks differentially expressed in metastatic OS. Expression profiling of OS tumors, and subsequent supervised network analysis, was performed to discover genetic networks differentially activated or organized in metastatic OS compared to localized OS. Broad trends among the profiles of metastatic tumors include aberrant activity of intracellular organization and translation networks, as well as disorganization of metabolic networks. The differentially activated PRKCε-RASGRP3-GNB2 network, which interacts with the disorganized DLG2 hub, was also found to be differentially expressed among OS cell lines with differing metastatic capacity in xenograft models. PRKCε transcript was more abundant in some metastatic OS tumors; however the difference was not significant overall. In functional studies, PRKCε was not found to be involved in migration of M132 OS cells, but its protein expression was induced in M112 OS cells following IGF-1 stimulation. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessArticle Nuclear DNA-Content in Mesenchymal Lesions in Dogs: Its Value as Marker of Malignancy and Extent of Genomic Instability
Cancers 2012, 4(4), 1300-1317; doi:10.3390/cancers4041300
Received: 25 October 2012 / Revised: 16 November 2012 / Accepted: 26 November 2012 / Published: 3 December 2012
PDF Full-text (640 KB) | HTML Full-text | XML Full-text
Abstract
DNA-aneuploidy may reflect the malignant nature of mesenchymal proliferations and herald gross genomic instability as a mechanistic factor in tumor genesis. DNA-ploidy and -index were determined by flow cytometry in canine inflammatory or neoplastic mesenchymal tissues and related to clinico-pathological features, biological behavior
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DNA-aneuploidy may reflect the malignant nature of mesenchymal proliferations and herald gross genomic instability as a mechanistic factor in tumor genesis. DNA-ploidy and -index were determined by flow cytometry in canine inflammatory or neoplastic mesenchymal tissues and related to clinico-pathological features, biological behavior and p53 gene mutational status. Half of all sarcomas were aneuploid. Benign mesenchymal neoplasms were rarely aneuploid and inflammatory lesions not at all. The aneuploidy rate was comparable to that reported for human sarcomas with significant variation amongst subtypes. DNA-ploidy status in canines lacked a relation with histological grade of malignancy, in contrast to human sarcomas. While aneuploidy was related to the development of metastases in soft tissue sarcomas it was not in osteosarcomas. No relation amongst sarcomas was found between ploidy status and presence of P53 gene mutations. Heterogeneity of the DNA index between primary and metastatic sarcoma sites was present in half of the cases examined. Hypoploidy is more common in canine sarcomas and hyperploid cases have less deviation of the DNA index than human sarcomas. The variation in the presence and extent of aneuploidy amongst sarcoma subtypes indicates variation in genomic instability. This study strengthens the concept of interspecies variation in the evolution of gross chromosomal aberrations during cancer development. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available

Review

Jump to: Research

Open AccessReview Proton Radiotherapy for Pediatric Sarcoma
Cancers 2014, 6(1), 112-127; doi:10.3390/cancers6010112
Received: 20 October 2013 / Revised: 17 December 2013 / Accepted: 18 December 2013 / Published: 14 January 2014
Cited by 3 | PDF Full-text (659 KB) | HTML Full-text | XML Full-text
Abstract
Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The
[...] Read more.
Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The physical properties of proton beam radiotherapy provide a distinct advantage over standard photon radiation by eliminating excess dose deposited beyond the target volume, thereby reducing both the dose of radiation delivered to non-target structures as well as the total radiation dose delivered to a patient. Dosimetric studies comparing proton plans to IMRT and 3D conformal radiation have demonstrated the superiority of protons in numerous pediatric malignancies and data on long-term clinical outcomes and toxicity is emerging. In this article, we review the existing clinical and dosimetric data regarding the use of proton beam radiation in malignant bone and soft tissue sarcomas. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessReview Novel Systemic Therapies in Advanced Liposarcoma: A Review of Recent Clinical Trial Results
Cancers 2013, 5(2), 529-549; doi:10.3390/cancers5020529
Received: 21 March 2013 / Revised: 16 April 2013 / Accepted: 2 May 2013 / Published: 10 May 2013
Cited by 11 | PDF Full-text (362 KB) | HTML Full-text | XML Full-text
Abstract
Liposarcoma is one of the most common adult soft tissue sarcomas and consists of three histologic subtypes (well and dedifferentiated, myxoid/round cell, and pleomorphic). Surgery is the mainstay of treatment for localized disease; however for unresectable or metastatic disease, effective treatment options are
[...] Read more.
Liposarcoma is one of the most common adult soft tissue sarcomas and consists of three histologic subtypes (well and dedifferentiated, myxoid/round cell, and pleomorphic). Surgery is the mainstay of treatment for localized disease; however for unresectable or metastatic disease, effective treatment options are currently limited. In the past decade, a better understanding of the distinct genetic and molecular aberrations for each of the three histologic subtypes has led to the development of several novel systemic therapies. Data from phase I and early phase II clinical trials have been reported. Despite challenges with conducting clinical trials in liposarcoma, preliminary results for several of these novel, biology-driven therapies are encouraging. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessReview Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect
Cancers 2013, 5(2), 519-528; doi:10.3390/cancers5020519
Received: 4 March 2013 / Revised: 9 April 2013 / Accepted: 3 May 2013 / Published: 8 May 2013
Cited by 2 | PDF Full-text (863 KB) | HTML Full-text | XML Full-text
Abstract
There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study
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There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study reviews the diagnostic criteria used to designate atypical neurofibroma from low-grade MPNSTs and reviews existing literature the natural history of each of these tumors to see if the distinction is, in fact, of importance. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available
Open AccessReview Hypoxia-Inducible Factors: Mediators of Cancer Progression; Prognostic and Therapeutic Targets in Soft Tissue Sarcomas
Cancers 2013, 5(2), 320-333; doi:10.3390/cancers5020320
Received: 23 January 2013 / Revised: 12 March 2013 / Accepted: 26 March 2013 / Published: 2 April 2013
Cited by 14 | PDF Full-text (382 KB) | HTML Full-text | XML Full-text
Abstract
Soft-tissue sarcomas remain aggressive tumors that result in death in greater than a third of patients due to either loco-regional recurrence or distant metastasis. Surgical resection remains the main choice of treatment for soft tissue sarcomas with pre- and/or post-operational radiation and neoadjuvant
[...] Read more.
Soft-tissue sarcomas remain aggressive tumors that result in death in greater than a third of patients due to either loco-regional recurrence or distant metastasis. Surgical resection remains the main choice of treatment for soft tissue sarcomas with pre- and/or post-operational radiation and neoadjuvant chemotherapy employed in more advanced stage disease. However, in recent decades, there has been little progress in the average five-year survival for the majority of patients with high-grade soft tissue sarcomas, highlighting the need for improved targeted therapeutic agents. Clinical and preclinical studies demonstrate that tumor hypoxia and up-regulation of hypoxia-inducible factors (HIFs) is associated with decreased survival, increased metastasis, and resistance to therapy in soft tissue sarcomas. HIF-mediated gene expression regulates many critical aspects of tumor biology, including cell survival, metabolic programming, angiogenesis, metastasis, and therapy resistance. In this review, we discuss HIFs and HIF-mediated genes as potential prognostic markers and therapeutic targets in sarcomas. Many pharmacological agents targeting hypoxia-related pathways are in development that may hold therapeutic potential for treating both primary and metastatic sarcomas that demonstrate increased HIF expression. Full article
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma) Print Edition available

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