Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

Science.gov (United States)

Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors. Get detailed information about clinical presentation, diagnosis, prognosis, and treatment of newly diagnosed and recurrent soft tissue sarcoma in this summary for clinicians.

Hypoxia-Inducible Factors: Mediators of Cancer Progression; Prognostic and Therapeutic Targets in Soft Tissue Sarcomas

International Nuclear Information System (INIS)

Sadri, Navid; Zhang, Paul J.

2013-01-01

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

Soft tissue sarcoma of the extremity.

LENUS (Irish Health Repository)

Cooper, T M

2012-02-03

A retrospective review of 33 cases of soft tissue sarcoma of the extremity presenting over a 10 year period was undertaken. The history, patterns of referral, diagnostic investigations, procedures undertaken and outcomes were studied. We found there was a frequent delay in diagnosis and sometimes misinterpretation of biopsy specimens. Patients were seen by a variety of specialists from disciplines such as general surgery, plastic surgery, orthopaedic surgery and rheumatology. Considerable progress has been made in the treatment of soft tissue sarcomas, often allowing local control of the tumour without amputation. We believe there should be early referral of patients having these tumours to a centre where a combined multidisciplinary approach can be undertaken.

New Therapeutic Targets in Soft Tissue Sarcoma

Science.gov (United States)

Demicco, Elizabeth G; Maki, Robert G; Lev, Dina C.; Lazar, Alexander J

2012-01-01

Soft tissue sarcomas are an uncommon and diverse group of more than 50 mesenchymal malignancies. The pathogenesis of many of these is poorly understood, but others have begun to reveal the secrets of their inner workings. With considerable effort over recent years, soft tissue sarcomas have increasingly been classified on the basis of underlying molecular alterations. In turn, this has allowed the development and application of targeted agents in several specific, molecularly defined, sarcoma subtypes. This review will focus the rationale for targeted therapy in sarcoma, with emphasis on the relevance of specific molecular factors and pathways in both translocation-associated sarcomas and in genetically complex tumors. In addition, we will address some of the early successes in sarcoma targeted therapy as well as a few challenges and disappointments in this field. Finally we will discuss several possible opportunities represented by poorly understood, but potentially promising new therapeutic targets, as well as several novel biologic agents currently in preclinical and early phase I/II trials. This will provide the reader with context for understanding the current state this field and a sense of where it may be headed in the coming years. PMID:22498582

Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study

NARCIS (Netherlands)

Lorigan, Paul; Verweij, Jaap; Papai, Zsuzsa; Rodenhuis, Sjoerd; Le Cesne, Axel; Leahy, Michael G.; Radford, John A.; van Glabbeke, Martine M.; Kirkpatrick, Anne; Hogendoorn, Pancras C. W.; Blay, Jean-Yves

2007-01-01

PURPOSE: Single-agent doxorubicin remains the standard treatment for advanced soft tissue sarcomas. Combining doxorubicin with standard-dose ifosfamide has not been shown to improve survival and is associated with a significantly increased toxicity; it is not known whether higher dose single-agent

Extremity Soft Tissue Sarcoma: A Review of 19 Cases. | Eyesan ...

African Journals Online (AJOL)

Background: Although soft tissue sarcoma is a rare tumour, it accounts for a significant proportion of malignancies seen in many orthopaedic practices. The objectives of this study are to evaluate the pattern of presentation of extremity soft tissue sarcoma and the treatment outcome in our patients. Method: This is a 3 year ...

Drugs Approved for Soft Tissue Sarcoma

Science.gov (United States)

This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

MicroRNAs in the Tumor Biology of Soft Tissue Sarcomas

NARCIS (Netherlands)

C.M.M. Gits (Caroline)

2013-01-01

markdownabstract__Abstract__ Soft tissue sarcomas represent a rare, heterogeneous group of mesenchymal tumors. In sarcomas, histological classification, prediction of clinical behaviour and prognosis, and targeted treatment is often a challenge. A better understanding of the biology of soft

Soft tissue sarcoma - Compliance with guidelines

NARCIS (Netherlands)

Nijhuis, PHA; Schaapveld, M; Otter, R; Hoekstra, HJ

2001-01-01

BACKGROUND. Because soft tissue sarcomas (STS) are rare, guidelines for the diagnosis and treatment of patients with STS were developed. Because the diagnostic management is essential for definitive treatment, adherence to these guidelines is important. METHODS. Primary STS registered by the

Soft Tissue Sarcomas In Children And Adolescents

International Nuclear Information System (INIS)

Bajciova, V.

2008-01-01

Soft tissue sarcomas are rare tumors, they may occur at any age. It is heterogenous group of different histology types, different biology and different clinical behavior. Different treatment strategy is used for children and adults. Adolescents with sarcomas could be managed by both pediatric and medical oncologists. Decision regarding location of treatment should be based on the best patient interest. (author)

Intensity of recent years in the investigation of soft tissue sarcoma.

Science.gov (United States)

Blay, Jean-Yves

2017-06-01

The European Medicines Agency's approval in 2007 of trabectedin for treatment of advanced soft tissue sarcoma (STS) - the first new agent approved in this indication for more than 30 years - coincided with the start of an exciting era in sarcoma research. Over the past 10 years, several other cytotoxic agents and targeted therapies have been approved for use in advanced STS and research efforts continue to intensify. In parallel, the virtual explosion of new evidence has presented its own set of challenges in terms of interpreting the data and understanding how it may apply to individual patients. This review provides a critical appraisal of several key studies published in the field of advanced STS in the past decade.

Preoperative radiotherapy for bone and soft tissue sarcoma

International Nuclear Information System (INIS)

Matsumoto, Seiichi; Kawaguchi, Noriyoshi; Amino, Katsuhisa; Manabe, Jun; Yamashita, Takashi; Kaneta, Kouichi; Furuya, Kohtaro; Isobe, Yasushi.

1989-01-01

The role of preoperative radiotherapy was evaluated in 16 cases with soft tissue sarcoma and 13 cases with osteosarcoma. Nine osteosarcoma cases underwent radiotherapy of whole lesion, and 4 cases had radiotherapy only of the surgically uncurable portion. There were no local recurrences in M0 cases, but skin necrosis occurred in the whole radiation group. As for the soft tissue sarcomas, local recurrence was not seen in virgin cases, but two cases which had received previous treatment showed local recurrence. There were no cases with severe side effects. Partial radiotherapy was effective as preoperative treatment for osteosarcoma. Preoperative radiotherapy is better than postoperative radiotherapy from many standpoints. (author)

Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

OpenAIRE

Agarwala Sandeep

2006-01-01

Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53%) of these originate from the striated muscles and are called rhabdomyosarcomas (RMS) the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retrope...

Isolated limb perfusion with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma : The value of adjuvant radiotherapy

NARCIS (Netherlands)

Thijssens, KMJ; van Ginkel, RJ; Pras, E; Suurmeijer, AJH; Hoekstra, HJ

Background: The aim was to investigate the value of adjuvant radiotherapy for locally advanced soft tissue sarcoma after hyperthermic isolated limb perfusion (ILP) with tumor necrosis factor alpha and melphalan followed by limb-saving surgery. Methods: From 1991 to 2003, 73 patients (median age, 54

THE POTENTIAL VALUE OF ULTRASOUND IN DIAGNOSIS OF SOFT TISSUE SARCOMA (LITERATURE REVIEW

Directory of Open Access Journals (Sweden)

I. G. Frolova

2015-01-01

Full Text Available Literature data on the potential value of ultrasound imaging in diagnosis of soft tissue sarcoma were analyzed. Ultrasound in B-regime was used to assess the extent of soft tissue sarcoma, Doppler ultrasonography was used to study tumor vascularization and sonoelastography was useful to differentiate benign from malignant tumors of soft tissues. The analysis of diagnostic value of ultrasound in detection of soft tissue lesions was carried out.  Criteria characterizing various histological types of tumors were identified.

Imaging of the most frequent superficial soft-tissue sarcomas

International Nuclear Information System (INIS)

Morel, Melanie; Taieb, Sophie; Ceugnart, Luc; Penel, Nicolas; Mortier, Laurent; Vanseymortier, Luc; Robin, Y.M.; Gosset, Pierre; Cotten, Anne

2011-01-01

Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)

Imaging of the most frequent superficial soft-tissue sarcomas

Energy Technology Data Exchange (ETDEWEB)

Morel, Melanie; Taieb, Sophie; Ceugnart, Luc [Centre Oscar Lambret, Department of Radiology, Lille (France); Penel, Nicolas [Centre Oscar Lambret, Department of Oncology, Lille (France); Mortier, Laurent [Centre Hospitalier Universitaire de Lille, Department of Dermatology, Hopital Claude Huriez, Lille (France); Vanseymortier, Luc [Centre Oscar Lambret, Department of Surgery, Lille (France); Robin, Y.M. [Centre Oscar Lambret, Departement of Pathology, Lille (France); Gosset, Pierre [Groupement Hospitalier de l' Institut Catholique-Faculte Libre de Medecine de Lille, Department of Pathology, Hopital Saint-Philibert, Lomme (France); Cotten, Anne [Centre Hospitalier Universitaire de Lille, Department of Musculoskeletal Radiology, Centre Hopital Roger Salengro, Lille (France)

2011-03-15

Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)

Pitfalls in soft tissue sarcoma imaging: chronic expanding hematomas.

Science.gov (United States)

Jahed, Kiarash; Khazai, Behnaz; Umpierrez, Monica; Subhawong, Ty K; Singer, Adam D

2018-01-01

Solid or nodular enhancement is typical of soft tissue sarcomas although high grade soft tissue sarcomas and those with internal hemorrhage often appear heterogeneous with areas of nonenhancement and solid or nodular enhancement. These MRI findings often prompt an orthopedic oncology referral, a biopsy or surgery. However, not all masses with these imaging findings are malignant. We report the multimodality imaging findings of two surgically proven chronic expanding hematomas (CEH) with imaging features that mimicked sarcomas. A third case of nonenhancing CEH of the lower extremity is also presented as a comparison. It is important that in the correct clinical scenario with typical imaging findings, the differential diagnosis of a chronic expanding hematoma be included in the workup of these patients. An image-guided biopsy of nodular tissue within such masses that proves to be negative for malignancy should not necessarily be considered discordant. A correct diagnosis may prevent a morbid unnecessary surgery and may indicate the need for a conservative noninvasive follow-up with imaging.

Bone and soft tissue sarcomas during pregnancy: A narrative review of the literature

Directory of Open Access Journals (Sweden)

George Zarkavelis

2016-07-01

Full Text Available Bone or soft tissue sarcomas are rarely diagnosed during pregnancy. Until today 137 well documented cases have been reported in the English literature between 1963 and 2014. Thirty-eight pregnant mothers were diagnosed with osteosarcoma, Ewing’s sarcoma or chondrosarcoma, whereas 95 other cases of soft tissue sarcomas of various types have been documented. We present the clinical picture and therapeutic management of this coexistence.

Histologic assessment of peritumoral edema in soft tissue sarcoma

International Nuclear Information System (INIS)

White, Lawrence M.; Wunder, Jay S.; Bell, Robert S.; O'Sullivan, Brian; Catton, Charles; Ferguson, Peter; Blackstein, Martin; Kandel, Rita A.

2005-01-01

Purpose: To evaluate whether satellite tumor cells can be identified histologically in the tissues surrounding a soft tissue sarcoma and whether their presence correlates with increased T 2 -weighted signal intensity on MRI. Methods and Materials: Fifteen patients with a high-grade extremity or truncal soft tissue sarcoma underwent preoperative MRI. The extent of high T 2 -weighted signal changes in the tissues surrounding tumor, thought to represent peritumoral edema/reactive changes, was determined. Twelve patients received i.v. gadolinium, and contrast enhancement was determined. All patients underwent surgical resection in the absence of preoperative chemotherapy or radiotherapy. The presence of tumor cells in the surrounding tissues was determined histologically in representative paraffin-embedded sections and correlated with the MRI findings. Results: The extent of peritumoral T 2 -weighted MRI signal changes ranged from 0 to 7.1 cm (mean, 2.5 cm); contrast enhancement ranged from 0 to 5.3 cm (mean, 1.1 cm). Sarcoma cells were identified histologically in the tissues beyond the tumor in 10 of 15 cases. In 6 cases, tumor cells were located within 1 cm of the tumor margin, and in 4 cases, malignant cells were found at a distance >1 cm and up to a maximum of 4 cm. The location of tumor cells beyond the margin did not correlate with tumor size nor did it correlate with the location or extent of peritumoral changes. Conclusion: The ability to identify tumor cells beyond the margin of a soft tissue sarcoma has important implications in planning appropriate targets for treatment. This could influence the use of new radiotherapy technologies such as intensity-modulated radiotherapy that aim to minimize treatment volumes through conformal planning

Intraoperative radiotherapy in the multidisciplinary management of soft tissue sarcomas

International Nuclear Information System (INIS)

Calvo, F.A.; Abuchaibe, O.; Escude, L.; Santos, M.; Villas, C.; Beguiristain, J.L.; Amillo, S.; Canadell, J.

1989-01-01

From 1984 to 1989 locally advanced stages of soft tissue sarcomas have been systematically approached with maximal surgical resection, IORT (10-20 Gy) boost to the tumor bed (high risk or residual disease areas) and conventional fractionated external beam postoperative radiotherapy. Chemotherapy was given to patients with high cellular grade sarcomas. Nineteen patients with localized soft tissue sarcomas of the extremities were treated and local control was obtained in 18/19 cases (95%). Distant metastasis developed in 2 patients and 15 patients are alive with no evidence of disease. The median follow-up time of the entire group is 27+ months (range 2+ to 43+ months). Severe IORT toxicity consisting in unreversible peripheral neuropathy has been observed in 3 patients. Another group of 24 patients had tumor located in central anatomical areas (3 head and neck, 7 trunk, 6 retroperitoneal, 3 glutens, 3 pelvis, 1 rectal, 1 bladder). Local control has been obtained in 15 patients (62%). Distant metastasis developed in 3 patients and 11 are alive and free of malignant disease. The median follow-up in this group is 14.5+ months (range from 4 to 50+ months). Severe neuropathy was observed in 1 patient. Severe neurological damage is a preoccupying finding that merits a word of caution in the development of IORT protocols, as what single dose can be selected in areas including nerves in treatment field

HEMIPELVECTOMY AND INTRAOPERATIVE RADIOTHERAPY FOR BONE AND SOFT-TISSUE SARCOMAS OF THE PELVIC GIRDLE

NARCIS (Netherlands)

HOEKSTRA, HJ; SINDELAR, WF; SZABO, BG; KINSELLA, TJ

1995-01-01

Current treatment of locally advanced bone and soft tissue sarcomas of the pelvic girdle are associated with a high local and distant failure rate, and local tumor control after hemipelvectomy can be a significant problem. IORT has been used in conjunction with hemipelvectomy, both conventional

Oxidative stress and antioxidant status in primary bone and soft tissue sarcoma

International Nuclear Information System (INIS)

Nathan, Fatima M; Singh, Vivek A; Dhanoa, Amreeta; Palanisamy, Uma D

2011-01-01

Oxidative stress is characterised by an increased level of reactive oxygen species (ROS) that disrupts the intracellular reduction-oxidation (redox) balance and has been implicated in various diseases including cancer. Malignant tumors of connective tissue or sarcomas account for approximately 1% of all cancer diagnoses in adults and around 15% of paediatric malignancies per annum. There exists no information on the alterations of oxidant/antioxidant status of sarcoma patients in literature. This study was aimed to determine the levels of oxidative stress and antioxidant defence in patients with primary bone and soft tissue sarcoma and to investigate if there exists any significant differences in these levels between both the sarcomas. The study cohort consisted of 94 subjects; 20 soft tissue sarcoma, 27 primary bone sarcoma and 47 healthy controls. Malondialdehyde (MDA) and protein carbonyls were determined to assess their oxidative stress levels while antioxidant status was evaluated using catalase (CAT), superoxide dismutase (SOD), thiols and trolox equivalent antioxidant capacity (TEAC). Sarcoma patients showed significant increase in plasma and urinary MDA and serum protein carbonyl levels (p < 0.05) while significant decreases were noted in TEAC, thiols, CAT and SOD levels (p < 0.05). No significant difference in oxidative damage was noted between both the sarcomas (p > 0.05). In conclusion, an increase in oxidative stress and decrease in antioxidant status is observed in both primary bone and soft tissue sarcomas with a similar extent of damage. This study offers the basis for further work on whether the manipulation of redox balance in patients with sarcoma represents a useful approach in the design of future therapies for bone disease

Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma

DEFF Research Database (Denmark)

Judson, Ian; Verweij, Jaap; Gelderblom, Hans

2014-01-01

BACKGROUND: Effective targeted treatment is unavailable for most sarcomas and doxorubicin and ifosfamide-which have been used to treat soft-tissue sarcoma for more than 30 years-still have an important role. Whether doxorubicin alone or the combination of doxorubicin and ifosfamide should be used...

Histology and imaging of soft tissue sarcomas.

Science.gov (United States)

Kind, Michèle; Stock, Nathalie; Coindre, Jean Michel

2009-10-01

Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

Histology and imaging of soft tissue sarcomas

International Nuclear Information System (INIS)

Kind, Michele; Stock, Nathalie; Coindre, Jean Michel

2009-01-01

Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

Histology and imaging of soft tissue sarcomas

Energy Technology Data Exchange (ETDEWEB)

Kind, Michele [Departement d' Imagerie Medicale, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France)], E-mail: kind@bergonie.org; Stock, Nathalie; Coindre, Jean Michel [Departement de Pathologie, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France); Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, 33076 Bordeaux Cedex (France)

2009-10-15

Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

Isolated limb perfusion with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma : Three time periods at risk for amputation

NARCIS (Netherlands)

van Ginkel, Robert J.; Thijssens, Katja M. J.; Pras, Elisabeth; van der Graaf, Winette T. A.; Suurmeijer, Albert J. H.; Hoekstra, Harald J.

Background: The aim of this study was to investigate the long-term limb salvage rate and overall survival after isolated limb perfusion (ILP) with tumor necrosis factor alpha and melphalan for locally advanced soft tissue sarcoma (STS). Methods: From 1991 to 2003, 73 patients (36 men, 37 women,

PML expression in soft tissue sarcoma: prognostic and predictive value in alkylating agents/antracycline-based first line therapy.

Science.gov (United States)

Vincenzi, Bruno; Santini, Daniele; Schiavon, Gaia; Frezza, Anna Maria; Silletta, Marianna; Crucitti, Pierfilippo; Casali, Paolo; Dei Tos, Angelo P; Rossi, Sabrina; Rizzo, Sergio; Badalamenti, Giuseppe; Tomasino, Rosa Maria; Russo, Antonio; Butrynski, James E; Tonini, Giuseppe

2012-04-01

Soft tissue sarcomas are aggressive tumors representing alkylating agents/antracycline-based first line therapy. One hundred eleven patients affected by locally advanced and metastatic soft tissue sarcoma were selected. PML expression was evaluated by immunohistochemical analysis in pathological samples and in the corresponding normal tissue from each case. PML immunohistochemical results were correlated with prognosis and with radiological response to alkylating agents/antracycline-based first line therapy. PML expression was significantly reduced in synovial sarcomas (P < 0.0001), in myofibroblastic sarcomas (P < 0.0001), angiosarcomas (P < 0.0001), in leiomyosarcomas (P = 0.003), in mixoid liposarcomas (P < 0.0001), and in dedifferentiated liposarcomas (P < 0.0001). No significant difference was found for pleomorphic sarcoma [31.8 (95% CI: 16.7-41.0); P = 0.21]. and pleomorphic liposarcomas (P = 0.51). Loss of PML expression was found to be statistically correlated with TTP (P < 0.0001), median duration of response (P = 0.007), and OS (P = 0.02). No correlation was observed between PML expression and treatment efficacy. PML IHC expression is down-regulated in synovial sarcomas, myofibroblastic sarcomas, angiosarcomas, liposarcoma, and leiomyosarcomas and its expression correlated with prognosis. Copyright © 2011 Wiley Periodicals, Inc.

Vorinostat in refractory soft tissue sarcomas - Results of a multi-centre phase II trial of the German Soft Tissue Sarcoma and Bone Tumour Working Group (AIO).

Science.gov (United States)

Schmitt, Thomas; Mayer-Steinacker, Regine; Mayer, Frank; Grünwald, Viktor; Schütte, Jochen; Hartmann, Jörg T; Kasper, Bernd; Hüsing, Johannes; Hajda, Jacek; Ottawa, Gregor; Mechtersheimer, Gunhild; Mikus, Gerd; Burhenne, Jürgen; Lehmann, Lorenz; Heilig, Christoph E; Ho, Anthony D; Egerer, Gerlinde

2016-09-01

New treatment options for patients with metastatic Soft Tissue Sarcoma are urgently needed. Preclinical studies suggested activity of vorinostat, a histone deacetylase inhibitor. A multi-centre, open-label, non-randomised phase II trial to investigate the efficacy and safety of vorinostat in patients with locally advanced or metastatic Soft Tissue Sarcoma failing 1st-line anthracycline-based chemotherapy was initiated. Patients were treated with vorinostat 400 mg po qd for 28 d followed by a treatment-free period of 7 d, representing a treatment cycle of 5 weeks. Restaging was performed every three cycles or at clinical progression. Between 06/10 and 09/13, 40 Soft Tissue Sarcoma patients were treated with vorinostat at seven participating centres. Patients had received 1 (n=8, 20%), 2 (n=10, 25%) or ≥3 (n=22, 55%) previous lines of chemotherapy. Best response after three cycles of treatment was stable disease (n=9, 23%). Median progression-free survival and overall survival were 3.2 and 12.3 months, respectively. Six patients showed long-lasting disease stabilisation for up to ten cycles. Statistical analyses failed to identify baseline predictive markers in this subgroup. Major toxicities (grade ≥III) included haematological toxicity (n=6, 15%) gastrointestinal disorders (n=5, 13%), fatigue (n=4, 10%), musculoskeletal pain (n=4, 10%), and pneumonia (n=2, 5%). In a heavily pre-treated patient population, objective response to vorinostat was low. However, a small subgroup of patients had long-lasting disease stabilisation. Further studies aiming to identify predictive markers for treatment response as well as exploration of combination regimens are warranted. NCT00918489 (ClinicalTrials.gov) EudraCT-number: 2008-008513-19. Copyright © 2016 Elsevier Ltd. All rights reserved.

Quality of life and patients' expectations in soft tissue sarcoma.

Science.gov (United States)

Jones, Robin L; Cesne, Axel Le

2018-05-01

Assessment of health-related quality of life (HRQoL) is essential for holistic care. Greater efforts are required to incorporate HRQoL measures into clinical trials and daily practice. Considerable HRQoL data are available for localized soft tissue sarcomas (STS), particularly in the orthopedic setting. In future, HRQoL is expected to become increasingly important in the evaluation of palliative therapy in advanced STS. A patient-centric approach is advocated for STS management. Greater awareness of STS by nonspecialist clinicians, and timely referral to specialized sarcoma reference centers, is crucial for patient welfare. The patient is central to shared decision-making during consultations and during case review in tumor boards. The management approach to STS should be collaborative, involving a multidisciplinary team, multiple centers and patient advocacy groups.

A phase IIb multicentre study comparing the efficacy of trabectedin to doxorubicin in patients with advanced or metastatic untreated soft tissue sarcoma: The TRUSTS trial

NARCIS (Netherlands)

Bui-Nguyen, B.; Butrynski, J.E.; Penel, N.; Blay, J.Y.; Isambert, N.; Milhem, M.; Kerst, J.M.; Reyners, A.K.; Litiere, S.; Marreaud, S.; Collin, F.; Graaf, W.T.A. van der

2015-01-01

PURPOSE: To evaluate whether trabectedin as first-line chemotherapy for advanced/metastatic soft tissue sarcoma prolongs progression-free survival (PFS), compared to doxorubicin and, in the phase IIb part here, to select the most appropriate trabectedin treatment schedule (3-hour or 24-hour

CMG2 Expression Is an Independent Prognostic Factor for Soft Tissue Sarcoma Patients

Directory of Open Access Journals (Sweden)

Thomas Greither

2017-12-01

Full Text Available The capillary morphogenesis gene 2 (CMG2, also known as the anthrax toxin receptor 2 (ANTXR2, is a transmembrane protein putatively involved in extracellular matrix (ECM adhesion and tissue remodeling. CMG2 promotes endothelial cell proliferation and exhibits angiogenic properties. Its downregulation is associated with a worsened survival of breast carcinoma patients. Aim of this study was to analyze the CMG2 mRNA and protein expression in soft tissue sarcoma and their association with patient outcome. CMG2 mRNA was measured in 121 tumor samples of soft tissue sarcoma patients using quantitative real-time PCR. CMG2 protein was evaluated in 52 tumor samples by ELISA. CMG2 mRNA was significantly correlated with the corresponding CMG2 protein expression (rs = 0.31; p = 0.027. CMG2 mRNA expression was associated with the mRNA expressions of several ECM and tissue remodeling enzymes, among them CD26 and components of the uPA system. Low CMG2 mRNA expression was correlated with a worsened patients’ disease-specific survival in Kaplan-Meier analyses (mean patient survival was 25 vs. 96 months; p = 0.013, especially in high-stage tumors. A decreased CMG2 expression is a negative prognostic factor for soft tissue sarcoma patients. CMG2 may be an interesting candidate gene for the further exploration of soft tissue sarcoma genesis and progression.

CMG2 Expression Is an Independent Prognostic Factor for Soft Tissue Sarcoma Patients.

Science.gov (United States)

Greither, Thomas; Wedler, Alice; Rot, Swetlana; Keßler, Jacqueline; Kehlen, Astrid; Holzhausen, Hans-Jürgen; Bache, Matthias; Würl, Peter; Taubert, Helge; Kappler, Matthias

2017-12-07

The capillary morphogenesis gene 2 (CMG2), also known as the anthrax toxin receptor 2 (ANTXR2), is a transmembrane protein putatively involved in extracellular matrix (ECM) adhesion and tissue remodeling. CMG2 promotes endothelial cell proliferation and exhibits angiogenic properties. Its downregulation is associated with a worsened survival of breast carcinoma patients. Aim of this study was to analyze the CMG2 mRNA and protein expression in soft tissue sarcoma and their association with patient outcome. CMG2 mRNA was measured in 121 tumor samples of soft tissue sarcoma patients using quantitative real-time PCR. CMG2 protein was evaluated in 52 tumor samples by ELISA. CMG2 mRNA was significantly correlated with the corresponding CMG2 protein expression (r s = 0.31; p = 0.027). CMG2 mRNA expression was associated with the mRNA expressions of several ECM and tissue remodeling enzymes, among them CD26 and components of the uPA system. Low CMG2 mRNA expression was correlated with a worsened patients' disease-specific survival in Kaplan-Meier analyses (mean patient survival was 25 vs. 96 months; p = 0.013), especially in high-stage tumors. A decreased CMG2 expression is a negative prognostic factor for soft tissue sarcoma patients. CMG2 may be an interesting candidate gene for the further exploration of soft tissue sarcoma genesis and progression.

Epirubicin is not Superior to Doxorubicin in the Treatment of Advanced Soft Tissue Sarcomas.The Experience of the EORTC Soft Tissue and Bone Sarcoma Group

DEFF Research Database (Denmark)

Nielsen, Ole Steen; Dombernowsky, Per; Mouridsen, Henning T

2000-01-01

studies the EORTC STBSG tested whether epirubicin (epi) is an alternative to standard dose dox in the treatment of chemonaive patients with advanced soft tissue sarcoma. The present report gives the final results of these studies.Patients/Methods. In the first study 210 patients were randomized to receive...... either dox or epi both at a dose of 75 mg/m(2) given as bolus injection at 3-week intervals. In the second study 334 patients were randomized to dox 75 mg/m(2), epi 150 mg/m(2) or epi 50 mg/m(2) days 1-3, all given as bolus injection at 3-week intervals.Results. In the first study no differences...... in median survival and duration of response were found. Of 167 evaluable patients the response rate was slightly in favour of dox (23% vs 18%) but at the expense of more toxicity.These data could suggest that increasing the epi dose may lead to a greater antineoplastic effect with acceptable toxicity...

Clinical practice guideline: 2006 update of recommendations for the radiotherapeutic management of patients with soft tissue sarcoma (sarcoma of the extremity, uterine sarcoma and retroperitoneal sarcoma)

International Nuclear Information System (INIS)

Le Pechoux, C.; Pautier, P.; Le Cesne, A.; Delannes, M.; Bui, B.N.; Blay, J.Y.; Bonichon, F.; Bonvalot, S.; Morice, P.; Chevalier-Place, A.; Taieb, S.; Coindre, J.M.; Ray-Coquard, I.; Stoeckle, E.

2006-01-01

Context. - The National French Federation of Comprehensive Cancer Centres (FNCLCC) initiated the update of clinical practice guideline for the management of patients with soft tissue sarcoma in collaboration with the French Sarcoma Group (GSF-GETO), specialists from French public universities, general hospitals and private clinics and with the French National Cancer Institute. This work is based on the methodology developed in the 'Standards, Options and Recommendations' (SOR) project. Objectives - To update SOR guidelines for the management of patients with oft tissue sarcoma previously validated in 1995. Methods. -The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines have been developed, they are reviewed by independent reviewers. Results. - This article presents the updated recommendations for radiotherapeutic management. The main recommendations are: 1) irradiation before or after surgical treatment is the standard for soft tissue sarcoma of the extremity and uterine sarcoma; 2) no systematic irradiation should be done in case of retroperitoneal sarcoma. (author)

Surgical considerations when reporting MRI studies of soft tissue sarcoma of the limbs

Energy Technology Data Exchange (ETDEWEB)

De la Hoz Polo, Marcela [Kings College Hospital, Radiology Department, London, Brixton (United Kingdom); Dick, Elizabeth [St Mary' s Hospital, Imperial College Healthcare NHS Trust, Radiology Department, London (United Kingdom); Bhumbra, Rej [Newham and The Royal London Hospitals, Trauma and Orthopaedic Department, Barts Health Orthopaedic Centre, London, Whitechapel (United Kingdom); Pollock, Rob [Royal National Orthopaedic Hospital, Trauma and Orthopaedic Department, Stanmore (United Kingdom); Sandhu, Ranbir [University Hospitals Birmingham, Radiology Department, Queen Elizabeth Hospital, Birmingham (United Kingdom); Saifuddin, Asif [Royal National Orthopaedic Hospital, Radiology Department, Stanmore (United Kingdom)

2017-12-15

Soft tissue sarcomas (STS) are rare tumours that require prompt diagnosis and treatment at a specialist centre. Magnetic resonance imaging (MRI) has become the modality of choice for identification, characterisation, biopsy planning and staging of soft tissue masses. MRI enables both the operating surgeon and patient to be optimally prepared prior to surgery for the likelihood of margin-negative resection and to anticipate possible sacrifice of adjacent structures and consequent loss of function. The aim of this review is to aid the radiologist in performing and reporting MRI studies of soft tissue sarcomas, with particular reference to the requirements of the surgical oncologist. (orig.)

The modified Glasgow prognostic score in patients undergoing surgery for bone and soft tissue sarcoma.

Science.gov (United States)

Morhij, Rossel; Mahendra, Ashish; Jane, Mike; McMillan, Donald C

2017-05-01

The prognostic significance of markers of the systemic inflammatory response in patients with soft tissue and bone sarcomas remains unclear. Therefore, the present study aimed to compare the prognostic value of markers of the systemic inflammatory response in patients undergoing surgery for primary soft tissue and bone sarcoma. Patients who underwent resection of primary soft tissue/bone sarcoma between 2008 and 2012 and had pre-operative measurements of the systemic inflammatory response [C-reactive protein, albumin, white cell, neutrophil, lymphocyte and platelet counts, and the combination of C-reactive protein and albumin (mGPS)] were included in the study (n = 111). The majority of the patients were ≤50 years old (84%), were female (63%), had soft tissue sarcoma (62%), and had tumours >10 cm (52%), mostly of high grade (85%). The median follow-up of survivors was 50 months (range 34-78); 24 (21%) developed local recurrence, 35 (31%) developed distant metastases and 30 (30%) died of their cancer. On univariate analysis, tumour size (P sarcoma. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Management and prognosis of patients with high-grade soft tissue sarcomas

International Nuclear Information System (INIS)

Alvegaard, T.A.

1989-01-01

Doxorubicin is one of the most active single agents in metastatic soft tissue sarcoma. This was the rationale for the evaluation og doxorubicin in an adjuvant situation for patients with high-grade soft tissue sarcoma in a randomized, Scandinavian, multicenter joint care program, which was carried out from January 1981 to February 1986, and included 240 patients. These patients comprised the base series for the investigations of the importance of the surgical margin and radiotherapy for local tumor control; the accuracy of malignancy grading and tumor typing; prognostic factors, including DNA content; and epidemiologic risk factors. The results showed that the use of doxorubicin as adjuvant chemotherapy did not have clinical benefit in patients with high-grade soft tissue sarcoma. The overall local tumor control was high (94 %) for radically operated on extremity-located tumors due to strict classification of surgical margins. The only risk factor for local recurrence was marginal surgery without radiotherapy with a four times higher risk than after compartmental or wide surgery. Twelve percent of the operations reported as radical were classified as marginal, demonstrating the importance of reevaluation of surgical margins, and in these patients the local recurrence rate was 37 %. The 5-year metastasis-free survival for the whole patients series was 55 %, with the extremes 79 % for the patients with no or only one risk factor and 0 % for those with four or five risk factors. The five prognostic factors found could be used for selection of high-risk patients for adjuvant chemotherapy in the future. The epidemiologic study gave limited support for an association between occupational phenoxy-acid exposure and soft tissue sarcoma development. (98 refs.)

Histologic response of soft tissue sarcoma to radiation therapy

International Nuclear Information System (INIS)

Willett, C.G.; Schiller, A.L.; Suit, H.D.; Mankin, H.J.; Rosenberg, A.

1987-01-01

Twenty-seven patients with soft tissue sarcoma had preoperative radiotherapy, limb-sparing marginal surgical resection and whole-mount tumor histologic analysis. Incisional biopsy specimens before radiotherapy were reviewed for tumor type, grade, and extent of necrosis. Preoperative radiotherapy was given in either of two regimens: 13 patients received a mean total dose of 5250 cGy in one daily 180 to 200 cGy fractions and 14 patients a mean total dose of 4770 cGy in two daily fractions of 180 to 200 cGy separated by 4 hours. Twenty-one specimens had at least 80% necrosis or severely altered cells, a 3+ to 4+ response. Grade and size of the tumor appeared to be indicators of response to treatment rather than histologic type. Three of five patients (60%) with Grade 1, eight of 11 patients (73%) with Grade 2 lesions, and ten of 11 patients (91%) with Grade 3 tumors had 80% or greater necrosis or severely altered cells. For tumors 10 cm or less in greatest diameter, the 3+ to 4+ histologic response was seen in 12 of 14 patients (86%) whereas for lesions greater than 10 cm, this response was observed in nine of 13 patients (69%). For patients with Grade 2 or 3 soft tissue sarcoma, 13 of 14 patients (93%) treated with two fractions per day and two of four patients (50%) receiving one fraction per day exhibited significant response. All six patients treated twice daily for lesions greater than 10 cm had 3+ to 4+ histologic response compared to three of seven (43%) patients treated once per day. Therefore, grade and size of soft tissue sarcoma are important predictors of response to radiotherapy and preoperative twice daily radiotherapy may more likely permit the conservative surgical excision of sarcomas of borderline resectability

Amputations for extremity soft tissue sarcoma in an era of limb salvage treatment : Local control and survival

NARCIS (Netherlands)

Stevenson, Marc G; Musters, Annelie H; Geertzen, Jan H B; van Leeuwen, Barbara L; Hoekstra, Harald J; Been, Lukas B

2018-01-01

BACKGROUND: Despite multimodality limb salvage treatment (LST) for locally advanced extremity soft tissue sarcoma (ESTS), some patients still need an amputation. Indications for amputation and oncological outcome for these patients are described. METHODS: Between 1996 and 2016, all patients who

Treatment Outcome Following Transarterial Chemoembolization in Advanced Bone and Soft Tissue Sarcomas

Energy Technology Data Exchange (ETDEWEB)

Jiang, Chunyu; Wang, Jianbo, E-mail: a602131499@163.com [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Radiology (China); Wang, Yonggang [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Oncology (China); Zhao, Jungong; Zhu, Yueqi; Ma, Xu; Zhou, Jia [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of Radiology (China); Yan, Xuebing [Shanghai Jiaotong University Affiliated Sixth People’s Hospital, Department of General Surgery (China)

2016-10-15

PurposeTransarterial chemoembolization (TACE) is used to treat unresectable bone and soft tissue sarcoma (STS) and as a pre-surgical adjuvant treatment. However, its efficiency for advanced STS is undetermined. This study evaluated TACE’s efficiency in treating advanced STS and prognostic factors for patient survival.Materials and MethodsWe enrolled 39 patients with unresectable STS who underwent TACE as an alternative treatment during 2010–2014, with overall survival (OS) as the primary end point. Cancer pain was evaluated by visual analogue scores (VAS) before and after TACE procedures. Factors that affect survival were evaluated by multivariate analyses (Cox proportional hazard model).ResultsMean OS after TACE was 23.7 ± 2.1 months, with 1-year OS 71.5 %, 2-year OS 45.8 %, and 3-year OS 32.5 %. Lesion number and tumor stage were key predictors of survival. TACE was found to decrease cancer pain VAS and increase relapse interval. Size of polyvinyl alcohol (PVA) particle diameter (P = 0.03) and imaging response (P = 0.044) were also found to affect relapse interval.ConclusionTACE was an effective treatment for advanced STS, with a 32.5 % 3-year OS rate, and led to lower cancer pain VAS and longer relapse intervals than chemoinfusion only. Smaller PVA particles are preferable during the TACE procedure.

Peripheral soft tissue ewing's sarcoma: a rare case report

Directory of Open Access Journals (Sweden)

Farzana Shegufta

2013-07-01

Full Text Available A 22 years male patient presented with gradual left forearm swelling for 6 months. X ray forearm revealed large soft tissue swelling with tiny calcification and mild scalloping at inner aspect of ulna and ultrasonogram (USG revealed soft tissue mass having calcification and necrotic areas within and spectral Doppler showed arterial type of blood flow with no augmentation. Later computerized tomography (CT scan showed soft tissue mass with necrotic area and calcification with no bony involvement. Magnetic resonance imaging (MRI with contrast revealed a large heterogeneously enhancing lobulated mixed intensity lesion in antero-medial compartment of the left forearm involving flexor group of muscles causing displacement of fat plane. MRI and subsequent histopathology of the lesion revealed it as a rare soft tissue Ewing’s sarcoma / primitive neuroectodermal tumor (PNET in extremity. Ibrahim Med. Coll. J. 2013; 7(2: 43-46

Endosialin and Associated Protein Expression in Soft Tissue Sarcomas: A Potential Target for Anti-Endosialin Therapeutic Strategies

Directory of Open Access Journals (Sweden)

Daniel J. O’Shannessy

2016-01-01

Full Text Available Endosialin (CD248, TEM-1 is expressed in pericytes, tumor vasculature, tumor fibroblasts, and some tumor cells, including sarcomas, with limited normal tissue expression, and appears to play a key role in tumor-stromal interactions, including angiogenesis. Monoclonal antibodies targeting endosialin have entered clinical trials, including soft tissue sarcomas. We evaluated a cohort of 94 soft tissue sarcoma samples to assess the correlation between gene expression and protein expression by immunohistochemistry for endosialin and PDGFR-β, a reported interacting protein, across available diagnoses. Correlations between the expression of endosialin and 13 other genes of interest were also examined. Within cohorts of soft tissue diagnoses assembled by tissue type (liposarcoma, leiomyosarcoma, undifferentiated sarcoma, and other, endosialin expression was significantly correlated with a better outcome. Endosialin expression was highest in liposarcomas and lowest in leiomyosarcomas. A robust correlation between protein and gene expression data for both endosialin and PDGFR-β was observed. Endosialin expression positively correlated with PDGFR-β and heparin sulphate proteoglycan 2 and negatively correlated with carbonic anhydrase IX. Endosialin likely interacts with a network of extracellular and hypoxia activated proteins in sarcomas and other tumor types. Since expression does vary across histologic groups, endosialin may represent a selective target in soft tissue sarcomas.

The Edinburgh experience of treating sarcomas of soft tissues and bone with neutron irradiation

International Nuclear Information System (INIS)

Duncan, W.; Arnott, S.J.; Jack, W.J.L.

1986-01-01

The experience of treating 30 patients with sarcomas of soft tissue and bone with d(15)+Be neutron irradiation is reported. The local control of measurable soft-tissue sarcomas was 38.5% (minimum follow-up 2 years), which is similar to that expected after photon therapy. The radiation morbidity was unacceptably high (50%). Bone tumours did not respond well; in only one out of nine was lasting local tumour control achieved. (author)

Developments in the management of advanced soft-tissue sarcoma – olaratumab in context

Directory of Open Access Journals (Sweden)

Moroncini G

2018-02-01

Full Text Available Gianluca Moroncini,1,* Elena Maccaroni,2,* Ilaria Fiordoliva,2 Chiara Pellei,2 Armando Gabrielli,1 Rossana Berardi2 1Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy; 2Medical Oncology Unit, Università Politecnica delle Marche, Azienda Ospedaliero-Universitaria Ospedali Riuniti Umberto I, GM Lancisi, G Salesi, Ancona, Italy *These authors contributed equally to this work Abstract: Lartruvo® (olaratumab is a fully human immunoglobulin G subclass 1 (IgG1 monoclonal antibody that inhibits platelet-derived growth factor receptor alpha (PDGFRα. The antitumor activity of olaratumab has been tested in vitro and in vivo, and inhibition of tumor growth has been observed in cancer cell lines, including glioblastoma and leiomyosarcoma cells. It represents the first-in-class antibody to be approved by regulatory authorities for the treatment of advanced soft-tissue sarcomas (STSs in combination with doxorubicin, based on the results of the Phase Ib/II trial by Tap et al. The median progression-free survival (PFS, which was the primary end point of the study, was improved for patients treated with olaratumab plus doxorubicin compared to those treated with doxorubicin monotherapy (6.6 vs 4.1 months, respectively; HR 0.672, 95% CI 0.442–1.021, p=0.0615. Moreover, final analysis of overall survival (OS showed a median OS of 26.5 months with olaratumab plus doxorubicin vs 14.7 months with doxorubicin, with a gain of 11.8 months (HR 0.46, 95% CI 0.30–0.71, p=0.0003. In October 2016, olaratumab was admitted in the Accelerated Approval Program by the US Food and Drug Administration (FDA for use in combination with doxorubicin for the treatment of adult patients with STSs. In November 2016, the European Medicines Agency (EMA granted conditional approval for olaratumab in the same indication under its Accelerated Assessment Program. A double-blind, placebo-controlled, randomized Phase III study (ANNOUNCE

High-grade soft tissue sarcoma arising in a desmoid tumor: case report and review of the literature.

Science.gov (United States)

Bertucci, François; Faure, Marjorie; Ghigna, Maria-Rosa; Chetaille, Bruno; Guiramand, Jérôme; Moureau-Zabotto, Laurence; Sarran, Anthony; Perrot, Delphine

2015-01-01

Desmoid tumors are rare benign monoclonal fibroblastic tumors. Their aggressiveness is local with no potential for metastasis or dedifferentiation. Here we report on a 61-year-old patient who presented a locally advanced breast desmoid tumor diagnosed 20 years after post-operative radiotherapy for breast carcinoma. After 2 years of medical treatment, a high-grade undifferentiated pleomorphic soft tissue sarcoma arose within the desmoid tumor. Despite extensive surgery removing both tumors, the patient showed locoregional relapse by the sarcoma, followed by multimetastatic progression, then death 25 months after the surgery. The arising of a soft tissue sarcoma in a desmoid tumor is an exceptional event since our case is the fourth one reported so far in literature. It reinforces the need for timely and accurate diagnosis when a new mass develops in the region of a preexisting desmoid tumor, and more generally when a desmoid tumor modifies its clinical or radiological aspect.

Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma

International Nuclear Information System (INIS)

Roberge, David; Skamene, Tanya; Nahal, Ayoub; Turcotte, Robert E.; Powell, Tom; Freeman, Carolyn

2010-01-01

Purpose: To report radiological and pathological response to neo-adjuvant radiotherapy for extremity and trunk soft-tissue sarcomas. Materials/methods: Fifty patients were identified retrospectively. All patients had MRI imaging pre and post neo-adjuvant external beam radiotherapy. Tumor volumes were measured in 3D on T1 Gadolinium enhanced sequences. Pathological treatment response was quantified in terms of percentage of treatment-related necrosis for each case. Results: Histopathologic responses to treatment varied from 0% to 100%. The median pathological treatment response was 67.5% for low-grade sarcomas and 50% for high-grade sarcomas. The median decrease in tumor volume was 13.8% for non-myxoid low-grade sarcomas, 82.1% for myxoid liposarcomas and <1% for high-grade sarcomas. A partial response on MRI (volume reduction ≥ 50%) was highly predictive of a good pathological response (p < 0.001). Patients with stable disease on imaging or volumetric progression had wide ranging pathological responses. Conclusions: Soft-tissue sarcomas show significant pathological treatment responses in the form of hyaline fibrosis, necrosis and granulation tissue. Despite this, there is minimal early volumetric response to radiation, especially for high-grade tumors. Although radiological partial response was predictive of pathological response, the significance of radiological progression was unclear. Myxoid liposarcoma tumor type was predictive of both pathological and radiological tumor response.

MIB-1 expression and iododeoxyuridine labelling in soft tissue sarcomas

DEFF Research Database (Denmark)

Jensen, V; Høyer, M; Sørensen, Flemming Brandt

1996-01-01

We investigated the relationship between immunohistochemical estimates of proliferative activity and expression of bcl-2 protein and mutant p53 protein in 23 cases of soft tissue sarcoma. Furthermore, the reproducibility of estimates of proliferative activity was analysed and correlations between...

Treatment of Vascular Soft Tissue Sarcomas With Razoxane, Vindesine, and Radiation

International Nuclear Information System (INIS)

Rhomberg, Walter; Wink, Anna; Pokrajac, Boris; Eiter, Helmut; Hackl, Arnulf; Pakisch, Brigitte; Ginestet, Angela; Lukas, Peter; Poetter, Richard Prof.

2009-01-01

Purpose: In previous studies, razoxane and vindesine together with radiotherapy was proved to be effective in soft tissue sarcomas (STS). Because razoxane leads to a redifferentiation of pathological tumor blood vessels, it was of particular interest to study the influence of this drug combination in vascular soft tissue sarcomas. Methods and Materials: This open multicenter Phase II study was performed by the Austrian Society of Radiooncology. Among 13 evaluable patients (10 angiosarcomas and 3 hemangio-pericytomas), 9 had unresectable measurable disease, 3 showed microscopic residuals, and 1 had a resection with clear margins. They received a basic treatment with razoxane and vindesine supported by radiation therapy. Outcome measures were objective response rates, survival time, and the incidence of distant metastases. Results: In nine patients with measurable vascular soft tissue sarcomas (eight angiosarcomas and one hemangiopericytoma), 6 complete remissions, 2 partial remissions, and 1 minor remission were achieved, corresponding to a major response rate of 89%. A maintenance therapy with razoxane and vindesine of 1 year or longer led to a suppression of distant metastases. The median survival time from the start of the treatment is 23+ months (range, 3-120+) for 12 patients with macroscopic and microscopic residual disease. The progression-free survival at 6 months was 75%. The combined treatment was associated with a low general toxicity, but attention must be given to increased normal tissue reactions. Conclusions: This trimodal treatment leads to excellent response rates, and it suppresses distant metastases when given as maintenance therapy.

Adult Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

Science.gov (United States)

Soft tissue sarcomas (STS) arise in any of the extremities, trunk, retroperitoneum, or head and neck. Treatment is determined by the tumor grade and options include surgery, radiation therapy, and chemotherapy. Get comprehensive information for STS and treatment in this clinician summary.

Trabectedin for Soft Tissue Sarcoma: Current Status and Future Perspectives.

Science.gov (United States)

Gordon, Erlinda M; Sankhala, K Kumar; Chawla, Neal; Chawla, Sant P

2016-07-01

Trabectedin (ET743, Yondelis(®), manufactured by Baxter Oncology GmbH, Halle/Westfalen, Germany, for Janssen Products, LP, Horsham, PA), derived from the marine ascidian, Ecteinascidia turbinata, is a natural alkaloid with multiple complex mechanisms of action. On 23 October 2015, 15 years after the results of the first Phase 1 clinical trial using trabectedin for chemotherapy-resistant solid malignancies was reported, and 8 years after its approval in Europe, the United States Food and Drug Administration (USFDA) finally approved trabectedin for the treatment of unresectable or metastatic liposarcoma or leiomyosarcoma that has failed a prior anthracycline-containing regimen. Approval was based on the results of a pivotal Phase 3 trial involving a 2:1 randomization of 518 patients (who were further stratified by soft tissue sarcoma subtype), in which a significant improvement in progression-free survival was reported in the trabectedin-treated group vs. the dacarbazine-treated group (p concept. In short, trabectedin is an old new drug with proven potential to impact the lives of patients with soft tissue sarcoma and other solid malignancies. Sarcoma Oncology Center, Santa Monica, CA 90405.

Soft Tissue Sarcoma—Health Professional Version

Science.gov (United States)

Soft tissue sarcomas are malignant tumors that arise in any of the mesodermal tissues of the extremities, trunk and retroperitoneum, or head and neck. Soft tissue sarcomas may be heterogeneous. Find evidence-based information on soft tissue sarcoma treatment and research.

Soft tissue sarcomas in the precision medicine era: new advances in clinical practice and future perspectives.

Science.gov (United States)

Badalamenti, Giuseppe; Messina, Carlo; De Luca, Ida; Musso, Emmanuela; Casarin, Alessandra; Incorvaia, Lorena

2018-04-04

Soft tissue sarcomas (STSs) represent a rare and heterogeneous group of solid tumours derived from mesenchymal progenitors and account for 1% of all adult malignancies. Although in the last decade anthracycline-based chemotherapy single agent or in combinations has been able to improve clinical benefits, prognosis is still poor and STSs represent an important unmet medical need. Continuous advances in cancer genetics and genomics have contributed to change management paradigms of STSs as it occurred for other solid tumours. Several treatments have been recently developed with the specific aim of targeting different cell pathways and immune-checkpoints that have been recognized to drive tumour progression. The following attempts to provide a review of literature focusing on the available data concerning novel treatments and future prospective for the management of metastatic STSs.

Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology.

Science.gov (United States)

von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Conrad, Ernest U; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Schuetze, Scott; Schupak, Karen D; Schwartz, Herbert S; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian

2016-06-01

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy. Copyright © 2016 by the National Comprehensive Cancer Network.

Effectiveness of Vascular Markers (Immunohistochemical Stains) in Soft Tissue Sarcomas.

Science.gov (United States)

Naeem, Namra; Mushtaq, Sajid; Akhter, Noreen; Hussain, Mudassar; Hassan, Usman

2018-05-01

To ascertain the effectiveness of IHC markers of vascular origin like CD31, CD34, FLI1 and ERG in vascular soft tissue sarcomas including angiosarcomas, Kaposi sarcomas, epithelioid hemangioendothelioma and a non-vascular soft tissue sarcoma (Epithelioid sarcoma). Descriptive study. Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from 2011 to 2017. Diagnosed cases of angiosarcomas (n=48), epithelioid hemangioendothelioma (n=9), Kaposi sarcoma (n=9) and epithelioid sarcoma (n=20) were selected. Immunohistochemical staining as performed on formalin fixed paraffin embedded sections. The sections were stained for the following markers: CD34 (VENTANA clone Q Bend 10), CD31 (Leica clone 1 A 10), FLI1 (CELL MARQUE clone MRQ-1) and ERG (CELL MARQUE clone EP111). A complete panel of CD34, CD31 and ERG was applied on 8/48 cases of angiosarcomas with triple positivity in 6 cases. Eight cases showed positivity for only CD31 and ERG and 2 cases showed positivity for only ERG. A complete panel of CD34, CD31 and ERG was applied on 3/9 cases of epithelioid hemangioendothelioma with positivity for all markers in 2 cases. Combined positivity for ERG and CD34 was seen in 2 cases and on 4 cases only CD31 immunohistochemical was solely applied with 100% positivity. FLI1 was not applied on any case. Among 9 cases of Kaposi sarcoma, ERG, CD34 and CD31 in combination were applied on only 1 case with triple positivity. Remaining cases show positivity for either CD34, CD31 or FLI1. Majority of cases of epithelioid sarcomas were diagnosed on the basis of cytokeratin and CD34 positivity with loss of INI1. The other vascular markers showed negativity in all cases. Among these four markers, ERG immunohistochemical stain is highly effective for endothelial differentiation due to its specific nuclear staining pattern in normal blood vessel endothelial cells (internal control) as well as neoplastic cells of vascular tumors and lack of background staining.

Preoperative Intensity-Modulated Radiotherapy Combined with Temozolomide for Locally Advanced Soft-Tissue Sarcoma

International Nuclear Information System (INIS)

Jakob, Jens; Wenz, Frederik; Dinter, Dietmar J.; Stroebel, Philipp; Hohenberger, Peter

2009-01-01

Purpose: To evaluate the toxicity and efficacy of preoperative intensity-modulated radiotherapy (IMRT) combined with temozolomide to improve local tumor control in soft-tissue sarcoma (STS). Patients and Methods: A cohort of 15 consecutive patients with nonmetastasized, primary high-grade or locally recurrent Stage III (n = 14) or IIb (n = 1) STS not amenable to surgical resection without significant organ or extremity function loss was prospectively investigated. Median tumor size was 9.8 cm, and most tumors were non-extremity sarcomas. Patients preoperatively received 50 mg/m 2 of temozolomide during IMRT (50.4 Gy). Resection was intended 6 weeks thereafter. Toxicity was assessed by the National Cancer Institute Common Terminology Criteria for Adverse Events version 3.0, and response was assessed by Response Evaluation Criteria in Solid Tumors. Results: Of 15 patients, 14 completed preoperative treatment. No Grade 4 toxicities occurred. Nausea and vomiting were the most frequent Grade 3 toxicities. The most frequent toxicities of any grade were dermatologic, gastrointestinal, and hematologic. Response was partial response in 5, stable disease in 7, and progressive disease in 2 patients. Ten patients underwent surgery: 7 were resected with clear margins (R0), and 2 patients had an R1 resection; in 1 patient the tumor was not resectable. Postoperative complications occurred in 4 patients. Five patients did not undergo surgery because of intercurrent metastatic disease, unresectable disease, or refusal. Conclusions: Preoperative chemoradiation with temozolomide and IMRT can be administered safely and with promising efficacy in patients with locally advanced STS.

Adjuvant Radiotherapy for Pediatric and Young Adult Nonrhabdomyosarcoma Soft-Tissue Sarcoma

International Nuclear Information System (INIS)

Smith, Kristy B.; Indelicato, Daniel J.; Knapik, Jacquelyn A.; Lagmay, Joanne P.; Morris, Christopher; Kirwan, Jessica M.; Zlotecki, Robert A.; Scarborough, Mark T.; Gibbs, C. Parker; Marcus, Robert B.

2011-01-01

Purpose: To evaluate the prognostic factors, outcomes, and complications in patients aged ≤30 years with resectable nonrhabdomyosarcoma soft-tissue sarcoma treated at the University of Florida with radiotherapy (RT) during a 34-year period. Methods and Materials: A total of 95 pediatric or young adult patients with nonrhabdomyosarcoma soft-tissue sarcoma were treated with curative intent with surgery and RT at the University of Florida between 1973 and 2007. The most common histologic tumor subtypes were synovial sarcoma in 22 patients, malignant fibrous histiocytoma in 19, and malignant peripheral nerve sheath tumor in 11 patients. The mean age at RT was 22 years (range, 6-30). Of the 95 patients, 73 had high-grade tumors; 45 had undergone preoperative RT and 50 postoperative RT. The prognostic factors for survival, local recurrence, and distant recurrence were analyzed. Results: The median follow-up was 7.2 years (range, 0.4-30.5). The actuarial 5-year local control rate was 88%. A microscopically negative margin was associated with superior local control. Although 83% of local recurrence cases initially developed in the absence of metastases, all patients with local failure ultimately died of their disease. The actuarial estimate of 5-year overall survival and disease-free survival was 65% and 63%, respectively. Of all the deaths, 92% were disease related. An early American Joint Committee on Cancer stage, tumor <8 cm, and the absence of neurovascular invasion were associated with superior disease-free survival. The National Cancer Institute Common Toxicity Criteria, version 3, Grade 3-4 treatment complication rate was 9%. No secondary malignancies were observed. Conclusion: In the present large single-institution study, we found positive margins and locally advanced features to be poor prognostic factors for both local progression and survival. The results from the present study have helped to characterize the therapeutic ratio of RT in pediatric and young

High dose rate brachytherapy for the treatment of soft tissue sarcoma of the extremity

International Nuclear Information System (INIS)

Speight, J.L.; Streeter, O.E.; Chawla, S.; Menendez, L.E.

1996-01-01

Purpose: we examined the role of preoperative neoadjuvant chemoradiation and adjuvant high-dose rate brachytherapy on the management of prognostically unfavorable soft tissue sarcomas of the extremities. Our goal was to examine the effect of high dose rate interstitial brachytherapy (HDR IBT) on reducing the risk of local recurrence following limb-sparing resection, as well as shortening treatment duration. Materials and methods: eleven patients, ranging in age from 31 to 73 years old, with soft tissue sarcoma of the extremity were treated at USC/Norris Comprehensive Cancer Center during 1994 and 1995. All patients had biopsy proven soft tissue sarcoma, and all were suitable candidates for limb-sparing surgery. All lesions were greater than 5cm in size and were primarily high grade. Tumor histologies included malignant fibrous histiocytoma (45%), liposarcoma (18%) and leiomyosarcoma, synovial cell sarcoma and spindle cell sarcoma (36%). Sites of tumor origin were the lower extremity (55%), upper extremity (18%) and buttock (9%), 1 patient (9%) had lesions in both the upper and lower extremity. Patients received HDR IBT following combined chemotherapy and external beam irradiation (EBRT) and en bloc resection of the sarcoma. Neoadjuvant chemotherapy consisted of three to four cycles of either Ifosfamide/Mesna with or without Adriamycin, or Mesna, Adriamycin, Ifosfamide and Dacarbazine. One patient received Cis-platin in addition to Ifos/Adr. A minimum of two cycles of chemotherapy were administered prior to EBRT. Additional cycles of chemotherapy were completed concurrently with EBRT but prior to HDR IBT. Preoperative EBRT doses ranging from 40 to 59.4 Gy were given in daily fractions of 180 to 200cGy. Following en bloc resection, HDR IBT was administered using the Omnitron tm 2000 remote afterloading system. Doses ranging from 13 to 30 Gy were delivered to the surgical tumor bed at depths of 0.5mm to 0.75mm from the radioactive source. Results: median follow-up was

Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy

International Nuclear Information System (INIS)

Kuten, A.; Sapir, D.; Cohen, Y.; Haim, N.; Borovik, R.; Robinson, E.

1985-01-01

Seven cases of soft tissue sarcoma developing after primary or postoperative radiotherapy for breast carcinoma are reported. The sarcomas occurred within the irradiated volume, after a latent period of 4-26 years. These cases conform well to established criteria for the diagnosis of radiation-induced sarcoma. Chemotherapy, consisting of the four-drug combination CYVADIC (cyclophosphamide, vincristine, adriamycin, DTIC) was employed in six of the seven patients. Only two of them achieved partial remission, lasting only 2 and 3 months, respectively. The effectiveness of adriamycin-containing chemotherapy regimens in soft tissue sarcomas as well as the remote hazard of radiation-related sarcoma in primary or postoperative breast irradiation are discussed

PET/MRI for Preoperative Planning in Patients with Soft Tissue Sarcoma

DEFF Research Database (Denmark)

Loft Jakobsen, Annika; Jensen, Karl Erik; L�fgren, Johan

2013-01-01

Clinical positron emission tomography (PET)/magnetic resonance imaging (MRI) acquisition protocols may improve the evaluation of soft tissue sarcomas (STS) prior to surgical planning. We examined two patients with lower extremity STS using a Siemens Biograph mMR PET/MRI scanner and the glucose...

Primary soft tissue Ewing's sarcoma of the maxillary sinus in elderly patients: presentation, management and prognosis.

Science.gov (United States)

Dutta, M; Ghatak, S; Biswas, G; Sen, A

2014-06-01

Nonosseous or soft tissue Ewing's sarcoma is a rare form of Ewing's sarcoma/primitive neuroectodermal tumour that seldom affects the head and neck region. Involvement of the nose and paranasal sinuses is extremely uncommon, with only eight of such patients being reported to date, mostly affecting adolescents and young adults. To our knowledge, this study is the first comprehensive report of primary soft tissue Ewing's sarcoma involving the paranasal sinuses in an elderly patient who successfully completed treatment. We herein discuss the pathogenesis, management and factors affecting the prognosis of this rare group of tumours involving the nose and paranasal sinuses, in relation to the available literature.

The Diagnostic and Prognostic Value of Hematological and Chemical Abnormalities in Soft Tissue Sarcoma: A Comparative Study in Patients with Benign and Malignant Soft Tissue Tumors.

Science.gov (United States)

Ariizumi, Takashi; Kawashima, Hiroyuki; Ogose, Akira; Sasaki, Taro; Hotta, Tetsuo; Hatano, Hiroshi; Morita, Tetsuro; Endo, Naoto

2018-01-01

The value of routine blood tests in malignant soft tissue tumors remains uncertain. To determine if these tests can be used for screening, the routine pretreatment blood test findings were retrospectively investigated in 359 patients with benign and malignant soft tissue tumors. Additionally, the prognostic potential of pretreatment blood abnormalities was evaluated in patients with soft tissue sarcomas. We compared clinical factors and blood tests findings between patients with benign and malignant soft tissue tumors using univariate and multivariate analysis. Subsequently, patients with malignant tumors were divided into two groups based on blood test reference values, and the prognostic significance of each parameter was evaluated. In the univariate analysis, age, tumor size, and tumor depth were significant clinical diagnostic factors. Significant increases in the granulocyte count, C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), and γ-glutamyl transpeptidase (γ-GTP) levels were found in patients with malignant soft tissue tumors. Multiple logistic regression showed that tumor size and ESR were independent factors that predicted malignant soft tissue tumors. The Kaplan-Meier survival analysis revealed that granulocyte counts, γ-GTP levels, and CRP levels correlated significantly with overall survival. Thus, pretreatment routine blood tests are useful diagnostic and prognostic markers for diagnosing soft tissue sarcoma. © 2018 by the Association of Clinical Scientists, Inc.

Singapore Cancer Network (SCAN) Guidelines for the Initial Evaluation, Diagnosis, and Management of Extremity Soft Tissue Sarcoma and Osteosarcoma.

Science.gov (United States)

2015-10-01

The SCAN sarcoma workgroup aimed to develop Singapore Cancer Network (SCAN) clinical practice guidelines for the initial evaluation, diagnosis, and management of extremity soft tissue sarcoma and osteosarcoma. The workgroup utilised a consensus approach to create high quality evidence-based clinical practice guidelines suited for our local setting. Various international guidelines from the fields of radiology, pathology, orthopaedic surgery, medical, radiation and paediatric oncology were reviewed, including those developed by von Mehren Metal (J Natl Compr Canc Netw 2014), the National Collaborating Centre for Cancer (2006), the European Sarcoma Network Working Group (2012) and Grimer RJ et al (Sarcoma 2008). Our clinical practice guidelines contextualised to the local patient will streamline care and improve clinical outcomes for patients with extremity soft tissue and osteosarcoma. These guidelines form the SCAN Guidelines 2015 for the initial evaluation, diagnosis, and management of extremity soft tissue sarcoma and osteosarcoma.

Soft-tissue sarcomas. Current aspects of diagnosis and therapy

International Nuclear Information System (INIS)

Hohenberger, P.

1996-01-01

The decisive factor for promising therapy of soft-tissue sarcomas is primary therapy provided in an experienced tumour unit. This centre must offer the entire spectrum of surgery (vascular, reconstruction and orthopaedic surgery) leading into an interdisciplinary treatment regimen. Initially, MRI would appear to be sufficient for diagnosis. On the other hand, the presence of an experienced pathologist conversant with all means of cytogenetic tumour classification is essential. For interdisciplinary therapy, a radiotherapist with hyperthermia equipment, nuclear medicine specialists and medical oncologists are indispensable. All personnel must be familiar with the special problems associated with sarcomas. The current trend is indeed toward a select number of centres where such skills are focused. (orig.) [de

Can MRI diffusion-weighted imaging identify postoperative residual/recurrent soft-tissue sarcomas?

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Mai Maher ElDaly

2018-01-01

Full Text Available Purpose: The aim of this study was to evaluate contrast-enhanced magnetic resonance imaging (CE-MRI and quantitative diffusion-weighted imaging (DWI with apparent diffusion coefficient (ADC mapping in the detection of recurrent/residual postoperative soft tissue sarcomas. Materials and Methods: This study included 36 patients; 27 patients had postoperative recurrent/residual soft tissue sarcomas and 9 patients had postoperative and treatment-related changes (inflammation/fibrosis. The DWI was obtained with 3 b values including 0, 400, and 800 s/mm2. Calculation of the ADC value of the lesion was done via placing the region of interest (ROI to include the largest area of the lesion. ADC values were compared to histopathology. Results: Our results showed that including CE-MRI improved the diagnostic accuracy and sensitivity in recurrence detection compared to conventional non-enhanced sequences. However, it showed low specificity (55.56% with a high false-positive rate that may lead to an unnecessary biopsy of a mass such as region of postoperative scar tissue. Conclusion: The joint use of gadolinium-enhanced MRI and quantitative DWI with ADC mapping offer added value in the detection of recurrent/residual postoperative soft tissue sarcoma. This combined use increased both the diagnostic sensitivity and specificity with a cut-off average ADC value for detecting nonmyxoid recurrent/residual lesions ≤1.3 × 10−3 mm2/s (100% specificity and 90.48% sensitivity. Our results showed limited value of DWI with ADC mapping in assessing myxoid sarcomatous tumor recurrences.

Role of radiation therapy in management of patients with sarcoma of soft tissue

International Nuclear Information System (INIS)

Spiro, Ira J.

1996-01-01

Soft tissue sarcomas (STS) are relatively rare malignant neoplasms arising from the mesenchymal connective tissues. There are some 5600 newly diagnosed patients with STS per year. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. Etiologic factors, including occupational risks, the role of environmental carcinogens, radiation and genetic diseases in the development of these tumors will be mode. The molecular biology of soft tissue sarcomas including the role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) will be reviewed. Cytogenetic alternations with an emphasis on molecular diagnostic techniques will be reviewed. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of various imaging modalities. The timing and type of biopsy (including FNA, core needle biopsy, incisional biopsy or excisional biopsy) for tumors at various sites and sizes will be addressed. Assessment of histopathologic subtype of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of electron beam techniques, or 3) post-operative brachytherapy. Results of these various treatment options with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and

Role of radiation therapy in management of patients with sarcoma of soft tissue

International Nuclear Information System (INIS)

Spiro, Ira J.; Suit, Herman D.

1997-01-01

Soft tissue sarcomas (STS) are relatively rare malignant neoplasms arising from the mesenchymal connective tissues. There are some 5600 newly diagnosed patients with STS per year. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. Etiologic factors, including occupational risks, the role of environmental carcinogens, radiation and genetic diseases in the development of these tumors will be made. The molecular biology of soft tissue sarcomas including the role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) will be reviewed. Cytogenetic alternations with an emphasis on molecular diagnostic techniques will be reviewed. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of various imaging modalities. The timing and type of biopsy (including FNA, core needle biopsy, incisional biopsy or excisional biopsy) for tumors at various sites and sizes will be addressed. Assessment of histopathologic subtype of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of electron beam techniques, or 3) post-operative brachytherapy. Results of these various treatment options with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and

Ether à go-go potassium channel expression in soft tissue sarcoma patients

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Stühmer Walter

2006-10-01

Full Text Available Abstract Background The expression of the human Eag1 potassium channel (Kv10.1 is normally restricted to the adult brain, but it has been detected in both tumour cell lines and primary tumours. Our purpose was to determine the frequency of expression of Eag1 in soft tissue sarcoma and its potential clinical implications. Results We used specific monoclonal antibodies to determine the expression levels of Eag1 in soft tissue sarcomas from 210 patients by immunohistochemistry. Eag1 was expressed in 71% of all tumours, with frequencies ranging from 56% (liposarcoma to 82% (rhabdomyosarcoma. We detected differences in expression levels depending on the histological type, but no association was seen between expression of this protein and sex, age, grade or tumour size. Four cell lines derived from relevant sarcoma histological types (fibrosarcoma and rhabdomyosarcoma were tested for Eag1 expression by real-time RT-PCR. We found all four lines to be positive for Eag1. In these cell lines, blockage of Eag1 by RNA interference led to a decrease in proliferation. Conclusion Eag1 is aberrantly expressed in over 70% sarcomas. In sarcoma cell lines, inhibition of Eag1 expression and/or function leads to reduced proliferation. The high frequency of expression of Eag1 in primary tumours and the restriction of normal expression of the channel to the brain, suggests the application of this protein for diagnostic or therapeutic purposes.

Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

Science.gov (United States)

Messiou, C; Moskovic, E; Vanel, D; Morosi, C; Benchimol, R; Strauss, D; Miah, A; Douis, H; van Houdt, W; Bonvalot, S

2017-07-01

Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

MINI REVIEW ARTICLE:Immunohistochemistry of Epithelioid Soft Tissue Sarcomas, Literature Review Based on Case Studies

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Megha Joshi

2012-07-01

Full Text Available Neoplasms with epithelioid histology may be diagnostically challenging. Immunohisto chemistry (IHC can aid in confirming thedifferential diagnosis of mesotheliomas, melanomas, lymphomas, and soft tissue sarcomas, all tumors that can present with an epithelioid histology. Immunohistochemistry can also assist in confirming the type of sarcomas. Using cases diagnosed in acommunity hospital setting over a ten year period, the use of IHC in sarcomas will be illustrated.

Role of radiation therapy in management of patients with sarcoma of soft tissue

International Nuclear Information System (INIS)

Suit, Herman D.; Spiro, Ira J.

1995-01-01

Soft tissue sarcomas (STS) are malignant neoplasms arising from the mesenchymal connective and supporting tissues. These tumors occur at all anatomic sites within the body and are of many histologic subtypes. There are some 5600 newly diagnosed patients with STS per year. Epidemiological and etiologic factors, including the role of environmental carcinogens and radiation in the development of these tumors will be made. The role of several oncogenes and suppressor genes (e.g. Rb, p53, MDM2) and cytogenetic alterations will be reviewed. Consideration of the epidemiology and role of environmental carcinogens and radiation in the development of these tumors will be made. The natural history of these tumors will be described with reference to local invasion and spread to regional and distal sites. The evaluation of the patients suspected of having a sarcoma of soft tissue will then be considered including the relative roles of CT, MRI, PET, and US. The place of core needle biopsy, incisional biopsy or excisional biopsy for tumors at various sites and sizes will be addressed. The histopathologic subtype assessment of the tumor by standard H and E stains, immunohistochemistry, electron microscopy and cytogenetic studies will then be discussed. The principal role for radiation in the management of patients with sarcoma of soft tissue is in combination with surgery. This may be: 1) pre-operative and or post-operative use of external beam photons, electrons, and protons, and 2) intra-operative use of brachytherapy or intra-operative election beam techniques. Results of treatment with respect to local control, disease-free survival and overall survival will be considered for each of the various techniques with respect to size, grade, histologic type, surgical margin status, anatomic site, primary vs. recurrent disease. Similarly, the factors associated with delay in wound healing are to be considered and strategies to reduce wound morbidity. Functional outcome after limb

Preclinical evaluation of oncolytic vaccinia virus for therapy of canine soft tissue sarcoma.

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Ivaylo Gentschev

Full Text Available Virotherapy using oncolytic vaccinia virus (VACV strains is one promising new strategy for canine cancer therapy. In this study we describe the establishment of an in vivo model of canine soft tissue sarcoma (CSTS using the new isolated cell line STSA-1 and the analysis of the virus-mediated oncolytic and immunological effects of two different Lister VACV LIVP1.1.1 and GLV-1h68 strains against CSTS. Cell culture data demonstrated that both tested VACV strains efficiently infected and destroyed cells of the canine soft tissue sarcoma line STSA-1. In addition, in our new canine sarcoma tumor xenograft mouse model, systemic administration of LIVP1.1.1 or GLV-1h68 viruses led to significant inhibition of tumor growth compared to control mice. Furthermore, LIVP1.1.1 mediated therapy resulted in almost complete tumor regression and resulted in long-term survival of sarcoma-bearing mice. The replication of the tested VACV strains in tumor tissues led to strong oncolytic effects accompanied by an intense intratumoral infiltration of host immune cells, mainly neutrophils. These findings suggest that the direct viral oncolysis of tumor cells and the virus-dependent activation of tumor-associated host immune cells could be crucial parts of anti-tumor mechanism in STSA-1 xenografts. In summary, the data showed that both tested vaccinia virus strains and especially LIVP1.1.1 have great potential for effective treatment of CSTS.

Cytokine-induced killer cells eradicate bone and soft-tissue sarcomas.

Science.gov (United States)

Sangiolo, Dario; Mesiano, Giulia; Gammaitoni, Loretta; Leuci, Valeria; Todorovic, Maja; Giraudo, Lidia; Cammarata, Cristina; Dell'Aglio, Carmine; D'Ambrosio, Lorenzo; Pisacane, Alberto; Sarotto, Ivana; Miano, Sara; Ferrero, Ivana; Carnevale-Schianca, Fabrizio; Pignochino, Ymera; Sassi, Francesco; Bertotti, Andrea; Piacibello, Wanda; Fagioli, Franca; Aglietta, Massimo; Grignani, Giovanni

2014-01-01

Unresectable metastatic bone sarcoma and soft-tissue sarcomas (STS) are incurable due to the inability to eradicate chemoresistant cancer stem-like cells (sCSC) that are likely responsible for relapses and drug resistance. In this study, we investigated the preclinical activity of patient-derived cytokine-induced killer (CIK) cells against autologous bone sarcoma and STS, including against putative sCSCs. Tumor killing was evaluated both in vitro and within an immunodeficient mouse model of autologous sarcoma. To identify putative sCSCs, autologous bone sarcoma and STS cells were engineered with a CSC detector vector encoding eGFP under the control of the human promoter for OCT4, a stem cell gene activated in putative sCSCs. Using CIK cells expanded from 21 patients, we found that CIK cells efficiently killed allogeneic and autologous sarcoma cells in vitro. Intravenous infusion of CIK cells delayed autologous tumor growth in immunodeficient mice. Further in vivo analyses established that CIK cells could infiltrate tumors and that tumor growth inhibition occurred without an enrichment of sCSCs relative to control-treated animals. These results provide preclinical proof-of-concept for an effective strategy to attack autologous sarcomas, including putative sCSCs, supporting the clinical development of CIK cells as a novel class of immunotherapy for use in settings of untreatable metastatic disease.

Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

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Ansari Tayyaba Z

2011-05-01

Full Text Available Abstract Background Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. Methods This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. Results Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02 and surgical margin status (p = 0.001 were statistically significant for determination of relapse of disease. Conclusion The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

Indian data on bone and soft tissue sarcomas: A summary of published study results

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Anant Ramaswamy

2016-01-01

Full Text Available Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS, chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.

Expression of neural cell adhesion molecules and neurofilament protein isoforms in Ewing's sarcoma of bone and soft tissue sarcomas of other than rhabdomyosarcoma

NARCIS (Netherlands)

Molenaar, W.M.; Muntinghe, F.L.H.

1999-01-01

In a previous study, it was shown that rhabdomyosarcomas widely express "neural" markers, such as neural cell adhesion molecules (N-CAM) and neurofilament protein isoforms, In the current study, a series of Ewing's sarcomas of bone and soft tissue sarcomas other than rhabdomyosarcoma was probed for

Retrospective review of soft tissue sarcoma of head and neck in a ...

African Journals Online (AJOL)

Background: Soft tissue sarcomas like other malignancies, impact negatively on patients and their caregivers as well as pose a challenge to the managing physician with variable treatment outcomes. A review of related studies on Medline has shown the paucity of the literature on the disease in the West African sub-region.

The relationship between tumor oxygenation and cell proliferation in human soft tissue sarcomas

International Nuclear Information System (INIS)

Nordsmark, Marianne; Hoeyer, Morten; Keller, Johnny; Nielsen, Ole Steen; Jensen, Oluf Myhre; Overgaard, Jens

1996-01-01

Purpose: In malignant tumors the oxygenation status and tumor cell proliferation are known to influence local tumor control after radiotherapy. However, the relationship between oxygenation status and tumor cell kinetics in human tumors has not yet been described. Newly developed clinically applicable techniques such as oxygen electrode measurements and assessment of tumor cell proliferation rates have been suggested as promising predictive assays. The purpose of the present study was to characterize tumor oxygenation status in soft tissue sarcomas and to compare this with tumor cell kinetics and clinical parameters. Methods and Materials: Pretreatment tumor oxygenation status was measured by polarographic oxygen needle electrodes and evaluated as the median pO 2 and the percentage of pO 2 values ≤ 5 mmHg and ≤ 2.5 mmHg in 22 patients with primary soft tissue sarcomas. All tumors were characterized by histology, grade of malignancy, the level of microscopic necrosis, the level of effective hemoglobin, and magnetic resonance imaging estimation of tumor volume. The tumor cell potential doubling time and labeling index were measured by flow cytometric and immunohistochemical analysis of tumor biopsy specimens after in vivo incorporation of iododeoxyuridine. Results: There was a significant correlation between the median pO 2 and the tumor cell potential doubling time (p = 0.041), whereas no correlation was found between the level of hypoxia expressed by the percentage of pO 2 values ≤ 2.5 and ≤ 5 mmHg, respectively, and tumor cell potential doubling time. Furthermore, no correlation was found between either of the three tumor oxygenation parameters and labeling index. The material represented large intertumor heterogeneity in oxygenation status, cell kinetics, and tumor volume, and no correlation was found between oxygenation status and either volume, histopathology, grade of malignancy, or effective hemoglobin. Conclusion: This report is the first to suggest

Multidisciplinary ''limb salvage'' treatment of soft tissue and skeletal sarcomas

International Nuclear Information System (INIS)

Weisenburger, T.H.; Eilber, F.R.; Grant, T.T.; Morton, D.L.; Mirra, J.J.; Steinberg, M.; Rickles, D.

1984-01-01

The goal of treating primary bone and soft tissue tumors, as with most other malignancies, is to control the disease locally and systematically while preserving as much function as possible. For soft tissue sarcomas the results following radical excision and post-operative radiation therapy with preservation of the limb have equaled the control rates of amputation. However, local recurrence rates of approximately 25-30% are reported for high-grade lesions of the proximal lower extremity. Amputations provides excellent local control for osteosarcoma but the functional results may be less than optimal. In an attempt to achieve limb salvage for these tumors, a multidisciplinary protocol was developed using intra-arterial doxorubicin, pre-operative radiation therapy and limb-sparing radical wide excision followed by post-operative chemotherapy for presumed micro-metastatic disease

SOFT TISSUE SARCOMAS – A CROS S SECTIONAL STUDY AT MNJIO

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Ramesh

2015-07-01

Full Text Available BACKGROUND: Sarcomas are an uncommon group of cancers with a wide distribution in the various anatomical regions of the body and even wider and confusing range of histopathological diagnosis. AIMS: To study the mode of presentation, histology, treatment modalities use d and outcomes both in terms of function and disease free survivals of all patients treated in the Department of surgical oncology in our Institute. MATERIALS & METHODS: All patients of soft tissue sarcoma presenting to out department were studied and data collected in a proforma. Histology was established by Trucut/open biopsy. Patients after clinical examination were evaluated f or distant disease in lungs by x - ray chest followed by CT scan . RESULTS: Symptomatology and anatomical location in our study was similar to world literature but stage of presentation and tumour size is advanced in our study. CONCLUSIONS: Patients in our study presented late, probably due to lack of access to medical care, delay in diagnosis and delay in referral. Hence higher incidence of amputations and also higher incidence of need for reconstruction. Successful utilization of multi - modality treatment and availability of good reconstruction ensured good functional outcome.

Adult soft tissue sarcomas: conventional therapies and molecularly targeted approaches.

Science.gov (United States)

Mocellin, Simone; Rossi, Carlo R; Brandes, Alba; Nitti, Donato

2006-02-01

The therapeutic approach to soft tissue sarcomas (STS) has evolved over the past two decades based on the results from randomized controlled trials, which are guiding physicians in the treatment decision-making process. Despite significant improvements in the control of local disease, a significant number of patients ultimately die of recurrent/metastatic disease following radical surgery due to a lack of effective adjuvant treatments. In addition, the characteristic chemoresistance of STS has compromised the therapeutic value of conventional antineoplastic agents in cases of unresectable advanced/metastatic disease. Therefore, novel therapeutic strategies are urgently needed to improve the prognosis of patients with STS. Recent advances in STS biology are paving the way to the development of molecularly targeted therapeutic strategies, the efficacy of which relies not only on the knowledge of the molecular mechanisms underlying cancer development/progression but also on the personalization of the therapeutic regimen according to the molecular features of individual tumours. In this work, we review the state-of-the-art of conventional treatments for STS and summarize the most promising findings in the development of molecularly targeted therapeutic approaches.

Eribulin in the management of inoperable soft-tissue sarcoma: patient selection and survival

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Thomas C

2016-09-01

Full Text Available Colin Thomas, Sujana Movva Department of Hematology/Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA Abstract: Patients diagnosed with metastatic soft-tissue sarcoma (STS have a poor prognosis. Additionally, after failure of first-line therapy, there are relatively few treatment options from which to choose. The novel tubulin-binding drug, eribulin, with a unique mechanism of action from taxanes or vinca alkaloids, has shown clinical activity in several different types of cancers. Eribulin has been approved by the US Food and Drug Administration (FDA for patients with metastatic breast cancer previously treated with an anthracycline or a taxane and has recently been FDA approved for patients with unresectable or metastatic liposarcoma who have failed a previous anthracycline regimen. Here, we review current standard treatments of STS, a background of eribulin, the studies that have propelled eribulin to FDA approval for liposarcoma, and future directions of the drug. The benefits of eribulin in STS are discussed in detail, especially with regard to the recent pivotal Phase III study comparing eribulin to dacarbazine for leiomyosarcoma and adipocytic sarcoma. Keywords: eribulin, tubulin-binding drugs, soft-tissue sarcoma

Alveolar Soft Part Sarcoma.

Science.gov (United States)

Jaber, Omar I; Kirby, Patricia A

2015-11-01

Alveolar soft part sarcoma is a rare neoplasm usually arising in the soft tissues of the lower limbs in adults and in the head and neck region in children. It presents primarily as a slowly growing mass or as metastatic disease. It is characterized by a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in fusion of the transcription factor E3 (TFE3) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. This translocation is diagnostically useful because the tumor nuclei are positive for TFE3 by immunohistochemistry. Real-time polymerase chain reaction to detect the ASPSCR1-TFE3 fusion transcript on paraffin-embedded tissue blocks has been shown to be more sensitive and specific than detection of TFE3 by immunohistochemical stain. Cathepsin K is a relatively recent immunohistochemical stain that can aid in the diagnosis. The recent discovery of the role of the ASPSCR1-TFE3 fusion protein in the MET proto-oncogene signaling pathway promoting angiogenesis and cell proliferation offers a promising targeted molecular therapy.

Risk assessment of radio-chemotherapy in pediatric soft tissue sarcomas

OpenAIRE

Abaza, A.; El-Shanshoury, H.

2015-01-01

Soft tissue sarcomas (STS) are a group of rare and heterogenous cancers, that diverse a wide spectrum of histology and varied clinical behavior. The aim was to study, retrospectively and prospectively the adverse effects of therapy in STS patients attending the Pediatric Oncology Clinic, National Cancer Institute (NCI), Cairo University during the last 10 years. Files of 106 STS patients were revised for history, staging, investigations, treatment modalities and side effects of therapy. Radio...

Alveolar soft-part sarcoma of the orbit | Rose | African Journal of ...

African Journals Online (AJOL)

Alveolar soft-part sarcoma (ASPS) is a rare soft tissue tumour of uncertain cellular origin. It accounts for only 1% of all sarcomas, which themselves represent only a small proportion of human tumours. ASPS can arise in any soft tissue of the body, but there is an unexplained predilection for the right side. The most common ...

Utility Values for Advanced Soft Tissue Sarcoma Health States from the General Public in the United Kingdom

Directory of Open Access Journals (Sweden)

Julian F. Guest

2013-01-01

Full Text Available Soft tissue sarcomas are a rare type of cancer generally treated with palliative chemotherapy when in the advanced stage. There is a lack of published health utility data for locally advanced “inoperable”/metastatic disease (ASTS, essential for calculating the cost-effectiveness of current and future treatments. This study estimated time trade-off (TTO and standard gamble (SG preference values associated with four ASTS health states (progressive disease, stable disease, partial response, complete response among members of the general public in the UK (n=207. The four health states were associated with decreases in preference values from full health. Complete response was the most preferred health state (mean utility of 0.60 using TTO. The second most preferred health state was partial response followed by stable disease (mean utilities were 0.51 and 0.43, respectively, using TTO. The least preferred health state was progressive disease (mean utility of 0.30 using TTO. The utility value for each state was significantly different from one another (P<0.001. This study demonstrated and quantified the impact that different treatment responses may have on the health-related quality of life of patients with ASTS.

Quantitative dynamic ¹⁸FDG-PET and tracer kinetic analysis of soft tissue sarcomas.

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Rusten, Espen; Rødal, Jan; Revheim, Mona E; Skretting, Arne; Bruland, Oyvind S; Malinen, Eirik

2013-08-01

To study soft tissue sarcomas using dynamic positron emission tomography (PET) with the glucose analog tracer [(18)F]fluoro-2-deoxy-D-glucose ((18)FDG), to investigate correlations between derived PET image parameters and clinical characteristics, and to discuss implications of dynamic PET acquisition (D-PET). D-PET images of 11 patients with soft tissue sarcomas were analyzed voxel-by-voxel using a compartment tracer kinetic model providing estimates of transfer rates between the vascular, non-metabolized, and metabolized compartments. Furthermore, standard uptake values (SUVs) in the early (2 min p.i.; SUVE) and late (45 min p.i.; SUVL) phases of the PET acquisition were obtained. The derived transfer rates K1, k2 and k3, along with the metabolic rate of (18)FDG (MRFDG) and the vascular fraction νp, was fused with the computed tomography (CT) images for visual interpretation. Correlations between D-PET imaging parameters and clinical parameters, i.e. tumor size, grade and clinical status, were calculated with a significance level of 0.05. The temporal uptake pattern of (18)FDG in the tumor varied considerably from patient to patient. SUVE peak was higher than SUVL peak for four patients. The images of the rate constants showed a systematic pattern, often with elevated intensity in the tumors compared to surrounding tissue. Significant correlations were found between SUVE/L and some of the rate parameters. Dynamic (18)FDG-PET may provide additional valuable information on soft tissue sarcomas not obtainable from conventional (18)FDG-PET. The prognostic role of dynamic imaging should be investigated.

Automated planning volume definition in soft-tissue sarcoma adjuvant brachytherapy

International Nuclear Information System (INIS)

Lee, Eva K.; Fung, Albert Y.C.; Zaider, Marco; Brooks, J. Paul

2002-01-01

In current practice, the planning volume for adjuvant brachytherapy treatment for soft-tissue sarcoma is either not determined a priori (in this case, seed locations are selected based on isodose curves conforming to a visual estimate of the planning volume), or it is derived via a tedious manual process. In either case, the process is subjective and time consuming, and is highly dependent on the human planner. The focus of the work described herein involves the development of an automated contouring algorithm to outline the planning volume. Such an automatic procedure will save time and provide a consistent and objective method for determining planning volumes. In addition, a definitive representation of the planning volume will allow for sophisticated brachytherapy treatment planning approaches to be applied when designing treatment plans, so as to maximize local tumour control and minimize normal tissue complications. An automated tumour volume contouring algorithm is developed utilizing computational geometry and numerical interpolation techniques in conjunction with an artificial intelligence method. The target volume is defined to be the slab of tissue r cm perpendicularly away from the curvilinear plane defined by the mesh of catheters. We assume that if adjacent catheters are over 2r cm apart, the tissue between the two catheters is part of the tumour bed. Input data consist of the digitized coordinates of the catheter positions in each of several cross-sectional slices of the tumour bed, and the estimated distance r from the catheters to the tumour surface. Mathematically, one can view the planning volume as the volume enclosed within a minimal smoothly-connected surface which contains a set of circles, each circle centred at a given catheter position in a given cross-sectional slice. The algorithm performs local interpolation on consecutive triplets of circles. The effectiveness of the algorithm is evaluated based on its performance on a collection of

Automated planning volume definition in soft-tissue sarcoma adjuvant brachytherapy

Energy Technology Data Exchange (ETDEWEB)

Lee, Eva K. [Department of Radiation Oncology, Emory University School of Medicine, Atlanta, GA (United States); School of Industrial and Systems Engineering, Georgia Institute of Technology, Atlanta, GA (United States); Fung, Albert Y.C.; Zaider, Marco [Department of Medical Physics, Memorial Sloan Kettering Cancer Center, New York, NY (United States); Brooks, J. Paul [School of Industrial and Systems Engineering, Georgia Institute of Technology, Atlanta, GA (United States)

2002-06-07

In current practice, the planning volume for adjuvant brachytherapy treatment for soft-tissue sarcoma is either not determined a priori (in this case, seed locations are selected based on isodose curves conforming to a visual estimate of the planning volume), or it is derived via a tedious manual process. In either case, the process is subjective and time consuming, and is highly dependent on the human planner. The focus of the work described herein involves the development of an automated contouring algorithm to outline the planning volume. Such an automatic procedure will save time and provide a consistent and objective method for determining planning volumes. In addition, a definitive representation of the planning volume will allow for sophisticated brachytherapy treatment planning approaches to be applied when designing treatment plans, so as to maximize local tumour control and minimize normal tissue complications. An automated tumour volume contouring algorithm is developed utilizing computational geometry and numerical interpolation techniques in conjunction with an artificial intelligence method. The target volume is defined to be the slab of tissue r cm perpendicularly away from the curvilinear plane defined by the mesh of catheters. We assume that if adjacent catheters are over 2r cm apart, the tissue between the two catheters is part of the tumour bed. Input data consist of the digitized coordinates of the catheter positions in each of several cross-sectional slices of the tumour bed, and the estimated distance r from the catheters to the tumour surface. Mathematically, one can view the planning volume as the volume enclosed within a minimal smoothly-connected surface which contains a set of circles, each circle centred at a given catheter position in a given cross-sectional slice. The algorithm performs local interpolation on consecutive triplets of circles. The effectiveness of the algorithm is evaluated based on its performance on a collection of

Radiation-induced pseudotumor following therapy for soft tissue sarcoma

Energy Technology Data Exchange (ETDEWEB)

Moore, Lacey F.; Kransdorf, Mark J. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Buskirk, Steven J. [Mayo Clinic, Department of Radiation Oncology, Jacksonville, FL (United States); O' Connor, Mary I. [Mayo Clinic, Department of Orthopedic Surgery, Jacksonville, FL (United States); Menke, David M. [Mayo Clinic, Department of Pathology, Jacksonville, FL (United States)

2009-06-15

The purpose of this study was to describe the prevalence and imaging appearance of radiation induced pseudotumors in patients following radiation therapy for extremity soft tissue sarcomas. We retrospectively reviewed the serial magnetic resonance (MR) images of 24 patients following radiation therapy for extremity soft tissue sarcomas. A total of 208 exams were reviewed (mean, 8.7 exams per patient) and included all available studies following the start of radiation therapy. Exams were analyzed for the identification of focal signal abnormalities within the surgical bed suggesting local tumor recurrence. Histopathologic correlation was available in nine patients suspected of having local tumor recurrence. Additional information recorded included patient demographics, tumor type and location, radiation type, and dose. The study group consisted of 12 men and 12 women, having an average age of 63 years (range, 39-88 years). Primary tumors were malignant fibrous histiocytoma (n = 13), leiomyosarcoma (n = 6), liposarcoma (n = 3), synovial sarcoma (n = 1), and extraskeletal chondrosarcoma (n = 1). All lesions were high-grade sarcomas, except for two myxoid liposarcomas. Average patient radiation dose was 5,658 cGy (range, 4,500-8,040 cGy). Average follow-up time was 63 months (range, 3-204 months). Focal signal abnormalities suggesting local recurrence were seen in nine (38%) patients. Three of the nine patients with these signal abnormalities were surgically proven to have radiation-induced pseudotumor. The pseudotumors developed between 11 and 61 months following the initiation of radiation therapy (mean, 38 months), with an average radiation dose of 5,527 cGy (range, 5,040-6,500 cGy). MR imaging demonstrated a relatively ill-defined ovoid focus of abnormal signal and intense heterogeneous enhancement with little or no associated mass effect. MR imaging of radiation-induced pseudotumor typically demonstrates a relatively ill-defined ovoid mass-like focus of intense

Neoadjuvant chemotherapy for high-grade soft-tissue sarcomas of the limbs

International Nuclear Information System (INIS)

Ramos, Pedro; Gonzalez, Manuel; Perry, Fernando; Cardona, Andres Felipe

2005-01-01

Background: the use of neoadjuvant chemotherapy for high-grade soft-tissue sarcomas of the limbs continues to be an area of controversy; however, the number of clinical studies favoring the use of an anthracycline and iphosphamide-based regimen is increasing steadily. This approach may provide some advantages for facilitating the surgical resection of the tumor and for local disease control. The historical 5-year survival rate of approximately 50% in this high-risk group treated with local therapy alone represents a poor standard of care; thus, there is a need to incorporate systemic therapy early in the management of these patients. Objective: to describe the role of neoadjuvant chemotherapy in the treatment of soft-tissue sarcomas. Materials and methods: the records of 42 patients who attended the national cancer institute of Colombia in search for management of primary soft-tissue sarcomas were retrospectively reviewed. Ten patients with high-grade tumors larger than 8 cm, treated from June 2000 to February 2002 with neoadjuvant chemotherapy based on an anthracycline and iphosphamide regimen, plus vincristin and cisplatinum in selected cases, followed by surgery and adjuvant therapy with chemotherapy combined with local radiotherapy, were included. Evaluations of objective tumor response, survival, and toxicity were carried out. Results: after neoadjuvant therapy, s ix patients underwent conservative and limb-salvage surgery, three required radical interventions, and one refused surgical treatment. Seven experienced an objective response: it was complete in four and partial in three; the disease kept stable in two patients, and the tumor progressed in one case. After an average 46-month follow-up, four patients were permanently free of disease. Hematological and gastrointestinal toxicity was remarkable, and no patient had a long-term morbidity related to the treatment. Conclusions: this limited retrospective review suggests an advantage for the use of

Role of radiotherapy and chemotherapy in management of soft tissue sarcomas

International Nuclear Information System (INIS)

Harmer, C.; Frampton, M.; Wiltshaw, E.

1986-01-01

One hundred eighty patients with soft tissue sarcoma were registered at the Royal Marsden Hospital last year, and the majority of these were seen in the Combined Sarcoma Clinic. This clinic is run by surgeons, radiotherapists, and medical oncologists who see the patients together each week. All the patients have an adequate biopsy, or if surgery has been performed at an outside hospital the previous histology is reviewed. A multidisciplinary team therefore participates in all the important decisions regarding treatment, and the patients continue to be seen in combined consultation during their physiotherapy, rehabilitation, and follow-up. By concentrating large numbers of patients with these rare tumours the scene is set for improved treatment and, hopefully, improved survival

Benign mural nodules within fluid collections at MRI after soft-tissue sarcoma resection.

Science.gov (United States)

Lantos, Joshua E; Hwang, Sinchun; Panicek, David M

2014-06-01

The purpose of this study was to determine the prevalence and clinical significance of nodules within fluid collections on MRI after surgical resection of soft-tissue sarcoma. This retrospective study included 175 patients who underwent resection of primary soft-tissue sarcoma and whose postoperative MRI reports mentioned fluid. Images were reviewed to determine the presence of fluid collections of 1 cm or greater in diameter in the surgical bed and any nodule (measuring ≥ 0.7 cm) within the collection. Signal intensity and characteristics of each collection and rim and presence of septa or blood products were recorded. Size, signal intensity, and contrast enhancement of nodules were reviewed. Nodules were classified as benign or malignant on the basis of histologic results or clinical or MRI follow-up. Fluid collections were present in 75 patients. Of those, 45 collections (60%) showed homogeneous fluid signal intensity and 30 (40%) were heterogeneous; septa were present in 45 (60%) and blood products in 12 (16%). Most collections showed a thin rim (59%) and rim enhancement (88%). Nodules were present along the inner wall of six (8%) collections. Four (66%) nodules enhanced and two (33%) were T1 hyperintense. At follow-up MRI, two nodules were stable in size, one decreased, and three resolved. Nodules in three patients were biopsied; all were benign. Two other patients had no recurrence at follow-up, and another died at 3 months. A nodule within a postoperative fluid collection at MRI after soft-tissue sarcoma resection generally does not represent tumor recurrence; short-interval follow-up MRI is recommended rather than immediate biopsy.

A prospective study of proton reirradiation for recurrent and secondary soft tissue sarcoma.

Science.gov (United States)

Guttmann, David M; Frick, Melissa A; Carmona, Ruben; Deville, Curtiland; Levin, William P; Berman, Abigail T; Chinniah, Chidambaram; Hahn, Stephen M; Plastaras, John P; Simone, Charles B

2017-08-01

Proton reirradiation for sarcoma has not been previously described. We hypothesized that this strategy would provide favorable toxicity and survival outcomes. Patients with soft tissue sarcoma in a previously-irradiated field were enrolled on a prospective trial of proton reirradiation. The primary endpoint was provider-reported acute toxicity. Secondary endpoints included late toxicities, local control, and overall survival. 23 patients underwent proton reirradiation. Median time between radiation courses was 40.7months (range 10-272). No grade 4-5 toxicities were observed. One patient (4%) experienced acute grade 3 dysphagia. Common grade 2 acute toxicities were fatigue (26%), anorexia (17%), and urinary incontinence (13%). There were two grade 3 late wound infections (10%) and one grade 3 late wound complication (5%). Grade 2 late complications included lymphedema (10%), fracture (5%), and fibrosis (5%). At a median follow-up of 36months, the 3-year cumulative incidence of local failure was 41% (95% CI [20-63%]). Median overall survival and progression-free survival were 44 and 29months, respectively. In extremity patients, amputation was spared in 7/10 (70%). Proton reirradiation of recurrent/secondary soft tissue sarcomas is well tolerated. While longer follow-up is needed, early survival outcomes in this high-risk population are encouraging. Copyright © 2017 Elsevier B.V. All rights reserved.

Microscopic histological characteristics of soft tissue sarcomas: analysis of tissue features and electrical resistance.

Science.gov (United States)

Tosi, A L; Campana, L G; Dughiero, F; Forzan, M; Rastrelli, M; Sieni, E; Rossi, C R

2017-07-01

Tissue electrical conductivity is correlated with tissue characteristics. In this work, some soft tissue sarcomas (STS) excised from patients have been evaluated in terms of histological characteristics (cell size and density) and electrical resistance. The electrical resistance has been measured using the ex vivo study on soft tissue tumors electrical characteristics (ESTTE) protocol proposed by the authors in order to study electrical resistance of surgical samples excised by patients in a fixed measurement setup. The measurement setup includes a voltage pulse generator (700 V, 100 µs long at 5 kHz, period 200 µs) and an electrode with 7 needles, 20 mm-long, with the same distance arranged in a fixed hexagonal geometry. In the ESTTE protocol, the same voltage pulse sequence is applied to each different tumor mass and the corresponding resistance has been evaluated from voltage and current recorded by the equipment. For each tumor mass, a histological sample of the volume treated by means of voltage pulses has been taken for histological analysis. Each mass has been studied in order to identify the sarcoma type. For each histological sample, an image at 20× or 40× of magnification was acquired. In this work, the electrical resistance measured for each tumor has been correlated with tissue characteristics like the type, size and density of cells. This work presents a preliminary study to explore possible correlations between tissue characteristics and electrical resistance of STS. These results can be helpful to adjust the pulse voltage intensity in order to improve the electrochemotherapy efficacy on some histotype of STS.

Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

Directory of Open Access Journals (Sweden)

Agarwala Sandeep

2006-01-01

Full Text Available Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53% of these originate from the striated muscles and are called rhabdomyosarcomas (RMS the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS. Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retroperitoneum. Pathologically RMS is now classified as superior, intermediate and poor outcome histologies. For stratification of treatment and also comparison of results the RMS are now staged both by the clinical grouping and the TNM staging systems. The ultimate outcome depends on the site, extent of disease and histology. Currently, approximately 70% of the patients survive for 5 years or more and are probably cured. This is credited to the use of multi-modal, risk-adapted therapy, refinements in tumor grouping and better supportive care which has emerged out of cooperative studies like Intergroup Rhabdomyosarcoma Study (IRS and the International Society of Pediatric Oncology studies (SIOP. The treatment involves chemotherapy, radiotherapy and organ/function preserving surgery. The gold standard chemotherapy is still vincristine, actinomycin D and cyclophosphamide (VAC regime with high doses of intensity bone marrow rescue with colony stimulating factors. The NRSTS are rare and of heterogenous histologies and so it has been difficult to arrive at a treatment strategy for these. What is definitely understood is that these are usually immature and poorly differentiated tumors that respond poorly to chemotherapy and so surgical resection forms the mainstay of treatment with adjuvant radiotherapy and chemotherapy to prevent local recurrences. In all likelihood, the molecular analysis of RMS will further refine current classification schemes and knowledge of genetic features of

Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

NARCIS (Netherlands)

Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

2010-01-01

BACKGROUND: Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects.

Genetic profiling differentiates second primary tumors from metastases in adult metachronous soft tissue sarcoma

DEFF Research Database (Denmark)

Fernebro, Josefin; Carneiro, Ana; Rydholm, Anders

2008-01-01

Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH) was applied to 30 mu...

Adult Head and Neck Soft Tissue Sarcomas: Treatment and Outcome

Directory of Open Access Journals (Sweden)

Rabindra P. Singh

2008-01-01

Thirty-six patients were seen, of whom 24 were treated at this unit, the remainder only receiving advice. The median age of the patients was 46 years. Most of the sarcomas were deep and of high or intermediate grade with a median size of 5.5 cm. Eleven different histological subtypes were identified. Wide excision was possible only in 21% of the cases. 42% of the patients developed local recurrence and 42% developed metastatic disease usually in the lungs. Overall survival was 49% at 5 years. Tumour size was the most important prognostic factor. Adult head and neck soft tissue sarcomas have a high mortality rate with a high risk of local recurrence and metastatic disease. The rarity of the disease would suggest that centralisation of care could lead to increased expertise and better outcomes.

Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

NARCIS (Netherlands)

Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

2012-01-01

Background Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects. This is an

Cyclophosphamide versus ifosfamide for paediatric and young adult bone and soft tissue sarcoma patients

NARCIS (Netherlands)

Mulder, Renée L.; Paulides, Marios; Langer, Thorsten; Kremer, Leontien C. M.; van Dalen, Elvira C.

2015-01-01

Alkylating agents, such as cyclophosphamide and ifosfamide, play a major role in the improved survival of children and young adults with bone and soft tissue sarcoma. However, there is still controversy as to their comparative anti-tumour efficacy and possible adverse effects. This is the second

Light ion irradiation for unfavorable soft tissue sarcoma

International Nuclear Information System (INIS)

Linstadt, D.; Castro, J.R.; Phillips, T.L.; Petti, P.L.; Collier, J.M.; Daftari, I.; Schoethaler, R.; Rayner, A.

1990-09-01

Between 1978 and 1989, 32 patients with unfavorable soft tissue sarcoma underwent light ion (helium, neon) irradiation with curative intent at Lawrence Berkeley Laboratory. The tumors were located in the trunk in 22 patients and head and neck in 10. Macroscopic tumor was present in 22 at the time of irradiation. Two patients had tumors apparently induced by previous therapeutic irradiation. Follow-up times for surviving patients ranged from 4 to 121 months (median 27 months). The overall 3-year actuarial local control rate was 62%; the corresponding survival rate was 50%. The 3-year actuarial control rate for patients irradiated with macroscopic tumors was 48%, while none of the patients with microscopic disease developed local recurrence (100%). The corresponding 3-year actuarial survival rates were 40% (macroscopic) and 78% (microscopic). Patients with retroperitoneal sarcoma did notably well; the local control rate and survival rate were 64% and 62%, respectively. Complications were acceptable; there were no radiation related deaths, while two patients (6%) required operations to correct significant radiation-related injuries. These results appear promising compared to those achieved by low -LET irradiation, and suggest that this technique merits further investigation

Therapeutic options and postoperative wound complications after extremity soft tissue sarcoma resection and postoperative external beam radiotherapy.

Science.gov (United States)

Abouarab, Mohamed H; Salem, Iman L; Degheidy, Magdy M; Henn, Dominic; Hirche, Christoph; Eweida, Ahmad; Uhl, Matthias; Kneser, Ulrich; Kremer, Thomas

2018-02-01

Soft tissue sarcomas occur most commonly in the lower and upper extremities. The standard treatment is limb salvage surgery combined with radiotherapy. Postoperative radiotherapy is associated with wound complications. This systematic review aims to summarise the available evidence and review the literature of the last 10 years regarding postoperative wound complications in patients who had limb salvage surgical excision followed by direct closure vs flap coverage together with postoperative radiotherapy and to define the optimal timeframe for adjuvant radiotherapy after soft tissue sarcomas resection and flap reconstruction. A literature search was performed using PubMed. The following keywords were searched: limb salvage, limb-sparing, flaps, radiation therapy, radiation, irradiation, adjuvant radiotherapy, postoperative radiotherapy, radiation effects, wound healing, surgical wound infection, surgical wound dehiscence, wound healing, soft tissue sarcoma and neoplasms. In total, 1045 papers were retrieved. Thirty-seven articles were finally selected after screening of abstracts and applying dates and language filters and inclusion and exclusion criteria. Plastic surgery provides a vast number of reconstructive flap procedures that are directly linked to decreasing wound complications, especially with the expectant postoperative radiotherapy. This adjuvant radiotherapy is better administered in the first 3-6 weeks after reconstruction to allow timely wound healing and avoid local recurrence. © 2017 Medicalhelplines.com Inc and John Wiley & Sons Ltd.

Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy

DEFF Research Database (Denmark)

Young, Robin J; Litière, Saskia; Lia, Michela

2017-01-01

BACKGROUND: The European Organization for Research and Treatment of Cancer (EORTC) 62012 study was a Phase III trial of doxorubicin versus doxorubicin-ifosfamide chemotherapy in 455 patients with advanced soft tissue sarcoma (STS). Analysis of the main study showed that combination chemotherapy...... improved tumor response and progression-free survival, but differences in overall survival (OS) were not statistically significant. We analyzed factors prognostic for tumor response and OS, and assessed histological subgroup and tumor grade as predictive factors to identify patients more likely to benefit...... patients had improved tumor response compared to other histological subgroups, whilst patients with metastases other than lung, liver or bone had a poorer response [odds ratio (OR) 0.42, 95% confidence interval (CI) 0.23-0.78; p = 0.006]. Patients with bone metastases had reduced OS [hazard ratio (HR) 1...

Phase II Study of Neoadjuvant Bevacizumab and Radiotherapy for Resectable Soft Tissue Sarcomas

International Nuclear Information System (INIS)

Yoon, Sam S.; Duda, Dan G.; Karl, Daniel L.; Kim, Tae-Min; Kambadakone, Avinash R.; Chen, Yen-Lin; Rothrock, Courtney; Rosenberg, Andrew E.; Nielsen, G. Petur; Kirsch, David G.; Choy, Edwin; Harmon, David C.; Hornicek, Francis J.; Dreyfuss, Jonathan; Ancukiewicz, Marek

2011-01-01

Purpose: Numerous preclinical studies have demonstrated that angiogenesis inhibitors can increase the efficacy of radiotherapy (RT). We sought to examine the safety and efficacy of bevacizumab (BV) and RT in soft tissue sarcomas and explore biomarkers to help determine the treatment response. Methods and Materials: Patients with ≥5 cm, intermediate- or high-grade soft tissue sarcomas at significant risk of local recurrence received neoadjuvant BV alone followed by BV plus RT before surgical resection. Correlative science studies included analysis of the serial blood and tumor samples and serial perfusion computed tomography scans. Results: The 20 patients had a median tumor size of 8.25 cm, with 13 extremity, 1 trunk, and 6 retroperitoneal/pelvis tumors. The neoadjuvant treatment was well tolerated, with only 4 patients having Grade 3 toxicities (hypertension, liver function test elevation). BV plus RT resulted in ≥80% pathologic necrosis in 9 (45%) of 20 tumors, more than double the historical rate seen with RT alone. Three patients had a complete pathologic response. The median microvessel density decreased 53% after BV alone (p <.05). After combination therapy, the median tumor cell proliferation decreased by 73%, apoptosis increased 10.4-fold, and the blood flow, blood volume, and permeability surface area decreased by 62–72% (p <.05). Analysis of gene expression microarrays of untreated tumors identified a 24-gene signature for treatment response. The microvessel density and circulating progenitor cells at baseline and the reduction in microvessel density and plasma soluble c-KIT with BV therapy also correlated with a good pathologic response (p <.05). After a median follow-up of 20 months, only 1 patient had developed local recurrence. Conclusions: The results from the present exploratory study indicated that BV increases the efficacy of RT against soft tissue sarcomas and might reduce the incidence of local recurrence. Thus, this regimen warrants

Impact of geriatric factors on surgical and prognostic outcomes in elderly patients with soft-tissue sarcoma.

Science.gov (United States)

Tsuda, Yusuke; Ogura, Koichi; Kobayashi, Eisuke; Hiruma, Toru; Iwata, Shintaro; Asano, Naofumi; Kawai, Akira; Chuman, Hirokazu; Ishii, Takeshi; Morioka, Hideo; Kobayashi, Hiroshi; Kawano, Hirotaka

2017-05-01

Patients aged ≥65 years requiring surgery for soft-tissue sarcoma are a concern in an aging society. We aimed to reveal the association of clinical/geriatric factors with survival period or postoperative events in such patients who underwent surgery. We enrolled patients aged ≥65 years who underwent surgery for localized soft-tissue sarcoma at five institutions. We retrospectively collected clinical/geriatric factors and laboratory data, and analyzed their association with outcomes using univariate and multivariate analyses. Among the 202 patients included, mean age at presentation was 73 years. Surgical margin was R0 in 139 patients (69%). The Eastern Cooperative Oncology Group performance status was ≥2 in 15 (7%). Thirty patients (15%) showed thinness (body mass index sarcoma-specific survival (hazard ratio for R1 vs. R0, 3.17; P = 0.001) and event-free survival (hazard ratio for R1 vs. R0, 2.56; P sarcoma-specific survival (hazard ratio for ≥2 vs. 0 or 1, 2.15; P = 0.038), and higher sensitivity-modified Glasgow prognostic score was significantly associated with poor event-free survival (hazard ratio for ≥1 vs. 0, 1.74; P = 0.046). Severe thinness (body mass index sarcoma patients. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group Response Score

International Nuclear Information System (INIS)

Schaefer, Inga-Marie; Hornick, Jason L.; Barysauskas, Constance M.; Raut, Chandrajit P.; Patel, Sagar A.; Royce, Trevor J.; Fletcher, Christopher D.M.; Baldini, Elizabeth H.

2017-01-01

Purpose: To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). Methods and Materials: For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Results: Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Conclusion: Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials.

Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group Response Score.

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Schaefer, Inga-Marie; Hornick, Jason L; Barysauskas, Constance M; Raut, Chandrajit P; Patel, Sagar A; Royce, Trevor J; Fletcher, Christopher D M; Baldini, Elizabeth H

2017-06-01

To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials. Copyright © 2017 Elsevier Inc. All rights reserved.

Histologic Appearance After Preoperative Radiation Therapy for Soft Tissue Sarcoma: Assessment of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group Response Score

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Schaefer, Inga-Marie; Hornick, Jason L. [Department of Pathology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Barysauskas, Constance M. [Department of Biostatistics and Computational Biology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Raut, Chandrajit P. [Division of Surgical Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Patel, Sagar A.; Royce, Trevor J. [Department of Radiation Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Fletcher, Christopher D.M. [Department of Pathology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States); Baldini, Elizabeth H., E-mail: ebaldini@partners.org [Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts (United States); Department of Radiation Oncology, Brigham and Women' s Hospital, Harvard Medical School, Boston, Massachusetts (United States)

2017-06-01

Purpose: To critically assess the prognostic value of the European Organization for Research and Treatment of Cancer–Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) response score and define histologic appearance after preoperative radiation therapy (RT) for soft tissue sarcoma (STS). Methods and Materials: For a cohort of 100 patients with STS of the extremity/trunk treated at our institution with preoperative RT followed by resection, 2 expert sarcoma pathologists evaluated the resected specimens for percent residual viable cells, necrosis, hyalinization/fibrosis, and infarction. The EORTC response score and other predictors of recurrence-free survival (RFS) and overall survival (OS) were assessed by Kaplan-Meier and proportional hazard models. Results: Median tumor size was 7.5 cm; 92% were intermediate or high grade. Most common histologies were unclassified sarcoma (34%) and myxofibrosarcoma (25%). Median follow-up was 60 months. The 5-year local recurrence rate was 5%, 5-year RFS was 68%, and 5-year OS was 75%. Distribution of cases according to EORTC response score tiers was as follows: no residual viable tumor for 9 cases (9% pathologic complete response); <1% viable tumor for 0, ≥1% to <10% for 9, ≥10% to <50% for 44, and ≥50% for 38. There was no association between EORTC-STBSG response score and RFS or OS. Conversely, hyalinization/fibrosis was a significant independent favorable predictor for RFS (hazard ratio 0.49, P=.007) and OS (hazard ratio 0.36, P=.02). Conclusion: Histologic evaluation after preoperative RT for STS showed a 9% pathologic complete response rate. The EORTC-STBSG response score and percent viable cells were not prognostic. Hyalinization/fibrosis was associated with favorable outcome, and if validated, may become a valid endpoint for neoadjuvant trials.

Anthracycline-induced cardiotoxicity in patients with paediatric bone sarcoma and soft tissue sarcoma.

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Bini, Ilaria; Asaftei, Sebastian D; Riggi, Chiara; Tirtei, Elisa; Manicone, Rosaria; Biasin, Eleonora; Basso, Maria Eleonora; Agnoletti, Gabriella; Fagioli, Franca

2017-11-01

Anthracycline cardiotoxicity is an important side-effect in long-term childhood cancer survivors. We evaluated the incidence of and factors associated with anthracycline cardiotoxicity in a population of patients diagnosed with bone or soft tissue sarcoma. Materials and methods We retrospectively enrolled patients diagnosed with bone or soft tissue sarcoma, from 1995 to 2011, treated with anthracycline chemotherapy at our Centre and with a follow-up echocardiography carried out ⩾3 years from cardiotoxic therapy completion. Cardiac toxicity was graded using Common Terminology Criteria for Adverse Events version 4.0. A total of 82 patients were eligible. The median age at treatment was 11.9 years (1.44-18). We evaluated the median cumulative anthracycline dose, age at treatment, sex, thoracic radiotherapy, hematopoietic stem cell transplantation, and high-dose cyclophosphamide treatment as possible risk factors for cardiotoxicity. The median cumulative anthracycline dose was 390.75 mg/m2 (80-580). Of the 82 patients, 12 (14.6%) developed cardiotoxicity with grade ⩾2 ejection fraction decline: four patients were asymptomatic and did not receive any treatment; six patients were treated with pharmacological heart failure therapy; one patient with severe cardiomyopathy underwent heart transplantation and did not need any further treatment; and one patient died while waiting for heart transplantation. The median time at cardiac toxicity, from the end of anthracycline frontline chemotherapy, was 4.2 years (0.05-9.6). Cumulative anthracycline dose ⩾300 mg/m2 (p 0.04) was the only risk factor for cardiotoxicity on statistical analyses. In our population, the cumulative incidence of cardiotoxicity is comparable to rates in the literature. This underlines the need for primary prevention and lifelong cardiac toxicity surveillance programmes in long-term childhood cancer survivors.

Radiosensitivity and repair capacity of two xenografted human soft tissue sarcomas to photons and fast neutrons

International Nuclear Information System (INIS)

Budach, V.; Stuschke, M.; Budach, W.; Krause, U.; Streffer, C.; Sack, H.

1989-01-01

The radiation response, the relative biological effectiveness (RBE) and sublethal damage repair of two xenografted human soft tissue sarcomas after single doses and fractionated irradiation with 60 Co and 5.8 MeV fast neutrons are presented. (author)

A rheumatoid nodule mimicking soft tissue sarcoma on MR: a case report

International Nuclear Information System (INIS)

Choi, Joon Il; Kong, Keun Young; Kang, Heung Sik

2000-01-01

We report a case of rheumatoid nodule of the right knee in a 55-year-old woman which on the basis of MR images was misdiagnosed as soft tissue sarcoma. Reumatoid nodules occur in up to 20% of adult patients with rheumatoid arthritis and most patients with rheumatoid nodules are rheumatoid factor positive. The nodules are most commonly located between the skin and an underlying bony prominence. In this case, the patient had a history of rheumatoid arthritis and a soft tissue mass was present in the lateral aspect of the right knee. T1-weighted images revealed a mass of similar signal intensity to that of muscle, and on T2-weighted images, a central portion of high signal intensity was noted. Enhanced T1-weighted images demonstrated peripheral rim enhancement. The mass extended to subcutaneous tissue, with penetration of lateral patellar retinaculum. Excisional biopsy was performed, and the pathologic diagnosis was rheumatoid nodule. Although its radiologic findings are non-specific, rheumatoid nodule should -in appropriate clinical setting- be included in the differential diagnosis of a predominantly solid or centrally necrotic periarticular soft tissue. (author)

Current concepts in non-gastrointestinal stromal tumor soft tissue sarcomas: A primer for radiologists

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Baheti, Akahay D. [Dept. of Radiology, Tata Memorial Centre, Mumbai (India); Tirumani, Harika [Dept. of Radiology, University of Arkansas for Medical Sciences, Little Rock (United States); O' Neill, Alibhe; Jagannathan, Jyothi P. [Dept. of Imaging, Dana-Farber Cancer Institute, Boston (United States)

2017-01-15

Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist.

Current concepts in non-gastrointestinal stromal tumor soft tissue sarcomas: A primer for radiologists

International Nuclear Information System (INIS)

Baheti, Akahay D.; Tirumani, Harika; O'Neill, Alibhe; Jagannathan, Jyothi P.

2017-01-01

Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist

Intraoperative extracorporeal autogenous irradiated tendon grafts for functional limb salvage surgery of soft tissue sarcomas of the wrist and hand.

Science.gov (United States)

Omori, Shinsuke; Hamada, Kenichiro; Outani, Hidetatsu; Oshima, Kazuya; Joyama, Susumu; Tomita, Yasuhiko; Naka, Norifumi; Araki, Nobuhito; Yoshikawa, Hideki

2015-05-12

In patients with soft tissue sarcoma of the wrist and hand, limb salvage operation is extremely challenging for surgeons in attempting a complete tumor resection with negative surgical margins. In this study, we report four patients with soft tissue sarcoma of the wrist and hand treated by limb salvage operation with intraoperative extracorporeal autogenous irradiated tendon grafts. The patients were all male, and the mean age at the time of surgery was 45 years. Histological diagnoses included clear cell sarcoma in two patients, synovial sarcoma in one, and angiosarcoma in one. All four patients had high grade tumors, wherein three had American Joint Committee on Cancer (AJCC) stage III disease and one with AJCC stage IV disease. The tumors were resected en bloc with involved tendons. The tendons were isolated from the resected tissues, irradiated ex vivo, and re-implanted into the host tendons. In one patient, the bone was resected additionally because of tumor invasion to the bone. Hand function was evaluated using Musculoskeletal Tumor Society (MSTS) rating system. Of the four patients, three died of distant metastatic disease. The remaining patient lives and remains disease-free. The mean follow-up period was 33 months. One patient had local recurrence outside the irradiated graft at 20 months after surgery. The functional rating was 22. Lower scores were seen in patients with reconstruction of flexor tendons than extensor tendons. Limb salvage operation with intraoperative extracorporeal autogenous irradiated tendon grafts is an acceptable method in selected patients with soft tissue sarcoma of the wrist and hand.

Effectiveness and Morbidity Associated With Reirradiation in Conservative Salvage Management of Recurrent Soft-Tissue Sarcoma

International Nuclear Information System (INIS)

Indelicato, Daniel J.; Meadows, Kenyon; Gibbs, Charles P.; Morris, Christopher G.; Scarborough, Mark T.; Zlotecki, Robert A.

2009-01-01

Purpose: The management of isolated local recurrence of soft-tissue sarcoma is therapeutically complex, and functional conservative management is preferable to radical or amputative salvage surgery. This study reviews University of Florida experience using conservative resection and reirradiation to manage isolated local recurrences of soft-tissue sarcoma. Methods and Materials: Between 1976 and 2005, a total of 14 patients who underwent primary conservative resection and irradiation developed isolated local recurrence and were managed with salvage conservative resection and reirradiation. Of the patients treated, 3 had tumors of the distal extremity, 8 had tumors of the proximal extremity, and 3 had tumors of the trunk. At the time of recurrence, 64% of tumors were greater than 5 cm and 79% were high grade. In combination with gross total resection, 13 of 14 patients received external beam radiotherapy with or without brachytherapy, and 1 patient was treated with brachytherapy alone. Two patients received chemotherapy. Results: The median follow-up was 30 months, and no living patients were lost during follow-up. From retreatment, the 5-year actuarial overall survival, cause-specific survival, and local control rates were 40%, 40%, and 18% respectively. There was a 50% incidence of serious complication requiring either reoperation or leading to permanent functional impairment. Of the 14 patients, only 1 has remained disease free and without significant complications. No treatment factors, including achieving wide surgical margins or delivering higher radiation dosages, seemed to confer an advantage in local control. Conclusions: Salvage therapy for management of locally recurrent soft-tissue sarcoma is challenging, and the effects of reoperation and reirradiation can be severe

Lymph Node Metastasis after a Soft Tissue Sarcoma of the Leg: A Case Report and a Review of the Literature

Directory of Open Access Journals (Sweden)

S. D. Nelen

2013-01-01

Full Text Available Introduction. Soft tissue sarcomas (STSs represent 1 percent of all adult malignancies and sarcomas only rarely spread to the regional lymph nodes. Case Presentation. We present a case of a woman with a dermatofibrosarcoma protuberans and a sarcoma not therwise specified of the lower extremity. The patient had no distant metastasis during follow-up, but did develop a regional lymph nodemetastasis (RLNM in the groin. We reviewed the literature about RLNM in STSs. Discussion. Reviewing the literature we see that within specific histological types RLNM occurs as often as distant metastasis. Furthermore RLNM occurs in over 10% for specific histological types and in 24% of all patients with a soft tissue sarcoma of the lower extremity. Except for radical lymphadenectomy with a 5-year survival rate of 46% there is no appropriate treatment. Conclusion. The risk for a RLNM in certain histological types and anatomical locations might transcend the risk for a distant lung metastasis.

Development of Soft Tissue Sarcomas in Ribosomal Proteins L5 and S24 Heterozygous Mice

Czech Academy of Sciences Publication Activity Database

Kazerounian, S.; Ciarlini, P.D.S.C.; Yuan, D.; Ghazvinian, R.; Alberich-Jorda, Meritxell; Joshi, M.; Zhang, H.; Beggs, A.H.; Gazda, H.T.

2016-01-01

Roč. 7, č. 1 (2016), s. 32-36 ISSN 1837-9664 R&D Projects: GA MŠk LK21307 Institutional support: RVO:68378050 Keywords : Ribosomal proteins RPL5 and RPS24 * Diamond-Blackfan anemia * Soft tissue sarcoma Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.916, year: 2016

No evidence of increased risk of soft tissue sarcomas in the neighborhood of a steel foundry in Verona

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Marta Benedetti

2010-12-01

Full Text Available The aim of the present study was to evaluate the possible association between occurrence of soft tissue sarcomas in Verona (Italy and residence near a steel foundry, whose emissions of dioxin-like compounds may be relevant. Exposure to total suspended particulate (TSP emitted from the plant as estimated by ADMS-urban dispersion model was used as an indirect index of exposure to dioxin-like compounds. Verona municipality was divided in six subareas according to the decreasing levels of estimated TSP exposure, and soft tissue sarcomas cases were mapped according to residence at time of diagnosis. Standardized incidence ratios were computed. No statistically significant departures between observed and expected cases were detected as illustrated by trend-test results.

[Soft tissue sarcomas: the role of histology and molecular pathology for differential diagnosis].

Science.gov (United States)

Poremba, C

2006-01-01

Soft tissue sarcomas include a wide spectrum of different entities. The so-called small round blue cell tumors and spindle cell tumors are difficult to classify based solely on conventional histology. To identify different subtypes of tumors special histochemical and immunohistochemical techniques are necessary. Analysis of protein expression by immunohistochemistry provides a helpful tool to investigate the histogenesis of tumors. A basic spectrum of antibodies should be included to study these tumors: Desmin and myogenin (or MyoD1) for skeletal differentiation; S-100, NSE, CD56, and synaptophysin for neural/neuroendocrine differentiation; CD3, CD20, and CD79 alpha for malignant lymphomas; CD34, sm-actin, and beta-catenin for spindle cell tumors; additional antigens, e. g. Ki-67 and p 53, for estimation of proliferation and tumor suppressor gene malfunctions. Nevertheless, the molecular analysis of soft tissue sarcomas is necessary for demonstration of specific translocations or gene defects to specify and proof a diagnosis. For this purpose, RT-PCR for RNA expression analysis of gene fusion transcripts and multi-color FISH for analysis of chromosomal rearrangements are used. Further investigations, using DNA microrrays may help to subclassify such tumors, with respect to prognosis or prediction of therapeutic response.

Intraoperative Radiation Therapy for Locally Advanced and Recurrent Soft-Tissue Sarcomas in Adults

International Nuclear Information System (INIS)

Tran, Phuoc T.; Hara, Wendy; Su Zheng; Lin, H. Jill; Bendapudi, Pavan K.; Norton, Jeffrey; Teng, Nelson; King, Christopher R.; Kapp, Daniel S.

2008-01-01

Purpose: To analyze the outcomes of and identify prognostic factors for patients treated with surgery and intraoperative radiotherapy (IORT) for locally advanced and recurrent soft-tissue sarcoma in adults from a single institution. Methods and Materials: We retrospectively reviewed 50 consecutive patients treated with IORT to 62 sites of disease. Primary sites included retroperitoneum-pelvis (78%), extremity (8%), and other (14%). Seventy percent of patients had recurrent disease failing prior surgery (70%) and/or radiation (32%). Mean disease-free interval (DFI) before IORT was 1.9 years (range, 2 weeks-5.4 years). The IORT was delivered with orthovoltage X-rays using individually sized beveled cone applicators. Clinical characteristics were as follows: mean tumor size, 10 cm (range, 1-25 cm); high-grade histologic subtype (72%); and mean dose, 1,159 cGy (range, 600-1,600 cGy). Postoperative radiation or chemotherapy was administered to 37% of IORT Sites and 32% of patients, respectively. Outcomes measured were infield control (IFC), locoregional control (LRC), distant metastasis-free survival (DMFS), disease-specific survival (DSS), and treatment-related complications. Mean and median follow-up of alive patients were 59 and 35 months, respectively. Results: Kaplan-Meier 5-year IFC, LRC, DMFS, and DSS probabilities for the entire group were 55%, 26%, 51%, and 25%, respectively. Prognostic factors found to be significant (p < 0.05) on multivariate analysis were prior DFI and tumor size for LRC, extremity location and leiomyosarcoma histologic subtype for DMFS, and prior DFI for DSS. Our cohort had five Grade 3/4 complications associated with treatment or a 5-year Kaplan-Meier Grade 3/4 complication-free survival rate of 85%. Conclusions: IORT after tumor reductive surgery is well tolerated and seems to confer IFC in carefully selected patients

Inter- and intra-observer variation in soft-tissue sarcoma target definition.

Science.gov (United States)

Roberge, D; Skamene, T; Turcotte, R E; Powell, T; Saran, N; Freeman, C

2011-08-01

To evaluate inter- and intra-observer variability in gross tumor volume definition for adult limb/trunk soft tissue sarcomas. Imaging studies of 15 patients previously treated with preoperative radiation were used in this study. Five physicians (radiation oncologists, orthopedic surgeons and a musculoskeletal radiologist) were asked to contour each of the 15 tumors on T1-weighted, gadolinium-enhanced magnetic resonance images. These contours were drawn twice by each physician. The volume and center of mass coordinates for each gross tumor volume were extracted and a Boolean analysis was performed to measure the degree of volume overlap. The median standard deviation in gross tumor volumes across observers was 6.1% of the average volume (range: 1.8%-24.9%). There was remarkably little variation in the 3D position of the gross tumor volume center of mass. For the 15 patients, the standard deviation of the 3D distance between centers of mass ranged from 0.06 mm to 1.7 mm (median 0.1mm). Boolean analysis demonstrated that 53% to 90% of the gross tumor volume was common to all observers (median overlap: 79%). The standard deviation in gross tumor volumes on repeat contouring was 4.8% (range: 0.1-14.4%) with a standard deviation change in the position of the center of mass of 0.4mm (range: 0mm-2.6mm) and a median overlap of 93% (range: 73%-98%). Although significant inter-observer differences were seen in gross tumor volume definition of adult soft-tissue sarcoma, the center of mass of these volumes was remarkably consistent. Variations in volume definition did not correlate with tumor size. Radiation oncologists should not hesitate to review their contours with a colleague (surgeon, radiologist or fellow radiation oncologist) to ensure that they are not outliers in sarcoma gross tumor volume definition. Protocols should take into account variations in volume definition when considering tighter clinical target volumes. Copyright © 2011 Société française de radioth

Soft tissue sarcoma: how can posttreatment alterations be distinguished from recurrences?; Weichteilsarkome: Wie lassen sich posttherapeutische Veraenderungen von Rezidiven unterscheiden

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Noebauer-Huhmann, I.M. [Universitaetsklinik fuer Radiologie und Nuklearmedizin, Abteilung fuer Neuroradiologie und Muskuloskeletale Radiologie, Wien (Austria); Grieser, T. [Klinikum Augsburg, Klinik fuer Diagnostische und Interventionelle Radiologie und Neuroradiologie, Augsburg (Germany)

2017-11-15

The recognition of recurrent soft tissue sarcomas and the differentiation from post-treatment alterations is complex. This article aims to assist the clinical radiologist in the systematic evaluation of local follow-up imaging in soft tissue sarcoma patients. Soft tissue sarcomas encompass multiple entities with different recurrence rates and follow-up intervals. Approved and up to date recommendations are provided, including imaging techniques. The past medical history of the patient, the clinical situation and previous therapies should be known in detail, including surgery, radiation therapy and chemotherapy. Previous imaging results should be consulted, if available. This article describes the time-dependent imaging spectrum of local post-therapeutic as well as local treatment-related complications. These include early complications, such as seromas, hematomas and infections, as well as late complications, including edema, fibrosis and joint stiffness, and also inflammatory pseudotumors, which may occur after variable time intervals. The imaging appearance of local recurrent and radiation-associated sarcoma are elucidated. In particular, magnetic resonance imaging (MRI) criteria are provided, which may help in differentiating post-therapeutic alterations from recurrent soft tissue sarcomas. (orig.) [German] Die Erkennung von Weichteilsarkomrezidiven und ihre Differenzierung von posttherapeutischen Veraenderungen sind komplex. Der Beitrag soll dem klinisch taetigen Radiologen helfen, lokale Nachkontrollen systematisch zu beurteilen. Voraussetzungen sind einerseits die genaue Kenntnis der klinischen und therapeutischen Anamnese und der aktuellen klinischen Situation sowie andererseits eine adaequate Untersuchungstechnik, fuer die Empfehlungen gegeben werden, und ein Vergleich mit der initialen Bildgebung. Der Beitrag gibt einen Ueberblick ueber Rezidivhaeufigkeiten und Therapiemodalitaeten wie der Operation, Bestrahlung und Chemotherapie. Er beschreibt das MR

Calcific haemorrhagic bursitis anterior to the knee mimicking a soft tissue sarcoma: report of two cases

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Stahnke, M.; Davies, A.M. [Department of Radiology, Royal Orthopaedic Hospital, Woodlands, B31 2AP, Northfield, Birmingham (United Kingdom); Mangham, D.C. [Department of Pathology, Royal Orthopaedic Hospital, Woodlands, B31 2AP, Northfield, Birmingham (United Kingdom)

2004-06-01

We describe the radiological and pathological findings of two cases of calcific haemorrhagic bursitis, one involving the superficial infrapatellar bursa and the other the prepatellar bursa. It was the presence of dystrophic calcification within the lesion that suggested a mineralizing soft tissue sarcoma such as synovial sarcoma. As the radiographic and MR features of the two conditions can be similar but the appropriate management very different, rare calcifying haemorrhagic bursitis needs to be included in the differential diagnosis of masses adjacent to the knee joint showing calcification. (orig.)

Calcific haemorrhagic bursitis anterior to the knee mimicking a soft tissue sarcoma: report of two cases

International Nuclear Information System (INIS)

Stahnke, M.; Davies, A.M.; Mangham, D.C.

2004-01-01

We describe the radiological and pathological findings of two cases of calcific haemorrhagic bursitis, one involving the superficial infrapatellar bursa and the other the prepatellar bursa. It was the presence of dystrophic calcification within the lesion that suggested a mineralizing soft tissue sarcoma such as synovial sarcoma. As the radiographic and MR features of the two conditions can be similar but the appropriate management very different, rare calcifying haemorrhagic bursitis needs to be included in the differential diagnosis of masses adjacent to the knee joint showing calcification. (orig.)

Improved Survival With Radiation Therapy in High-Grade Soft Tissue Sarcomas of the Extremities: A SEER Analysis

International Nuclear Information System (INIS)

Koshy, Matthew; Rich, Shayna E.; Mohiuddin, Majid M.

2010-01-01

Purpose: The benefit of radiation therapy in extremity soft tissue sarcomas remains controversial. The purpose of this study was to determine the effect of radiation therapy on overall survival among patients with primary soft tissue sarcomas of the extremity who underwent limb-sparing surgery. Methods and Materials: A retrospective study from the Surveillance, Epidemiology, and End Results (SEER) database that included data from January 1, 1988, to December 31, 2005. A total of 6,960 patients constituted the study population. Overall survival curves were constructed using the Kaplan-Meir method and for patients with low- and high-grade tumors. Hazard ratios were calculated based on multivariable Cox proportional hazards models. Results: Of the cohort, 47% received radiation therapy. There was no significant difference in overall survival among patients with low-grade tumors by radiation therapy. In high-grade tumors, the 3-year overall survival was 73% in patients who received radiation therapy vs. 63% for those who did not receive radiation therapy (p < 0.001). On multivariate analysis, patients with high-grade tumors who received radiation therapy had an improved overall survival (hazard ratio 0.67, 95% confidence interval 0.57-0.79). In patients receiving radiation therapy, 13.5% received it in a neoadjuvant setting. The incidence of patients receiving neoadjuvant radiation did not change significantly between 1988 and 2005. Conclusions: To our knowledge, this is the largest population-based study reported in patients undergoing limb-sparing surgery for soft tissue sarcomas of the extremities. It reports that radiation was associated with improved survival in patients with high-grade tumors.

Postradiation osteogenic sarcoma of bone and soft tissues. A clinicopathologic study of 66 patients

International Nuclear Information System (INIS)

Huvos, A.G.; Woodard, H.Q.; Cahan, W.G.; Higinbotham, N.L.; Stewart, F.W.; Butler, A.; Bretsky, S.S.

1985-01-01

Sixty-six patients with well-documented osteogenic sarcomas arising in bones and soft tissues after exposure to x-rays, which represent approximately 5.5 percent of all osteogenic sarcomas registered since 1921 at this institution, were studied. These secondary sarcomas occurred in equal proportion in both sexes, with the sixth decade of life being the most common age. In 42 patients, the bone had been normal at the time of irradiation, whereas in 24, the radiation was directed against an osseous tumor or tumor-like lesion. The median latent period was 10.5 years in both groups, ranging from 3.5 to 33 years. The radiation varied from diagnostic quality to 1 MeV x-rays. The dose was variable, but none was less than 2000 rads. Postradiation osteogenic sarcomas most commonly arose in the bones of the pelvic and shoulder regions. Histologically, the sarcomas were mostly of the fibrous type (46%) and radiographically showed a destructive bone lesion with or without signs of radiation osteitis. The cumulative disease-free survival rate at 5 years was 17%, with a median survival estimate of 1 year

Lung Metastasis of Primary Alveolar Soft-Part Sarcoma Occurring 20 Years after Initial Treatment

Directory of Open Access Journals (Sweden)

R. F. Falkenstern-Ge

2013-01-01

Full Text Available A 30-year old woman was referred to our center because of suspicion of a primary lung tumor of the right upper lobe. Histological examination of the lung lesion revealed lung metastasis of a previously treated alveolar soft part sarcoma of the musculus vastus medialis of the right femur, which was resected 20 years ago. Alveolar soft-part sarcoma is a rare malignant tumor that occurs most often in the soft tissue of lower limbs. It is a slow-growing malignant soft tissue tumor arising in muscle tissue, usually in young adults. Due to pleural and extensive mediastinal infiltration with bilateral lung metastases, a systemic treatment with chemotherapy doxorubicin and ifosfamide was initiated. Late metastases from previously treated alveolar part sarcoma should be considered in patients with suspicious lung lesions even if surgical treatment was performed a long time ago.

Stereotactic Radiotherapy in the Treatment of Lung Metastases from Bone and Soft-tissue Sarcomas.

Science.gov (United States)

Frakulli, Rezarta; Salvi, Fabrizio; Balestrini, Damiano; Parisi, Alessandro; Palombarini, Marcella; Cammelli, Silvia; Rocca, Michele; Salone, Mariacristina; Longhi, Alessandra; Ferrari, Stefano; Morganti, Alessio G; Frezza, Giovanni

2015-10-01

The purpose of this study was to evaluate local control and toxicity in a group of patients treated with stereotactic body radiotherapy (SBRT) for lung metastases (LM) from bone and soft tissue sarcomas. From October 2010 to July 2014, patients with LM from sarcomas not suitable for surgery were treated with daily cone-beam computed tomography-guided SBRT. The dose administered ranged from 30 to 60 Gy in 3-8 fractions. Acute and late toxicity were scored according to Common Terminology Criteria for Adverse Events version 4.0. A total of 24 patients with 68 LM from sarcomas were treated with SBRT. The median follow-up after SBRT was 17 months (range=11-51 months). Two-year actuarial lesion local control and overall survival were 85.9% and 66.4%, respectively. No G3 or greater acute and late toxicities were observed. SBRT is a safe and effective treatment for LM from sarcoma and might be used as an alternative option in patients unfit for surgery. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

A prognostic profile of hypoxia-induced genes for localised high-grade soft tissue sarcoma

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Aggerholm-Pedersen, Ninna; Sørensen, Brita Singers; Overgaard, Jens

2016-01-01

sarcoma (STS). METHODS: The hypoxia-induced gene quantification was performed by real-time quantitative PCR (RT-qPCR) of formalin-fixed, paraffin-embedded tissue samples. The gene expression cut-points were determined in a test cohort of 55 STS patients and used to allocate each patient into a more......BACKGROUND: For decades, tumour hypoxia has been pursued as a cancer treatment target. However, prognostic and predictive biomarkers are essential for the use of this target in the clinic. This study investigates the prognostic value of a hypoxia-induced gene profile in localised soft tissue...

Combining Targeted Agents With Modern Radiotherapy in Soft Tissue Sarcomas

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Wong, Philip; Houghton, Peter; Kirsch, David G.; Finkelstein, Steven E.; Monjazeb, Arta M.; Xu-Welliver, Meng; Dicker, Adam P.; Ahmed, Mansoor; Vikram, Bhadrasain; Teicher, Beverly A.; Coleman, C. Norman; Machtay, Mitchell; Curran, Walter J.

2014-01-01

Improved understanding of soft-tissue sarcoma (STS) biology has led to better distinction and subtyping of these diseases with the hope of exploiting the molecular characteristics of each subtype to develop appropriately targeted treatment regimens. In the care of patients with extremity STS, adjunctive radiation therapy (RT) is used to facilitate limb and function, preserving surgeries while maintaining five-year local control above 85%. In contrast, for STS originating from nonextremity anatomical sites, the rate of local recurrence is much higher (five-year local control is approximately 50%) and a major cause of death and morbidity in these patients. Incorporating novel technological advancements to administer accurate RT in combination with novel radiosensitizing agents could potentially improve local control and overall survival. RT efficacy in STS can be increased by modulating biological pathways such as angiogenesis, cell cycle regulation, cell survival signaling, and cancer-host immune interactions. Previous experiences, advancements, ongoing research, and current clinical trials combining RT with agents modulating one or more of the above pathways are reviewed. The standard clinical management of patients with STS with pretreatment biopsy, neoadjuvant treatment, and primary surgery provides an opportune disease model for interrogating translational hypotheses. The purpose of this review is to outline a strategic vision for clinical translation of preclinical findings and to identify appropriate targeted agents to combine with radiotherapy in the treatment of STS from different sites and/or different histology subtypes. PMID:25326640

Prognostic value of proliferation in pleomorphic soft tissue sarcomas

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Seinen, Jojanneke M; Jönsson, Mats; Bendahl, Pär-Ola O

2012-01-01

= 1.6-12.1), Top2a (hazard ratio = 2.2, CI = 1.2-3.5) and high S-phase fraction (hazard ratio = 1.8, CI = 1.2-3.7) significantly correlated with risk for metastasis. When combined with currently used prognostic factors, Ki-67, S-phase fraction and Top2a fraction contributed to refined identification...... of prognostic risk groups. Proliferation, as assessed by expression of Ki-67 and Top2a and evaluation of S-phase fraction and applied to statistical decision-tree models, provides prognostic information in soft tissue sarcomas of the extremity and trunk wall. Though proliferation contributes independently...... to currently applied prognosticators, its role is particularly strong when few other factors are available, which suggests a role in preoperative decision-making related to identification of high-risk individuals who would benefit from neoadjuvant therapy....

Hypoxia-inducible factor 1α predicts recurrence in high-grade soft tissue sarcoma of extremities and trunk wall

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Nyström, H; Jönsson, M; Werner-Hartman, L

2017-01-01

BACKGROUND AND AIM: Sarcomas are of mesenchymal origin and typically show abundant tumour stroma and presence of necrosis. In search for novel biomarkers for personalised therapy, we determined the prognostic impact of stromal markers, hypoxia and neovascularity in high-grade soft tissue leiomyos...

Analysis of Dietary Intake during Consecutive-Day Chemotherapy for Bone and Soft-Tissue Sarcomas

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Yuta Hori

2018-01-01

Full Text Available BackgroundBone and soft tissue sarcomas are commonly treated with consecutive-day chemotherapy regimens consisting of multiple anticancer agents. Chemotherapy-induced nausea and vomiting (CINV is a serious adverse effect of these regimens and may result in decreased energy intake during chemotherapy. Decreased energy intake may lead to undernutrition and may cause adverse effects on patient quality of life and survival.MethodsPatients with bone and soft tissue sarcomas who received consecutive-day chemotherapy were retrospectively evaluated. CINV and dietary energy intake were assessed, as well as the occurrences of hiccups and constipation during chemotherapy.ResultsA total of 13 patients, 10 males and 3 females, with a total 16 chemotherapy courses were included in the study. All patients received antiemetic prophylaxis. The CINV control rate, defined as no emesis and no rescue therapy, gradually decreased from chemotherapy day 1 (94% to day 5 (75%. Four patients experienced emesis, two of whom had been treated with a cisplatin-containing regimen. Decreased dietary energy intake was possibly associated with CINV during chemotherapy. Anorexia was grade 2 except for one case of grade 3. The incidences of hiccups and constipation were high on days 3–5.ConclusionAntiemetic prophylaxis treatment did not prevent emesis due to consecutive-day chemotherapy, especially with cisplatin-containing regimens, in patients with bone and soft-tissue tumors. Dietary energy intake decreased during chemotherapy, and this appeared to be associated with CINV. In addition, the incidence of hiccups and constipation increased during the course of consecutive-day chemotherapy regimens. Although these results are based on a small number of patients, it may be important to observe nutritional status during chemotherapy, as this may reflect a patient’s general condition. Nutritional counseling might be useful in supporting nutritional status in patients undergoing

Synovial sarcoma

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Sucari S.C. Vlok

2014-12-01

Full Text Available Synovial sarcoma is a malignant, predominantly juxta-articular, soft-tissue tumour representing approximately 10% of all soft-tissue sarcomas. Frequently initially incorrectly diagnosed as a benign lesion, it should be considered as a diagnosis when a young adult patient presents with a calcified juxta-articular soft-tissue mass of insidious onset.

Statistics of bone sarcoma in Japan: Report from the Bone and Soft Tissue Tumor Registry in Japan.

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Ogura, Koichi; Higashi, Takahiro; Kawai, Akira

2017-01-01

No previous reports to date have characterized the national profiles of bone sarcoma overall. In the present study, we aimed to describe the nationwide statistics of bone sarcoma in Japan by analyzing data from the Bone and Soft Tissue Tumor (BSTT) Registry in Japan, which is a nationwide organ-specific cancer registry for bone and soft tissue tumor. We identified 2773 patients with bone sarcomas using the BSTT Registry during 2006-2012. We extracted the data regarding patient demographics, treatment, and prognosis at the last follow-up for each patient. There was a slight male preponderance. The age distribution had 2 peaks overall: one in the second decade and the other in the sixth to seventh decade with the proportion of the elderly patients over 60 years approximately 30%. The most frequent tumor locations were the lower extremity (N = 1342; 48.4%) and the trunk (N = 1038; 37.4%). We also showed the significant association between disease-specific survival and patient's age, histologic grade and subtype, tumor size and location, and limb salvage status based on 1401 patients with bone sarcoma, and demonstrated the worst disease-specific survival in the elderly patients. The present study is the first study to have analyzed data from the BSTT Registry and has provided an overview of the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of patients with bone sarcoma in Japan based on cases assumed to have received relatively uniform treatment strategies. It is essential to document our data regarding the outcomes of elderly patients so that other countries showing similar population aging trends can learn from our experiences. Copyright © 2016 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.

Prevalence of bone and soft tissue tumors.

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Yücetürk, Güven; Sabah, Dündar; Keçeci, Burçin; Kara, Ahmet Duran; Yalçinkaya, Selçuk

2011-01-01

Multidisciplinary approach is a necessity for the appropriate diagnosis and treatment of bone and soft tissue tumors. The Ege University Musculoskeletal Tumor Council offers consultation services to other hospitals in the Aegean region. Since 1988 the Council has met weekly and spent approximately 1,500 hours evaluating almost 6,000 patients with suspected skeletal system tumors. Our objective was to present the data obtained from this patient group. A total of 5,658 patients, suspected to have a musculoskeletal tumor, were evaluated retrospectively. Multiple records of the patients due to multiple attendance to the Council were excluded. The prevalance of the bone and soft tissue tumors in these patients were analysed. Malignant mesenchymal tumors accounted for 39.7% of the total patients, benign tumors for 17%, tumor-like lesions for 17.8% and metastatic carsinomas for 8.6%. Malignant bone tumors were 50.2% and malignant soft tissue tumors were 49.8% of all the sarcomas. Among the malignant bone tumors the most common was osteosarcomas at a rate of 33.6%, followed by Ewing-PNET at 25.5%, chondrosarcomas at 19.4% and haematopoietic tumors at 17.6%. Pleomorphic sarcomas (24.5%), liposarcoma (16.4%), synovial sarcoma (13%) and undifferential sarcomas (8.8%) were the most common types of malignant sof tissue tumors. Benign soft tissue tumors (48%), benign cartilage tumors (28%), giant cell tumor (15%) and osteogenic tumors (9%) were found among the benign tumors. Hemangioma, lipoma, agressive fibromatosis, enchondroma, solitary chondroma and osteoid osteoma were the most common tumors in their groups. Lung (27%), breast (24%), gastrointestinal system (10.5%) and kidney (8.2%) carcinomas were the most common primary sites of the bone metastasis. Turkey still lacks a comprehensive series indicating the incidence and diagnostic distribution of bone and soft tissue tumors. The presented data would add to our knowledge on the specific rates of the bone and soft tissue

Diagnostic value of 18F-FDG-PET/CT for the follow-up and restaging of soft tissue sarcomas in adults.

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Kassem, T W; Abdelaziz, O; Emad-Eldin, S

2017-10-01

The purpose of this study was to evaluate the clinical utility of 2-[ 18 F] fluoro-2-deoxy-D-glucose ( 18 FDG) positron emission tomography (PET)/computed tomography (CT) ( 18 F-FDG-PET/CT) in the follow-up of adult patients with soft tissue sarcomas. We prospectively evaluated 37 consecutive patients with known soft tissue sarcoma with 18 F-FDG-PET/CT examination for suspected recurrence of disease. They were 21 men and 16 women with a mean age of 49.6±10.6 (SD) years (range, 34-75years). The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of 18 F-FDG-PET/CT examination were calculated on a per patient basis. 18 F-FDG-PET/CT showed an overall diagnostic accuracy of 91.8%, sensitivity of 90% and a specificity of 100%. The positive predictive value and negative predictive value were 100 and 70%, respectively. The 18 F-FDG-PET/CT interpretations were correct in 34/37 patients (91.8%). Incorrect interpretations occurred in three patients (8.1%). Reasons for false negative findings were low 18 F-FDG uptake of local recurrence in one patient and low 18 F-FDG uptake of subcentimetric inguinal lymph node metastases. 18 F-FDG-PET/CT has a high diagnostic value in the follow-up of patients with soft tissue sarcoma. Copyright © 2017 Editions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

On clinical usefulness of Tl-201 scintigraphy for the management of malignant soft tissue tumors

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Terui, Shoji; Terauchi, Takashi; Abe, Hiroyuki; Fukuma, Hisatoshi; Beppu, Yasuo; Chuman, Koichi; Yokoyama, Ryohei

1994-01-01

The purpose of this study was to investigate Tl-201 as a tumor scanning agent in patients with malignant soft tissue sarcomas and to establish the sensitivity of this type of scintigraphy concerning local recurrences or metastases that may remain clinically suspected. Seventy-eight patients with malignant soft tissue sarcomas and 22 with benign soft tissue tumors were studied. Of these 78 malignant soft tissue sarcomas patients, the sensitivity of Tl-201 (81.2%) was higher than that of Ga-67 (68.8%). Thirty-three out of 78 patients received a total of 95 consecutive scintigraphic follow-up examinations. Therapeutic effects was assessed by comparing the results of Tl-201 examinations with the clinical findings. Of these 33 patients, the therapeutic effects observed were as follows: complete remission 1, partial remission 8, progress of disease 1, and no remarkable change 23. Tl-201 scintigraphy has proved itself very useful not only in clinically detecting the malignant soft tissue sarcomas and in assessing therapeutic effects on these diseases, but also in assessing the follow-up patients with malignant soft tissue sarcomas. (author)

Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology.

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von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Keedy, Vicki; Kim, Edward; Koon, Henry; Mayerson, Joel; McCarter, Martin; McGarry, Sean V; Meyer, Christian; Morris, Zachary S; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Petersen, Ivy A; Pfeifer, John D; Riedel, Richard F; Ruo, Bernice; Schuetze, Scott; Tap, William D; Wayne, Jeffrey D; Bergman, Mary Anne; Scavone, Jillian L

2018-05-01

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations. Copyright © 2018 by the National Comprehensive Cancer Network.

Evaluation of Soft Tissue Sarcoma Tumors Electrical Conductivity Anisotropy Using Diffusion Tensor Imaging for Numerical Modeling on Electroporation

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Ghazikhanlou-sani K.

2016-06-01

Full Text Available Introduction: There is many ways to assessing the electrical conductivity anisotropy of a tumor. Applying the values of tissue electrical conductivity anisotropy is crucial in numerical modeling of the electric and thermal field distribution in electroporation treatments. This study aims to calculate the tissues electrical conductivity anisotropy in patients with sarcoma tumors using diffusion tensor imaging technique. Materials and Method: A total of 3 subjects were involved in this study. All of patients had clinically apparent sarcoma tumors at the extremities. The T1, T2 and DTI images were performed using a 3-Tesla multi-coil, multi-channel MRI system. The fractional anisotropy (FA maps were performed using the FSL (FMRI software library software regarding the DTI images. The 3D matrix of the FA maps of each area (tumor, normal soft tissue and bone/s was reconstructed and the anisotropy matrix was calculated regarding to the FA values. Result: The mean FA values in direction of main axis in sarcoma tumors were ranged between 0.475–0.690. With assumption of isotropy of the electrical conductivity, the FA value of electrical conductivity at each X, Y and Z coordinate axes would be equal to 0.577. The gathered results showed that there is a mean error band of 20% in electrical conductivity, if the electrical conductivity anisotropy not concluded at the calculations. The comparison of FA values showed that there is a significant statistical difference between the mean FA value of tumor and normal soft tissues (P<0.05. Conclusion: DTI is a feasible technique for the assessment of electrical conductivity anisotropy of tissues. It is crucial to quantify the electrical conductivity anisotropy data of tissues for numerical modeling of electroporation treatments.

Feasibility of combined modality therapy for localized high-grade soft tissue sarcomas in adults

International Nuclear Information System (INIS)

Blum, R.H.; Greenberger, J.S.; Wilson, R.E.; Corson, J.M.

1979-01-01

Seventeen consecutive patients with localized, high grade soft tissue sarcomas had resection of their primary tumor, radiation therapy and chemotherapy. The soft tissue sarcoma was primary in 14 patients and regionally recurrent in 3 patients. Chemotherapy consisted of cyclophosphamide 500 mg/M 2 day 1, Adriamycin (ADR) 60 mg/M 2 day 2, and DTIC 400 mg/M 2 days 1 and 2, given every 21 days to a maximum ADR dose of 450 mg/M 2 . Cyclophosphamide and DTIC were then given to a total duration of 1 year. Radiation therapy consisted of 4000 to 5000 rad by megavoltage photons in 5 weeks, and in selected cases, an additional 1500 to 2000 rad by electron beam boost in the tumor bed delivered over 2 additional weeks. Following surgery, 12 patients were treated sequentially with an interval of chemotherapy, radiation therapy and then the completion of chemotherapy. The added morbidity of this sequential approach is minimal: one patient of 12 had delayed primary healing of her wound, 1 of 10 patients required a break in radiation therapy because of skin erythema. Four patients were treated with intensive pre-chemotherapy radiation therapy because of inadequate surgical margins. The median time on study was 18 months from onset of treatment (range, 8 to 41 months). Although there have been no local, regional or distant recurrences, the follow-up time is inadequate to assess the therapeutic benefit of this combined modality treatment

Multiple cerebral metastasis of alveolar soft-part sarcoma in the retroperitoneum

International Nuclear Information System (INIS)

Ishizaka, Hiroaki; Mori, Kazuo; Moroki, Jiro; Miyake, Hitoshi; Kamei, Toshiaki.

1987-01-01

Alveolar soft-part sarcoma is a rare disease which has thus for been recognized as a malignant soft tissue tumor only with much uncertainty. This report describes a 29-year-old man with multiple cerebral metastasis of alveolar soft-part sarcoma, presumably originating in the retroperitoneum. He was admitted to our hospital with complaints of severe headache, nausea, and left homonymous hemianopsia. A CT scan showed a slight high-density area, with a marked peritumoral low density in the right occipital lobe. Vertebral angiography showed a tumor stain which appeared in the early arterial phase and lasted to the late venous one. Arteriovenous shunting was also remarkable. In the enhanced CT, a small high-density area was also noted in the left frontal lobe. Right occipital craniotomy was carried out, and a total removal was performed. Specimens of the tumor showed typical features of alveolar soft-part sarcoma histologically. Whole-brain radiotherapy-chemotherapy did not show any definite effect. A second operation for the left frontal tumor was carried out five months later. Histological and electron-microscopical examination again showed typical features of alveolar soft-part sarcoma. Regarding the choice of the treatment of this tumor, we recommended total removal of the tumor as the best management. (author)

Results of total lung irradiation and chemotherapy in comparison with partial lung irradiation in metastatic undifferentiated soft tissue sarcomas

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Zamboglou, N.; Fuerst, G.; Pape, H.; Bannach, B.; Schmitt, G.; Molls, M.

1988-07-01

The poor prognosis of patients with unresectable pulmonary metastases of soft tissue sarcoma is well known. In order to evaluate the beneficial effect of radiotherapy, we have treated 44 patients with pulmonary metastases of grade 3 soft tissue sarcoma from 1980 to 1986. In 36 patients the treatment volume was restricted to the single metastases up to a dose of 50 to 60 (9 to 10 Gy/week). The survival rate at one year was 18% and at two years 6%. Eight patients were treated with a combined regimen, consisting of cisplatin and ifosfamide with simultaneous whole lung irradiation. Irradiation was performed with 8 or 16 MV photons at a hyperfractionation of 2x0,8 Gy/day (8 Gy/week). After a dose of 12 Gy, the single metastases were boosted up to 50 to 60 Gy, with a second course of chemotherapy. In six of eight patients complete remissions were achieved, one patient showed a partial remission. The survival rate at 27 months was 50%. The patients with partial remission died from pulmonary progression at 23 months. One patient died after twelve months from a loco-regional recurrence in the tonsillar fossa without evidence of pulmonary disease. Side effects included alopecia and moderate bone marrow suppression approximately twelve days after each chemotherapy cycle. Pulmonary fibrosis was observed only at the high dose volume without impairment of respiratory function. From these observations the conclusion is drawn that whole lung irradiation simultaneously with cisplatin and ifosfamide chemotherapy provides good palliative results without relevant morbidity in patients with high grade unresectable pulmonary metastases of soft tissue sarcomas.

Exposure-survival analyses of pazopanib in renal cell carcinoma and soft tissue sarcoma patients: opportunities for dose optimization.

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Verheijen, R B; Swart, L E; Beijnen, J H; Schellens, J H M; Huitema, A D R; Steeghs, N

2017-12-01

Pazopanib is an angiogenesis inhibitor approved for the treatment of renal cell carcinoma and soft tissue sarcoma. Post hoc analysis of a clinical trial demonstrated a relationship between pazopanib trough concentrations (C min ) and treatment efficacy. The aim of this study was to explore the pharmacokinetics and exposure-survival relationships of pazopanib in a real-world patient cohort. Renal cell cancer and soft tissue sarcoma patients who had at least one pazopanib plasma concentration available were included. Using calculated C min values and a threshold of > 20 mg/L, univariate and multivariate exposure-survival analyses were performed. Sixty-one patients were included, of which 16.4% were underexposed (mean C min   20 mg/L was related to longer progression free survival in renal cell cancer patients (34.1 vs. 12.5 weeks, n = 35, p = 0.027) and the overall population (25.0 vs. 8.8 weeks, n = 61, p = 0.012), but not in the sarcoma subgroup (18.7 vs. 8.8 weeks, n = 26, p = 0.142). In multivariate analysis C min  > 20 mg/L was associated with hazard ratios of 0.25 (p = 0.021) in renal cancer, 0.12 (p = 0.011) in sarcoma and 0.38 (p = 0.017) in a pooled analysis. This study confirms that pazopanib C min  > 20 mg/L relates to better progression free survival in renal cancer and points towards a similar trend in sarcoma patients. C min monitoring of pazopanib can help identify patients with low C min for whom individualized treatment at a higher dose may be appropriate.

Clinical implications of determination of safe surgical margins by using a combination of CT and 18FDG-positron emission tomography in soft tissue sarcoma

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Yoshioka Takako

2011-07-01

Full Text Available Abstract Background To determine safe surgical margins for soft tissue sarcoma, it is essential to perform a general evaluation of the extent of tumor, responses to auxiliary therapy, and other factors preoperatively using multiple types of diagnostic imaging. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT is a tool for diagnostic imaging that has recently spread rapidly in clinical use. At present, the roles played by FDG-PET/CT in determination of margins for surgical resection of sarcoma are unclear. The present study was undertaken to explore the roles of FDG-PET/CT in determination of surgical margins for soft tissue sarcoma and to examine whether PET can serve as a standard means for setting the margins of surgical resection during reduced surgery. Methods The study involved 7 patients with sarcoma who underwent surgery in our department and in whom evaluation with FDG-PET/CT was possible. Sarcoma was histologically rated as MFH in 6 cases and leiomyosarcoma in 1 case. In all cases, sarcoma was superficial (T1a or T2a. The tumor border was defined by contrast-enhanced MRI, and SUVs were measured at intervals of 1 cm over a 5-cm long area from the tumor border. Mapping of viable tumor cells was carried out on whole-mount sections of resected tissue, and SUVs were compared with histopathological findings. Results Preoperative maximum SUVs (SUV-max of the tumor averaged 11.7 (range: 3.8-22.1. Mean SUV-max was 2.2 (range: 0.3-3.8 at 1 cm from the tumor border, 1.1 (0.85-1.47 at 2 cm, 0.83 (0.65-1.15 at 3 cm, 0.7 (0.42-0.95 at 4 cm, and 0.64 (0.45-0.82 at 5 cm. When resected tissue was mapped, tumor cells were absent in the areas where SUV-max was below 1.0. Conclusions Our findings suggest that a safe surgical margin free of viable tumor cells can be ensured if the SUV cut-off level is set at 1.0. FDG-PET/CT is promising as a diagnostic imaging technique for setting of safe minimal margins for surgical

Phase 1/2 study of immunotherapy with dendritic cells pulsed with autologous tumor lysate in patients with refractory bone and soft tissue sarcoma.

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Miwa, Shinji; Nishida, Hideji; Tanzawa, Yoshikazu; Takeuchi, Akihiko; Hayashi, Katsuhiro; Yamamoto, Norio; Mizukoshi, Eishiro; Nakamoto, Yasunari; Kaneko, Shuichi; Tsuchiya, Hiroyuki

2017-05-01

There are limited options for the curative treatment of refractory bone and soft tissue sarcomas. The purpose of this phase 1/2 study was to assess the immunological and clinical effects of dendritic cells (DCs) pulsed with autologous tumor lysate (TL) in patients with advanced bone and soft tissue sarcomas. Thirty-seven patients with metastatic or recurrent sarcomas were enrolled in this study. Peripheral blood mononuclear cells obtained from the patients were suspended in media containing interleukin 4 (IL-4) and granulocyte-macrophage colony-stimulating factor. Subsequently, these cells were treated with TL, tumor necrosis factor α, and OK-432. The DCs were injected into the inguinal or axillary region. One treatment course comprised 6 weekly DC injections. The toxicity, clinical response (tumor volume, serum interferon-γ [IFN-γ], and serum IL-12), and oncological outcomes were observed. In total, 47 courses of DC therapy were performed in 37 patients. No severe adverse events or deaths associated with the DC injections were observed in the study patients. Increased serum IFN-γ and IL-12 levels were observed 1 month after the DC injection. Among the 37 patients, 35 patients were assessed for clinical responses: 28 patients showed tumor progression, 6 patients had stable disease, and 1 patient showed a partial response 8 weeks after the DC injection. The 3-year overall and progression-free survival rates of the patients were 42.3% and 2.9%, respectively. Although DC therapy appears safe and resulted in an immunological response in patients with refractory sarcoma, it resulted in an improvement of the clinical outcome in only a small number of patients. Cancer 2017;123:1576-1584. © 2017 American Cancer Society. © 2017 American Cancer Society.

Tumor-targeting Salmonella typhimurium A1-R is a highly effective general therapeutic for undifferentiated soft tissue sarcoma patient-derived orthotopic xenograft nude-mouse models.

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Igarashi, Kentaro; Kawaguchi, Kei; Kiyuna, Tasuku; Miyake, Kentaro; Miyake, Masuyo; Singh, Arun S; Eckardt, Mark A; Nelson, Scott D; Russell, Tara A; Dry, Sarah M; Li, Yunfeng; Yamamoto, Norio; Hayashi, Katsuhiro; Kimura, Hiroaki; Miwa, Shinji; Tsuchiya, Hiroyuki; Singh, Shree Ram; Eilber, Fritz C; Hoffman, Robert M

2018-03-18

Undifferentiated soft tissue sarcoma (USTS) is a recalcitrant and heterogeneous subgroup of soft tissue sarcoma with high risk of metastasis and recurrence. Due to heterogeneity of USTS, there is no reliably effective first-line therapy. We have generated tumor-targeting Salmonella typhimurium A1-R (S. typhimurium A1-R), which previously showed strong efficacy on single patient-derived orthotopic xenograft (PDOX) models of Ewing's sarcoma and follicular dendritic cell sarcoma. In the present study, tumor resected from 4 patients with a biopsy-proven USTS (2 undifferentiated pleomorphic sarcoma [UPS], 1 undifferentiated sarcoma not otherwise specified [NOS] and 1 undifferentiated spindle cell sarcoma [USS]) were grown orthotopically in the biceps femoris muscle of mice to establish PDOX models. One USS model and one UPS model were doxorubicin (DOX) resistant. One UPS and the NOS model were partially sensitive to DOX. DOX is first-line therapy for these diseases. S. typhimurium A1-R arrested tumor growth all 4 models. In addition to arresting tumor growth in each case, S. typhimurium A1-R was significantly more efficacious than DOX in each case, thereby surpassing first-line therapy. These results suggest that S. typhimurium A1-R can be a general therapeutic for USTS and possibly sarcoma in general. Published by Elsevier Inc.

Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas

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Chang, A.E.; Steinberg, S.M.; Culnane, M.; Lampert, M.H.; Reggia, A.J.; Simpson, C.G.; Hicks, J.E.; White, D.E.; Yang, J.J.; Glatstein, E. (National Cancer Institute, Bethesda, MD (USA))

1989-09-01

We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone.

Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas

International Nuclear Information System (INIS)

Chang, A.E.; Steinberg, S.M.; Culnane, M.

1989-01-01

We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone

Evaluation of Quality of Life at Progression in Patients with Soft Tissue Sarcoma

Directory of Open Access Journals (Sweden)

Stacie Hudgens

2017-01-01

Full Text Available Introduction. Soft Tissue Sarcoma (STS is a rare malignancy of mesodermal tissue, with international incidence estimates between 1.8 and 5 per 100,000 per year. Understanding quality of life (QoL and the detrimental impact of disease progression is critical for long-term care and survival. Objectives. The primary objective was to explore the relationship between disease progression and health-related quality of life (HRQoL using data from Eisai’s study (E7389-G000-309. Methods. This was a 1 : 1 randomized, open-label, multicenter, Phase 3 study comparing the efficacy and safety of eribulin versus dacarbazine in patients with advanced STS. The QoL analysis was conducted for the baseline and progression populations using the European Organization for Research and Treatment of Cancer 30-item core QoL questionnaire (EORTC QLQ-C30. Results. There were no statistical differences between the two treatment arms at baseline for any domain (p>0.05; n=452. Of the 399 patients who experienced disease progression (unadjusted and adjusting for histology, dacarbazine patients had significantly lower Global Health Status, Physical Functioning scores, and significantly worse Nausea and Vomiting, Insomnia, and Appetite Loss (p<0.05. Conclusions. These results indicate differences in HRQoL overall and at progression between dacarbazine and eribulin patients, with increases in symptom severity observed among dacarbazine patients.

Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study

DEFF Research Database (Denmark)

Issels, Rolf D; Lindner, Lars H; Verweij, Jaap

2010-01-01

The optimum treatment for high-risk soft-tissue sarcoma (STS) in adults is unclear. Regional hyperthermia concentrates the action of chemotherapy within the heated tumour region. Phase 2 studies have shown that chemotherapy with regional hyperthermia improves local control compared with chemother...

Volume fractions of DCE-MRI parameter as early predictor of histologic response in soft tissue sarcoma: A feasibility study.

Science.gov (United States)

Xia, Wei; Yan, Zhuangzhi; Gao, Xin

2017-10-01

To find early predictors of histologic response in soft tissue sarcoma through volume transfer constant (K trans ) analysis based on dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). 11 Patients with soft tissue sarcoma of the lower extremity that underwent preoperative chemoradiotherapy followed by limb salvage surgery were included in this retrospective study. For each patient, DCE-MRI data sets were collected before and two weeks after therapy initiation, and histologic tumor cell necrosis rate (TCNR) was reported at surgery. The DCE-MRI volumes were aligned by registration. Then, the aligned volumes were used to obtain the K trans variation map. Accordingly, three sub-volumes (with increased, decreased or unchanged K trans ) were defined and identified, and fractions of the sub-volumes, denoted as F + , F - and F 0 , respectively, were calculated. The predictive ability of volume fractions was determined by using area under a receiver operating characteristic curve (AUC). Linear regression analysis was performed to investigate the relationship between TCNR and volume fractions. In addition, the K trans values of the sub-volumes were compared. The AUC for F - (0.896) and F 0 (0.833) were larger than that for change of tumor longest diameter ΔD (0.625) and the change of mean K trans ΔK trans ¯ (0.792). Moreover, the regression results indicated that TCNR was directly proportional to F 0 (R 2 =0.75, P=0.0003), while it was inversely proportional to F - (R 2 =0.77, P=0.0002). However, TCNR had relatively weak linear relationship with ΔK trans ¯ (R 2 =0.64, P=0.0018). Additionally, TCNR did not have linear relationship with DD (R 2 =0.16, P=0.1246). The volume fraction F - and F 0 have potential as early predictors of soft tissue sarcoma histologic response. Copyright © 2017 Elsevier B.V. All rights reserved.

High expression of CD109 antigen regulates the phenotype of cancer stem-like cells/cancer-initiating cells in the novel epithelioid sarcoma cell line ESX and is related to poor prognosis of soft tissue sarcoma.

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Makoto Emori

Full Text Available Epithelioid sarcoma (ES is a relatively rare, highly malignant soft tissue sarcoma. The mainstay of treatment is resection or amputation. Currently other therapeutic options available for this disease are limited. Therefore, a novel therapeutic option needs to be developed. In the present study, we established a new human ES cell line (ESX and analyzed the characteristics of its cancer stem-like cells/cancer-initiating cells (CSCs/CICs based on ALDH1 activity. We demonstrated that a subpopulation of ESX cells with high ALDH1 activity (ALDH(high cells correlated with enhanced clonogenic ability, sphere-formation ability, and invasiveness in vitro and showed higher tumorigenicity in vivo. Next, using gene expression profiling, we identified CD109, a GPI-anchored protein upregulated in the ALDH(high cells. CD109 mRNA was highly expressed in various sarcoma cell lines, but weakly expressed in normal adult tissues. CD109-positive cells in ESX predominantly formed spheres in culture, whereas siCD109 reduced ALDH1 expression and inhibited the cell proliferation in vitro. Subsequently, we evaluated the expression of CD109 protein in 80 clinical specimens of soft tissue sarcoma. We found a strong correlation between CD109 protein expression and the prognosis (P = 0.009. In conclusion, CD109 might be a CSC/CIC marker in epithelioid sarcoma. Moreover, CD109 is a promising prognostic biomarker and a molecular target of cancer therapy for sarcomas including ES.

Children’s Oncology Group’s 2013 Blueprint for Research: Soft Tissue Sarcomas

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Hawkins, Douglas S.; Spunt, Sheri L.; Skapek, Stephen X.

2013-01-01

In the US, approximately 850-900 children are diagnosed each year with soft tissue sarcomas (STS). Key findings from recent clinical trials include safe reduction in therapy for low risk rhabdomyosarcoma (RMS), validation of FOXO1 fusion as a prognostic factor, a modest improvement in outcome for high-risk RMS, and a biologically-designed non-cytotoxic therapy for pediatric desmoid tumor. Planned Phase 2 trials include targeted agents for VEGF/PDGF, mTOR, and IGF-1R for children with RMS and VEGF for children with non-RMS STS (NRSTS). For RMS, COG Phase 3 trials potentially will explore VEGF/mTOR inhibition or chemotherapy interval compression. For NRSTS, a COG Phase 3 trial will explore VEGF inhibition. PMID:23255356

Head and neck soft tissue sarcomas treated with radiation therapy

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Lucas K. Vitzthum

2016-06-01

Full Text Available Head and neck soft tissue sarcomas (HNSTSs are rare and heterogeneous cancers in which radiation therapy (RT has an important role in local tumor control (LC. The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC, disease-free survival (DFS, overall survival (OS, and predictors thereof were assessed. Forty-eight patients with HNSTS were evaluated. Five-year Kaplan-Meier estimates of LC, LRC, DFS, and OS were 87, 73, 63, and 83%, respectively. Angiosarcomas were found to be associated with worse LC, LRC, DFS, and OS. Patients over the age of 60 had lower rates of DFS. HNSTSs comprise a diverse group of tumors that can be managed with various treatment regimens involving RT. Angiosarcomas have higher recurrence and mortality rates.

Brachytherapy in the conservative treatment of soft tissue sarcomas extending to neurovascular structures: an analysis of 38 cases

International Nuclear Information System (INIS)

Thomas, L.; Delannes, M.; Stoeckle, E.; Martel, P.; Pigneux, J.; Daly-Schveitzer, N.; Bui, B.N.; Chevreau, C.; Kantor, G.

1996-01-01

To evaluate the tolerance of neurovascular structures to brachytherapy, a retrospective review of our series was undertaken. Between May 1986 and January 1994, 85 patients with soft tissue sarcomas underwent conservative surgery and low-dose rate interstitial irradiation. Thirty-eight patients had tumors extending to neurovascular structures. Brachytherapy was part of initial treatment in 30 patients and was done in 7 cases for recurrent sarcomas. Afterloading catethers for brachytherapy were inserted intraoperatively and placed direct upon or under the neurovascular structures in the tumor bed. A mean dose of 20 Gy was delivered to the target volume. Thirty patients received 45 to 50 Gy of postoperative external irradiation. With a median follow-up of 39 months, the 3-year actuarial survival was 82.9%, the 3-year disease-free survival was 71.9% and the 3-year actuarial local control was 91%. The 3-year actuarial incidence of distant metastase was 28%. Acute side effects occurred in 12 patients requiring conservative surgical procedures in 6 cases. Significant late toxicity occurred in 8 patients : 2 lymphoedemas interfering with normal activity, 1 partial artery stenosis, 5 peripheral neuropathy (2 grade 2, 3 grade 3). Late toxicity has led to significant impairment of mobility in 4 patients. Limb preservation was achieved in every patient, no amputation was required. We conclude that integration of brachytherapy in the conservative treatment of soft tissue sarcomas extending to neurovascular structures can provide excellent local control with an acceptable level of toxicity

Fast neutron radiotherapy for soft tissue and cartilaginous sarcomas at high risk for local recurrence

International Nuclear Information System (INIS)

Schwartz, David L.; Einck, John; Bellon, Jennifer; Laramore, George E.

2001-01-01

Purpose: The practice policy at the University of Washington has been to employ fast neutron radiotherapy for soft tissue sarcoma lesions with prognostic features predictive for poor local control. These include gross residual disease/inoperable disease, recurrent disease, and contaminated surgical margins. Cartilaginous sarcomas have also been included in this high-risk group. This report updates and expands our previously described experience with this approach. Methods and Materials: Eighty-nine soft tissue sarcoma lesions in 72 patients were treated with neutron radiotherapy in our department between 1984 and 1996. Six patients, each with solitary lesions, were excluded from analysis due to lack of follow-up. Seventy-three percent were treated with fast neutron radiation alone, the rest with a combination of neutrons and photons. Median neutron dose was 18.3 nGy (range 4.8-22). Forty-two patients with solitary lesions were treated with curative intent. Thirty-one patients (including 7 previously treated with neutrons) with 41 lesions were treated with the goal of local palliation. Tumors were predominantly located in the extremity and torso. Thirty of 35 (85%) of curative group patients treated postoperatively had close or positive surgical margins. Thirty-four (82%) lesions treated for palliation were unresectable. Thirty-five patients (53%) were treated at the time of recurrence. Median tumor size at initial presentation was 8.0 cm (range 0.6-29), median treated gross disease size was 5.0 cm (range 1-22), and 46/69 evaluable lesions (67%) were judged to be of intermediate to high histologic grade. Fourteen patients (21%) had chondrosarcomas. Results: Median follow-up was 6 months (range 2-47) and 38 months (range 2-175) for the palliative and curative groups, respectively. Kaplan-Meier estimates were obtained for probability of local relapse-free survival (68%), distant disease-free survival (59%), cause-specific survival (68%), and overall survival (66%) at

Genetic susceptibility to bone and soft tissue sarcomas: a field synopsis and meta-analysis.

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Benna, Clara; Simioni, Andrea; Pasquali, Sandro; De Boni, Davide; Rajendran, Senthilkumar; Spiro, Giovanna; Colombo, Chiara; Virgone, Calogero; DuBois, Steven G; Gronchi, Alessandro; Rossi, Carlo Riccardo; Mocellin, Simone

2018-04-06

The genetic architecture of bone and soft tissue sarcomas susceptibility is yet to be elucidated. We aimed to comprehensively collect and meta-analyze the current knowledge on genetic susceptibility in these rare tumors. We conducted a systematic review and meta-analysis of the evidence on the association between DNA variation and risk of developing sarcomas through searching PubMed, The Cochrane Library, Scopus and Web of Science databases. To evaluate result credibility, summary evidence was graded according to the Venice criteria and false positive report probability (FPRP) was calculated to further validate result noteworthiness. Integrative analysis of genetic and eQTL (expression quantitative trait locus) data was coupled with network and pathway analysis to explore the hypothesis that specific cell functions are involved in sarcoma predisposition. We retrieved 90 eligible studies comprising 47,796 subjects (cases: 14,358, 30%) and investigating 1,126 polymorphisms involving 320 distinct genes. Meta-analysis identified 55 single nucleotide polymorphisms (SNPs) significantly associated with disease risk with a high (N=9), moderate (N=38) and low (N=8) level of evidence, findings being classified as noteworthy basically only when the level of evidence was high. The estimated joint population attributable risk for three independent SNPs (rs11599754 of ZNF365/EGR2 , rs231775 of CTLA4 , and rs454006 of PRKCG ) was 37.2%. We also identified 53 SNPs significantly associated with sarcoma risk based on single studies.Pathway analysis enabled us to propose that sarcoma predisposition might be linked especially to germline variation of genes whose products are involved in the function of the DNA repair machinery. We built the first knowledgebase on the evidence linking DNA variation to sarcomas susceptibility, which can be used to generate mechanistic hypotheses and inform future studies in this field of oncology.

Epidemiological study of soft-tissue sarcomas in Ireland.

LENUS (Irish Health Repository)

Bhatt, Nikita

2015-11-21

Soft-tissue sarcomas (STS) account for 1% of adult and 7% of pediatric malignancies. Histopathology and classification of these rare tumors requires further refinements. The aim of this paper is to describe the current incidence and survival of STS from 1994 to 2012 in Ireland and compare these with comparably coded international published reports. This is a retrospective, population study based on the data from the National Cancer Registry of Ireland (NCRI). Incidence and relative survival rates for STS in Ireland were generated. Incidence of STS based on gender, age and anatomical location was examined. Annual mean incidence rate (European Age Standardized) in Ireland between 1994 and 2012 was 4.48 ± 0.15 per 100,000 person-years. The overall relative 5-year survival rate of STS for the period 1994-2011 in Ireland was 56%, which was similar to that reported in the U.K. but lower than in most of Europe and U.S.A. Survival rate fluctuated over the period examined, declining slightly in females but showing an increase in males. STS incidence trends in Ireland were comparable to international reports. Survival trends of STS were significantly different between Ireland and other European countries, requiring further study to understand causation.

Trabectedin and Eribulin: Where Do They Fit in the Management of Soft Tissue Sarcoma?

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Ratan, Ravin; Patel, Shreyaskumar R

2017-06-01

Trabectedin and eribulin are two agents that have been recently approved for the treatment of specific soft tissue sarcoma subtypes. They have proved to be a much-needed line of additional treatment for patients with these rare tumors, but their activity remains admittedly modest in most cases. Further exploitation of these novel agents is likely to require a more granular understanding of the salient mechanisms of action. For example, if as some studies suggest, eribulin derives its benefit from restructuring of tumor vasculature to improve efficacy of subsequent lines of therapy, then patients may benefit from its use earlier in the treatment pathway. The sequencing of trabectedin with other agents is also worth examining. In a disease like myxoid liposarcoma, consideration should be given to using trabectedin before other salvage regimens like gemcitabine and docetaxel, given its tolerability and excellent efficacy against this sarcoma subtype. Also, to be further investigated is the use of trabectedin in sarcoma subtypes which were excluded from the phase III study, but in which activity has been documented in earlier trials and subsequent reports. Combinations of trabectedin with other agents, particularly doxorubicin, have been explored, but the data to date do not support the routine use of these regimens.

The financial burden of reexcising incompletely excised soft tissue sarcomas: a cost analysis.

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Alamanda, Vignesh K; Delisca, Gadini O; Mathis, Shannon L; Archer, Kristin R; Ehrenfeld, Jesse M; Miller, Mark W; Homlar, Kelly C; Halpern, Jennifer L; Schwartz, Herbert S; Holt, Ginger E

2013-09-01

Although survival outcomes have been evaluated between those undergoing a planned primary excision and those undergoing a reexcision following an unplanned resection, the financial implications associated with a reexcision have yet to be elucidated. A query for financial data (professional, technical, indirect charges) for soft tissue sarcoma excisions from 2005 to 2008 was performed. A total of 304 patients (200 primary excisions and 104 reexcisions) were identified. Wilcoxon rank sum tests and χ2 or Fisher's exact tests were used to compare differences in demographics and tumor characteristics. Multivariable linear regression analyses were performed with bootstrapping techniques. The average professional charge for a primary excision was $9,694 and $12,896 for a reexcision (pfinancial burden nearly doubles.

The effect of delayed postoperative irradiation on local control of soft tissue sarcomas of the extremity and torso

International Nuclear Information System (INIS)

Schwartz, David L.; Einck, John; Hunt, Karen; Bruckner, James; Conrad, Ernest; Koh, W.-J.; Laramore, George E.

2002-01-01

Purpose: The impact of delayed adjuvant radiotherapy in patients treated by surgical resection for peripheral or torso soft tissue sarcoma has not been well characterized. We retrospectively examined this issue in an institutional patient cohort. Methods and Materials: One hundred two adult patients were treated at the University of Washington Medical Center between 1981 and 1998 with postoperative radiotherapy for cure of a newly diagnosed soft tissue sarcoma. Of this group, 58 patients had primary intermediate- or high-grade disease of the extremity or torso (50 extremity/8 torso). Tumor histology was predominantly malignant fibrohistiocytoma, synovial cell sarcoma, and leiomyosarcoma. The group was dichotomized according to time interval from definitive resection to the start of adjuvant radiation. Twenty-six patients had a short delay, defined as <4 months, and 32 patients had a long delay of ≥4 months. Both groups were balanced with regard to site, size, margin status, and tumor depth; however, the long-delay group had a larger proportion of high histologic grade lesions and was treated more frequently with chemotherapy (31/32 [97%] for long-delay patients vs. 14/26 [54%] for short-delay patients). Median follow-up was 49.5 months (range: 7-113 months). Median follow-up for patients still alive was 54 months (range: 9-113 months). Survival outcomes were estimated by the Kaplan-Meier method. Results: Overall local relapse-free survival at 5 years from the time of definitive resection was 74%. On univariate analysis, estimated 5-year local relapse-free survival was significantly improved in the short-delay group (88% vs. 62% for the long-delay group, p = 0.048 by log rank). Overall distant relapse-free survival, disease-free survival, and overall survival at 5 years were 77%, 68%, and 86%, respectively. These survival outcomes were statistically equivalent in both radiation delay groups. There was no evidence to suggest that delaying adjuvant systemic therapy

A prognostic model for soft tissue sarcoma of the extremities and trunk wall based on size, vascular invasion, necrosis, and growth pattern

DEFF Research Database (Denmark)

Carneiro, Ana; Bendahl, Par-Ola; Engellau, Jacob

2011-01-01

type, necrosis, and grade. METHODS:: Whole-tumor sections from 239 soft tissue sarcomas of the extremities were reviewed for the following prognostic factors: size, vascular invasion, necrosis, and growth pattern. A new prognostic model, referred to as SING (Size, Invasion, Necrosis, Growth...

Agreement Among RTOG Sarcoma Radiation Oncologists in Contouring Suspicious Peritumoral Edema for Preoperative Radiation Therapy of Soft Tissue Sarcoma of the Extremity

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Bahig, Houda [Department of Radiation Oncology, Centre Hospitalier de l' Université de Montréal, Montreal, QC (Canada); Roberge, David, E-mail: david.roberge.chum@ssss.gouv.qc.ca [Department of Radiation Oncology, Centre Hospitalier de l' Université de Montréal, Montreal, QC (Canada); Bosch, Walter [Department of Radiation Oncology, Washington University, St Louis, Missouri (United States); Levin, William [Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania (United States); Petersen, Ivy; Haddock, Michael [Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (United States); Freeman, Carolyn [Division of Radiation Oncology, McGill University Health Centre, Montreal, QC (Canada); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Abrams, Ross A. [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States); Indelicato, Danny J. [Department of Radiation Oncology, University of Florida Medical Center, Jacksonville, Florida (United States); Baldini, Elizabeth H. [Department of Radiation Oncology, Brigham and Women' s Hospital and Dana-Farber Cancer Institute, Boston, Massachusetts (United States); Hitchcock, Ying [Department of Radiation Oncology, University of Utah Medical Center, Salt Lake City, Utah (United States); Kirsch, David G. [Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Kozak, Kevin R. [Department of Human Oncology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin (United States); Wolfson, Aaron [Department of Radiation Oncology, University of Miami Miller School of Medicine, Miami, Florida (United States); and others

2013-06-01

Purpose: Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Methods and Materials: Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm{sup 3} (7-413 cm{sup 3}), 280 cm{sup 3} and 360 cm{sup 3}. The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm{sup 3} (24-565 cm{sup 3}) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm{sup 3} (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm{sup 3}, respectively. There were 3 large tumors with >30 cm{sup 3} of SE not included in the CTV3cm volume. Conclusion: Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant

Agreement among RTOG sarcoma radiation oncologists in contouring suspicious peritumoral edema for preoperative radiation therapy of soft tissue sarcoma of the extremity.

Science.gov (United States)

Bahig, Houda; Roberge, David; Bosch, Walter; Levin, William; Petersen, Ivy; Haddock, Michael; Freeman, Carolyn; Delaney, Thomas F; Abrams, Ross A; Indelicato, Danny J; Baldini, Elizabeth H; Hitchcock, Ying; Kirsch, David G; Kozak, Kevin R; Wolfson, Aaron; Wang, Dian

2013-06-01

Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm(3) (7-413 cm(3)), 280 cm(3) and 360 cm(3). The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm(3) (24-565 cm(3)) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm(3) (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm(3), respectively. There were 3 large tumors with >30 cm(3) of SE not included in the CTV3cm volume. Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant expansion of the CTVs is required to cover SE. Copyright © 2013 Elsevier Inc. All rights

Agreement Among RTOG Sarcoma Radiation Oncologists in Contouring Suspicious Peritumoral Edema for Preoperative Radiation Therapy of Soft Tissue Sarcoma of the Extremity

International Nuclear Information System (INIS)

Bahig, Houda; Roberge, David; Bosch, Walter; Levin, William; Petersen, Ivy; Haddock, Michael; Freeman, Carolyn; DeLaney, Thomas F.; Abrams, Ross A.; Indelicato, Danny J.; Baldini, Elizabeth H.; Hitchcock, Ying; Kirsch, David G.; Kozak, Kevin R.; Wolfson, Aaron

2013-01-01

Purpose: Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Methods and Materials: Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm 3 (7-413 cm 3 ), 280 cm 3 and 360 cm 3 . The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm 3 (24-565 cm 3 ) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm 3 (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm 3 , respectively. There were 3 large tumors with >30 cm 3 of SE not included in the CTV3cm volume. Conclusion: Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant expansion of the CTVs is required to cover SE

ALK-negative anaplastic large cell lymphoma mimicking a soft tissue sarcoma

Directory of Open Access Journals (Sweden)

Rachel Hudacko

2011-01-01

Full Text Available Anaplastic lymphoma kinase protein (ALK-negative anaplastic large cell lymphoma (ALCL has a vast morphologic spectrum and may mimic many other types of malignancies both cytologically and histologically. There are only a few published case reports/series describing the cytomorphologic features of ALCL on fine-needle aspiration (FNA biopsy specimens. We describe a case of ALK-negative ALCL mimicking a high-grade soft tissue sarcoma of the thigh in a 62-year-old man. The characteristic morphologic findings on FNA and core biopsy along with the immunophenotypic profile are described and reviewed. The diagnosis of ALCL on FNA biopsy may be difficult, but can be done successfully with the use of ancillary tests. Therefore, it must be considered in the differential diagnosis of lesions with pleomorphism, anaplasia, and wreath-like or horseshoe-shaped nuclei to ensure that adequate material is obtained for ancillary studies.

Soft tissue sarcoma nomograms and their incorporation into practice.

Science.gov (United States)

Callegaro, Dario; Miceli, Rosalba; Mariani, Luigi; Raut, Chandrajit P; Gronchi, Alessandro

2017-08-01

The accurate prediction of prognosis in patients with soft tissue sarcoma (STS) is a challenging issue. Extreme variability in the clinical and pathological characteristics of this family of tumors hinders the simple stratification of patients into meaningful prognostic cohorts. Precision medicine tools for the prediction of prognosis, such as nomograms, enable personalized computation of outcome based on clinical and pathological characteristics of both patient and tumor. The eighth edition of the American Joint Committee on Cancer staging manual moved from a "population-based" to a "personalized" approach endorsing high-quality nomograms to improve clinician prediction ability in definite patient subgroups. The first nomogram for STS was published in 2002, and this was followed by several prognostic predictors offered to clinicians. Focusing on a specific STS subgroup or site, nomograms can take into consideration highly specific factors relevant only in that particular scenario, thereby maximizing prognostic ability. The objective of this review was to critically evaluate available nomograms for patients with STS to provide clinicians and researchers with a choice of the most optimal tool for each specific patient. Cancer 2017;123:2802-20. © 2017 American Cancer Society. © 2017 American Cancer Society.

Complex surgery for locally advanced bone and soft tissue sarcomas of the shoulder girdle.

Science.gov (United States)

Lesenský, Jan; Mavrogenis, Andreas F; Igoumenou, Vasilios G; Matejovsky, Zdenek; Nemec, Karel; Papagelopoulos, Panayiotis J; Fabbri, Nicola

2017-08-01

Surgical management of primary musculoskeletal tumors of the shoulder girdle is cognitively and technically demanding. Over the last decades, advances in the medical treatments, imaging and surgical techniques have fostered limb salvage surgery and reduced the need for amputation. Despite well-accepted general principles, an individualized approach is often necessary to accommodate tumor extension, anatomical challenges and patient characteristics. A combination of techniques is often required to achieve optimal oncologic and durable functional outcome. Goal of this article is to review approach and management of patients with locally advanced sarcomas of the shoulder girdle requiring major tumor surgery, to illustrate principles of surgical strategy, outcome and complications, and to provide useful guidelines for the treating physicians.

A prediction model for treatment decisions in high-grade extremity soft-tissue sarcomas: Personalised sarcoma care (PERSARC).

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van Praag, Veroniek M; Rueten-Budde, Anja J; Jeys, Lee M; Laitinen, Minna K; Pollock, Rob; Aston, Will; van der Hage, Jos A; Dijkstra, P D Sander; Ferguson, Peter C; Griffin, Anthony M; Willeumier, Julie J; Wunder, Jay S; van de Sande, Michiel A J; Fiocco, Marta

2017-09-01

To support shared decision-making, we developed the first prediction model for patients with primary soft-tissue sarcomas of the extremities (ESTS) which takes into account treatment modalities, including applied radiotherapy (RT) and achieved surgical margins. The PERsonalised SARcoma Care (PERSARC) model, predicts overall survival (OS) and the probability of local recurrence (LR) at 3, 5 and 10 years. Development and validation, by internal validation, of the PERSARC prediction model. The cohort used to develop the model consists of 766 ESTS patients who underwent surgery, between 2000 and 2014, at five specialised international sarcoma centres. To assess the effect of prognostic factors on OS and on the cumulative incidence of LR (CILR), a multivariate Cox proportional hazard regression and the Fine and Gray model were estimated. Predictive performance was investigated by using internal cross validation (CV) and calibration. The discriminative ability of the model was determined with the C-index. Multivariate Cox regression revealed that age and tumour size had a significant effect on OS. More importantly, patients who received RT showed better outcomes, in terms of OS and CILR, than those treated with surgery alone. Internal validation of the model showed good calibration and discrimination, with a C-index of 0.677 and 0.696 for OS and CILR, respectively. The PERSARC model is the first to incorporate known clinical risk factors with the use of different treatments and surgical outcome measures. The developed model is internally validated to provide a reliable prediction of post-operative OS and CILR for patients with primary high-grade ESTS. LEVEL OF SIGNIFICANCE: level III. Copyright © 2017 Elsevier Ltd. All rights reserved.

Postoperative radiation boost does not improve local recurrence rates in extremity soft tissue sarcomas

International Nuclear Information System (INIS)

Alamanda, Vignesh K.; Schwartz, Herbert S.; Holt, Ginger E.; Song, Yanna; Shinohara, Eric

2014-01-01

The standard of care for extremity soft tissue sarcomas continues to be negative-margin limb salvage surgery. Radiotherapy is frequently used as an adjunct to decrease local recurrence. No differences in survival have been found between preoperative and postoperative radiotherapy regimens. However, it is uncertain if the use of a postoperative boost in addition to preoperative radiotherapy reduces local recurrence rates. This retrospective review evaluated patients who received preoperative radiotherapy (n = 49) and patients who received preoperative radiotherapy with a postoperative boost (n=45). The primary endpoint analysed was local recurrence, with distant metastasis and death due to sarcoma analysed as secondary endpoints. Wilcoxon rank-sum test and either χ 2 or Fisher's exact test were used to compare variables. Multivariable regression analyses were used to take into account potential confounders and identify variables that affected outcomes. No differences in the proportion or rate of local recurrence, distant metastasis or death due to sarcoma were observed between the two groups (P>0.05). The two groups were similarly matched with respect to demographics such as age, race and sex and tumour characteristics including excision status, tumour site, size, depth, grade, American Joint Committee on Cancer stage, chemotherapy receipt and histological subtype (P>0.05). The postoperative boost group had a larger proportion of patients with positive microscopic margins (62% vs 10%; P<0.001). No differences in rates of local recurrence, distant metastasis or death due to sarcoma were found in patients who received both pre- and postoperative radiotherapy when compared with those who received only preoperative radiotherapy.

Outcome and Toxicity of an Ifosfamide-Based Soft Tissue Sarcoma Treatment Protocol in Children. The Importance of Local Therapy

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S. Murray Yule

1998-01-01

Full Text Available Background. Although the survival of children with soft tissue sarcoma (STS has improved considerably, the outcome of patients with metastatic disease, and those with primary tumours of the extremities or parameningeal sites remains disappointing. We describe the clinical outcome of an ifosfamide-based regimen with local therapy directed only to children who failed to achieve a complete response to initial chemotherapy.

Adult soft tissue sarcomas of the head and neck. Treatment outcome after combined surgery and irradiation or radiotherapy alone

International Nuclear Information System (INIS)

Willers, H.; Spiro, I.J.; Efird, J.T.; Rosenberg, A.E.; Wang, C.C.

1997-01-01

To analyse the experience treating soft tissue sarcomas of the head and neck at the Massachusetts General Hospital, Boston. Detailed results have been published previously. After 5 years, patients with angiosarcomas (n=11) and patients with other tumor types (n=46) had locoregional control rates of 24% and 69%, distant failure rates of 58% and 17%, and overall survival rates of 31% and 74%, respectively (p [de

Preoperative chemotherapy of bone and soft tissue sarcomas. Evaluation with dynamic MR imaging

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Ando, Yoko; Fukatsu, Hiroshi; Isomura, Takayuki; Itoh, Shigeki; Ishigaki, Takeo; Yamamura, Shigeki; Sugiura, Hideshi; Satoh, Keiji.

1995-01-01

Dynamic MR imaging and conventional angiography were performed in eleven patients with musculoskeletal malignant tumors before and after preoperative chemotherapy in order to evaluate its effect. Dynamic MRI was obtained with GRASS (TR/TE/FA=50/10-13/30) or SE (TR/TE=150-350/20). Although resected specimen in one case of osteosarcoma had the necrotic ratio of more than 90%, it had marked early enhancement in dynamic MRI, and microscopic examination revealed fibrotic necrosis with many capillaries. In soft tissue sarcomas with hemorrhage and/or cystic change, dynamic MRI findings did not necessarily correlate with the chemotherapy effect. Dynamic MRI was more useful than angiography because of its ability to show tumor vascularity and of its non-invasiveness. (author)

Gross tumor volume and clinical target volume: soft-tissue sarcoma of the extremities

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Lartigau, E.; Kantor, G.; Lagarde, P.; Taieb, S.; Ceugnart, L.; Vilain, M.O.; Penel, N.; Depadt, G.

2001-01-01

Soft tissue sarcomas of the extremities are currently treated with more conservative and functional approaches, combining surgery, radiotherapy and chemotherapy. The role of external beam radiotherapy and brachytherapy has been defined through randomized studies performed in the 80's and 90's. However, the ubiquity of tumour location for these tumours makes difficult a systematic definition of local treatments. Tumour volume definition is based on pre and post surgical imaging (MRI) and on described pathological report. The clinical target volume will take into account quality of the resection and anatomical barriers and will be based on an anatomy and not only on safety margins around the tumour bed. General rules for this irradiation (doses, volumes) and principal results will be presented. (authors)

Down-regulated E-cadherin expression is associated with poor five-year overall survival in bone and soft tissue sarcoma: results of a meta-analysis.

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Ning Wang

Full Text Available To conduct a meta-analysis to evaluate the prognostic role of E-cadherin expression in bone and soft tissue sarcomas.The PubMed, EMBASE, and Web of Science databases were searched using terms related to E-cadherin, sarcoma, and prognosis for all articles published in English before March 2014. Pooled effect was calculated from the available data to evaluate the association between negative E-cadherin expression and 5-year overall survival and tumor clinicopathological features in sarcoma patients. Pooled odds ratios (OR and risk ratios (RR with 95% confidence intervals (CI were calculated using a fixed-effects model.Eight studies met the selection criteria and reported on 812 subjects. A total of 496 subjects showed positive E-cadherin expression (59.9%. Negative E-cadherin expression in bone and soft tissue sarcomas was correlated with lower 5-year overall survival (OR = 3.831; 95% CI: 2.246-6.534, and was associated with higher clinical stage (RR = 1.446; 95% CI: 1.030-2.028 and with male sex (RR = 0.678; 95% CI: 0.493-0.933.In the E-cadherin negative group, 5-year overall survival was significantly worse than in the E-cadherin positive group. However, further studies are required to confirm these results.

Defining the incidence and clinical significance of lymph node metastasis in soft tissue sarcoma.

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Keung, Emily Z; Chiang, Yi-Ju; Voss, Rachel K; Cormier, Janice N; Torres, Keila E; Hunt, Kelly K; Feig, Barry W; Roland, Christina L

2018-01-01

The incidence and clinical significance of lymph node metastasis (LNM, N1) in soft tissue sarcoma (STS) is unclear. Recent studies have focused on extremity/trunk STS (ETSTS). We sought to define the subgroup of patients with LNM at sarcoma diagnosis across all disease sites and histologies. We identified and categorized 89,870 STS patients from the National Cancer Data Base (1998-2012) by nodal stage. Pathologically confirmed LNM (pN1) were identified in 1404 patients; 1750 had clinically suspicious but not pathologically confirmed LNM (cN1). Survival analyses were performed by Kaplan-Meier method. Of 3154 patients (3.5%) with pN1 or cN1 LNM at presentation, 1310 had synchronous distant metastasis (M1). LNM affected a small proportion of patients (5.8% head/neck, 5.3% intrathoracic, 5.1% intra-abdominal, 2.0% ETSTS). Angiosarcoma (6%), epithelioid (13%), clear cell (16%), and small cell sarcoma (19%) had the highest incidence of LNM, although liposarcoma, fibrous histiocytoma, and leiomyosarcoma accounted for the greatest number of LNM patients. For pN1M0 disease, median overall survival (OS) was 28.2 months, varying by histology. Among patients with pN1M0 STS, angiosarcoma, clear cell sarcoma, leiomyosarcoma, and fibrous histiocytoma were associated with worse median OS (19.4, 23.8, 27.1, and 29.3 months) compared to epithelioid sarcoma and liposarcoma (49.6 and 56.0 months, p < 0.001). Despite clinical suspicion, pathologic LN evaluation in STS is inconsistently performed. LNM occurs across anatomic disease sites and is unevenly distributed across histologies. Although M1 disease portends poor prognosis regardless of LN status, LNM predicts worse OS in a histology-dependent manner in M0 disease. Published by Elsevier Ltd.

Safety of concurrent adjuvant radiotherapy and chemotherapy for locally advanced soft tissue sarcoma.

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Greto, Daniela; Loi, Mauro; Saieva, Calogero; Muntoni, Cristina; Delli Paoli, Camilla; Becherini, Carlotta; Ciabatti, Cinzia; Perna, Marco; Campanacci, Domenico; Terziani, Francesca; Beltrami, Giovanni; Scoccianti, Guido; Bonomo, Pierluigi; Meattini, Icro; Desideri, Isacco; Simontacchi, Gabriele; Mangoni, Monica; Livi, Lorenzo

2018-04-01

This retrospective study analyzes the safety and feasibility of concurrent chemoradiotherapy (CRT) in adjuvant treatment of soft tissue sarcoma (STS). A total of 158 patients with STS were retrospectively analyzed. Anthracycline-based computed tomography was performed in high-risk patients. Acute radiotherapy toxicity and chemotherapy-related toxicity were assessed according to the Common Terminology Criteria for Adverse Events 4.0; late radiotherapy toxicity was recorded according to Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer criteria. Fifty-four (34.2%) patients received CRT. Mean follow up was 5.4 years (range .2-21.1 years). Local DFS-recurrence-free survival, distant DFS-relapse-free survival, and overall survival were 79.1%, 76.4%, and 64.6%, respectively, at last follow-up. Leukopenia occurred in 11.4% of patients. Skin acute toxicity developed in 60.1% of patients and determined interruption of radiotherapy treatment in 19 (12%) patients. Nineteen patients (12%) experienced moderate fibrosis (grade 2). Mild and moderate joint stiffness was recorded in 16 (10.1%) patients. Size ≥5 cm was the only predictor of local recurrence at multivariate analysis (hazard ratio [HR] 9.65, 95% confidence interval [CI] 1.28-72.83, p = .028). Age and stage resulted as independent distant relapse predictors (HR 4.77, 95% CI 1.81-12.58, p = .002 and HR 4.83, CI 1.41-16.57, p = .012, respectively). At Cox regression univariate analysis, Karnofsky Performance Status, size, and stage were significant survival predictors (HR 2.23, 95% CI 1.02-4.87, p = .045; HR 2.88, 95% CI 1.10-7.52, p = .031; HR 2.59, 95% CI 1.11-6.04, p = .028). Concurrent CRT is a well-tolerated treatment option with no additional toxicity compared to exclusive radiotherapy or sequential CRT.

Synovial sarcoma: MR evaluation in 23 patients

International Nuclear Information System (INIS)

Galant, J.; Marti-Bonmati, L.; Lafuente, J.; Hernandez, L.; Soler, R.; Saez, F.

1997-01-01

The synovial sarcoma is one of the most common soft tissue sarcomas. MR is the technique of choice to determine to local extension of malignant soft tissue tumors. To assess the clinical and MR imaging parameters associated with synovial sarcomas that aid in establishing their diagnosis. We review the clinical findings and images of 23 histologically confirmed synovial sarcomas that were studied by MR. Synovial sarcomas usually develop in young adults as soft tissue tumors, preferentially in the deep tissues of an extremity in close proximity to a joint. They are characterized as having a lobulated contour and septa, frequently infiltrating neighboring tissues at some point, and are heterogeneous. The presence of hemorrhage, as well as infiltration of the fascia in subcutaneous tumors, suggests the diagnosis of synovial sarcoma. The development of perilesional edema is not uncommon. Although, logically, the clinical and radiological features of synovial sarcomas can overlap with those of other soft tissue tumors, the findings described here are fairly characteristic of these lesions: thus, when present, they should serve to orient the diagnostic process. (Author) 16 refs

Durable clinical activity of single-agent bevacizumab in a nonagenarian patient with metastatic alveolar soft part sarcoma.

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Mir, Olivier; Boudou-Rouquette, Pascaline; Larousserie, Frédérique; Blanchet, Benoit; Babinet, Antoine; Anract, Philippe; Goldwasser, François

2012-08-01

Alveolar soft part sarcoma is a rare malignancy usually considered resistant to conventional chemotherapy, but recent data suggest that the multikinase inhibitors sunitinib and cediranib could be active in this setting. A 90-year-old lady with alveolar soft part sarcoma of the leg and lung metastases was started on sunitinib 37.5 mg daily. The treatment was poorly tolerated with grade 3 hypertension and grade 3 thrombocytopenia, which persisted after dose reduction to 25 mg daily. The patient was subsequently started on bevacizumab 10 mg/kg every 2 weeks, resulting in a marked improvement in pain and a partial response on lung metastases for 16 months and ongoing. Agents targeting the vascular endothelial growth factor-signalling pathway seem to exert clinically relevant and prolonged activity against alveolar soft part sarcoma and deserve further evaluation in the treatment of this rare soft tissue sarcoma.

Expression of human Piwi-like genes is associated with prognosis for soft tissue sarcoma patients

International Nuclear Information System (INIS)

Greither, Thomas; Taubert, Helge; Koser, Franziska; Kappler, Matthias; Bache, Matthias; Lautenschläger, Christine; Göbel, Steffen; Holzhausen, Hans-Jürgen; Wach, Sven; Würl, Peter

2012-01-01

Argonaute genes are essential for RNA interference, stem cell maintenance and differentiation. The Piwi-like genes, a subclass of the Argonaute genes, are expressed mainly in the germline. These genes may be re-expressed in tumors, and expression of the Piwi-like genes is associated with prognosis in several types of tumors. We measured the expression of Piwi-like mRNAs (Piwi-like 2–4) in 125 soft tissue sarcoma (STS) samples by qPCRs. Statistical tests were applied to study the correlation of expression levels with tumor-specific survival for STS patients. In multivariate Cox’s regression analyses, we showed that low Piwi-like 2 and Piwi-like 4 mRNA expression were significantly associated with a worse prognosis (RR = 1.87; p = 0.032 and RR = 1.82; p = 0.039). Low expression of both genes was associated with a 2.58-fold increased risk of tumor-related death (p = 0.01). Piwi-like 4 and combined Piwi-like 2 and 4 mRNA levels correlated significantly with prognosis (RR = 3.53; p = 0.002 and RR = 5.23; p = 0.004) only for female but not for male patients. However, combined low Piwi-like 2 and 3 transcript levels were associated with worse survival (RR = 5.90; p = 0.02) for male patients. In this study, we identified a significant association between the expression of Piwi-like 2 and 4 mRNAs and the tumor-specific survival of soft tissue sarcoma patients. Furthermore, a connection between sex and the impact of Piwi-like mRNA expressions on STS patients’ prognosis was shown for the first time

MRI findings associated with microscopic residual tumor following unplanned excision of soft tissue sarcomas in the extremities

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Wang, Lee; Chelala, Lydia; Jose, Jean; Subhawong, Ty K. [University of Miami Miller School of Medicine/Jackson Memorial Hospital, Department of Radiology, Miami, FL (United States); Pretell-Mazzini, Juan [University of Miami Miller School of Medicine, Department of Orthopaedic Surgery, Division of Musculoskeletal Oncology, Miami, FL (United States); Kerr, Darcy A. [University of Miami Miller School of Medicine, Department of Pathology, Miami, FL (United States); Yang, Xuan [University of Miami Miller School of Medicine, Department of Public Health Sciences, Miami, FL (United States)

2018-02-15

MRI is often used to determine the presence of residual disease following unplanned excisions (UPE) of soft tissue sarcomas (STS). We sought to identify MRI features associated with histologic evidence of residual disease after TBE. This was an IRB-approved retrospective review of 27 patients with R1-type UPE of STS over a 32-month period, with subsequent MRI and TBE. MRI studies were retrospectively evaluated to determine depth of tissue involvement, presence of nodular enhancement, and maximum length of soft tissue edema normalized to extremity size. MRI findings were correlated with histology from unplanned excision and TBE. Among the 21 subjects, there were 13 males and 8 females, mean age 58. Eighteen of 21 STS were grade 2 or 3. Deep compartments were involved in 5/21 cases. Original margins were positive in 17/21 UPE, with inadequate margin assessment in the remaining 4 cases. Residual tumor was present at TBE in 11/21 cases; it was found in 4/6 cases with nodular enhancement and 7/15 cases without nodular enhancement (sensitivity = 0.36; specificity = 0.80; PPV = 0.67; NPV = 0.53). Increased extent of soft tissue edema increased the likelihood of residual tumor at TBE (OR = 35.0; 95% CI = 1.6 to 752.7; p = 0.023). Nodular enhancement is neither sensitive nor specific in predicting residual microscopic tumor in TBE following UPE. Extensive soft tissue edema on MRI after UPE increases the likelihood of finding a residual microscopic tumor, justifying ample margins at TBE and consideration of adjuvant therapy. (orig.)

What is current practice in soft tissue sarcoma grading?

International Nuclear Information System (INIS)

Golouh, R.; Bracko, M.

2001-01-01

Purpose. Most published grading systems of soft tissue sarcomas (STS) are somewhat subjective and it seems that there is no definite consensus among experts which of them is the most effective. The aim of this study was to collect data from practicing pathologists and to get some insight in the practice of STS grading. Subjects. A questionnaire was sent to 135 pathologists chosen randomly. Results. There were 88 responders from 30 countries from 5 continents. Most responders (85%) grade STS, more frequently in Europe than in non-European countries. Three-grade system is preferred by both European and non-European pathologists, who use it in almost 77% and 67%, respectively. They apply the criteria set by FNCLCC in 37.3%, by NCI in 24%, by Broders in 12% and by Markhede in 1.4%. In Europe, FNCLCC system is the most widely used. Beside classical histological criteria, other modern methods are applied by more than one half of the responders. Immunohistochemical evaluation of proliferation markers is the method most widely used, followed by molecular markers and DNA flow cytometry. Conclusion. The results of our study indicate that most pathologists consider histologic grade of STS as a valuable, however not completely satisfactory predictor of a patient's survival. (author)

Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

Science.gov (United States)

2018-02-27

Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

Primary clear cell sarcoma of bone

International Nuclear Information System (INIS)

Choi, J.H.; Gu, M.J.; Kim, M.J.; Bae, Y.K.; Choi, W.H.; Shin, D.S.; Cho, K.H.

2003-01-01

Clear cell sarcoma is a rare soft tissue sarcoma of young adults with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses. Primary clear cell sarcoma of bone is extremely rare. We report a case of primary clear cell sarcoma of the right first metatarsal in a 48-year-old woman and provide a literature review of the entity. (orig.)

Follow-up of recurrences of limb soft tissue sarcomas in patients with localized disease: performance of ultrasound

International Nuclear Information System (INIS)

Tagliafico, Alberto; Truini, Mauro; Spina, Bruno; Cambiaso, Paolo; Zaottini, Federico; Bignotti, Bianca; Derchi, Lorenzo E.; Martinoli, Carlo; Calabrese, Massimo

2015-01-01

To evaluate diagnostic performance of ultrasound in the detection of local recurrences in patients with localized soft tissue sarcomas of the limb. An analysis of patients treated for soft tissue sarcomas between 2005 and April 2014 was performed. Sixty-eight patients (men/women, 36:32; age range, 18-84 years) were evaluated. Sensitivity, specificity with 95 % confidence intervals (CIs), positive predictive value (PPV), pre-test probability (the prevalence), negative predictive value (NPV), likelihood ratio for positive results (LH+), accuracy and post-test probability (post-P) of ultrasound were reported on a per patient basis using surgical findings and clinical follow-up as reference standard. Effects of independent variables (US equipment, age and sex, body mass index, radiologist) were considered. Comparison with MR was also performed. The overall sensitivity and specificity were 0.88 (0.60-0.94) and 0.94 (0.86-0.98). PPV, pre-test probability, NPV, LH+, accuracy and post-P: 0.83/0.25/0.96/14.9/0.92/0.83. There were two false negative cases both graded as G3 and deeply located and three false positive US cases. Diagnostic accuracy was not dependent by US machine (p = 0.08), age and sex (p = 0.16), body mass index (p = 0.07) and radiologists (p = 0.07). Diagnostic accuracy of ultrasound was relatively high. Negative US results excluded the presence of a local recurrence with acceptable accuracy. (orig.)

Soft tissue sarcoma after treatment for breast cancer

International Nuclear Information System (INIS)

Karlsson, Per; Holmberg, Erik; Johansson, Karl-Axel; Kindblom, Lars-Gunnar; Carstensen, John; Wallgren, Arne

1996-01-01

In a register study all women in the West of Sweden Health Care Region with a breast cancer diagnosed between 1960 and 1980 (n = 13 490) were followed up in the Swedish Cancer Register to the end of 1988 for later occurrence of a soft tissue sarcoma (STS). Nineteen sarcomas were reported, whereas 8.7 were expected and the relative risk (RR) was 2.2 (CI 95% 1.3-3.4). The absolute risk was (1.7(10 4 )) person years (PY) in comparison with 0.8 expected. To obtain a more detailed analysis of the associations between arm lymphoedema, radiotherapy and STS development, and to control the quality of the register data, a case control study was also performed. Clinical records from the different hospitals in the region were collected for all the 19 cases as well as for three selected controls per case. The histopathology of the cases were reviewed, and one of the cases was reclassified as a malignant melanoma and excluded from further analysis. Thirteen of the cases were clustered around the treated breast area. To quantify the exposure to radiotherapy, the integral dose was estimated. The presence of lymphedema was included as a binary variable in the analysis. The exact conditional randomisation test indicated a significant correlation between the integral dose and the development of an STS (p = 0.008) and this association was still significant after stratification for arm oedema. A conditional logistic regression analysis with STS as the dependent variable and the integral dose as the explanatory variable gave an odds ratio (OR) of(5.2(100)) J (CI 95% 1.3-21.2), and if this regression was restricted only to the STS developing in the radiation fields the OR was(3.2(100)) J (CI 95% 0.8-12.9). Thus, the excess of STS in this breast cancer cohort was very low ((0.9(10 4 )) PY). However the integral dose correlates well to the development of STS and can be useful in quantifying even small risks of secondary malignancies in the breast cancer population

Long-term results of preoperative intra-arterial doxorubicin combined with neoadjuvant radiotherapy, followed by extensive surgical resection for locally advanced soft tissue sarcomas of the extremities

International Nuclear Information System (INIS)

Nijhuis, P.H.A.; Pras, E.; Sleijfer, D.T.; Molenaar, W.M.; Schraffordt Koops, H.; Hoekstra, H.J.

1999-01-01

Background and purpose: In the 1980s a combined modality therapy of intraarterial doxorubicin, neoadjuvant radiotherapy and surgery was initiated at the Groningen University Hospital as a limb-saving treatment for locally advanced, primarily irresectable high-grade soft tissue sarcomas (STS) of the extremities. This study presents the short- and long-term results.Patients and methods: Between 1983 and 1987, 11 patients were treated with intraarterial doxorubicin, preoperative radiotherapy (10x3.5 Gy) and surgical resection. Non-radical resections received additional postoperative radiotherapy of 20-30 Gy.Results: The limb-salvage rate was 91%, without local recurrences during a median hollow-up of 84 months. Six patients died (55%); five from metastatic disease (45%). There were five long-term survivors with a median follow-up of 10 years. Three patients (60%) suffered serious late complications, resulting in disabilitating limb function. Conclusion: Although this approach is feasible as a limb-saving treatment for these unfavorable STS, long-term morbidity is high. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

Evolution of Randomized Trials in Advanced/Metastatic Soft Tissue Sarcoma: End Point Selection, Surrogacy, and Quality of Reporting.

Science.gov (United States)

Zer, Alona; Prince, Rebecca M; Amir, Eitan; Abdul Razak, Albiruni

2016-05-01

Randomized controlled trials (RCTs) in soft tissue sarcoma (STS) have used varying end points. The surrogacy of intermediate end points, such as progression-free survival (PFS), response rate (RR), and 3-month and 6-month PFS (3moPFS and 6moPFS) with overall survival (OS), remains unknown. The quality of efficacy and toxicity reporting in these studies is also uncertain. A systematic review of systemic therapy RCTs in STS was performed. Surrogacy between intermediate end points and OS was explored using weighted linear regression for the hazard ratio for OS with the hazard ratio for PFS or the odds ratio for RR, 3moPFS, and 6moPFS. The quality of reporting for efficacy and toxicity was also evaluated. Fifty-two RCTs published between 1974 and 2014, comprising 9,762 patients, met the inclusion criteria. There were significant correlations between PFS and OS (R = 0.61) and between RR and OS (R = 0.51). Conversely, there were nonsignificant correlations between 3moPFS and 6moPFS with OS. A reduction in the use of RR as the primary end point was observed over time, favoring time-based events (P for trend = .02). In 14% of RCTs, the primary end point was not met, but the study was reported as being positive. Toxicity was comprehensively reported in 47% of RCTs, whereas 14% inadequately reported toxicity. In advanced STS, PFS and RR seem to be appropriate surrogates for OS. There is poor correlation between OS and both 3moPFS and 6moPFS. As such, caution is urged with the use of these as primary end points in randomized STS trials. The quality of toxicity reporting and interpretation of results is suboptimal. © 2016 by American Society of Clinical Oncology.

Wound Complications in Preoperatively Irradiated Soft-Tissue Sarcomas of the Extremities

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Rosenberg, Lewis A. [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States); Esther, Robert J. [Department of Orthopedics, University of North Carolina, Chapel Hill, North Carolina (United States); Erfanian, Kamil [Department of Surgery, University of North Carolina, Chapel Hill, North Carolina (United States); Green, Rebecca [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States); Kim, Hong Jin; Sweeting, Raeshell [Department of Surgery, University of North Carolina, Chapel Hill, North Carolina (United States); Tepper, Joel E., E-mail: tepper@med.unc.edu [Department of Radiation Oncology, University of North Carolina, Chapel Hill, North Carolina (United States)

2013-02-01

Purpose: To determine whether the involvement of plastic surgery and the use of vascularized tissue flaps reduces the frequency of major wound complications after radiation therapy for soft-tissue sarcomas (STS) of the extremities. Methods and Materials: This retrospective study evaluated patients with STS of the extremities who underwent radiation therapy before surgery. Major complications were defined as secondary operations with anesthesia, seroma/hematoma aspirations, readmission for wound complications, or persistent deep packing. Results: Between 1996 and 2010, 73 patients with extremity STS were preoperatively irradiated. Major wound complications occurred in 32% and secondary operations in 16% of patients. Plastic surgery closed 63% of the wounds, and vascularized tissue flaps were used in 22% of closures. When plastic surgery performed closure the frequency of secondary operations trended lower (11% vs 26%; P=.093), but the frequency of major wound complications was not different (28% vs 38%; P=.43). The use of a vascularized tissue flap seemed to have no effect on the frequency of complications. The occurrence of a major wound complication did not affect disease recurrence or survival. For all patients, 3-year local control was 94%, and overall survival was 72%. Conclusions: The rates of wound complications and secondary operations in this study were very similar to previously published results. We were not able to demonstrate a significant relationship between the involvement of plastic surgery and the rate of wound complications, although there was a trend toward reduced secondary operations when plastic surgery was involved in the initial operation. Wound complications were manageable and did not compromise outcomes.

Wound Complications in Preoperatively Irradiated Soft-Tissue Sarcomas of the Extremities

International Nuclear Information System (INIS)

Rosenberg, Lewis A.; Esther, Robert J.; Erfanian, Kamil; Green, Rebecca; Kim, Hong Jin; Sweeting, Raeshell; Tepper, Joel E.

2013-01-01

Purpose: To determine whether the involvement of plastic surgery and the use of vascularized tissue flaps reduces the frequency of major wound complications after radiation therapy for soft-tissue sarcomas (STS) of the extremities. Methods and Materials: This retrospective study evaluated patients with STS of the extremities who underwent radiation therapy before surgery. Major complications were defined as secondary operations with anesthesia, seroma/hematoma aspirations, readmission for wound complications, or persistent deep packing. Results: Between 1996 and 2010, 73 patients with extremity STS were preoperatively irradiated. Major wound complications occurred in 32% and secondary operations in 16% of patients. Plastic surgery closed 63% of the wounds, and vascularized tissue flaps were used in 22% of closures. When plastic surgery performed closure the frequency of secondary operations trended lower (11% vs 26%; P=.093), but the frequency of major wound complications was not different (28% vs 38%; P=.43). The use of a vascularized tissue flap seemed to have no effect on the frequency of complications. The occurrence of a major wound complication did not affect disease recurrence or survival. For all patients, 3-year local control was 94%, and overall survival was 72%. Conclusions: The rates of wound complications and secondary operations in this study were very similar to previously published results. We were not able to demonstrate a significant relationship between the involvement of plastic surgery and the rate of wound complications, although there was a trend toward reduced secondary operations when plastic surgery was involved in the initial operation. Wound complications were manageable and did not compromise outcomes.

Clinic-radiology findings at limp-sparing surgery of alveolar sarcoma

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Tzekov, A.; Penchev, V.; Niagolova, S.; Kovachev, V.

2007-01-01

Soft-tissue sarcomas are a rare and heterogeneous group of tumors. Alveolar soft part sarcoma is a rare sarcoma of an unclear cause. It is representing 0.2-1 percent of soft tissue sarcomas. Unique biologic behaviors of soft-tissue sarcomas features include formation of a reactive zone, intra-compartmental growth, and, rarely, the presence of skip metastases. Although the soft-tissue sarcomas have a unique morphology, biological behavior, and prognosis, they share biological characteristics and are treated in a similar fashion. The concept of limb-sparing surgery, or limb salvage, has gradually evolved over the past 25 years. Prior to this the basic principles of surgical oncology for the extremities consisted solely of determining the correct level at which to perform an amputation. The three stages of a limb-sparing procedure are as follows: tumor resection skeletal reconstruction and soft-tissue coverage and muscle transfers to restore function. Imaging diagnostic is a key step in the limp-sparing surgery of a soft tissue sarcomas at relation of: preoperatively - the diagnosis of lesion, the decision for biopsy, about location of the lesion and what is the safest anatomic route to that location, possibly lung and bone metastases; postoperative - checking the position of the prosthesis, inflammatory, mechanic and oncology complications. (authors)

Pre- and Postoperative Chemotherapy in Localized Extremity Soft Tissue Sarcoma: A European Organization for Research and Treatment of Cancer Expert Survey.

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Rothermundt, Christian; Fischer, Galina F; Bauer, Sebastian; Blay, Jean-Yves; Grünwald, Viktor; Italiano, Antoine; Kasper, Bernd; Kollár, Attila; Lindner, Lars H; Miah, Aisha; Sleijfer, Stefan; Stacchiotti, Silvia; Putora, Paul Martin

2018-04-01

The management of localized extremity soft tissue sarcomas (STS) is challenging and the role of pre- and postoperative chemotherapy is unclear and debated among experts. Medical oncology experts of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group were asked to participate in this survey on the use of pre- and postoperative chemotherapy in STS. Experts from 12 centers in Belgium, France, Germany, Great Britain, Italy, Switzerland, and The Netherlands agreed to participate and provided their treatment algorithm. Answers were converted into decision trees based on the objective consensus methodology. The decision trees were used as a basis to identify consensus and discrepancies. Several criteria used for decision-making in extremity STS were identified: chemosensitivity, fitness, grading, location, and size. In addition, resectability and resection status were relevant in the pre- and postoperative setting, respectively. Preoperative chemotherapy is considered in most centers for marginally resectable tumors only. Yet, in some centers, neoadjuvant chemotherapy is used routinely and partially combined with hyperthermia. Although most centers do not recommend postoperative chemotherapy, some offer this treatment on a regular basis. Radiotherapy is an undisputed treatment modality in extremity STS. Due to lacking evidence on the utility of pre- and postoperative chemotherapy in localized extremity STS, treatment strategies vary considerably among European experts. The majority recommended neoadjuvant chemotherapy for marginally resectable grade 2-3 tumors; the majority did not recommend postoperative chemotherapy in any setting. The management of localized extremity soft tissue sarcomas (STS) is challenging and the role of pre- and postoperative chemotherapy is unclear and debated among experts. This study analyzed the decision-making process among 12 European experts on systemic therapy for STS. A wide range of

Detection of EWS/FLI-1 fusion in non-Ewing soft tissue tumors.

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Trancău, I O; Huică, R; Surcel, M; Munteanu, A; Ursaciuc, C

2015-01-01

EWS/FLI-1 fusion mainly appears in Ewing's sarcoma or the primitive neuroectodermal tumors and represents a genomic marker for these tumors. However, it can appear with lower frequency in other soft tissue tumors. The paper investigates the presence of EWS/FLI-1 fusion in clinically diagnosed sarcoma belonging to different non-Ewing connective tissue tumors in order to search for a possible new biomarker valuable for investigators. 20 patients with soft tissue tumors, who underwent surgery, were tested. Intra-operative samples of normal and tumor tissue were collected for histopathological diagnosis and genetics determinations. The patients' RNA from tumor and normal peritumoral tissue was extracted and EWS/FLI-1 fusion screened by quantitative real-time PCR. The relative expression of the fusion in the tumor sample was compared to the similar expression in normal tissue. The amplification in the threshold zone was shown by 5 samples (25%): 2 clear cell sarcoma, 1 fibrosarcoma, 1 malignant tumor of nerve sheath, 1 metastatic adenocarcinoma. We differentiated between the unspecific amplification and concluded that these are weak positive results. Genomic investigation may establish the tumor malignancy and its possible affiliation earlier than histopathology. It can support the screening of EWS/FLI-1 fusion in a larger variety of clinically diagnosed soft tissue tumors.

Magnetic resonance imaging appearance of soft-tissue metastases: our experience at an orthopedic oncology center

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Sammon, Jennifer; Jain, Abhishek; Bleakney, Robert; Mohankumar, Rakesh

2017-01-01

To assess the prevalence and magnetic resonance imaging appearance of metastasis presenting as a soft-tissue mass. A retrospective chart review was performed on 51 patients who presented to an orthopedic oncology center with soft-tissue masses, with a histology-proven diagnosis of soft-tissue metastasis, over a 14-year period. Their magnetic resonance imaging, primary origin, and follow-up have been assessed. Soft-tissue metastasis was identified in patients ranging from 18 to 85 years old. Most (80%) of the masses were located deep to the deep fascia. In our cohort of patients, melanoma was the most common primary malignancy contributing to soft-tissue metastasis (21.8%). Among soft-tissue metastasis from solid organs, breast and lung were the most frequent (9.1% each). Five patients had soft-tissue metastases from an unknown primary. Imaging diagnosis of soft-tissue metastases is challenging as it can demonstrate imaging appearances similar to primary soft-tissue sarcoma. The presence of a known malignancy may not be evident in everyone, and even if available, histopathology will be necessary for diagnosis if this is the only site of recurrence/metastasis to differentiate from a primary soft-tissue sarcoma. Moreover, soft-tissue metastasis may be the initial presentation of a malignancy. Primary malignancies with soft-tissue metastasis carry a poor prognosis; hence, prompt diagnosis and management in essential. (orig.)

Magnetic resonance imaging appearance of soft-tissue metastases: our experience at an orthopedic oncology center

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Sammon, Jennifer; Jain, Abhishek; Bleakney, Robert; Mohankumar, Rakesh [Mount Sinai Hospital and University of Toronto, Division of Musculoskeletal Imaging, Joint Department of Medical Imaging, Toronto, Ontario (Canada)

2017-04-15

To assess the prevalence and magnetic resonance imaging appearance of metastasis presenting as a soft-tissue mass. A retrospective chart review was performed on 51 patients who presented to an orthopedic oncology center with soft-tissue masses, with a histology-proven diagnosis of soft-tissue metastasis, over a 14-year period. Their magnetic resonance imaging, primary origin, and follow-up have been assessed. Soft-tissue metastasis was identified in patients ranging from 18 to 85 years old. Most (80%) of the masses were located deep to the deep fascia. In our cohort of patients, melanoma was the most common primary malignancy contributing to soft-tissue metastasis (21.8%). Among soft-tissue metastasis from solid organs, breast and lung were the most frequent (9.1% each). Five patients had soft-tissue metastases from an unknown primary. Imaging diagnosis of soft-tissue metastases is challenging as it can demonstrate imaging appearances similar to primary soft-tissue sarcoma. The presence of a known malignancy may not be evident in everyone, and even if available, histopathology will be necessary for diagnosis if this is the only site of recurrence/metastasis to differentiate from a primary soft-tissue sarcoma. Moreover, soft-tissue metastasis may be the initial presentation of a malignancy. Primary malignancies with soft-tissue metastasis carry a poor prognosis; hence, prompt diagnosis and management in essential. (orig.)

Results of a phase I dose escalation study of eltrombopag in patients with advanced soft tissue sarcoma receiving doxorubicin and ifosfamide

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Chawla, Sant P; Staddon, Arthur; Hendifar, Andrew; Messam, Conrad A; Patwardhan, Rita; Kamel, Yasser Mostafa

2013-01-01

The objective of this Phase I dose escalation study was to explore the safety and tolerability of eltrombopag, an oral, nonpeptide, thrombopoietin receptor agonist, in patients with advanced soft tissue sarcoma (STS) and thrombocytopenia due to treatment with doxorubicin and ifosfamide (AI) combination chemotherapy. Patients aged 18 or older with histologically confirmed, locally advanced or metastatic STS were treated with 1 cycle of AI followed by AI with eltrombopag starting at Cycle 2, using 2 different dosing schedules. The study design included an eltrombopag dose escalation phase starting at 75 mg daily to determine the optimal biological dose (OBD). Eighteen patients were enrolled and 15 received at least 1 dose of chemotherapy; 3 patients withdrew prior to receiving eltrombopag. Seven, 4, and 1 patients received 75 mg, 100 mg, and 150 mg eltrombopag daily, respectively. No dose-limiting toxicities were reported. Due to slow recruitment, the study was closed prior to identifying an OBD. The most common hematologic adverse events (AEs) were thrombocytopenia (80%), neutropenia (73%), and anemia (67%). The most common nonhematologic AEs were fatigue (53%), alanine aminotransferase increased, constipation, and nausea (47% each). Eleven of 12 patients who received eltrombopag completed at least 2 chemotherapy cycles; all had increased platelet counts on Day 1 of Cycle 2 (cycle with eltrombopag) compared to Day 1 of Cycle 1 (cycle without eltrombopag). Although data are limited, safety data were consistent with the known toxicities of AI combination chemotherapy or the side effect profile of eltrombopag seen in other studies. Available data suggest a potential pre- and post-chemotherapy dosing scheme for eltrombopag when administered with AI chemotherapy, and support further investigation of eltrombopag treatment in patients with chemotherapy-induced thrombocytopenia

Feasibility and efficacy of external beam radiotherapy after hyperthermic isolated limb perfusion with TNF-α and melphalan for limb-saving treatment in locally advanced extremity soft-tissue sarcoma

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Olieman, Annette F.T.; Pras, Elisabeth; Ginkel, Robert J. van; Molenaar, Willemina M.; Koops, Heimen Schraffordt; Hoekstra, Harald J.

1998-01-01

Purpose: Hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor-α (TNF-α), interferon-γ (IFN-γ), and melphalan is associated with a dramatic antitumor effect in locally advanced extremity soft-tissue sarcomas (STS). The aim of this study was to demonstrate the feasibility and efficacy of adjuvant radiotherapy after HILP with TNF-α, IFN-γ, and melphalan and delayed surgical resection. Methods and Materials: Between 1991 and 1995, 34 patients--16 males and 18 females, median age 50 (range 18-80) years--underwent HILP for locally advanced extremity STS. Resection of the residual tumor mass was performed in most patients after 6-8 weeks. Fifteen patients with histopathological viable tumor after resection received adjuvent 60-70 Gy external beam radiotherapy (EBRT) (44%, HILP + EBRT group). Nineteen patients received HILP without adjuvent EBRT (56%, HILP-only group). Five patients in the HILP-only group had also distant metastases (15%) and received HILP as a palliative treatment. Treatment morbidity, local recurrences, and regional and distant metastases were scored. Results: During a median follow-up of 34 months (range 8-54), limb salvage was achieved in 29 patients (85%): 14 patients after HILP + EBRT and 15 patients after HILP only. None of the patients from the HILP + EBRT group developed local recurrences; however, five patients from the HILP-only did (26%) (p < 0.05). Regional metastases were observed in one patient from the HILP + EBRT group (7%) and in two patients from the HILP-only group who were treated with curative intent (14%). Distant metastases occurred in four patients after HILP + EBRT (27%) and in four patients after HILP only with curative intent (29%). The mean morbidity (subjective, objective, medical management, and analytical evaluation) score in both groups was, respectively, 0.33 for skin and subcutaneous tissue and for muscle and soft tissue, 0.34 (HILP + EBRT group) and 0.33 (HILP-only group). Conclusion: Adjuvent

Detection of soft-tissue sarcoma recurrence: added value of functional MR imaging techniques at 3.0 T.

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Del Grande, Filippo; Subhawong, Ty; Weber, Kristy; Aro, Michael; Mugera, Charles; Fayad, Laura M

2014-05-01

To determine the added value of functional magnetic resonance (MR) sequences (dynamic contrast material-enhanced [DCE] and quantitative diffusion-weighted [DW] imaging with apparent diffusion coefficient [ADC] mapping) for the detection of recurrent soft-tissue sarcomas following surgical resection. This retrospective study was approved by the institutional review board. The requirement to obtain informed consent was waived. Thirty-seven patients referred for postoperative surveillance after resection of soft-tissue sarcoma (35 with high-grade sarcoma) were studied. Imaging at 3.0 T included conventional (T1-weighted, fluid-sensitive, and contrast-enhanced T1-weighted imaging) and functional (DCE MR imaging, DW imaging with ADC mapping) sequences. Recurrences were confirmed with biopsy or resection. A disease-free state was determined with at least 6 months of follow-up. Two readers independently recorded the signal and morphologic characteristics with conventional sequences, the presence or absence of arterial enhancement at DCE MR imaging, and ADCs of the surgical bed. The accuracy of conventional MR imaging in the detection of recurrence was compared with that with the addition of functional sequences. The Fisher exact and Wilcoxon rank sum tests were used to define the accuracy of imaging features, the Cohen κ and Lin interclass correlation were used to define interobserver variability, and receiver operating characteristic analysis was used to define a threshold to detect recurrence and assess reader confidence after the addition of functional imaging to conventional sequences. There were six histologically proved recurrences in 37 patients. Sensitivity and specificity of MR imaging in the detection of tumor recurrence were 100% (six of six patients) and 52% (16 of 31 patients), respectively, with conventional sequences, 100% (six of six patients) and 97% (30 of 31 patients) with the addition of DCE MR imaging, and 60% (three of five patients) and 97% (30 of

A Longitudinal Study of Functional Outcomes in Patients with Limb Salvage Surgery for Soft Tissue Sarcoma

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Eunsun Oh

2018-01-01

Full Text Available Background. Many studies have reported on the surgical outcomes of soft tissue sarcoma. However, there was no longitudinal cohort study. Because time is the most valuable factor for functional recovery, adjusting time value was the key for finding the causal relationship between other risk factors and postoperative function. Therefore, existing cross-sectional studies can neither fully explain the causal relationship between the risk factors and the functional score nor predict functional recovery. The aim of this study was to determine important predictive factors that affect postoperative functional outcomes and longitudinal changes in functional outcomes in patients who had undergone limb-sparing surgery (LSS for soft tissue sarcoma (STS. Methods. Between January 2008 and December 2014, we retrospectively enrolled 150 patients who had undergone LSS for STS and had been assessed for postoperative functional outcomes with questionnaires. To evaluate functional outcomes, we used the Musculoskeletal Tumor Society (MSTS score and Toronto Extremity Salvage Score (TESS. Multivariate generalized estimating equation (GEE analysis was used to identify the predictive factors, including size, stage, and anatomic location of tumor, bone resection, flap reconstruction, age, and time after surgery. Each continuous variable such as age and time after surgery was explored for statistically significant cutoff points using the Wilcoxon rank sum test. Results. Functional scores significantly improved until the second year after surgery and plateaued for the rest of the 5-year period. Age p<0.0001, bone resection p=0.0004, and time after surgery p<0.0001 were identified as significant predictive factors. The functional score was significantly higher in patients younger than 47 years old. Conclusions. Functional outcomes can improve until the second year after surgery. Patients who were older than 47 and underwent bone resection may have poor final functional

Primary clear cell sarcoma of bone: a unique site of origin

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Gelczer, R.K.; Wenger, D.E.; Wold, L.E.

1999-01-01

Clear cell sarcoma is a rare soft tissue neoplasm, accounting for less than 1% of soft tissue sarcomas. We are presenting a case of a clear cell sarcoma of bone which, to our knowledge, is the only report of a primary clear cell sarcoma of bone. (orig.)

Doxorubicin plus evofosfamide versus doxorubicin alone in locally advanced, unresectable or metastatic soft-tissue sarcoma (TH CR-406/SARC021): an international, multicentre, open-label, randomised phase 3 trial.

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Tap, William D; Papai, Zsuzsanna; Van Tine, Brian A; Attia, Steven; Ganjoo, Kristen N; Jones, Robin L; Schuetze, Scott; Reed, Damon; Chawla, Sant P; Riedel, Richard F; Krarup-Hansen, Anders; Toulmonde, Maud; Ray-Coquard, Isabelle; Hohenberger, Peter; Grignani, Giovanni; Cranmer, Lee D; Okuno, Scott; Agulnik, Mark; Read, William; Ryan, Christopher W; Alcindor, Thierry; Del Muro, Xavier F Garcia; Budd, G Thomas; Tawbi, Hussein; Pearce, Tillman; Kroll, Stew; Reinke, Denise K; Schöffski, Patrick

2017-08-01

Evofosfamide is a hypoxia-activated prodrug of bromo-isophosphoramide mustard. We aimed to assess the benefit of adding evofosfamide to doxorubicin as first-line therapy for advanced soft-tissue sarcomas. We did this international, open-label, randomised, phase 3, multicentre trial (TH CR-406/SARC021) at 81 academic or community investigational sites in 13 countries. Eligible patients were aged 15 years or older with a diagnosis of an advanced unresectable or metastatic soft-tissue sarcoma, of intermediate or high grade, for which no standard curative therapy was available, an Eastern Cooperative Oncology Group performance status of 0-1, and measurable disease by Response Evaluation Criteria in Solid Tumors version 1.1. Patients were randomly assigned (1:1) to receive doxorubicin alone (75 mg/m 2 via bolus injection administered over 5-20 min or continuous intravenous infusion for 6-96 h on day 1 of every 21-day cycle for up to six cycles) or doxorubicin (given via the same dose procedure) plus evofosfamide (300 mg/m 2 intravenously for 30-60 min on days 1 and 8 of every 21-day cycle for up to six cycles). After six cycles of treatment, patients in the single-drug doxorubicin group were followed up expectantly whereas patients with stable or responsive disease in the combination group were allowed to continue with evofosfamide monotherapy until documented disease progression. A web-based central randomisation with block sizes of two and four was stratified by extent of disease, doxorubicin administration method, and previous systemic therapy. Patients and investigators were not masked to treatment assignment. The primary endpoint was overall survival, analysed in the intention-to-treat population. Safety analyses were done in all patients who received any amount of study drug. This study was registered with ClinicalTrials.gov, number NCT01440088. Between Sept 26, 2011, and Jan 22, 2014, 640 patients were enrolled and randomly assigned to a treatment group (317 to

Radionuclide imaging of soft tissue neoplasms

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Chew, F.S.; Hudson, T.M.; Enneking, W.F.

1981-01-01

Two classes of radiopharmaceuticals may be used for imaging tumors of the musculoskeletal system. The first is comprised of soft tissue or tumor specific agents such as gallium-67, bleomycin, and radionuclide-labeled antibodies, which may be useful for detecting and localizing these tumors. The other class of tracer is comprised of those with avidity for bone. The 99mTc-labeled-phosphate skeletal imaging compounds have been found to localize in a variety of soft tissue lesions, including benign and malignant tumors. In 1972, Enneking began to include bone scans in the preoperative evaluation of soft tissue masses. Later, he and his associates reported that these scans were useful in planning operative treatment of sarcomas by detecting involvement of bone by the tumors. Nearly all malignant soft tissue tumors take up bone-seeking radiopharmaceuticals, and bone involvement was indicated in two-thirds of the scans we reviewed. About half of benign soft tissue lesions had normal scans, but the other half showed uptake within the lesion and a few also showed bone involvement. Careful, thorough imaging technique is essential to proper evaluation. Multiple, high-resolution static gamma camera images in different projections are necessary to adequately demonstrate the presence or absence of soft tissue abnormality and to define the precise relationship of the tumor to the adjacent bone

Does an Algorithmic Approach to Using Brachytherapy and External Beam Radiation Result in Good Function, Local Control Rates, and Low Morbidity in Patients With Extremity Soft Tissue Sarcoma?

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Klein, Jason; Ghasem, Alex; Huntley, Samuel; Donaldson, Nathan; Keisch, Martin; Conway, Sheila

2018-03-01

High-dose-rate brachytherapy (HDR-BT) and external-beam radiation therapy (EBRT) are two modalities used in the treatment of soft tissue sarcoma. Previous work at our institution showed early complications and outcomes for patients treated with HDR-BT, EBRT, or a combination of both radiation therapy modalities. As the general indications for each of these approaches to radiation therapy differ, it is important to evaluate the use of each in an algorithmic way, reflecting how they are used in contemporary practice at sites that use these treatments. QUESTION/PURPOSES: (1) To determine the proportions of intermediate- and long-term complications associated with the use of brachytherapy in the treatment of primary high-grade extremity soft tissue sarcomas; (2), to characterize the long-term morbidity of the three radiation treatment groups using the Radiation Therapy Oncology Group/ European Organization for Research and Treatment of Cancer (RTOG/EORTC) Late Radiation Morbidity Scoring Scheme; (3) to determine whether treatment with HDR-BT, EBRT, and HDR-BT+EBRT therapy, in combination with limb-salvage surgery, results in acceptable local control in this high-risk group of sarcomas. We retrospectively studied data from 171 patients with a diagnosis of high-grade extremity soft tissue sarcoma treated with limb-sparing surgery and radiation therapy between 1990 and 2012 at our institution, with a mean followup of 72 months. Of the 171 patients, 33 (20%) were treated with HDR-BT, 128 (75%) with EBRT, and 10 (6%) with HDR-BT+EBRT. We excluded 265 patients with soft tissue sarcomas owing to axial tumor location, previous radiation to the affected extremity, incomplete patient records, patients receiving primary amputation, recurrent tumors, pediatric patients, low- and intermediate-grade tumors, and rhabdoid histology. Fifteen patients (9%) were lost to followup for any reason including died of disease or other causes during the first 12 months postoperatively. This

A general aspect on soft-tissue sarcoma and c-kit expression in primitive neuroectodermal tumor and Ewings sarcoma

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Kara, Ismail O.; Sahin, B.; Gonlusen, G.; Ergin, M.; Erdogan, S.

2005-01-01

Within soft-tissue sarcoma, primitive neuroectodermal tumors have been shown to cover a wide spectrum of small round cell sarcomas, including Ewings sarcomas (Es) and primitive neuroectodermal tumors (PET). The role of the stem cell factor/kit pathway has been investigated in different human tumors especially in chronic metallically leukemia and gastrointestinal stromal tumor and an autocrine loop has been assumed in small cell lung carcinoma, and recently in Es and PET. Our aim is to investigate the c-kit expression in Es and PET and also to assessed if c-kit has any role in disease process. We thoroughly searched the archives of the Department of Pathology, Faculty of Medicine, Cukurova University Turkey, between 2000 and 2004; and found 14 ES and 14 PNET paraffin embedded tissues. We carried out the detection of the c-kit expression by immunohistochemical staining. The patients median age was 23.7 +/-14.6 (12 male and 16 female). Five were diagnosed as metastatic disease whereas 23 were diagnosed as non-metastatic disease at admission. The mean follow up period was 38.9 +/- 22.3 months. The main localization of the disease was lower extremity (32.1%), and others were as follows: head and neck 25%, thorax and abdomen 14.3%, pelvic and upper extremity 7.1% (11 were localized skeletal and 17 were extraskeletal). According to treatment modalities, 10 were treated with surgery alone, 11 with surgery and chemotherapy, and 7 with surgery, radiation therapy and also with chemotherapy. The primary tumor was lower than 5 cm in its dimension in 21 patients. While in 5 patients, tumor was more than 5 cm but did not exceed 10 cm, it was >10 cm in 2 patients. The c-kit expression was positive in 7 patients both cytoplasmic and membranously, whereas 8 patients were positive cytoplasmically. In 5 PNET patients, c-kit expression were stained immunohistochemically in over 50% and in 3 of ES patients. There was no significant correlation between c-kit expression and gender

Outcome of primary soft tissue sarcoma of the knee and elbow

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Alektiar, Kaled M.; McKee, Andrea B.; Jacobs, Jordan M.; McKee, Brady J.; Healey, John H.; Brennan, Murray F.

2002-01-01

Purpose: Concern is frequently raised regarding the tolerance of irradiation over a joint space. The purpose of this study was to determine the outcome in terms of relapse and potential complications in patients with knee and elbow soft tissue sarcoma treated with limb-sparing surgery with or without adjuvant radiotherapy (RT). Methods and Materials: A review of our prospective database between June 1982 and December 1999 identified 86 adult patients with primary soft tissue sarcoma arising from the knee (n=65; 76%) or elbow (n=21; 24%) treated with limb-sparing surgery. Tumors had high-grade histologic features in 72% and were >5 cm in 48% of patients. Adjuvant RT was given to 46 (54%) of 86 patients. The type of RT was postoperative external beam RT in 63% and brachytherapy in 37%. Of the 46 patients who received RT, 85% (n=39) had deep, 78% (n=36) high-grade, and 54% (n=25) >5-cm tumors. Complications were assessed in terms of wound complications requiring repeated surgery, bone fracture, nerve damage, and joint stiffness. Results: With a median follow-up of 48 months (range 4-175), the 5-year actuarial rate of local control, distant control, and overall survival was 75% (95% confidence interval [CI] 64-85%), 82% (95% CI 73-91%), and 81% (95% CI 71-91%), respectively. The 5-year local control rate for patients who received RT was 80% vs. 71% for those who did not (p=0.3). The type of RT did not significantly influence the local control rate. Patients treated with external beam RT had a 5-year local control rate of 84% compared with 73% for those treated with brachytherapy (p=0.4). On multivariate analysis, tumor size >5 cm retained its significance as an independent predictor of poor local control (p=0.04; relative risk 3; 95% CI 1-6). In addition, high-grade histologic features emerged as an independent predictor of local recurrence (p=0.02; relative risk 4; 95% CI 1-20). No statistically significant difference was found between the RT and no-RT group in terms

Concomitant radiation therapy and doxorubicin by continuous Infusion in advanced malignancies - A phase I-II study - evidence of synergistic effect in Soft tissue sarcomas and hepatomas

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Rosenthal, C.J.; Bhutiani, I.; Rotman, M.

1986-01-01

This paper attempts to establish the dose and the duration of administration of doxorubicin infusion having the best therapeutic index and to determine the dose of radiation which in combination with the optimal dose of doxorubicin administered by continuous infusion would have the best therapeutic index. The authors attempt to find the nature and the incidence of side effects of these combined modalities of therapy. The therapeutic effect of the optimal doxorubicin infusion dose in combination with concomitant radiotherapy is assessed in a preliminary study and it was found to have a significant enhancing effect in a few cases of hepatomas and in most of the cases of recurrent and metastatic soft tissue sarcomas

overall cost in recurrent soft-tissue sarcoma

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Arash O. Naghavi

2017-01-01

Full Text Available Purpose : Adjuvant brachytherapy (AB with immediate (IR and staged reconstruction (SR are distinct treatment modalities available for patients with recurrent soft tissue sarcoma (STS. Although SR may offer local control and toxicity benefit, it requires additional upfront procedures, and there is no evidence that it improves overall survival. With the importance of value-based care, our goal is to identify which technique is more cost effective. Material and methods : A retrospective review of 22 patients with recurrent extremity STS treated with resection followed by AB alone. Hospital charges were used to compare the cost between SR and IR at the time of initial treatment, at 6-month intervals following surgery, and cumulative cost comparisons at 18 months. Results : Median follow-up was 31 months. Staged reconstruction (n = 12 was associated with an 18-month local control benefit (85% vs. 42%, p = 0.034, compared to IR (n = 10. Staged reconstruction had a longer hospital stay during initial treatment (10 vs. 3 days, p = 0.002, but at 18 months, the total hospital stay was no longer different (11 vs. 11 days. Initially, there was no difference in the cost of SR and IR. With longer follow-up, cost eventually favored SR, which was attributed primarily to the costs associated with local failure (LF. On multivariate analysis, cost of initial treatment was associated with length of hospital stay (~$4.5K per hospital day, p < 0.001, and at 18 months, the cumulative cost was ~175K lower with SR (p = 0.005 and $58K higher with LF (p = 0.02. Conclusions : In recurrent STS, SR has a longer initial hospital stay when compared to IR. At 18 months, SR had lower rates of LF, translating to lower total costs for the patient. SR is the more cost-effective brachytherapy approach in the treatment of STS, and should be considered as healthcare transitions into value-based medicine.

Targeted therapy for sarcomas

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Forscher C

2014-03-01

Full Text Available Charles Forscher,1 Monica Mita,2 Robert Figlin3 1Sarcoma Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 2Experimental Therapeutics Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 3Academic Development Program, Samuel Oschin Comprehensive Cancer Institute, and Division of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Abstract: Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing's sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing's sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. Keywords: sarcoma, targeted agents, tyrosine kinase inhibitors, mTor inhibition

Occurrence of Comorbidities before and after Soft Tissue Sarcoma Diagnosis

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Myrthe P. P. van Herk-Sukel

2012-01-01

Full Text Available Background. Data is limited on the burden of common comorbidities, such as cardiovascular disease (CVD, respiratory disease and diabetes, or comorbidities related to cancer and its treatment, such as anemia and depression, in patients with soft tissue sarcoma (STS. Patients and Methods. From the Dutch Pathology Registry linked to the PHARMO database (including data on drug use and hospitalizations, 533 patients with STS were selected during 2000–2007 and matched 1 : 10 to cancer-free controls. The occurrences of comorbidities were assessed in the 12 months before and after STS diagnosis. Results. STS patients were 2–4 times more likely to have comorbidities at diagnosis compared with cancer-free controls. The incidence of CVD, anemia, and depression after STS diagnosis differed significantly from cancer-free controls and decreased during followup from 40–124 per 1,000 person-years (py during the first six months to 11–38 per 1,000 py more than 12 months after diagnosis. The incidence of respiratory disease and diabetes among STS patients remained stable during followup (5–21 per 1,000 py and did not differ significantly from cancer-free controls. Conclusions. STS patients were more likely to have comorbidities before cancer diagnosis and to develop CVD, anemia, and depression after diagnosis compared to cancer-free controls.

[Histopathological diagnostic concordance in bone and soft tissue sarcomas between two comprehensive cancer centers from eastern and western Europe: a collaborative experience].

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Somcutian, Oana; Buiga, Rares; Galatir, Mihaela; Tudor Eniu, Dan; Rachieru, Claudiu; Coza, Daniela; Terrier, Philippe

2015-01-01

This study aims to assess the degree of concordance of histological diagnosis of bone and soft tissue sarcomas between a Comprehensive Cancer Center (CCC) of Eastern Europe - not specialized in this area of pathology - and an important CCC of Western Europe, which is one of the coordinators of a clinical reference network in sarcoma pathology. The goal is to have an overview of the sarcomatous pathology in a region of Eastern Europe and to discover diagnostic discrepancies between the two centers, while determining their cause. The initial diagnosis was compared with the revised diagnosis on 110 specimens from 88 patients with bone or soft tissue sarcomas from East-European CCC, in a one-year period of time. Complete diagnostic agreement was observed in 55 cases (62.5%), a partial agreement in 23 cases (26.1%) and a major disagreement in 10 cases (11.4%). Major discrepancies of the histological type was observed in only 3 cases (3.4%): one case of discordance benign/malignant and 2 cases of discordance mesenchymal/non mesenchymal. Minor histological discrepancies - not affecting the management of the patient - were observed in 18 cases (20.4%). A major discordance in grading - potentially changing the management of the patient - was noted in 7 cases (7.9%), and a minor discrepancy in 5 cases (5.7%). Some histological types were clearly overdiagnosed, like "adult fibrosarcomas" and "malignant peripheral nerve sheet tumors" (MPNST), mostly converted after the audit into "undifferentiated spindle cell sarcomas" or other types of sarcomas. Some "unclassified" sarcomas and "undifferentiated pleomorphic sarcomas" could be re-classified with the aid of an extensive panel of antibodies. Overall, immunohistochemistry was responsible, but not in exclusivity, for half of the minor discrepancies, and for 2 out of 3 cases of major histological discrepancies. Otherwise, the main cause of discrepancies was the difficulties in the interpretation of the morphology. Molecular

Prognostic Metabolite Biomarkers for Soft Tissue Sarcomas Discovered by Mass Spectrometry Imaging

Science.gov (United States)

Lou, Sha; Balluff, Benjamin; Cleven, Arjen H. G.; Bovée, Judith V. M. G.; McDonnell, Liam A.

2017-02-01

Metabolites can be an important read-out of disease. The identification and validation of biomarkers in the cancer metabolome that can stratify high-risk patients is one of the main current research aspects. Mass spectrometry has become the technique of choice for metabolomics studies, and mass spectrometry imaging (MSI) enables their visualization in patient tissues. In this study, we used MSI to identify prognostic metabolite biomarkers in high grade sarcomas; 33 high grade sarcoma patients, comprising osteosarcoma, leiomyosarcoma, myxofibrosarcoma, and undifferentiated pleomorphic sarcoma were analyzed. Metabolite MSI data were obtained from sections of fresh frozen tissue specimens with matrix-assisted laser/desorption ionization (MALDI) MSI in negative polarity using 9-aminoarcridine as matrix. Subsequent annotation of tumor regions by expert pathologists resulted in tumor-specific metabolite signatures, which were then tested for association with patient survival. Metabolite signals with significant clinical value were further validated and identified by high mass resolution Fourier transform ion cyclotron resonance (FTICR) MSI. Three metabolite signals were found to correlate with overall survival ( m/z 180.9436 and 241.0118) and metastasis-free survival ( m/z 160.8417). FTICR-MSI identified m/z 241.0118 as inositol cyclic phosphate and m/z 160.8417 as carnitine.

Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

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Habib Reshadi

2014-06-01

Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

Directory of Open Access Journals (Sweden)

Habib Reshadi

2014-06-01

Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

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Faris Shweikeh

2014-01-01

Full Text Available Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing’s sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months (osteosarcoma, Ewing sarcoma, chordoma, angiosarcoma, and rhabdomyosarcoma, some at 24–36 months (malignant fibrous tumors, malignant peripheral nerve sheath tumors, and alveolar soft part sarcoma, and a few after 36 months (chondrosarcoma and liposarcoma. Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months (Ewing’s sarcoma, liposarcoma, malignant fibrous tumors, malignant peripheral nerve sheath tumors, angiosarcoma and chordomas. Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease.

Undifferentiated pleomorphic sarcoma: indolent, tail-like recurrence of a high-grade tumor

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Alpert, Justin S. [Memorial Sloan Kettering Cancer Center, Department of Radiology, New York, NY (United States); Boland, Patrick [Memorial Sloan Kettering Cancer Center, Division of Orthopaedic Surgery, Department of Surgery, New York, NY (United States); Weill Medical College of Cornell University, New York, NY (United States); Hameed, Meera [Memorial Sloan Kettering Cancer Center, Department of Pathology, New York, NY (United States); Panicek, David M. [Memorial Sloan Kettering Cancer Center, Department of Radiology, New York, NY (United States); Weill Medical College of Cornell University, New York, NY (United States)

2018-01-15

Recurrence of a soft tissue sarcoma typically manifests as a round or oval mass at imaging, and recurrent high-grade soft tissue sarcomas generally enlarge relatively rapidly. We present a case of high-grade undifferentiated pleomorphic sarcoma in the calf of a 48-year-old male that recurred as a thin, curvilinear ''tail'' of enhancing tissue at magnetic resonance imaging (MRI), with extremely indolent growth over a 7-year period. The unusual imaging finding of a slowly enlarging ''tail'' should not be dismissed as postoperative changes, even for a high-grade soft tissue sarcoma. (orig.)

Patterns of Local Recurrence and Dose Fractionation of Adjuvant Radiation Therapy in 462 Patients With Soft Tissue Sarcoma of Extremity and Trunk Wall

International Nuclear Information System (INIS)

Jebsen, Nina L.; Engellau, Jacob; Engström, Katarina; Bauer, Henrik C.; Monge, Odd R.; Muren, Ludvig P.; Eide, Geir E.; Trovik, Clement S.; Bruland, Øyvind S.

2013-01-01

Purpose: To study the impact of dose fractionation of adjuvant radiation therapy (RT) on local recurrence (LR) and the relation of LR to radiation fields. Methods and Materials: LR rates were analyzed in 462 adult patients with soft tissue sarcoma who underwent surgical excision and adjuvant RT at five Scandinavian sarcoma centers from 1998 to 2009. Medical records were reviewed for dose fractionation parameters and to determine the location of the LR relative to the radiation portals. Results: Fifty-five of 462 patients developed a LR (11.9%). Negative prognostic factors included intralesional surgical margin (hazard ratio [HR]: 7.83, 95% confidence interval [CI]: 3.08-20.0), high malignancy grade (HR: 5.82, 95% CI: 1.31-25.8), age at diagnosis (HR per 10 years: 1.27, 95% CI: 1.03-1.56), and malignant peripheral nerve sheath tumor histological subtype (HR: 6.66, 95% CI: 2.56-17.3). RT dose was tailored to margin status. No correlation between RT dose and LR rate was found in multiple Cox regression analysis. The majority (65%) of LRs occurred within the primary RT volume. Conclusions: No significant dose–response effect of adjuvant RT was demonstrated. Interestingly, patients given 45-Gy accelerated RT (1.8 Gy twice daily/2.5 weeks) had the best local outcome. A total dose of 50 Gy in 25 fractions seemed adequate following wide margin surgery. The risk of LR was associated with histopathologic subtype, which should be included in the treatment algorithm of adjuvant RT in soft tissue sarcoma

Patterns of Local Recurrence and Dose Fractionation of Adjuvant Radiation Therapy in 462 Patients With Soft Tissue Sarcoma of Extremity and Trunk Wall

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Jebsen, Nina L., E-mail: nina.louise.jebsen@helse-bergen.no [Department of Clinical Medicine, Faculty of Medicine and Dentistry, University of Bergen, Bergen (Norway); Department of Oncology, Haukeland University Hospital, Bergen (Norway); Engellau, Jacob [Department of Oncology, Skåne University Hospital, Lund (Sweden); Engström, Katarina [Department of Oncology, Sahlgrenska University Hospital, Gothenburg (Sweden); Bauer, Henrik C. [Department of Molecular Medicine and Surgery, Section for Orthopaedics and Sports Medicine, Karolinska University Hospital, Karolinska Institute, Stockholm (Sweden); Monge, Odd R. [Department of Oncology, Haukeland University Hospital, Bergen (Norway); Muren, Ludvig P. [Department of Physics and Technology, University of Bergen, Bergen (Norway); Department of Medical Physics, Aarhus University and Aarhus University Hospital, Aarhus (Denmark); Eide, Geir E. [Centre for Clinical Research, Haukeland University Hospital, Bergen (Norway); Department of Public Health and Primary Health Care, University of Bergen, Bergen (Norway); Trovik, Clement S. [Department of Clinical Medicine, Faculty of Medicine and Dentistry, University of Bergen, Bergen (Norway); Department of Oncology, Haukeland University Hospital, Bergen (Norway); Bruland, Øyvind S. [Department of Oncology, The Norwegian Radium Hospital, Oslo University Hospital, Oslo (Norway); Institute of Clinical Medicine, University of Oslo, Oslo (Norway)

2013-08-01

Purpose: To study the impact of dose fractionation of adjuvant radiation therapy (RT) on local recurrence (LR) and the relation of LR to radiation fields. Methods and Materials: LR rates were analyzed in 462 adult patients with soft tissue sarcoma who underwent surgical excision and adjuvant RT at five Scandinavian sarcoma centers from 1998 to 2009. Medical records were reviewed for dose fractionation parameters and to determine the location of the LR relative to the radiation portals. Results: Fifty-five of 462 patients developed a LR (11.9%). Negative prognostic factors included intralesional surgical margin (hazard ratio [HR]: 7.83, 95% confidence interval [CI]: 3.08-20.0), high malignancy grade (HR: 5.82, 95% CI: 1.31-25.8), age at diagnosis (HR per 10 years: 1.27, 95% CI: 1.03-1.56), and malignant peripheral nerve sheath tumor histological subtype (HR: 6.66, 95% CI: 2.56-17.3). RT dose was tailored to margin status. No correlation between RT dose and LR rate was found in multiple Cox regression analysis. The majority (65%) of LRs occurred within the primary RT volume. Conclusions: No significant dose–response effect of adjuvant RT was demonstrated. Interestingly, patients given 45-Gy accelerated RT (1.8 Gy twice daily/2.5 weeks) had the best local outcome. A total dose of 50 Gy in 25 fractions seemed adequate following wide margin surgery. The risk of LR was associated with histopathologic subtype, which should be included in the treatment algorithm of adjuvant RT in soft tissue sarcoma.

Prediction of treatment response and metastatic disease in soft tissue sarcoma

Science.gov (United States)

Farhidzadeh, Hamidreza; Zhou, Mu; Goldgof, Dmitry B.; Hall, Lawrence O.; Raghavan, Meera.; Gatenby, Robert A.

2014-03-01

Soft tissue sarcomas (STS) are a heterogenous group of malignant tumors comprised of more than 50 histologic subtypes. Based on spatial variations of the tumor, predictions of the development of necrosis in response to therapy as well as eventual progression to metastatic disease are made. Optimization of treatment, as well as management of therapy-related side effects, may be improved using progression information earlier in the course of therapy. Multimodality pre- and post-gadolinium enhanced magnetic resonance images (MRI) were taken before and after treatment for 30 patients. Regional variations in the tumor bed were measured quantitatively. The voxel values from the tumor region were used as features and a fuzzy clustering algorithm was used to segment the tumor into three spatial regions. The regions were given labels of high, intermediate and low based on the average signal intensity of pixels from the post-contrast T1 modality. These spatially distinct regions were viewed as essential meta-features to predict the response of the tumor to therapy based on necrosis (dead tissue in tumor bed) and metastatic disease (spread of tumor to sites other than primary). The best feature was the difference in the number of pixels in the highest intensity regions of tumors before and after treatment. This enabled prediction of patients with metastatic disease and lack of positive treatment response (i.e. less necrosis). The best accuracy, 73.33%, was achieved by a Support Vector Machine in a leave-one-out cross validation on 30 cases predicting necrosis treatment and metastasis.

Acute and long-term adverse effects of postoperative irradiation on limb function of combined modality limb sparing therapy for extermity of soft tissue sarcoma

International Nuclear Information System (INIS)

Glinski, B.; Dymek, P.; Walasek, T.; Pecak, M.; Rogowska, M.

2008-01-01

The aim of this study is to focus on the aspect of functional outcome for early and late normal tissues damage for patients undergoing postoperative radiotherapy after limb preservation surgery for extremity soft tissues sarcoma (ESTS). The review of the literature suggests that approximately 50% of patients with ESTS have significant impairments, but synthesis of the results is difficult because of the heterogeneity of outcomes used to evaluate function. Future studies require the use of standardized definitions, and reliable and functional outcome measures. (author)

A Systematic Overview of Radiation Therapy Effects in Soft Tissue Sarcomas

International Nuclear Information System (INIS)

Strander, Hans; Turesson, Ingela; Cavallin-Staahl, Eva

2003-01-01

A systematic review of radiation therapy trials in several tumour types was performed by The Swedish Council of Technology Assessment in Health Care (SBU). The procedures for evaluation of the scientific literature are described separately. This synthesis of the literature on radiation therapy for soft tissue sarcomas (STS) is based on data from five randomized trials. Moreover, data from 6 prospective studies, 25 retrospective studies and 3 other articles were used. In total, 39 scientific articles are included, involving 4,579 patients. The results were compared with those of a similar overview from 1996 which included 3,344 patients. The conclusions reached can be summarized as follows: The well-established prognostic factors for tumour-related death from STS--histological grade, tumour size and age--are well documented. The importance of superficial versus deep site as well as the anatomic site is also reaffirmed to some extent. There is strong evidence that adjuvant radiotherapy improves the local control rate in combination with conservative surgery in the treatment of STS of extremities and trunk in patients with negative, marginal or minimal microscopic positive surgical margins. A local control rate of 90% has been achieved. Improvement is obtained with radiotherapy added in the case of intralesional surgery, but the local control rate is somewhat lower. More studies are needed on this issue. For STS in other anatomic sites, retroperitoneum, head and neck, breast and uterus, there is only weak indication of a benefit for the local control rate, with the use of adjuvant radiotherapy. There is still insufficient data to establish that preoperative radiotherapy is favourable compared to postoperative radiotherapy for local control in patients presenting primarily with large tumours. One small study has shown a possible survival benefit for preoperative radiotherapy. There is fairly good evidence to suggest that the preoperative setting results in more wound

Clear cell sarcoma of the abdominal wall with peritoneal sarcomatosis: CT features

International Nuclear Information System (INIS)

Sabate, J.M.; Fernandez, A.; Torrubia, S.; Villanueva, A.; Monill, J.M.

1999-01-01

Clear cell sarcoma, also called malignant melanoma of soft parts, is an uncommon neoplasm that involves tendons or aponeuroses of the lower extremity. The CT features of a clear cell sarcoma arising from the abdominal wall with later peritoneal dissemination are described. Peritoneal sarcomatosis from soft tissue sarcomas is a very rare condition previously unreported in the radiologic literature. Metastases to peritoneal surfaces must therefore be considered a possible site for systemic dissemination of soft tissue sarcomas. (orig.)

Prognostic relevance of {sup 18}F-FDG PET uptake in patients with locally advanced, extremity soft tissue sarcomas undergoing neoadjuvant isolated limb perfusion with TNF-α and melphalan

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Andreou, Dimosthenis [Muenster University Hospital, Department of General Orthopedics and Tumor Orthopedics, Muenster (Germany); HELIOS Klinikum Berlin-Buch, Department of Orthopedic Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Boldt, Henrike [HELIOS Klinikum Berlin-Buch, Department of Nuclear Medicine, Berlin (Germany); Pink, Daniel [HELIOS Klinikum Bad Saarow, Department of Hematology, Oncology and Palliative Care, Sarcoma Center Berlin-Brandenburg, Bad Saarow (Germany); Jobke, Bjoern [HELIOS Klinikum Berlin-Buch, Department of Radiology, Berlin (Germany); Werner, Mathias [HELIOS Klinikum Emil von Behring, Department of Pathology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Schuler, Markus [University Hospital Carl Gustav Carus Dresden, Department of Internal Medicine I, Dresden (Germany); Reichardt, Peter [HELIOS Klinikum Berlin-Buch, Department of Interdisciplinary Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany); Tunn, Per-Ulf [HELIOS Klinikum Berlin-Buch, Department of Orthopedic Oncology, Sarcoma Center Berlin-Brandenburg, Berlin (Germany)

2014-06-15

The objective of this study was to determine whether {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET) can adequately assess the risk of systemic disease progression in patients with primary, localized, high-grade soft tissue sarcomas of the extremities undergoing neoadjuvant isolated limb perfusion (ILP) with tumour necrosis factor and melphalan. This was a retrospective analysis of the files of 35 patients who underwent a PET or PET/CT scan prior to and after ILP followed by surgical resection with curative intent between 2006 and 2012. SUV{sub max1} was defined as the maximum standardized uptake value (SUV) at diagnosis, SUV{sub max2} as the maximum SUV after ILP and ΔSUV{sub max} as the percentage difference between SUV{sub max1} and SUV{sub max2}. The median follow-up was 40 months for all patients. The median SUV{sub max1} amounted to 7.6, while the median SUV{sub max2} was 4.7. The median ΔSUV{sub max} was -44 %. Overall survival (OS) probability at 2 and 5 years amounted to 78 and 70 %, respectively, while metastasis-free survival (MFS) probability at 2 and 5 years was 67 and 64 %, respectively. Receiver-operating characteristic (ROC) curve analysis showed that both SUV{sub max2} and ΔSUV{sub max} could predict systemic disease progression, while SUV{sub max1} could not adequately identify patients who went on to develop metastatic disease. The optimal cut-off value was 6.9 for SUV{sub max2} and -31 % for ΔSUV{sub max}. Patients with an SUV{sub max2} <6.9 had a 2-year MFS of 80 %, compared to 31 % for patients with an SUV{sub max2} ≥ 6.9 (p < 0.001). Patients with a ΔSUV{sub max} < -31 %, i.e. patients with a higher metabolic response, had an MFS of 76 % at 2 years, compared to 42 % for patients with a ΔSUV{sub max} ≥ -31 % (p = 0.050). SUV{sub max} after ILP for primary, locally advanced, non-metastatic high-grade soft tissue sarcomas of the extremities appears to be significantly correlated with prognosis. Whether patients

Imaging of benign and malignant soft tissue masses of the foot

International Nuclear Information System (INIS)

Waldt, Simone; Rummeny, Ernst J.; Woertler, Klaus; Rechl, Hans

2003-01-01

The foot is a relatively uncommon site of neoplastic and non-neoplastic soft tissue tumors. Although it contains a relatively small amount of somatic soft tissue elements, the foot is considerably rich in tendons, fasciae, retinaculae, and synovium. Corresponding to this distribution of soft tissue elements, some soft tissue lesions, such as giant cell tumor of tendon sheath, fibromatosis, and synovial sarcoma, are commonly seen in this location. Vascular tumors represent common soft tissue masses of the foot as well. Magnetic resonance imaging is the modality of choice in the assessment of soft tissue tumors. The presence of a suspected lesion can be confirmed and tumor margins can be defined accurately. In general, MRI does not provide histologic specificity, but considering some MR features may often help in correctly distinguishing benign from malignant lesions. In addition, characteristic features of the most common benign tumors (i.e., fibromatosis, cavernous hemangioma) and reactive processes of the foot (ganglion cyst, Morton's neuroma) often suggest a specific diagnosis. (orig.)

Soft tissue changes and its stability as a sequlae to mandibular advancement.

Science.gov (United States)

Uppada, Uday Kiran; Sinha, Ramen; Reddy, D Sreenatha; Paul, Dushyanth

2014-01-01

To predict the changes and evaluate the stability that occurs in the soft tissues following the skeletal movement subsequent to surgical advancement of the mandible through bilateral sagittal split osteotomy and to provide the patient reliable information with regard to esthetic changes that can be expected following the treatment. Twenty adult patients diagnosed with skeletal class II malocclusion and underwent bilateral sagittal split osteotomy for mandibular advancement by a mean of 8 mm using rigid fixation were included in the study. Soft tissue changes brought about by the surgical procedure and their stability over a period of time were evaluated prospectively using 12 linear (4 vertical and 8 horizontal) and 4 angular measurements on profile cephalograms which were taken preoperatively after the pre-surgical orthodontics (T1) and postoperatively with duration of 1 month (T2) and 6 months (T3) respectively. It was observed that compared to the linear measurements, the angular measurements showed significant changes. The improvement in the esthetic outcome is a direct reflection of the angular changes whereas the linear changes played a contributing role. Following mandibular advancement surgery the profiles of the patients was perceived to have improved with reduction in the facial convexity, an increase in the lower facial height, decrease in the depth of the mentolabial sulcus and improvement in the lip competency with lengthening, straightening and thinning of the lower lip. The soft tissue response and its stability depends on the stability of the surgical procedure itself, postsurgical growth and remodeling of the hard tissues and soft tissue changes as a result of maturation and aging.

Neoadjuvant chemoradiation (modified Eilber protocol) versus adjuvant radiotherapy in the treatment of extremity soft tissue sarcoma

International Nuclear Information System (INIS)

Lehane, Chris; Parasyn, Andrew; Ho, Frederick; Thompson, Stephen R.; Smee, Robert; Links, David; Crowe, Phil; Lewis, Craig; Friedlander, Michael; Williams, Janet

2016-01-01

Local control for extremity soft tissue sarcomas (STS) requires surgery combined with radiotherapy, usually given pre-operatively or post-operatively. The modified Eilber protocol, a neoadjuvant chemoradiation regimen, has been reported with excellent local control rates. This retrospective single-centre study compared outcomes for patients treated with the modified Eilber protocol with those treated with standard adjuvant radiotherapy. Twenty-nine patients were treated with modified Eilber protocol. Thirty-four patients received adjuvant radiotherapy. Three patients (10%) in the Eilber group and five patients (15%) in the Adjuvant group developed local recurrence (P = 0.87). Major acute wound complications were noted in four patients in each group (P = 0.55). One patient (3.4%) in the Eilber group developed Grade 3 or 4 late toxicities after 1 year compared with nine patients (27%) in the Adjuvant group (P = 0.02). Patients with a diagnosis of extremity STS were retrospectively reviewed from the Prince of Wales Hospital Sarcoma Database from 1995 to 2012. Sixty-three patients underwent curative surgery with either neoadjuvant Eilber chemoradiotherapy (Eilber) or adjuvant radiotherapy (Adjuvant). Neoadjuvant chemoradiation (Eilber protocol) provided similar rates of local control when compared with adjuvant radiotherapy. Acute wound complication rates were similar but there was less severe late toxicity in the Eilber group.

Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

International Nuclear Information System (INIS)

Stacy, G.S.; Nair, L.

2007-01-01

The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult

Adjuvant radiotherapy with brachytherapy boost in soft tissue sarcomas

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Annalisa Cortesi

2017-06-01

Full Text Available Purpose: The standard primary treatment for soft tissue sarcoma (STS is a wide surgical resection, preceded or followed by radiotherapy. Purpose of this retrospective study was to assess the efficacy of perioperative brachytherapy (BRT plus postoperative external beam radiation therapy (EBRT in patients with intermediate-high risk STS. Material and methods : BRT delivered dose was 20 Gy. External beam radiation therapy was delivered with 3D-technique using multiple beams. The prescribed dose was 46 Gy to the PTV. Neoadjuvant and adjuvant chemotherapy (CHT was used in patients with potentially chemosensitive histological subtypes. The primary aim of the study was to analyze overall survival (OS and local control (LC in a large patient population treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Secondary objective was to identify prognostic factors for patients outcome in terms of LC, disease-free survival (DFS, and OS. Results : From 2000 to 2011, 107 patients presenting 2-3 grade (FNLCC primary or recurrent STS were treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Five-year LC and OS were 80.9% and 87.4%, respectively. At univariate analysis, a higher LC was recorded in primary vs. recurrent tumors (p = 0.015, and in lower limb tumors vs. other sites (p = 0.027. An improved DFS was recorded in patients with lower limb tumors vs. other sites (p = 0.034. Conclusions : The combination of BRT and EBRT was able to achieve satisfactory results even in a patients population with intermediate-high risk STS. Patients with recurrent or other than lower limb sited tumors show a worse LC.

Adjuvant radiotherapy with brachytherapy boost in soft tissue sarcomas

Science.gov (United States)

Cortesi, Annalisa; Galuppi, Andrea; Arcelli, Alessandra; Romani, Fabrizio; Mattiucci, Gian Carlo; Bianchi, Giuseppe; Ferrari, Stefano; Ferraro, Andrea; Farioli, Andrea; Gambarotti, Marco; Righi, Alberto; Macchia, Gabriella; Deodato, Francesco; Cilla, Savino; Buwenge, Milly; Valentini, Vincenzo; Morganti, Alessio Giuseppe; Donati, Davide; Cammelli, Silvia

2017-01-01

Purpose The standard primary treatment for soft tissue sarcoma (STS) is a wide surgical resection, preceded or followed by radiotherapy. Purpose of this retrospective study was to assess the efficacy of perioperative brachytherapy (BRT) plus postoperative external beam radiation therapy (EBRT) in patients with intermediate-high risk STS. Material and methods BRT delivered dose was 20 Gy. External beam radiation therapy was delivered with 3D-technique using multiple beams. The prescribed dose was 46 Gy to the PTV. Neoadjuvant and adjuvant chemotherapy (CHT) was used in patients with potentially chemosensitive histological subtypes. The primary aim of the study was to analyze overall survival (OS) and local control (LC) in a large patient population treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Secondary objective was to identify prognostic factors for patients outcome in terms of LC, disease-free survival (DFS), and OS. Results From 2000 to 2011, 107 patients presenting 2-3 grade (FNLCC) primary or recurrent STS were treated with surgery, perioperative BRT, and adjuvant EBRT ± CHT. Five-year LC and OS were 80.9% and 87.4%, respectively. At univariate analysis, a higher LC was recorded in primary vs. recurrent tumors (p = 0.015), and in lower limb tumors vs. other sites (p = 0.027). An improved DFS was recorded in patients with lower limb tumors vs. other sites (p = 0.034). Conclusions The combination of BRT and EBRT was able to achieve satisfactory results even in a patients population with intermediate-high risk STS. Patients with recurrent or other than lower limb sited tumors show a worse LC. PMID:28725250

MR imaging of uncommon soft tissue tumors in the foot: a pictorial essay

Energy Technology Data Exchange (ETDEWEB)

Lee, Youn Joo; Chun, Kyung Ah; Kim, Jee Young; Sung, Mi Sook; Kim, Ki Tae [The Catholic University of Korea, Uijeongbu (Korea, Republic of)

2007-06-15

The large variety of masses occur in the foot. The foot is a comparatively rare site of soft tissue neoplasms. MRI has greatly improved the ability to detect and delineate soft tissue lesions and is now considered the gold-standard imaging technique in their investigation. Recently, we have encountered rare soft tissue tumors of the foot. The presented cases include benign masses such as granuloma annulare, angiomyoma, neural fibrolipoma, and giant cell tumor of tendon sheath, as well as malignant tumors such as melanoma, synovial sarcoma, rhabdomyosarcoma and extraskeletal myxoid chondrosarcoma. We wish to illustrate the MR findings of these uncommon soft tissue mors to aid in their diagnosis.

Recurrence and Mortality after Surgical Treatment of Soft Tissue ...

African Journals Online (AJOL)

In Africa, patients with soft tissue sarcomas generally present late and have to travel long distances to treatment centers. Many do not benefit from radiotherapy and chemotherapy after the surgical procedure due to inadequate resources. As such recurrence rates may be much higher than those reported elsewhere (11).

Molecular developmental biology

International Nuclear Information System (INIS)

Bogorad, L.

1986-01-01

This book contains nine chapters. The chapter titles are: Pathology of soft tissue sarcomas: Diagnostic strategy for adult soft tissue sarcomas: Staging of soft tissue sarcomas: Surgical treatment of soft tissue sarcomas: Radiotherapy; Chemotherapy in advanced soft tissue sarcomas: Adjuvant chemotherapy for soft tissue sarcomas; Intra-arterial infusion and perfusion chemotherapy for soft tissue sarcomas of the extremities; and Phase II new drug trials in soft tissue sarcomas

EPIDEMIOLOGY AND SURVIVAL OF PATIENTS WITH MALIGNANT TUMORS OF CONNECTIVE AND SOFT TISSUE

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V. M. Merabishvili

2015-01-01

Full Text Available Introduction. Malignant tumors of connective and soft tissue are met relatively rare, although in general in Russia each year more than 1.500 new cases are registered. On five administrative territories of Russia during a year there are recorded less than 5 new cases of malignant tumors of connective and soft tissue (Yamal-Nenets A.R. – 4; Tuva Republic – 0, Magadan Region – 3; Chukotka A.R. – 0; Jewish A.R. – 4. More seldom data on these patients’ survival are published. Purpose of study. To estimate dynamics of incidence of malignant tumors of connective and soft tissue on the basis of public reporting, to calculate the index accuracy and observed and relative survival rates by histological forms, including sarcomas. Material and methods. To perform a detailed study there were selected, for two periods of observation, respectively 1054 patients (1995–2001 and 919 patients (2002–2008. Estimation of survival was carried out using software, which had been developed together with Ltd. «Novel» (Director – T.L.Tsvetkova, Ph.D.. results of study. The most typical incidence rate for of malignant tumors of connective and soft tissue (S47, 49 that are presented by  cancer registries of different countries is from 1.5 to 2.5 0/   in men and 1.5–2.0 0/   in women. Dynamics  of morbidity of the Russian population, Moscow and St. Petersburg indicates that the level of standardized  incidence rates is in the range of 2.0 0/   in men and within 1.5 0/   in women. The mortality rate in 2013  was respectively for men and women in Russia in total 1.7 0/   and 1.13 0/   , in Moscow – 1.42 0/   and  1.24 0/   , in St. Petersburg – 1.88 0/   and 1.26 0/   . The index accuracy for both sexes in Russia is 0.88,  in Moscow – 1.2; in St. Petersburg – 1.4. This index should be used for the site of these diseases with high fatality. According to official data a one-year lethality of patients with tumors of connective and soft

Ultrasound for initial evaluation and triage of clinically suspicious soft-tissue masses

International Nuclear Information System (INIS)

Lakkaraju, A.; Sinha, R.; Garikipati, R.; Edward, S.; Robinson, P.

2009-01-01

Aim: To evaluate the efficacy of ultrasound as a first-line investigation in patients with a clinical soft-tissue mass. Methods: Three hundred and fifty-eight consecutive patients (155 male, 203 female, mean age 48 years) referred from primary and secondary care with soft-tissue masses underwent ultrasound evaluation. Five radiologists performed ultrasound using a 10-15 MHz linear transducer and recorded the referrer diagnosis, history, lesion size, anatomical location and depth, internal echogenicity, external margins (well-defined rim or infiltrative), and vascularity on power Doppler (absent or present, if present the pattern was listed as either linear or disorganized). A provisional ultrasound diagnosis was made using one of eight categories. Benign categories (categories 1-5) were referred back to a non-sarcoma specialist or original referrer for observation. Indeterminate or possible sarcomas (categories 6-8) were referred for magnetic resonance imaging (MRI) within 14 days. Additionally category 8 lesions were referred to the regional sarcoma service. Institutional and regional database follow-up was performed. Results: Two hundred and eighty-four of the 358 (79%) lesions were classified as benign (categories 1-5). On follow-up 15 of the 284 patients were re-referred but none (284/284) had a malignancy on follow-up (24-30 months). Overall at ultrasound 33 lesions were larger than 5 cm, 42 lesions were deep to deep fascia with 20 showing both features. In this subgroup of 95 patients there were six malignant tumours with the rest benign. Seventy-three of the 358 patients underwent MRI; the results of which indicated that there were 60 benign or non-tumours, 10 possible sarcomas, and three indeterminate lesions. Overall six of 12 (6/358, 1.68% of total patients) lesions deemed to represent possible sarcomas on imaging were sarcomas. Conclusion: Ultrasound is an effective diagnostic triage tool for the evaluation of soft-tissue masses referred from primary

Radiation Therapy for Control of Soft-Tissue Sarcomas Resected With Positive Margins

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DeLaney, Thomas F.; Kepka, Lucyna; Goldberg, Saveli I.; Hornicek, Francis J.; Gebhardt, Mark C.; Yoon, Sam S.; Springfield, Dempsey S.; Raskin, Kevin A.; Harmon, David C.; Kirsch, David G.; Mankin, Henry J.; Rosenberg, Andrew E.; Nielsen, G. Petur; Suit, Herman D.

2007-01-01

Purpose: Positive margins (PM) remain after surgery in some soft-tissue sarcoma (STS) patients. We investigated the efficacy of radiation therapy (RT) in STS patients with PM. Methods and Materials: A retrospective chart review was performed on 154 patients with STS at various anatomic sites with PM, defined as tumor on ink, who underwent RT with curative intent between 1970 and 2001. Local control (LC), disease-free survival (DFS), and overall survival (OS) rates were evaluated by univariate (log-rank) and multivariate analysis of prognostic and treatment factors. Results: At 5 years, actuarial LC, DFS, and OS rates were: 76%, 46.7%, and 65.2%, respectively. LC was highest with extremity lesions (p 64 Gy (p 64 Gy had higher 5-year LC, DFS, and OS rates of 85%, 52.1%, and 67.8% vs. 66.1%, 41.8%, and 62.9% if ≤64 Gy, p 50), also significantly influenced OS. By multivariate analysis, the best predictors of LC were site (extremity vs. other), p 64 vs. ≤64 Gy), p 64 Gy, superficial location, and extremity site are associated with improved LC. OS is worse in patients with tumors with lesions >5 cm, grossly positive margins, and after local failure

The Scandinavian Sarcoma Group Central Register: 6,000 patients after 25 years of monitoring of referral and treatment of extremity and trunk wall soft-tissue sarcoma.

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Trovik, Clement; Bauer, Henrik C F; Styring, Emelie; Sundby Hall, Kirsten; Vult Von Steyern, Fredrik; Eriksson, Sigvard; Johansson, Ingela; Sampo, Mika; Laitinen, Minna; Kalén, Anders; Jónsson, Halldór; Jebsen, Nina; Eriksson, Mikael; Tukiainen, Erkki; Wall, Najme; Zaikova, Olga; Sigurðsson, Helgi; Lehtinen, Tuula; Bjerkehagen, Bodil; Skorpil, Mikael; Egil Eide, Geir; Johansson, Elisabeth; Alvegard, Thor A

2017-06-01

Purpose - We wanted to examine the potential of the Scandinavian Sarcoma Group (SSG) Central Register, and evaluate referral and treatment practice for soft-tissue sarcomas in the extremities and trunk wall (STS) in the Nordic countries. Background - Based on incidence rates from the literature, 8,150 (7,000-9,300) cases of STS of the extremity and trunk wall should have been diagnosed in Norway, Finland, Iceland, and Sweden from 1987 through 2011. The SSG Register has 6,027 cases registered from this period, with 5,837 having complete registration of key variables. 10 centers have been reporting to the Register. The 5 centers that consistently report treat approximately 90% of the cases in their respective regions. The remaining centers have reported all the patients who were treated during certain time periods, but not for the entire 25-year period. Results - 59% of patients were referred to a sarcoma center untouched, i.e. before any attempt at open biopsy. There was an improvement from 52% during the first 5 years to 70% during the last 5 years. 50% had wide or better margins at surgery. Wide margins are now achieved less often than 20 years ago, in parallel with an increase in the use of radiotherapy. For the centers that consistently report, 97% of surviving patients are followed for more than 4 years. Metastasis-free survival (MFS) increased from 67% to 73% during the 25-year period. Interpretation - The Register is considered to be representative of extremity and trunk wall sarcoma disease in the population of Scandinavia, treated at the reporting centers. There were no clinically significant differences in treatment results at these centers.

Local control and survival in patients with soft tissue sarcomas treated with limb sparing surgery in combination with interstitial brachytherapy and external radiation

DEFF Research Database (Denmark)

Muhic, A.; Hovgaard, D.; Mork, Petersen M.

2008-01-01

%. Nineteen (49%) patients suffered from some degree of decreased force or function of the affected extremity, 16 (41%) suffered from oedema, 12 (31%) had persistent pain, 8 (21%) suffered from wound complications, and in 4 (10%) of these patients plastic surgery were required. CONCLUSION: Limb sparing...... surgery, combined with PDR BRT and EBRT can result in good local control in patients with soft tissue sarcomas. BRT is an effective modality with good cosmetic results and acceptable toxicity Udgivelsesdato: 2008/9...

Primary clear cell sarcoma of rib

International Nuclear Information System (INIS)

Hersekli, Murat Ali; Ozkoc, Gurkan; Akpinar, Sercan; Ozalay, Metin; Tandogan, Reha N.; Bircan, Sema; Tuncer, Ilhan

2005-01-01

Clear cell sarcoma (malignant melanoma of soft tissues) is a very rare soft tissue neoplasm. It generally arises in tendons and aponeuroses. Although metastasis of malignant melanoma to bone is not uncommon, primary clear cell sarcoma of bone is an extremely rare neoplasm. To our knowledge five cases have been reported in the English literature. We present a case of primary clear cell sarcoma of bone in a 28-year-old woman arising in the left ninth rib. We treated the patient with total excision of the mass and postoperative radiotherapy. The patient is alive and well without local recurrence or distant metastasis at 33 months after surgery. (orig.)

Angiography of histopathologic variants of synovial sarcoma

International Nuclear Information System (INIS)

Lois, J.F.; Fischer, H.J.; Mirra, J.M.; Gomes, A.S.; California Univ., Los Angeles

1986-01-01

Synovial sarcomas are rare soft tissue tumors which histopathologically can be divided into monophasic, biphasic and mixed variants. As part of a protocol for intra-arterial chemotherapy 12 patients with biopsy proven synovial sarcoma underwent angiography. The angiograms on these patients were reviewed to determine whether synovial sarcomas and their variants demonstrated a characteristic angiographic appearance. Synovial sarcomas appeared angiographically as soft tissue masses which showed a fine network of tumor vessels with an inhomogeneous capillary blush. Their degree of vascularity varied according to their histopathology. Monophasic synovial sarcomas demonstrated in general a higher degree of neovascularity than the biphasic form. This finding was also suggested by histopathologic analysis of the vessels in the tumor. Although angiography did not show a distinctive vascular pattern it may be useful to evaluate tumor size and vascularity. (orig.)

"In situ preparation": new surgical procedure indicated for soft-tissue sarcoma of a lower limb in close proximity to major neurovascular structures.

Science.gov (United States)

Matsumoto, Seiichi; Kawaguchi, Noriyoshi; Manabe, Jun; Matsushita, Yasushi

2002-02-01

When soft-tissue sarcomas occur near neurovascular structures, preoperative images cannot always reveal the accurate relationship between the tumor and these structures. Therefore, in some patients, neurovascular structures are sacrificed unnecessarily. In other patients, neurovascular structures are preserved with an inappropriate margin, followed by local recurrence. The objective of this study was to evaluate a new surgical method, "in situ preparation" (ISP), which enables the preparation of neurovascular bundles and the intraoperative evaluation of the surgical margin without contamination by tumor cells. With this method, additional procedures, including pasteurization, alcohol soaking, and distilled water soaking of the preserved neurovascular bundle can also be performed to preserve the continuity of vessels. Between April 1992 and December 1998, 18 patients with soft-tissue sarcoma were operated on using ISP. The tumor and neurovascular structure were lifted en bloc from the surgical bed and separated from the field by the use of a vinyl sheet. The consistency of the neurovascular structures was preserved. The tissue block could be freely turned around and the neurovascular structure was separated from the block through the nearest approach. The margin between the tumor and neurovascular structure was evaluated, and an additional procedure, such as pasteurization, alcohol soaking or distilled water soaking, was performed, according to the safety of the surgical margin. Only one patient showed recurrence after ISP. Complications after ISP were arterial occlusion in two patients and nerve palsy in three patients. The main cause of these complications was the long period of pasteurization; modified additional procedures could prevent such complications. ISP is a useful method with which to ensure a safe surgical margin and good functional results.

High-level expression of podoplanin in benign and malignant soft tissue tumors: immunohistochemical and quantitative real-time RT-PCR analysis.

Science.gov (United States)

Xu, Yongjun; Ogose, Akira; Kawashima, Hiroyuki; Hotta, Tetsuo; Ariizumi, Takashi; Li, Guidong; Umezu, Hajime; Endo, Naoto

2011-03-01

Podoplanin is a 38 kDa mucin-type transmembrane glycoprotein that was first identified in rat glomerular epithelial cells (podocytes). It is expressed in normal lymphatic endothelium, but is absent from vascular endothelial cells. D2-40 is a commercially available mouse monoclonal antibody which binds to an epitope on human podoplanin. D2-40 immunoreactivity is therefore highly sensitive and specific for lymphatic endothelium. Recent investigations have shown widespread applications of immunohistochemical staining with D2-40 in evaluating podoplanin expression as an immunohistochemical marker for diagnosis and prognosis in various tumors. To determine whether the podoplanin (D2-40) antibody may be useful for the diagnosis of soft tissue tumors, 125 cases, including 4 kinds of benign tumors, 15 kinds of malignant tumors and 3 kinds of tumor-like lesions were immunostained using the D2-40 antibody. Total RNA was extracted from frozen tumor tissue obtained from 41 corresponding soft tissue tumor patients and 12 kinds of soft tissue tumor cell lines. Quantitative real-time PCR reactions were performed. Immunohistochemical and quantitative real-time RT-PCR analyses demonstrated the expression of the podoplanin protein and mRNA in the majority of benign and malignant soft tissue tumors and tumor-like lesions examined, with the exception of alveolar soft part sarcoma, embryonal and alveolar rhabdomyosarcoma, extraskeletal Ewing's sarcoma/peripheral primitive neuro-ectodermal tumor and lipoma, which were completely negative for podoplanin. Since it is widely and highly expressed in nearly all kinds of soft tissue tumors, especially in spindle cell sarcoma, myxoid type soft tissue tumors and soft tissue tumors of the nervous system, podoplanin is considered to have little value in the differential diagnosis of soft tissue tumors.

Influence of site on the therapeutic ratio of adjuvant radiotherapy in soft-tissue sarcoma of the extremity

International Nuclear Information System (INIS)

Alektiar, Kaled M.; Brennan, Murray F.; Singer, Samuel

2005-01-01

Purpose: The ultimate goal of adjuvant radiotherapy (RT) in soft-tissue sarcoma of the extremity is to improve the therapeutic ratio by increasing local control while minimizing morbidity. Most efforts in trying to improve this ratio have focused on the sequencing of RT and surgery, with little attention to the potential influence of the tumor site. The purpose of this study was to determine the influence of tumor site on local control and complications in a group of patients with primary high-grade soft-tissue sarcoma of the extremity treated at a single institution with postoperative RT. Methods and Materials: Between July 1982 and December 2000, 369 adult patients with primary high-grade soft-tissue sarcoma of the extremity were treated with limb-sparing surgery and postoperative RT. Patients who underwent surgery or RT outside our institution were excluded. The tumor site was the upper extremity (UE) in 103 (28%) and the lower extremity (LE) in 266 (72%). The tumor was ≤5 cm in 98 patients (27%), and the microscopic margins were positive in 44 (12%). Of the 369 patients, 104 (28%) underwent postoperative external beam RT (EBRT), 233 (63%) postoperative brachytherapy (BRT), and 32 underwent a combination (9%); 325 (88%) received a 'conventional' radiation dose, defined as 60-70 Gy for EBRT, 45 Gy for BRT, and 45-50 Gy plus 15-20 Gy for EBRT plus BRT. Complications were assessed in terms of wound complications requiring repeat surgery, fracture, joint stiffness, edema, and Grade 3 or worse peripheral nerve damage. Results: The UE and LE groups were balanced with regard to age, depth, margin status, and type of RT (EBRT vs. BRT ± EBRT). However, more patients in the UE group had tumors ≤5 cm and more received a conventional radiation dose (p = 0.01 and P = 0.03, respectively). With a median follow-up of 50 months, the 5-year actuarial rate of local control, distant relapse-free survival, and overall survival for the whole population was 82% (95% confidence

Late effects after treatment of twenty children with soft tissue sarcomas of the head and neck. Experience at a single institution with a review of the literature

International Nuclear Information System (INIS)

Fromm, M.; Littman, P.; Raney, R.B.; Nelson, L.; Handler, S.; Diamond, G.; Stanley, C.

1986-01-01

Twenty children with soft tissue sarcomas of the head and neck, treated at the Children's Hospital of Philadelphia and the Hospital of the University of Pennsylvania from 1972 to 1981, were evaluated for the late deleterious effects of treatment. All patients received radiation therapy and combination chemotherapy with vincristine, dactinomycin, and cyclophosphamide; certain patients also received Adriamycin (doxorubicin). All had ophthalmologic, otologic, growth, and cosmetic evaluations; 15 also had dental and maxillofacial examinations. The median age at diagnosis was 6 years (range, 7 months-13 years). Median follow-up from time of diagnosis was 5.5 years with a minimum of 3 years in all but four patients. The major problems encountered were related to the eyes (xerophthalmia and cataracts), ears (hearing loss), teeth (maleruption and caries), glandular structures (xerostomia, hypopituitarism), and development (craniofacial deformity). It is concluded that children treated for soft tissue sarcomas of the head and neck with combined modality therapy, including radiation enhancers, may show a variety of late treatment-related adversities. These children require close multidisciplinary follow-up for detection of late effects in order that appropriate prophylactic or symptomatic treatment can be instituted to minimize their consequences

[Evaluation of the management of soft tissue sarcomas in Franche-Comté since the establishment of a multidisciplinary meeting at University Hospital. About 47 cases].

Science.gov (United States)

Haddad, J; Kalbacher, E; Piccard, M; Aubry, S; Chaigneau, L; Pauchot, J

2017-02-01

A multidisciplinary meeting (RCP) dedicated to the treatment of sarcoma was established in Franche-Comte in 2010. The goals of the study are: (a) To evaluate the treatment of sarcomas by confrontation with the existing literature; (b) To evaluate the influence of the multidisciplinary meeting on the management of sarcomas by hospitals at the regional level. This is a retrospective single center study from 2010 to 2015 on patients with sarcoma and peripheral soft tissue drawn from a Netsarc database (National Network of sarcomas) and communicating cancer record. A database Cleanweb especially dedicated is created. Forty-seven patients were included: ten sarcomas at the upper member 26 to the lower limbs, 11 on the trunk. Forty patients were operated on: ten out of the university hospital, 28 at the university hospital and two in a coordinating center. Ninety percent of patients treated at the university hospital were in accordance with the recommandations. None of the patients operated out of the university hospital benefited from medical care in accordance to the recommendations. There is an increase in the number of files sent by the hospitals out of the university hospital discussed in multidisciplinary meeting, before treatment. The creation of a dedicated multidisciplinary meeting sarcoma improves the medical management of these tumors and decreases inappropriate medical managements thanks to a better education of the regional physicians. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Cervical synovial sarcoma in a young boy | Fisher | South African ...

African Journals Online (AJOL)

Synovial sarcomas comprise about 8% of all tumours of somatic soft-tissues, and are the most common sarcomas of the 'hands and feet. Occasionally they may occur in the trunk, but they have rarely been reported in the neck. We present a case of cervical soft-tissue mass producing symptoms in a 12-year-old-boy.

Results of a Qualitative Study to Develop a Patient Reported Outcome Measure for Patients with 4 Subtypes of Soft Tissue Sarcoma

Directory of Open Access Journals (Sweden)

Anne M. Skalicky

2017-01-01

Full Text Available Objective. The objective of this research was to develop a disease-specific symptom inventory for soft tissue sarcoma. Methods. Literature review and clinical expert and patient interviews were conducted to determine disease-specific symptoms important to patients with one of the four STS subtypes. Clinical experts identified the most relevant STS symptom items from the item pool developed from literature review. Concept elicitation interviews were conducted with patients to elicit their STS symptom experiences followed by a completion of the draft symptom list via web survey. A cognitive interview was conducted on the comprehension and importance of the symptom items. Results. Eighty-three symptom items were compiled and discussed with three clinical experts who identified 26 symptoms specific to the four STS subtypes. A total sample of 27 STS participants with self-reported leiomyosarcoma (74%, undifferentiated sarcoma (15%, synovial sarcoma (7%, or liposarcoma (4% diagnosis completed the web survey and 10 were interviewed. The draft 12-item STS-specific symptom inventory includes abdominal pain, pressure in abdomen, early satiety, bloating, gastrointestinal pain, muscle pain, bone pain, heavy menstrual flow, shortness of breath, chest pain, cough, and painful menstruation. Conclusion. A number of symptoms are common across STS subtypes and may form a single STS symptom inventory.

Optimal management of primary retroperitoneal sarcoma: an update.

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Miah, Aisha B; Hannay, Jonathan; Benson, Charlotte; Thway, Khin; Messiou, Christina; Hayes, Andrew J; Strauss, Dirk C

2014-05-01

Soft tissue sarcomas are a group of heterogeneous neoplasms with more than 50 histological subtypes exhibiting major differences in terms of pathogenesis, genetic alterations and clinical behavior. Sarcomas represent approximately 1% of malignancies with retroperitoneal sarcomas representing 10-15% of all soft tissue sarcomas. Surgery is currently the only modality which offers the chance of cure. Surgery for retroperitoneal sarcomas presents specific challenges due their location in a complex space surrounded by vital structures and visceral organs often prohibiting resection with wide margins. Furthermore, even after complete resection local recurrence is common and the leading cause of death. In this article the authors describe the initial investigations, prognostic factors and optimal surgical management. The evidence and current research as regards the role of multimodality treatment is reviewed and discussed.

Intraduodenal sarcoma recurrence of retroperitoneal origin: an unusual cause for a duodenal obstruction

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Bao Jean J

2012-04-01

Full Text Available Abstract Soft tissue sarcomas are uncommon tumors, and intraduodenal soft tissue sarcoma manifestation is even more rare. Only three cases of intraduodenal sarcomas have been reported in the literature thus far. Here, we report a case of an intraduodenal recurrence of a retroperitoneal sarcoma causing bowel obstruction. This unusual recurrence pattern likely relates to the patient’s previous resection and radiation treatment, and highlights the benefits, limitations and follow-up strategies after multimodality treatment.

The radiographic and MRI features of gout referred as suspected soft tissue sarcoma: a review of the literature and findings from 27 cases

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Upadhyay, Neil [Imperial College Healthcare NHS Trust, Imaging Department, London (United Kingdom); Saifuddin, Asif [The Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom)

2014-09-24

Gout has been termed the ''great mimicker'', for its ability to resemble other pathological conditions. Whilst the typical imaging features of gout are well described, there is a relative paucity of literature describing more challenging cases of gout, where a malignant tumour has been considered in the differential diagnosis. The aim of this article is to provide an overview of the radiographic and MRI features of 27 cases of gout that were initially referred to our centre as suspected soft tissue sarcoma, alongside a review of the literature. (orig.)

The radiographic and MRI features of gout referred as suspected soft tissue sarcoma: a review of the literature and findings from 27 cases

International Nuclear Information System (INIS)

Upadhyay, Neil; Saifuddin, Asif

2015-01-01

Gout has been termed the ''great mimicker'', for its ability to resemble other pathological conditions. Whilst the typical imaging features of gout are well described, there is a relative paucity of literature describing more challenging cases of gout, where a malignant tumour has been considered in the differential diagnosis. The aim of this article is to provide an overview of the radiographic and MRI features of 27 cases of gout that were initially referred to our centre as suspected soft tissue sarcoma, alongside a review of the literature. (orig.)

Soluble Neural-cadherin as a novel biomarker for malignant bone and soft tissue tumors

International Nuclear Information System (INIS)

Niimi, Rui; Matsumine, Akihiko; Iino, Takahiro; Nakazora, Shigeto; Nakamura, Tomoki; Uchida, Atsumasa; Sudo, Akihiro

2013-01-01

Neural-cadherin (N-cadherin) is one of the most important molecules involved in tissue morphogenesis, wound healing, and the maintenance of tissue integrity. Recently, the cleavage of N-cadherin has become a focus of attention in the field of cancer biology. Cadherin and their ectodomain proteolytic shedding play important roles during cancer progression. The aims of this study are to investigate the serum soluble N-cadherin (sN-CAD) levels in patients with malignant bone and soft tissue tumors, and to evaluate the prognostic significance of the sN-CAD levels. We examined the level of serum sN-CAD using an ELISA in 80 malignant bone and soft tissue tumors (bone sarcoma, n = 23; soft tissue sarcoma, n = 50; metastatic cancer, n = 7) and 87 normal controls. The mean age of the patients was 51 years (range, 10–85 years) and the mean follow-up period was 43 months (range, 1–115 months). The median serum sN-CAD level was 1,267 ng/ml (range, 135–2,860 ng/ml) in all patients. The mean serum sN-CAD level was 1,269 ng/ml (range, 360–2,860 ng/ml) in sarcoma patients, otherwise 1,246 ng/ml (range, 135–2,140 ng/ml) in cancer patients. The sN-CAD levels in patient were higher than those found in the controls, who had a median serum level of 108 ng/ml (range, 0–540 ng/ml). The patients with tumors larger than 5 cm had higher serum sN-CAD levels than the patients with tumors smaller than 5 cm. The histological grade in the patients with higher serum sN-CAD levels was higher than that in the patients with lower serum sN-CAD levels. A univariate analysis demonstrated that the patients with higher serum sN-CAD levels showed a worse disease-free survival rate, local recurrence-free survival rate, metastasis-free survival rate, and overall survival rate compared to those with lower serum sN-CAD levels. In the multivariate analysis, sN-CAD was an independent factor predicting disease-free survival. sN-CAD is a biomarker for malignant bone and soft tissue tumors, and a

Conservative treatment of soft tissue sarcomas of the extremities. Functional evaluation with LENT-SOMA scales and the Enneking score

International Nuclear Information System (INIS)

Tawfiq, N.; Lagarde, P.; Thomas, L.; Kantor, G.; Stockle, E.; Bui, B.N.

2000-01-01

Objective. - The aim of this prospective study is the feasibility of late effects assessment by LENT-SOMA scales after conservative treatment of soft tissue sarcomas of the extremities and a comparison with the functional evaluation by the Enneking score. Patients and methods. - During the systematic follow-up consultations, a series of 32 consecutive patients was evaluated in terms of late effects by LENT SOMA scales and functional results by the Enneking score. The median time after treatment was 65 months. The treatment consisted of conservative surgery (all cases) followed by radiation therapy (29 cases), often combined with adjuvant therapy (12 concomitant radio-chemotherapy association cases out of 14). The assessment of the toxicity was retrospective for acute effects and prospective for the following late tissue damage: skin/subcutaneous tissues, muscles/soft tissues and peripheral nerves. Results. -According to the Enneking score, the global score for the overall series was high (24/30) despite four the scores zero for the psychological acceptance. According to LENT SOMA scales, a low rate of severe sequelae (grade 3-4) was observed. The occurrence of high-grade sequelae and their functional consequences were not correlated with quality of exeresis, dose of radiotherapy or use of concomitant chemotherapy. A complementarity was observed between certain factors of the Enneking score and some criteria of the LENTSOMA scales, especially of muscles/soft tissues. Conclusion. -The good quality of functional results was confirmed by the two mean scoring systems for late normal tissue damage. The routine use of LENT-SOMA seems to be more time consuming than the Enneking score (mean time of scoring: 1 3 versus five minutes). The LENT-SOMA scales are aimed at a detailed description of late toxicity and sequelae while the Enneking score provides a more global evaluation, including the psychological acceptance of treatment. The late effects assessment by the LENT

Synovial sarcoma of the abdominal wall

International Nuclear Information System (INIS)

Matushita, J.P.K.; Matushita, J.S.

1989-01-01

A case report of synovial sarcoma arising in the abdominal wall is presented. A brief review of the clinical and radiological features of synovial sarcoma is made. Pre-operative diagnosis of an abdominal wall synovial sarcoma is virtually impossible, but should be considered when a soft tissue swelling is found to show amorphous stippled calcification X-ray. (author) [pt

Retroperitoneal soft tissue sarcoma: An analysis of radiation and surgical treatment

International Nuclear Information System (INIS)

Ballo, Matthew T.; Zagars, Gunar K.; Pollock, Raphael E.; Benjamin, Robert S.; Feig, Barry W.; Cormier, Janice N.; Hunt, Kelly K.; Patel, Shreyaskumar R.; Trent, Jonathan C.; Beddar, Sam; Pisters, Peter W.T.

2007-01-01

Purpose: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. Methods and Materials: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. Results: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. Conclusions: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes

Identification of heterogeneity among soft tissue sarcomas by gene expression profiles from different tumors

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Skubitz Amy PN

2008-05-01

Full Text Available Abstract The heterogeneity that soft tissue sarcomas (STS exhibit in their clinical behavior, even within histological subtypes, complicates patient care. Histological appearance is determined by gene expression. Morphologic features are generally good predictors of biologic behavior, however, metastatic propensity, tumor growth, and response to chemotherapy may be determined by gene expression patterns that do not correlate well with morphology. One approach to identify heterogeneity is to search for genetic markers that correlate with differences in tumor behavior. Alternatively, subsets may be identified based on gene expression patterns alone, independent of knowledge of clinical outcome. We have reported gene expression patterns that distinguish two subgroups of clear cell renal carcinoma (ccRCC, and other gene expression patterns that distinguish heterogeneity of serous ovarian carcinoma (OVCA and aggressive fibromatosis (AF. In this study, gene expression in 53 samples of STS and AF [including 16 malignant fibrous histiocytoma (MFH, 9 leiomyosarcoma, 12 liposarcoma, 4 synovial sarcoma, and 12 samples of AF] was determined at Gene Logic Inc. (Gaithersburg, MD using Affymetrix GeneChip® U_133 arrays containing approximately 40,000 genes/ESTs. Gene expression analysis was performed with the Gene Logic Genesis Enterprise System® Software and Expressionist software. Hierarchical clustering of the STS using our three previously reported gene sets, each generated subgroups within the STS that for some subtypes correlated with histology, and also suggested the existence of subsets of MFH. All three gene sets also recognized the same two subsets of the fibromatosis samples that we had found in our earlier study of AF. These results suggest that these subgroups may have biological significance, and that these gene sets may be useful for sub-classification of STS. In addition, several genes that are targets of some anti-tumor drugs were found to

Diagnosis and treatment of sarcomas and related tumors

International Nuclear Information System (INIS)

1978-01-01

The Cancergram focuses on clinical aspects of sarcomas involving soft tissue and bone, and also includes abstracts on related malignant and benign tumors. Soft tissues are considered as all non-epithelial extra-skeletal tissues of the body, with the exception of the reticuloendothelial system, the neuroglia, and visceral and parenchymal organs. Included, therefore, are sarcomas of the vascular system, fatty tissue, muscle tissues, connective tissues, and synovial tissues. Bone tumors included are osteosarcoma, chondrosarcoma, adamantinoma, chrondroblastoma, Ewing's sarcoma, and other benign and malignant disorders of the bone. This Cancergram excludes disorders of the bone marrow, which are the subjects of separate Cancergrams (see series CT03 for leukemias, and series CT12 for multiple myeloma). The scope includes diagnosis and staging, supportive care, evaluation, and therapy. Selected abstracts concerning epidemiology, etiology and other pre-clinical studies will also be included where they have direct clinical relevance

A Phase I Study of the Combination of Temsirolimus with Irinotecan for Metastatic Sarcoma

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Verschraegen, Claire F., E-mail: claire.verschraegen@vtmednet.org [Department of Hematology/Oncology, The University of Vermont Cancer Center, 89 Beaumont Ave., Burlington, VT 05405 (United States); Movva, Sujana [Department of Medical Oncology, Fox Chase Cancer Center, Pennsylvania, PA 19111 (United States); Ji, Yongli [Department of Hematology/Oncology, The University of Vermont Cancer Center, 89 Beaumont Ave., Burlington, VT 05405 (United States); Schmit, Berndt [Department of Radiology, University of Pittsburgh Medical Center East, Pittsburgh, PA 15146 (United States); Quinn, Robert H. [Department of Orthopaedics, University of Texas Health Science Center San Antonio, San Antonio, TX 78229 (United States); Liem, Ben [Department of Radiation Oncology, University of New Mexico Cancer Center, Albuquerque, NM 87131 (United States); Bocklage, Therese [Department of Pathology, University of New Mexico Cancer Center, Albuquerque, NM 87131 (United States); Shaheen, Monte [Division of Hematology/Oncology, University of New Mexico Cancer Center, Albuquerque, NM 87131 (United States)

2013-04-11

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/m{sup 2} 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.

Thallium scintigraphy used in the evaluation of soft tissue sarcoma

International Nuclear Information System (INIS)

Pilloni, A.; Brooks, R.

1998-01-01

Full text: The use of thallium scintigraphy in tumour localisation is a promising diagnostic modality. Thallium has a good affinity for numerous tumour types including sarcomas. Its properties as a potassium analogue result in active uptake via the sodium- potassium ATP-ase pump enabling its use in evaluating tumour extent, viability, staging, localisation and treatment. This case illustrates the role of thallium in tumour staging. A 67-year-old woman with a fibrous histiocytoma resected from the right foot four months previously presented with pain in the ribs, lower back and feet. A bone scan was performed to assess the possibility of metastatic disease. Several abnormal foci were seen on the bone scan suggestive of metastatic disease. A thallium scan was performed to confirm the bone scan result after a subsequent CT scan showed no evidence of this extensive disease. Static images of the whole body were acquired on an Elscinct 400AG 20 minutes post i.v. injection of 3.5 mCi of thallium. Focal areas of increased thallium uptake were seen in the 11th rib posteriorly and in the left sacroiliac region corresponding with sites on the bone scan suggesting metastases. However, an extensive area of uptake was also observed in the right inguinal region which had no corresponding abnormalities in the bone study. This was thought to be tumour metastasis in an inguinal Iymph node, which was retrospectively confirmed on an earlier CT scan of the pelvis. This study demonstrates the usefulness of thallium in the detection of soft tissue tumours and metastases. Thallium also exhibits potential in the assessment of tumour extent and viability pre- and post-treatment. This potential needs to be explored further in patient management

Imaging diagnosis of Granulocytic Sarcoma in the skull base

International Nuclear Information System (INIS)

Zheng Shaoyan; Xie Jiming; Yang Zhiyun; Zhou Zhou; Li Shurong

2010-01-01

Objective: To improve the understanding and imaging diagnosis of granulocytic sarcoma in the skull base. Methods: Three cases of granulocytic sarcomas in the skull base are reported. The clinical features and imaging findings were analyzed. Results: The three cases occurred in children with acute myeloid leukemia. Two patients presented with oculomotor paralysis before the diagnosis of leukemia, the third patient with history of leukemia presented with headache. Diffuse infiltration of basal skull bone marrow and extracranial soft tissue masses were shown on MRI. The signal intensities of the masses were similar to that of gray matter on T 1 WI and T 2 WI with marked contrast enhancement. The soft tissue masses were located in the para-sellar region and surrounded the lateral wall of the maxillary sinus in one case. The soft tissue mass of the second case infiltrated the orbital cavity, cavernous sinus and oculomotor nerve. Tumor infiltrating the meninges, cranial nerves and paranasal sinuses was seen in the third patient. Conclusion: Cranial nerve paralysis can be the presenting symptom of basal skull granulocytic sarcoma in children. Granulocytic sarcoma should be considered in the different diagnosis when diffuse abnormal signal intensities in the basal skull bone marrow with solitary or multiple soft tissue masses are shown on MRI. (authors)

[{sup 18}F]FMISO and [{sup 18}F]FDG PET imaging in soft tissue sarcomas: correlation of hypoxia, metabolism and VEGF expression

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Rajendran, J.G.; Peterson, L.M.; Grierson, J.R.; Eary, J.F. [Division of Nuclear Medicine, Department of Radiology, University of Washington Medical Center, Box 356113, WA 98195, Seattle (United States); Wilson, D.C. [Radiation Oncology, British Columbia Cancer Control Agency, Vancouver, BC (Canada); Conrad, E.U.; Bruckner, J.D. [Department of Orthopedic Surgery, University of Washington Medical Center, Seattle, Washington (United States); Rasey, J.S.; Chin, L.K.; Hofstrand, P.D. [Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington (United States); Krohn, K.A. [Division of Nuclear Medicine, Department of Radiology, University of Washington Medical Center, Box 356113, WA 98195, Seattle (United States); Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington (United States)

2003-05-01

Hypoxia imparts resistance to radiotherapy and chemotherapy and also promotes a variety of changes in tumor biology through inducible promoters. The purpose of this study was to evaluate the use of positron emission tomography (PET) imaging with fluorine-18 fluoromisonidazole (FMISO) in soft tissue sarcomas (STS) as a measure of hypoxia and to compare the results with those obtained using [{sup 18}F]fluorodeoxyglucose (FDG) and other known biologic correlates. FDG evaluates energy metabolism in tumors while FMISO uptake is proportional to tissue hypoxia. FMISO uptake was compared with FDG uptake. Vascular endothelial growth factor (VEGF) expression was also compared with FMISO uptake. Nineteen patients with STS underwent PET scanning with quantitative determination of FMISO and FDG uptake prior to therapy (neo-adjuvant chemotherapy or surgery alone). Ten patients receiving neo-adjuvant chemotherapy were also imaged after chemotherapy but prior to surgical resection. Standardized uptake value (SUV) was used to describe FDG uptake; regional tissue to blood ratio ({>=}1.2 was considered significant) was used for FMISO uptake. Significant hypoxia was found in 76% of tumors imaged prior to therapy. No correlation was identified between pretherapy hypoxic volume (HV) and tumor grade (r=0.15) or tumor volume (r=0.03). The correlation of HV with VEGF expression was 0.39. Individual tumors showed marked heterogeneity in regional VEGF expression. The mean pixel-by-pixel correlation between FMISO and FDG uptake was 0.49 (range 0.09-0.79) pretreatment and 0.32 (range -0.46-0.72) after treatment. Most tumors showed evidence of reduced uptake of both FMISO and FDG following chemotherapy. FMISO PET demonstrates areas of significant and heterogeneous hypoxia in soft tissue sarcomas. The significant discrepancy between FDG and FMISO uptake seen in this study indicates that regional hypoxia and glucose metabolism do not always correlate. Similarly, we did not find any relationship

Whole-tumor apparent diffusion coefficient (ADC) histogram analysis to differentiate benign peripheral neurogenic tumors from soft tissue sarcomas.

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Nakajo, Masanori; Fukukura, Yoshihiko; Hakamada, Hiroto; Yoneyama, Tomohide; Kamimura, Kiyohisa; Nagano, Satoshi; Nakajo, Masayuki; Yoshiura, Takashi

2018-02-22

Apparent diffusion coefficient (ADC) histogram analyses have been used to differentiate tumor grades and predict therapeutic responses in various anatomic sites with moderate success. To determine the ability of diffusion-weighted imaging (DWI) with a whole-tumor ADC histogram analysis to differentiate benign peripheral neurogenic tumors (BPNTs) from soft tissue sarcomas (STSs). Retrospective study, single institution. In all, 25 BPNTs and 31 STSs. Two-b value DWI (b-values = 0, 1000s/mm 2 ) was at 3.0T. The histogram parameters of whole-tumor for ADC were calculated by two radiologists and compared between BPNTs and STSs. Nonparametric tests were performed for comparisons between BPNTs and STSs. P histogram parameters except kurtosis and entropy differed significantly between BPNTs and STSs. 3 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018. © 2018 International Society for Magnetic Resonance in Medicine.

Can Bone Tissue Engineering Contribute to Therapy Concepts after Resection of Musculoskeletal Sarcoma?

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Boris Michael Holzapfel

2013-01-01

Full Text Available Resection of musculoskeletal sarcoma can result in large bone defects where regeneration is needed in a quantity far beyond the normal potential of self-healing. In many cases, these defects exhibit a limited intrinsic regenerative potential due to an adjuvant therapeutic regimen, seroma, or infection. Therefore, reconstruction of these defects is still one of the most demanding procedures in orthopaedic surgery. The constraints of common treatment strategies have triggered a need for new therapeutic concepts to design and engineer unparalleled structural and functioning bone grafts. To satisfy the need for long-term repair and good clinical outcome, a paradigm shift is needed from methods to replace tissues with inert medical devices to more biological approaches that focus on the repair and reconstruction of tissue structure and function. It is within this context that the field of bone tissue engineering can offer solutions to be implemented into surgical therapy concepts after resection of bone and soft tissue sarcoma. In this paper we will discuss the implementation of tissue engineering concepts into the clinical field of orthopaedic oncology.

Analysis of gene expression profiles of soft tissue sarcoma using a combination of knowledge-based filtering with integration of multiple statistics.

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Anna Takahashi

Full Text Available The diagnosis and treatment of soft tissue sarcomas (STS have been difficult. Of the diverse histological subtypes, undifferentiated pleomorphic sarcoma (UPS is particularly difficult to diagnose accurately, and its classification per se is still controversial. Recent advances in genomic technologies provide an excellent way to address such problems. However, it is often difficult, if not impossible, to identify definitive disease-associated genes using genome-wide analysis alone, primarily because of multiple testing problems. In the present study, we analyzed microarray data from 88 STS patients using a combination method that used knowledge-based filtering and a simulation based on the integration of multiple statistics to reduce multiple testing problems. We identified 25 genes, including hypoxia-related genes (e.g., MIF, SCD1, P4HA1, ENO1, and STAT1 and cell cycle- and DNA repair-related genes (e.g., TACC3, PRDX1, PRKDC, and H2AFY. These genes showed significant differential expression among histological subtypes, including UPS, and showed associations with overall survival. STAT1 showed a strong association with overall survival in UPS patients (logrank p = 1.84 × 10(-6 and adjusted p value 2.99 × 10(-3 after the permutation test. According to the literature, the 25 genes selected are useful not only as markers of differential diagnosis but also as prognostic/predictive markers and/or therapeutic targets for STS. Our combination method can identify genes that are potential prognostic/predictive factors and/or therapeutic targets in STS and possibly in other cancers. These disease-associated genes deserve further preclinical and clinical validation.

Prognostic factors in adult soft-tissue sarcomas of the head and neck

International Nuclear Information System (INIS)

Le, Quynh-Thu X.; Fu, Karen K.; Kroll, Stew; Fitts, Linda; Massullo, Vincent; Ferrell, Linda; Kaplan, Michael J.; Phillips, Theodore L.

1997-01-01

Purpose: The main objectives of this study were (a) to review the treatment results of primary head and neck soft-tissue sarcoma at our institution, (b) to identify important prognostic factors in local control and survival, and (c) to assess the efficacy of salvage therapy. Methods and Materials: Sixty-five patients were treated at the University of California, San Francisco, between 1961 and 1993. Seventeen patients (27%) had low-grade, 10 (15%) had intermediate-grade, and 38 (58%) had high-grade sarcomas. Tumors were > 5 cm in 35 patients. Local management consisted of surgery alone in 14 patients (22%), surgery and radiotherapy in 40 (61%), and radiotherapy alone in 11 (17%) patients. The median follow-up was 64 months. Results: The 5-year actuarial local control rate of the entire group was 66%. Tumor size and grade were important predictors for local control on multivariate analysis. The actuarial local control rate at 5 years was 92% for T1 vs. 40% for T2 primaries (p = 0.004), and 80% for Grade 1-2 vs. 48% for Grade 3 tumors (p 0.01). None of the patients treated with radiotherapy alone with a dose of 50-65 Gy were controlled locally. Combined radiotherapy and surgery appeared to yield superior local control compared to surgery alone (77% vs. 59%); however, the difference was not statistically significant. The 5-year actuarial overall and cause-specific survivals were 56% and 60%, respectively. Unfavorable prognostic factors for cause-specific survival on multivariate analysis were age > 55 (p = 0.009), high tumor grade (p 0.0002), inadequate surgery (p = 0.008), and positive surgical margins (p 0.0009). In patients who underwent salvage therapy for treatment failure, the 5-year actuarial survival after salvage treatment was 26%. Conclusion: Tumor size and grade were important predictors for local control. Age, grade, adequacy of surgery, and status of surgical margins were significant prognostic factors for survival. There was a trend of improved local

Optically-tracked handheld fluorescence imaging platform for monitoring skin response in the management of soft tissue sarcoma

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Chamma, Emilie; Qiu, Jimmy; Lindvere-Teene, Liis; Blackmore, Kristina M.; Majeed, Safa; Weersink, Robert; Dickie, Colleen I.; Griffin, Anthony M.; Wunder, Jay S.; Ferguson, Peter C.; DaCosta, Ralph S.

2015-07-01

Standard clinical management of extremity soft tissue sarcomas includes surgery with radiation therapy. Wound complications (WCs) arising from treatment may occur due to bacterial infection and tissue breakdown. The ability to detect changes in these parameters during treatment may lead to earlier interventions that mitigate WCs. We describe the use of a new system composed of an autofluorescence imaging device and an optical three-dimensional tracking system to detect and coregister the presence of bacteria with radiation doses. The imaging device visualized erythema using white light and detected bacterial autofluorescence using 405-nm excitation light. Its position was tracked relative to the patient using IR reflective spheres and registration to the computed tomography coordinates. Image coregistration software was developed to spatially overlay radiation treatment plans and dose distributions on the white light and autofluorescence images of the surgical site. We describe the technology, its use in the operating room, and standard operating procedures, as well as demonstrate technical feasibility and safety intraoperatively. This new clinical tool may help identify patients at greater risk of developing WCs and investigate correlations between radiation dose, skin response, and changes in bacterial load as biomarkers associated with WCs.

Selection of suitable reference genes for normalization of genes of interest in canine soft tissue sarcomas using quantitative real-time polymerase chain reaction

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Zornhagen, K. W.; Kristensen, A. T.; Hansen, Anders Elias

2015-01-01

Quantitative real-time reverse transcription polymerase chain reaction (RT-qPCR) is a sensitive technique for quantifying gene expression. Stably expressed reference genes are necessary for normalization of RT-qPCR data. Only a few articles have been published on reference genes in canine tumours....... The objective of this study was to demonstrate how to identify suitable reference genes for normalization of genes of interest in canine soft tissue sarcomas using RT-qPCR. Primer pairs for 17 potential reference genes were designed and tested in archival tumour biopsies from six dogs. The geNorm algorithm...

Percutaneous computed tomography-guided core needle biopsy of soft tissue tumors: results and correlation with surgical specimen analysis

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Chojniak, Rubens; Grigio, Henrique Ramos; Bitencourt, Almir Galvao Vieira; Pinto, Paula Nicole Vieira; Tyng, Chiang J.; Cunha, Isabela Werneck da; Aguiar Junior, Samuel; Lopes, Ademar, E-mail: chojniak@uol.com.br [Hospital A.C. Camargo, Sao Paulo, SP (Brazil)

2012-09-15

Objective: To evaluate the efficacy of percutaneous computed tomography (CT)-guided core needle biopsy of soft tissue tumors in obtaining appropriate samples for histological analysis, and compare its diagnosis with the results of the surgical pathology as available. Materials and Methods: The authors reviewed medical records, imaging and histological reports of 262 patients with soft-tissue tumors submitted to CT-guided core needle biopsy in an oncologic reference center between 2003 and 2009. Results: Appropriate samples were obtained in 215 (82.1%) out of the 262 patients. The most prevalent tumors were sarcomas (38.6%), metastatic carcinomas (28.8%), benign mesenchymal tumors (20.5%) and lymphomas (9.3%). Histological grading was feasible in 92.8% of sarcoma patients, with the majority of them (77.9%) being classified as high grade tumors. Out of the total sample, 116 patients (44.3%) underwent surgical excision and diagnosis confirmation. Core biopsy demonstrated 94.6% accuracy in the identification of sarcomas, with 96.4% sensitivity and 89.5% specificity. A significant intermethod agreement about histological grading was observed between core biopsy and surgical resection (p < 0.001; kappa = 0.75). Conclusion: CT-guided core needle biopsy demonstrated a high diagnostic accuracy in the evaluation of soft tissue tumors as well as in the histological grading of sarcomas, allowing an appropriate therapeutic planning (author)

A case of clear cell sarcoma

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Juel, Jacob; Ibrahim, Rami Mossad

2017-01-01

INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made...... this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas. PRESENTATION OF CASE: A 22-year-old patient presented with a mass sized 2.6×2.7×2.7cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted...

Fluorodeoxyglucose positron emission tomography of soft tissue tumours: is a non-invasive determination of biological activity possible?

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Schulte, M.; Hartwig, E.; Sarkar, M.R.; Schultheiss, M. [Department of Trauma, Hand- and Reconstructive Surgery, University Hospital Ulm (Germany); Brecht-Krauss, D.; Guhlmann, A.; Diederichs, C.G.; Kotzerke, J.; Reske, S.N. [Department of Nuclear Medicine, University Hospital Ulm (Germany); Heymer, B. [Department of Pathology, University Hospital Ulm (Germany)

1999-06-01

Since musculoskeletal tumours comprise a large heterogeneous group of entities with different biological behaviour, clinical diagnosis of such lesions can be very difficult. The aim of this prospective study was to assess the usefulness of 2-[F-18]-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) in the non-invasive evaluation of soft tissue tumours. One hundred and two patients with suspected soft tissue neoplasms were investigated by FDG-PET. The uptake of FDG was evaluated semiquantitatively by determining the tumour to background ratio (TBR). All patients underwent biopsy, resulting in the histological detection of 39 high-grade sarcomas, 16 intermediate-grade sarcomas, 11 low-grade sarcomas, 25 benign tumours, 10 tumour-like lesions such as spontaneous myositis ossificans (n = 6) and one non-Hodgkin lymphoma. All lesions except for two lipomas disclosed an increased FDG uptake. Sarcomas showed significantly higher TBR values than latent or active benign lesions (P<0.001) and aggressive benign lesions (P<0.05). Using a TBR cut-off level of 3.0 for malignancy, sensitivity of FDG-PET was 97.0%, specificity 65.7% and accuracy 86.3%. From our data there are three main conclusions: (1) Except for patients with pseudotumoral myositis ossificans, lesions with a TBR >3 were sarcomas (91.7%) or aggressive benign tumours (8.3%). (2) Tumours with a TBR <1.5 were latent or active benign lesions, exclusively. (3) The group with intermediate TBR values (<3 and >1.5) comprised primarily latent or active benign lesions, but also four aggressive benign tumours and two low-grade sarcomas. Our data suggest that FDG-PET represents a useful tool for the evaluation of the biological activity of soft tissue neoplasms. (orig.) With 5 figs., 2 tabs., 26 refs.

Ewing`s Sarcoma

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Agnieszka Budny

2017-06-01

Full Text Available Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Clinical presentation is usually dominated by local bone pain and a mass. Magnetic resonance best defines the extent of the lesion. Patients diagnosed with Ewing's sarcoma within  last years show a improving  survival rate . Rehabilitation seems to be a crucial part of multimodal therapy.

Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation

International Nuclear Information System (INIS)

Pollack, Alan; Zagars, Gunar K.; Goswitz, Mary S.; Pollock, Raphael A.; Feig, Barry W.; Pisters, Peter W.T.

1998-01-01

Purpose: Radiotherapy for soft tissue sarcoma is typically preoperative or postoperative, with advocates of each. In this study, the relationship of the sequencing of radiotherapy and surgery to local control was examined. Methods and Materials: The cohort consisted of 453 patients with Grade 2-3 malignant fibrous histiocytoma, synovial sarcoma, or liposarcoma treated from 1965-1992. Retroperitoneal sarcomas were excluded. Median follow-up was 97 months. There were 3 groups of patients that were classified by the treatment administered at our institution: preoperative radiotherapy to a median dose of 50 Gy given before excision at MDACC (Preop; n = 128); postoperative radiotherapy to a median dose of 64 Gy given after excision at MDACC (Postop; n = 165); and radiotherapy to a median dose of 65 Gy without excision at MDACC (RT Alone; n = 160). Those in the RT Alone Group had gross total excision at an outside center prior to referral. Results: Histological classification, whether locally recurrent at referral, and final MDACC margins were independent determinants of local control in Cox proportional hazards multivariate analysis using the entire cohort. The type of treatment was not significant; however, tumor status at presentation (gross disease vs. excised) affected these findings greatly. Gross disease treated with Preop was controlled locally in 88% at 10 years, as compared to 67% with Postop (p = 0.01). This association was independently significant for patients treated primarily (not for recurrence). In contrast, for those presenting after excision elsewhere, 10-year local control was better with Postop (88% vs. 73%, p = 0.07), particularly for patients treated primarily (91% vs. 72%, p 0.02 in univariate analysis; p = 0.06 in multivariate analysis). Re-excision at MDACC (Postop) resulted in enhanced 10-year local control over that with RT Alone (88% vs. 75%, p = 0.06), and was confirmed to be an independent predictor in multivariate analysis (p = 0

Analyzing the "CareGap": assessing gaps in adherence to clinical guidelines in adult soft tissue sarcoma.

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Waks, Zeev; Goldbraich, Esther; Farkash, Ariel; Torresani, Michele; Bertulli, Rossella; Restifo, Nicola; Locatelli, Paolo; Casali, Paolo; Carmeli, Boaz

2013-01-01

Clinical decision support systems (CDSSs) are gaining popularity as tools that assist physicians in optimizing medical care. These systems typically comply with evidence-based medicine and are designed with input from domain experts. Nonetheless, deviations from CDSS recommendations are abundant across a broad spectrum of disorders, raising the question as to why this phenomenon exists. Here, we analyze this gap in adherence to a clinical guidelines-based CDSS by examining the physician treatment decisions for 1329 adult soft tissue sarcoma patients in northern Italy using patient-specific parameters. Dubbing this analysis "CareGap", we find that deviations correlate strongly with certain disease features such as local versus metastatic clinical presentation. We also notice that deviations from the guideline-based CDSS suggestions occur more frequently for patients with shorter survival time. Such observations can direct physicians' attention to distinct patient cohorts that are prone to higher deviation levels from clinical practice guidelines. This illustrates the value of CareGap analysis in assessing quality of care for subsets of patients within a larger pathology.

Phase II study of the safety and antitumor activity of the hypoxia-activated prodrug TH-302 in combination with doxorubicin in patients with advanced soft tissue sarcoma.

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Chawla, Sant P; Cranmer, Lee D; Van Tine, Brian A; Reed, Damon R; Okuno, Scott H; Butrynski, James E; Adkins, Douglas R; Hendifar, Andrew E; Kroll, Stew; Ganjoo, Kristen N

2014-10-10

TH-302, a prodrug of the cytotoxic alkylating agent bromo-isophosphoramide mustard, is preferentially activated in hypoxic conditions. This phase II study investigated TH-302 in combination with doxorubicin, followed by single-agent TH-302 maintenance therapy in patients with first-line advanced soft tissue sarcoma (STS) to assess progression-free survival (PFS), response rate, overall survival, safety, and tolerability. In this open-label phase II study, TH-302 300 mg/m(2) was administered intravenously on days 1 and 8 with doxorubicin 75 mg/m(2) on day 1 of each 21-day cycle. After six cycles, patients with stable and/or responding disease could receive maintenance monotherapy with TH-302. Ninety-one patients initiated TH-302 plus doxorubicin induction treatment. The PFS rate at 6 months (primary efficacy measure) was 58% (95% CI, 46% to 68%). Median PFS was 6.5 months (95% CI, 5.8 to 7.7 months); median overall survival was 21.5 months (95% CI, 16.0 to 26.2 months). Best tumor responses were complete response (n = 2 [2%]) and partial response (n = 30 [34%]). During TH-302 maintenance (n = 48), five patients improved from stable disease to partial response, and one patient improved from partial to complete response. The most common adverse events during induction were fatigue, nausea, and skin and/or mucosal toxicities as well as anemia, thrombocytopenia, and neutropenia. These were less severe and less frequent during maintenance. There was no evidence of TH-302-related hepatic, renal, or cardiac toxicity. PFS, overall survival, and tumor response compared favorably with historical outcomes achieved with other first-line chemotherapies for advanced STS. A phase III study of TH-302 is ongoing (NCT01440088). © 2014 by American Society of Clinical Oncology.

Vaccine-associated sarcomas in cats: a unique cancer model.

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McNiel, E A

2001-01-01

Epidemiologic evidence supports a relationship between vaccination of cats for rabies and feline leukemia virus with the development of soft tissue sarcomas at the site of administration. These tumors are locally invasive and histologically aggressive. As with high-grade soft tissue sarcoma in humans, combination treatment with radiation therapy and surgery provides for optimum tumor control. Feline vaccine-associated sarcoma has become a difficult issue for the veterinary profession for legal, ethical, and clinical reasons. Although most research efforts have focused on therapeutic intervention, this tumor has great potential to provide an informative model for carcinogenesis and genetic susceptibility applicable to cancer in all species, including humans.

The Epidemiology of Sarcoma

Directory of Open Access Journals (Sweden)

Burningham Zachary

2012-10-01

Full Text Available Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend

Advances in sarcoma gene mutations and therapeutic targets.

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Gao, Peng; Seebacher, Nicole A; Hornicek, Francis; Guo, Zheng; Duan, Zhenfeng

2018-01-01

Sarcomas are rare and complex malignancies that have been associated with a poor prognostic outcome. Over the last few decades, traditional treatment with surgery and/or chemotherapy has not significantly improved outcomes for most types of sarcomas. In recent years, there have been significant advances in the understanding of specific gene mutations that are important in driving the pathogenesis and progression of sarcomas. Identification of these new gene mutations, using next-generation sequencing and advanced molecular techniques, has revealed a range of potential therapeutic targets. This, in turn, may lead to the development of novel agents targeted to different sarcoma subtypes. In this review, we highlight the advances made in identifying sarcoma gene mutations, including those of p53, RB, PI3K and IDH genes, as well as novel therapeutic strategies aimed at utilizing these mutant genes. In addition, we discuss a number of preclinical studies and ongoing early clinical trials in sarcoma targeting therapies, as well as gene editing technology, which may provide a better choice for sarcoma patient management. Published by Elsevier Ltd.

Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial.

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Gronchi, Alessandro; Ferrari, Stefano; Quagliuolo, Vittorio; Broto, Javier Martin; Pousa, Antonio Lopez; Grignani, Giovanni; Basso, Umberto; Blay, Jean-Yves; Tendero, Oscar; Beveridge, Robert Diaz; Ferraresi, Virginia; Lugowska, Iwona; Merlo, Domenico Franco; Fontana, Valeria; Marchesi, Emanuela; Donati, Davide Maria; Palassini, Elena; Palmerini, Emanuela; De Sanctis, Rita; Morosi, Carlo; Stacchiotti, Silvia; Bagué, Silvia; Coindre, Jean Michelle; Dei Tos, Angelo Paolo; Picci, Piero; Bruzzi, Paolo; Casali, Paolo Giovanni

2017-06-01

Previous trials from our group suggested an overall survival benefit with five cycles of adjuvant full-dose epirubicin plus ifosfamide in localised high-risk soft-tissue sarcoma of the extremities or trunk wall, and no difference in overall survival benefit between three cycles versus five cycles of the same neoadjuvant regimen. We aimed to show the superiority of the neoadjuvant administration of histotype-tailored regimen to standard chemotherapy. For this international, open-label, randomised, controlled, phase 3, multicentre trial, patients were enrolled from 32 hospitals in Italy, Spain, France, and Poland. Eligible patients were aged 18 years or older with localised, high-risk (high malignancy grade, 5 cm or longer in diameter, and deeply located according to the investing fascia), soft-tissue sarcoma of the extremities or trunk wall and belonging to one of five histological subtypes: high-grade myxoid liposarcoma, leiomyosarcoma, synovial sarcoma, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma. Patients were randomly assigned (1:1) to receive three cycles of full-dose standard chemotherapy (epirubicin 60 mg/m 2 per day [short infusion, days 1 and 2] plus ifosfamide 3 g/m 2 per day [days 1, 2, and 3], repeated every 21 days) or histotype-tailored chemotherapy: for high-grade myxoid liposarcoma, trabectedin 1·3 mg/m 2 via 24-h continuous infusion, repeated every 21 days; for leiomyosarcoma, gemcitabine 1800 mg/m 2 on day 1 intravenously over 180 min plus dacarbazine 500 mg/m 2 on day 1 intravenously over 20 min, repeated every 14 days; for synovial sarcoma, high-dose ifosfamide 14 g/m 2 , given over 14 days via an external infusion pump, every 28 days; for malignant peripheral nerve sheath tumour, intravenous etoposide 150 mg/m 2 per day (days 1, 2, and 3) plus intravenous ifosfamide 3 g/m 2 per day (days 1, 2, and 3), repeated every 21 days; and for undifferentiated pleomorphic sarcoma, gemcitabine 900 mg/m 2 on days 1 and

Association Between Treatment at High-Volume Facilities and Improved Overall Survival in Soft Tissue Sarcomas.

Science.gov (United States)

Venigalla, Sriram; Nead, Kevin T; Sebro, Ronnie; Guttmann, David M; Sharma, Sonam; Simone, Charles B; Levin, William P; Wilson, Robert J; Weber, Kristy L; Shabason, Jacob E

2018-03-15

Soft tissue sarcomas (STS) are rare malignancies that require complex multidisciplinary management. Therefore, facilities with high sarcoma case volume may demonstrate superior outcomes. We hypothesized that STS treatment at high-volume (HV) facilities would be associated with improved overall survival (OS). Patients aged ≥18 years with nonmetastatic STS treated with surgery and radiation therapy at a single facility from 2004 through 2013 were identified from the National Cancer Database. Facilities were dichotomized into HV and low-volume (LV) cohorts based on total case volume over the study period. OS was assessed using multivariable Cox regression with propensity score-matching. Patterns of care were assessed using multivariable logistic regression analysis. Of 9025 total patients, 1578 (17%) and 7447 (83%) were treated at HV and LV facilities, respectively. On multivariable analysis, high educational attainment, larger tumor size, higher grade, and negative surgical margins were statistically significantly associated with treatment at HV facilities; conversely, black race and non-metropolitan residence were negative predictors of treatment at HV facilities. On propensity score-matched multivariable analysis, treatment at HV facilities versus LV facilities was associated with improved OS (hazard ratio, 0.87, 95% confidence interval, 0.80-0.95; P = .001). Older age, lack of insurance, greater comorbidity, larger tumor size, higher tumor grade, and positive surgical margins were associated with statistically significantly worse OS. In this observational cohort study using the National Cancer Database, receipt of surgery and radiation therapy at HV facilities was associated with improved OS in patients with STS. Potential sociodemographic disparities limit access to care at HV facilities for certain populations. Our findings highlight the importance of receipt of care at HV facilities for patients with STS and warrant further study into improving access to

Orbital Epithelioid Sarcoma: A Case Report

NARCIS (Netherlands)

Jurdy, Lama L.; Blank, Leo E.; Bras, Johannes; Saeed, Peerooz

2016-01-01

Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with

The role of imaging in the evaluation of extraskeletal Ewing’s sarcoma

Directory of Open Access Journals (Sweden)

Pieter Barnardt

2013-03-01

Full Text Available Primary bone tumours account for 5% of all adolescent and child cancers. When Ewing’s sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing’s sarcoma (ESS. Computed tomography (CT scanning and magnetic resonance imaging (MRI features of Ewing’s sarcoma are non-specific, and a radiological differential diagnosis should be considered. Ewing’s sarcoma is confirmed by features on histological analysis. In young people who present with soft-tissue tumours, ESS should be considered. In the management of patients with tumours, imaging techniques are useful for biopsy guidance, evaluating the possibility of resection, and tumour response to treatment.

A case of clear cell sarcoma-A rare malignancy

DEFF Research Database (Denmark)

Juel, Jacob; Ibrahim, Rami Mossad

2017-01-01

INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made...

Oncolytic Maraba Virus MG1 as a Treatment for Sarcoma.

Science.gov (United States)

Le Boeuf, Fabrice; Selman, Mohammed; Son, Hwan Hee; Bergeron, Anabel; Chen, Andrew; Tsang, Jovian; Butterwick, Derek; Arulanandam, Rozanne; Forbes, Nicole E; Tzelepis, Fanny; Bell, John C; Werier, Joel; Abdelbary, Hesham; Diallo, Jean-Simon

2017-09-15

The poor prognosis of patients with advanced bone and soft-tissue sarcoma has not changed in the past several decades, highlighting the necessity for new therapeutic approaches. Immunotherapies, including oncolytic viral (OV) therapy, have shown great promise in a number of clinical trials for a variety of tumor types. However, the effective application of OV in treating sarcoma still remains to be demonstrated. Although few pre-clinical studies using distinct OVs have been performed and demonstrated therapeutic benefit in sarcoma models, a side-by-side comparison of clinically relevant OV platforms has not been performed. Four clinically relevant OV platforms (Reovirus, Vaccinia virus, Herpes-simplex virus and Rhabdovirus) were screened for their ability to infect and kill human and canine sarcoma cell lines in vitro, and human sarcoma specimens ex vivo. In vivo treatment efficacy was tested in a murine model. The rhabdovirus MG1 demonstrated the highest potency in vitro. Ex vivo, MG1 productively infected more than 80% of human sarcoma tissues tested, and treatment in vivo led to a significant increase in long-lasting cures in sarcoma-bearing mice. Importantly, MG1 treatment induced the generation of memory immune response that provided protection against a subsequent tumor challenge. This study opens the door for the use of MG1-based oncolytic immunotherapy strategies as treatment for sarcoma or as a component of a combined therapy. © 2017 UICC.

Pre-operative radiotherapy in soft tissue tumors: Assessment of response by static post-contrast MR imaging compared to histopathology

International Nuclear Information System (INIS)

Einarsdottir, H.; Wejde, J.; Bauer, H.C.F.

2000-01-01

To evaluate if static post-contrast MR imaging was adequate to assess tumor viability after pre-operative radiotherapy in soft tissue sarcoma. Post-contrast MR imaging of 36 soft tissue sarcomas performed 0 - 54 days (median 13 days) after pre-operative radiotherapy, were retrospectively reviewed and compared to post-operative histopathology reports. The contrast enhancement of the tumor was visually graded as minor, moderate or extensive. From the post-operative histopathology reports, three types of tumor response to radiotherapy were defined: Poor, intermediate or good. The size of the tumors before and after radiation was compared. Even if most viable tumors enhanced more than non-viable tumors, there was major overlapping and significant contrast enhancement could be seen in tumors where histopathological examination revealed no viable tumor tissue. Based on histopathology, there were 12 good responders; 8 of these showed minor, 3 moderate and 1 extensive contrast enhancement on MR imaging. Sixteen tumors had an intermediate response; 3 showed minor, 8 moderate and 5 extensive enhancement. Eight tumors had poor response; none showed minor enhancement, 3 moderate and 5 extensive enhancement. Both increase and Decrease in tumor size was seen in lesions with a good therapy response. Static post-contrast MR imaging cannot reliably assess tumor viability after pre-operative radiotherapy in soft tissue sarcoma. In tumors with no viable tumor tissue, moderate and extensive contrast enhancement can be seen

Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum

DEFF Research Database (Denmark)

Nilbert, Mef; Therkildsen, Christina; Nissen, Anja

2009-01-01

-register to identify HNPCC families in which sarcomas had been diagnosed. Fourteen sarcomas were identified in families with mutations in MSH2, MSH6, and MLH1. The median age at sarcoma diagnosis was 43 (15-74) years. Soft tissue sarcomas predominated followed by uterine sarcomas and eight histopathological subtypes...

Synovial Sarcoma of the Buccal Mucosa: A Rare Case Report

Directory of Open Access Journals (Sweden)

Kumar T. S. Mahesh

2013-01-01

Full Text Available Synovial sarcoma (SS is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Synovial sarcoma has been reported rarely in the oral cavity. We report a very rare case of Synovial sarcoma of the buccal mucosa in a 24-year-old male patient.

Micro Regional Heterogeneity of ⁶⁴Cu-ATSM and ¹⁸F-FDG Uptake in Canine Soft Tissue Sarcomas

DEFF Research Database (Denmark)

Zornhagen, Kamilla Westarp; Hansen, Anders Elias; Oxboel, Jytte

2015-01-01

OBJECTIVES: Tumour microenvironment heterogeneity is believed to play a key role in cancer progression and therapy resistance. However, little is known about micro regional distribution of hypoxia, glycolysis and proliferation in spontaneous solid tumours. The overall aim was simultaneous...... investigation of micro regional heterogeneity of 64Cu-ATSM (hypoxia) and 18F-FDG (glycolysis) uptake and correlation to endogenous markers of hypoxia, glycolysis, proliferation and angiogenesis to better therapeutically target aggressive tumour regions and prognosticate outcome. METHODS: Exploiting...... the different half-lives of 64Cu-ATSM (13h) and 18F-FDG (2h) enabled simultaneous investigation of micro regional distribution of hypoxia and glycolysis in 145 tumour pieces from four spontaneous canine soft tissue sarcomas. Pairwise measurements of radioactivity and gene expression of endogenous markers...

Diagnostic value of MRI signs in differentiating Ewing sarcoma from osteomyelitis.

Science.gov (United States)

Kasalak, Ömer; Overbosch, Jelle; Adams, Hugo Ja; Dammann, Amelie; Dierckx, Rudi Ajo; Jutte, Paul C; Kwee, Thomas C

2018-01-01

Background The value of magnetic resonance imaging (MRI) signs in differentiating Ewing sarcoma from osteomyelitis has not be thoroughly investigated. Purpose To investigate the value of various MRI signs in differentiating Ewing sarcoma from osteomyelitis. Material and Methods Forty-one patients who underwent MRI because of a bone lesion of unknown nature with a differential diagnosis that included both Ewing sarcoma and osteomyelitis were included. Two observers assessed several MRI signs, including the transition zone of the bone lesion, the presence of a soft-tissue mass, intramedullary and extramedullary fat globules, and the penumbra sign. Results Diagnostic accuracies for discriminating Ewing sarcoma from osteomyelitis were 82.4% and 79.4% for the presence of a soft-tissue mass, and 64.7% and 58.8% for a sharp transition zone of the bone lesion, for readers 1 and 2 respectively. Inter-observer agreement with regard to the presence of a soft-tissue mass and the transition zone of the bone lesion were moderate (κ = 0.470) and fair (κ = 0.307), respectively. Areas under the receiver operating characteristic curve of the diameter of the soft-tissue mass (if present) were 0.829 and 0.833, for readers 1 and 2 respectively. Mean inter-observer difference in soft-tissue mass diameter measurement ± limits of agreement was 35.0 ± 75.0 mm. Diagnostic accuracies of all other MRI signs were all value in this setting.

Magnetic resonance imaging of low-grade fibromyxoid sarcoma

International Nuclear Information System (INIS)

Torriani, Martin; Ouellette, Hugue; Etchebehere, Mauricio; Amstalden, Eliane M.I.

2006-01-01

Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with a deceptively benign histologic appearance affecting predominantly young adults during the fourth decade of life. (MR) imaging features of a surgically confirmed case of LGFMS, affecting the shoulder is presented. A 30-year-old man presented with a 20-year history of a painless slow-growing mass in the right shoulder. Magnetic resonance images were obtained on a 2.0T scanner (Elscint, Haifa, Israel), demonstrating a well-defined soft tissue mass measuring 12.0 x 7.0 x 9.0 cm located between the deltoid muscle, rotator-cuff muscles, and proximal humerus. The differential diagnosis of LGFMS includes several benign and malignant neoplasms containing variable amounts of myxoid and fibrous tissue. Histologically, the most important differential diagnosis is with myxofibrosarcoma. Low-grade fibromyxoid sarcoma is a rare soft tissue tumor with slow growth and deceptively benign histologic appearance. The possibility of LGFMS must be considered when elaborating differential diagnostic possibilities for young adults with a large soft tissue mass exhibiting MR imaging characteristics of intermixed fibrous and myxoid tissue

Magnetic resonance imaging of low-grade fibromyxoid sarcoma

Energy Technology Data Exchange (ETDEWEB)

Torriani, Martin; Ouellette, Hugue [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States). Dept. of Radiology. Div. of Musculoskeletal Radiology; Etchebehere, Mauricio [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Ortopedia; Amstalden, Eliane M.I. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Patologia

2006-06-15

Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with a deceptively benign histologic appearance affecting predominantly young adults during the fourth decade of life. (MR) imaging features of a surgically confirmed case of LGFMS, affecting the shoulder is presented. A 30-year-old man presented with a 20-year history of a painless slow-growing mass in the right shoulder. Magnetic resonance images were obtained on a 2.0T scanner (Elscint, Haifa, Israel), demonstrating a well-defined soft tissue mass measuring 12.0 x 7.0 x 9.0 cm located between the deltoid muscle, rotator-cuff muscles, and proximal humerus. The differential diagnosis of LGFMS includes several benign and malignant neoplasms containing variable amounts of myxoid and fibrous tissue. Histologically, the most important differential diagnosis is with myxofibrosarcoma. Low-grade fibromyxoid sarcoma is a rare soft tissue tumor with slow growth and deceptively benign histologic appearance. The possibility of LGFMS must be considered when elaborating differential diagnostic possibilities for young adults with a large soft tissue mass exhibiting MR imaging characteristics of intermixed fibrous and myxoid tissue.

Long-term outcome of combined modality therapy in retroperitoneal and deep-trunk soft-tissue sarcoma: analysis of prognostic factors

International Nuclear Information System (INIS)

Youssef, Emad; Fontanesi, James; Mott, Michael; Kraut, Michael; Lucas, David; Mekhael, Hany; Ben-Josef, Edgar

2002-01-01

Purpose: To evaluate the long-term outcome of surgery and postoperative radiotherapy (RT) in retroperitoneal and deep-trunk soft-tissue sarcoma, and to identify the prognostic factors for local control, disease-free survival, and overall survival. Methods and Materials: Between January 1980 and December 1998, 60 patients with nonmetastatic retroperitoneal and deep-trunk soft-tissue sarcoma were treated at Wayne State University using combined surgery and RT. The location was retroperitoneal in 38 patients (63%) and deep trunk in 22 (27%). Forty-six patients (76%) were treated for primary disease and 14 (24%) for recurrent disease. The resection margins were negative in 24 patients (40%), close in 3 (5%), and positive in 33 (55%; 18 microscopic and 15 macroscopic). The median tumor size was 8.6 cm (range 2-55). External beam RT (EBRT; median dose 5220 cGy) was given to 44 patients (73%) and combined EBRT (median dose 4200 cGy) and brachytherapy (median dose 1600 cGy) to 16 patients (27%). Univariate and multivariate Cox regression analyses were conducted to identify the possible associations between patient age, race, gender, tumor site, histologic features, grade, size, stage, surgical margin, RT dose, modality (EBRT vs. EBRT plus brachytherapy), and presentation (primary vs. recurrent) and disease control. Results: The actuarial 5- and 10-year disease-free survival rate was 53% and 44%, respectively. Disease-free survival was significantly associated with female gender on univariate analysis (67% for female patients and 37% for male patients at 5 years, p=0.05). On multivariate analysis, both gender and surgical margin had borderline significance (p=0.06). The actuarial local control rate was 71% and 54% at 5 and 10 years, respectively. The median time to local relapse was 10.2 months, with 75% of all failures occurring within 29 months. The surgical margin status was significantly associated with local control (78% for patients with negative or close margins vs

Clinical experience with intravenous radiosensitizers in unresectable sarcomas

International Nuclear Information System (INIS)

Kinsella, T.J.; Glatstein, E.

1987-01-01

Traditionally, adult bone and soft tissue sarcomas have been considered to be ''radioresistant.'' Because of this philosophy, patients who present with locally advanced, unresectable sarcomas often are treated in a palliative fashion, usually with low-dose radiotherapy. Over the last 6 years, 29 patients with unresectable primary or metastatic sarcomas were treated using a combination of intravenous chemical radiosensitizers and high-dose irradiation. Twenty-two of 29 patients achieved clinical local control, with six patients having a complete clinical response. The time to tumor response is often several months or longer, which is in contrast to other tumor histologies (carcinomas, lymphomas), where tumor response usually occurs over several weeks. Several large tumors have shown only a minimal tumor response, yet were found to be sterilized in posttreatment biopsy or autopsy examination. Of 15 patients with primary sarcomas without metastases, 11 patients (73%) remain free of local tumor progression from 12 to 83 months. Adult high-grade sarcomas can be controlled with high-dose radiotherapy and intravenous radiosensitizers, although the precise role of these agents is unclear

Extraskeletal presentation of Ewing's Sarcoma.

Science.gov (United States)

Mangual, Danny; Bisbal-Matos, Luis A; Jiménez-Lee, Ricardo; Vélez, Román; Noy, Miguel

2018-03-01

The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

Computed tomography in soft-tissue lesions of the hand and forearm

International Nuclear Information System (INIS)

Schmitt, R.; Warmuth-Metz, M.; Lucas, D.; Feyerabend, T.; Schindler, G.; Lanz, U.

1990-01-01

Computed tomography was carried out in 32 patients with clinically equivocal soft-tissue lesions of the hand (24 times) and forearm (8 times). The CT scans were performed with the patients in standard positions; thin slices and zoom technique were used. All soft-tissue tumors were correctly diagnosed with regard to localization, size and infiltration of the surrounding tissue. The histological diagnosis was correct in tendon-sheath proliferations, deposits caused by metabolic disorders, epithelial and ganglion cysts, hemangiomas, lipomas and in one schwannoma. A malignancy was suspected and was proven to be correct in two cases. False-positive diagnoses of a malignant soft-tissue tumor were made in one case of an aggressive fibromatosis, in a rapidly progressive, ossifying myositis, and three times in the presence of postoperative scar tissue following the resection of a sarcoma. Finally, a case of proliferative myositis regarded as semimalignant was underrated by CT. The hand surgeon considered CT diagnostics to be very helpful in planning operations in an anatomically complex organ such as the hand. (orig.) [de

Low-dose-rate intraoperative brachytherapy combined with external beam irradiation in the conservative treatment of soft tissue sarcoma

International Nuclear Information System (INIS)

Delannes, M.; Thomas, L.; Martel, P.; Bonnevialle, P.; Stoeckle, E.; Chevreau, Ch.; Bui, B.N.; Daly-Schveitzer, N.; Pigneux, J.; Kantor, G.

2000-01-01

Purpose: Conservative treatment of soft tissue sarcomas most often implies combination of surgical resection and irradiation. The aim of this study was to evaluate low-dose-rate intraoperative brachytherapy, delivered as a boost, in the local control of primary tumors, with special concern about treatment complications. Methods and Materials: Between 1986 and 1995, 112 patients underwent intraoperative implant. This report focuses on the group of 58 patients with primary sarcomas treated by combination of conservative surgery, intraoperative brachytherapy, and external irradiation. Most of the tumors were located in the lower limbs (46/58--79%). Median size of the tumor was 10 cm, most of the lesions being T2-T3 (51/58--88%), Grade 2 or 3 (48/58--83%). The mean brachytherapy dose was 20 Gy and external beam irradiation dose 45 Gy. In 36/58 cases, iridium wires had to be placed on contact with neurovascular structures. Results: With a median follow-up of 54 months, the 5-year actuarial survival was 64.9%, with a 5-year actuarial local control of 89%. Of the 6 patients with local relapse, 3 were salvaged. Acute side effects, essentially wound healing problems, occurred in 20/58 patients, late side effects in 16/58 patients (7 neuropathies G2 to G4). No amputation was required. The only significant factor correlated with early side effects was the location of the tumor in the lower limb (p = 0.003), and with late side effects the vicinity of the tumor with neurovascular structures (p = 0.009). Conclusion: Brachytherapy allows early delivery of a boost dose in a reduced volume of tissue, precisely mapped by the intraoperative procedure. Combined with external beam irradiation, it is a safe and efficient treatment technique leading to high local control rates and limited functional impairment

Histopathological diagnostic discrepancies in soft tissue tumours referred to a specialist centre: reassessment in the era of ancillary molecular diagnosis.

Science.gov (United States)

Thway, Khin; Wang, Jayson; Mubako, Taka; Fisher, Cyril

2014-01-01

Introduction. Soft tissue tumour pathology is a highly specialised area of surgical pathology, but soft tissue neoplasms can occur at virtually all sites and are therefore encountered by a wide population of surgical pathologists. Potential sarcomas require referral to specialist centres for review by pathologists who see a large number of soft tissue lesions and where appropriate ancillary investigations can be performed. We have previously assessed the types of diagnostic discrepancies between referring and final diagnosis for soft tissue lesions referred to our tertiary centre. We now reaudit this 6 years later, assessing changes in discrepancy patterns, particularly in relation to the now widespread use of ancillary molecular diagnostic techniques which were not prevalent in our original study. Materials and Methods. We compared the sarcoma unit's histopathology reports with referring reports on 348 specimens from 286 patients with suspected or proven soft tissue tumours in a one-year period. Results. Diagnostic agreement was seen in 250 cases (71.8%), with 57 (16.4%) major and 41 (11.8%) minor discrepancies. There were 23 cases of benign/malignant discrepancies (23.5% of all discrepancies). 50 ancillary molecular tests were performed, 33 for aiding diagnosis and 17 mutational analyses for gastrointestinal stromal tumour to guide therapy. Findings from ancillary techniques contributed to 3 major and 4 minor discrepancies. While the results were broadly similar to those of the previous study, there was an increase in frequency of major discrepancies. Conclusion. Six years following our previous study and notably now in an era of widespread ancillary molecular diagnosis, the overall discrepancy rate between referral and tertiary centre diagnosis remains similar, but there is an increase in frequency of major discrepancies likely to alter patient management. A possible reason for the increase in major discrepancies is the increasing lack of exposure to soft tissue

Roentgenologic examination in Kaposi's sarcoma

International Nuclear Information System (INIS)

Kossovoj, A.L.

1990-01-01

Review of roentgenologic investigations into Kaposi's sarcoma is presented. It is shown that Kaposi's sarcoma is a disease injuring skin, osteal system, lungs and mediastinum, gastroeuteric tract and lymphatic nodes. Roentgenologic changes of soft tissues of limbs, osteal system, chest and gastroenteric tract organs are described. Manifestations of a tumor of any localization are quite different which makes it more difficult to perform roentgenologic diagnosis. An increase of Kaposi's sarcoma occurrence in patients suffering from aids as the disease increases is indicated

Adult soft tissue sarcomas of the head and neck treated by radiation and surgery or radiation alone: patterns of failure and prognostic factors

International Nuclear Information System (INIS)

Willers, Henning; Hug, Eugen G.; Spiro, Ira J.; Efird, Jimmy T.; Rosenberg, Andrew E.; Wang, C. C.

1995-01-01

Purpose: To analyze our experience treating soft tissue sarcomas of the head and neck in adults, and to identify patterns of failure and prognostic factors. Methods and Materials: The records of 57 patients with Stage M0 disease treated by radiation with or without surgery between 1972 and 1993 were reviewed. Median follow-up time was 4.3 years (range, 1.1-16.8 years). A group of potential prognostic factors was evaluated, including age at diagnosis, sex, initial tumor presentation (primary vs. recurrent), grade, T-stage, direct tumor extension, tumor depth, duration of treatment, and radiation dose. Results: The subset of angiosarcomas (11 out of 57 patients) had a considerably adverse effect on treatment outcome for the total group of sarcomas, with actuarial 5-year overall survival (OS), locoregional control (LRC), and freedom from distant metastasis (FDM) rates being 31%, 24%, and 42%, respectively. In contrast, for the remaining 46 patients with other histopathological tumor types, OS, LRC, and FDM rates were significantly higher (74%, 69%, and 83%, respectively). For this group of significant prognostic factors identified by uni- and multivariate analysis included tumor grade as a predictor of OS and T-stage as a predictor of LRC (p ≤ 0.050). Those patients who experienced a locoregional recurrence were at a significantly increased risk of dying (p = 0.004 in a multivariate model). All 17 patients without direct tumor extension to neurovascular structures, bone, contiguous organs, or skin remained free from distant failure. In contrast, 27% of 29 patients with direct extension had developed distant metastases at 5 years. In multivariate analysis, the absence of direct extension was a positive predictor of FDM (p = 0.007) and of OS (p = 0.034). Conclusions: 1) Angiosarcomas of the head and neck have a considerably poorer prognosis than other soft tissue sarcomas of this site. 2) In addition to tumor grade and size, direct tumor extension may be a useful

Racial and Ethnic Disparities in the Incidence and Trends of Soft Tissue Sarcoma Among Adolescents and Young Adults in the United States, 1995-2008.

Science.gov (United States)

Hsieh, Mei-Chin; Wu, Xiao-Cheng; Andrews, Patricia A; Chen, Vivien W

2013-09-01

The aim of this study was to examine racial/ethnic disparities in the incidence rates and trends of soft tissue sarcoma (STS) by gender, age, and histological type among adolescents and young adults (AYAs) aged 15-29 years. The 1995-2008 incidence data from 25 population-based cancer registries, covering 64% of the United States population, were obtained from the North American Association of Central Cancer Registries. The Surveillance, Epidemiology and End Results AYA site recode and International Classification of Diseases for Oncology, 3rd Edition, were adopted to categorize STS histological types and anatomic groups. Age-adjusted incidence rates and average annual percent change (AAPC) were calculated. The incidence of all STSs combined was 34% higher in males than females (95% CI: 1.28, 1.39), 60% higher among blacks than whites (95% CI: 1.52, 1.68), and slightly higher among Hispanics than whites. Compared with whites, blacks had significantly higher incidence of fibromatous neoplasms, and Hispanics had significantly higher incidence of liposarcoma. Whites were more likely to be diagnosed with synovial sarcoma than blacks. Black and Hispanic males had significantly higher Kaposi sarcoma incidence than white males. The AAPC of all STSs combined showed a significant decrease from 1995 to 2008 (AAPC=-2.1%; 95% CI: -3.2%, -1.0%). However, after excluding Kaposi sarcoma, there was no significant trend. The incidence rates of STS histological types in AYAs vary among racial/ethnic groups. The declining trends of STS are due mainly to decreasing incidence of Kaposi sarcoma in all races/ethnicities. Research to identify factors associated with racial/ethnic disparities in AYA STS is necessary.

Adjuvant chemotherapy in soft tissue sarcomas…Conflicts, consensus, and controversies

Directory of Open Access Journals (Sweden)

Jyoti Bajpai

2016-01-01

Full Text Available Soft tissue sarcomas (STSs are an uncommon and diverse group of more than 50 mesenchymal malignancies. Each of these histologic subtypes represents a unique disease with distinct biologic behavior and varying sensitivity to chemotherapy. The judicious use of adjuvant/neoadjuvant chemotherapy along with surgery and radiation in the treatment of localized STS has a role in improving patient outcomes by decreasing local and distant recurrences. There is evidence that the use of adjuvant chemotherapy to a mixed cohort of chemo sensitive and insensitive sarcoma subtypes results in limited benefit. Therefore, it is of paramount importance to identify the subpopulation with high metastatic potential and to identify effective histology-specific treatment options to these patients. Present perspective, will focus on the rationale for adjuvant chemotherapy in sarcoma, with emphasis on the histology driven chemotherapy. It will outline key therapeutic opportunities and hurdles in adjuvant medical treatment of sarcoma, focusing on specific subtypes that are on the verge of new breakthroughs, as well as those in which promise has not lived up to expectations.

Cervical Synovial Sarcoma In a Young Boy

African Journals Online (AJOL)

Synovial sarcomas comprise about 8% of all tumours of somatic soft-tissues, and are the most common sar- comas of the 'hands and feet. Occasionally they may occur in the trunk, but they have rarely been reported in the neck. We present a case of cervical soft-tissue mass pro- ducing symptoms in a 12-year-old-boy.

Indistinguishable genomic profiles and shared prognostic markers in undifferentiated pleomorphic sarcoma and leiomyosarcoma: different sides of a single coin?

DEFF Research Database (Denmark)

Carneiro, Ana; Francis, Princy; Bendahl, Pär-Ola

2009-01-01

Soft tissue sarcoma (STS) diagnostics and prognostics are challenging, particularly in highly malignant and pleomorphic subtypes such as undifferentiated pleomorphic sarcoma (UPS) and leiomyosarcoma (LMS). We applied 32K BAC arrays and gene expression profiling to 18 extremity soft tissue LMS and...

The tolerance of skin grafts to postoperative radiation therapy in patients with soft-tissue sarcoma

International Nuclear Information System (INIS)

Lawrence, W.T.; Zabell, A.; McDonald, H.D.

1986-01-01

During the last ten years at the National Cancer Institute, 11 patients have received 12 courses of postoperative adjuvant radiation therapy to skin grafts used for wound closure after the resection of soft-tissue sarcomas. The intervals between grafting and the initiation of radiation ranged between 3 and 20 weeks, and 4 patients received chemotherapy at the same time as their radiation. Ten of the 12 irradiated grafts remained intact after the completion of therapy. One graft had several small persistently ulcerated areas that required no further surgical treatment, and one graft required a musculocutaneous flap for reconstruction of a persistent large ulcer. Acute radiation effects on the grafted skin sometimes developed at slightly lower doses than usually seen with normal skin, but these acute effects necessitated a break in therapy on only five occasions. Concurrent chemotherapy and a relatively short interval between grafting and the initiation of radiation seemed to contribute to more severe radiation reactions. This experience indicates that postoperative adjuvant radiation therapy can be delivered to skin grafted areas without undue fear of complications, especially if the graft is allowed to heal adequately prior to initiating therapy and if chemotherapy is not given in conjunction with radiation

Gemcitabine treatment of rat soft tissue sarcoma with phosphatidyldiglycerol-based thermosensitive liposomes.

Science.gov (United States)

Limmer, Simone; Hahn, Jasmin; Schmidt, Rebecca; Wachholz, Kirsten; Zengerle, Anja; Lechner, Katharina; Eibl, Hansjörg; Issels, Rolf D; Hossann, Martin; Lindner, Lars H

2014-09-01

The pyrimidine analogue gemcitabine (dFdC) is frequently used in the treatment of patients with solid tumors. However, after i.v. application dFdC is rapidly inactivated by metabolization. Here, the potential of thermosensitive liposomes based on 1,2-dipalmitoyl-sn-glycero-3-phosphodiglycerol (DPPG2-TSL) were investigated as carrier and targeting system for delivery of dFdC in combination with local hyperthermia (HT). DPPG2-TSL were prepared by the lipid film hydration and extrusion method and characterized by dynamic light scattering, thin layer chromatography, phosphate assay and HPLC. In vivo experiments were performed in Brown Norway rats with a syngeneic soft tissue sarcoma. Local HT treatment was performed by light exposure. DPPG2-TSL were stable at 37°C in serum and showed a temperature dependent dFdC release >40°C. Plasma half-life of dFdC was strongly increased from 0.07 h (non-liposomal) to 0.53 h (liposomal, vesicle size 105 nm) or 2.59 h (liposomal, 129 nm). Therapy of BN175 tumors with dFdC encapsulated in DPPG2-TSL + HT showed significant improvement in tumor growth delay compared to non-liposomal dFdC without HT (p < 0.05), non-liposomal dFdC with HT (p < 0.01), and liposomal dFdC without HT (p < 0.05), respectively. Gemcitabine encapsulated in DPPG2-TSL in combination with local HT is a promising tool for the treatment of solid tumors. Therefore, these encouraging results ask for further investigation and evaluation.

Doxorubicin plus the IGF-1R antibody cixutumumab in soft tissue sarcoma: a phase I study using the TITE-CRM model.

Science.gov (United States)

Chugh, R; Griffith, K A; Davis, E J; Thomas, D G; Zavala, J D; Metko, G; Brockstein, B; Undevia, S D; Stadler, W M; Schuetze, S M

2015-07-01

Insulin-like growth factor receptor (IGF-1R) has been studied as an oncologic target in soft tissue sarcoma (STS), but its role in sarcoma biology is unclear. Anti-IGF-1R antibody cixutumumab demonstrated acceptable toxicity but limited activity as a single agent in STS. We carried out a dose-escalation study of cixutumumab with doxorubicin to evaluate safety and dosing of the combination. Eligible patients with advanced STS were treated with cixutumumab intravenously on days 1/8/15 at one of three dose levels (A: 1 mg/kg, B: 3 mg/kg, C: 6 mg/kg) with doxorubicin at 75 mg/m(2) as a 48 h infusion on day 1 of a 21 day cycle. After six cycles of the combination, patients could receive cixutumumab alone. The Time-to-Event Continual Reassessment Method was used to estimate the probability of dose-limiting toxicity (DLT) and to assign patients to the dose with an estimated probability of DLT≤20%. Between September 2008 and January 2012, 30 patients with advanced STS received a median of six cycles of therapy (range <1-22). Two DLTs were observed, grade 3 mucositis (dose level B) and grade 4 hyperglycemia (dose level C). Grade 2 and 3 reduced left ventricular ejection fraction was seen in three and two patients, respectively. Five partial responses were observed, and estimated progression-free survival was 5.3 months (95% confidence interval 3.0-6.3) in 26 response-assessable patients. Immunohistochemical staining of 11 available tumor samples for IGF-1R and phospho-IGF-1R was not significantly different among responders and non-responders, and serum analysis of select single-nucleotide polymorphisms did not predict for cardiotoxicity. The maximum tolerated dose was doxorubicin 75 mg/m(2) on day 1 and cixitumumab 6 mg/kg on days 1/8/15 of a 21 day cycle. Cardiac toxicity was observed and should be monitored in subsequent studies, which should be considered in STS only if a predictive biomarker of benefit to anti-IGF-1R therapy is identified. Clinical

Soft Tissue Sarcoma: A Prognostic Variables and Treatment Outcomes after Chemo - Radiotherapy and Conservative Surgery

International Nuclear Information System (INIS)

Abaza, A.; El-Shanshoury, H.

2015-01-01

Soft tissue sarcomas (STS) constitute a group of rare and heterogeneous cancers, that diverse a wide spectrum of histology and varied clinical behavior. The aim was to study, retrospectively the clinico-epidemiologic manifestation, response to therapy and prognostic factors of retroperitoneal (RPS), extremity and other STS patients treated with conservative surgery plus chemo - radiotherapy (CRT) attending the Pediatric Oncology Clinic, National Cancer Institute (NCI), Cairo University during 6 years period (inclusive). Files of 66 STS patients were revised for history, staging, investigations, treatment modalities and outcome of therapy. Their ages ranged from 6 months to 21 years. The male to female ratio was 1.87:1. Most of the patients belong to stage 3 and 1. Radiotherapy (RTH) and surgery remains the backbone of the multi - modality treatment plan. Complete remission (CR) was obtained in 80.3% of STS patients. Meanwhile, 6% of cases had disease progression and 6% died. The 2 - years overall survival rate (OS) and Disease - free survivals (DFS) we re 74.2% and 57.6% respectively. However, 20.8% of patients develop relapse>2 years after the end of treatment. Age at diagnosis, stage of the disease, tumor mass, pathological subtype and the RTH used were statistically significant prognostic variables affecting survival. Finally, the use of RTH in the treatment of STS gives positive impact on the survival of patients. Recommendation regarding further studies would be required to identify methods of increasing the radiation dose selectively. Also, using the newly developed radiation technologies to make dose escalation and sparing normal tissue

Diode laser soft-tissue surgery: advancements aimed at consistent cutting, improved clinical outcomes.

Science.gov (United States)

Romanos, Georgios E

2013-01-01

Laser dentistry and soft-tissue surgery, in particular, have become widely adopted in recent years. Significant cost reductions for dental lasers and the increasing popularity of CADCAM, among other factors, have contributed to a substantial increase in the installed base of dental lasers, especially soft-tissue lasers. New development in soft-tissue surgery, based on the modern understanding of laser-tissue interactions and contact soft-tissue surgery mechanisms, will bring a higher quality and consistency level to laser soft-tissue surgery. Recently introduced diode-laser technology enables enhanced control of side effects that result from tissue overheating and may improve soft-tissue surgical outcomes.

Detection of SYT-SSX mutant transcripts in formalin-fixed paraffin-embedded sarcoma tissues using one-step reverse transcriptase real-time PCR.

Science.gov (United States)

Norlelawati, A T; Mohd Danial, G; Nora, H; Nadia, O; Zatur Rawihah, K; Nor Zamzila, A; Naznin, M

2016-04-01

Synovial sarcoma (SS) is a rare cancer and accounts for 5-10% of adult soft tissue sarcomas. Making an accurate diagnosis is difficult due to the overlapping histological features of SS with other types of sarcomas and the non-specific immunohistochemistry profile findings. Molecular testing is thus considered necessary to confirm the diagnosis since more than 90% of SS cases carry the transcript of t(X;18)(p11.2;q11.2). The purpose of this study is to diagnose SS at molecular level by testing for t(X;18) fusion-transcript expression through One-step reverse transcriptase real-time Polymerase Chain Reaction (PCR). Formalin-fixed paraffin-embedded tissue blocks of 23 cases of soft tissue sarcomas, which included 5 and 8 cases reported as SS as the primary diagnosis and differential diagnosis respectively, were retrieved from the Department of Pathology, Tengku Ampuan Afzan Hospital, Kuantan, Pahang. RNA was purified from the tissue block sections and then subjected to One-step reverse transcriptase real-time PCR using sequence specific hydrolysis probes for simultaneous detection of either SYT-SSX1 or SYT-SSX2 fusion transcript. Of the 23 cases, 4 cases were found to be positive for SYT-SSX fusion transcript in which 2 were diagnosed as SS whereas in the 2 other cases, SS was the differential diagnosis. Three cases were excluded due to failure of both amplification assays SYT-SSX and control β-2-microglobulin. The remaining 16 cases were negative for the fusion transcript. This study has shown that the application of One-Step reverse transcriptase real time PCR for the detection SYT-SSX transcript is feasible as an aid in confirming the diagnosis of synovial sarcoma.

A Review: Molecular Aberrations within Hippo Signaling in Bone and Soft Tissue Sarcomas

Directory of Open Access Journals (Sweden)

Michael D Deel

2015-09-01

Full Text Available The Hippo signaling pathway is an evolutionarily conserved developmental network vital for the regulation of organ size, tissue homeostasis, repair and regeneration, and cell fate. The Hippo pathway has also been shown to have tumor suppressor properties. Hippo transduction involves a series of kinases and scaffolding proteins that are intricately connected to proteins in developmental cascades and in the tissue microenvironment. This network governs the downstream Hippo transcriptional coactivators YAP and TAZ, which bind to and activate the output of TEADs, as well as other transcription factors responsible for cellular proliferation, self-renewal, differentiation, and survival. Surprisingly, there are few oncogenic mutations within the core components of the Hippo pathway. Instead, dysregulated Hippo signaling is a versatile accomplice to commonly mutated cancer pathways. For example, YAP and TAZ can be activated by oncogenic signaling from other pathways, or serve as coactivators for classical oncogenes. Emerging evidence suggests that Hippo signaling couples cell density and cytoskeletal structural changes to morphogenic signals, and conveys a mesenchymal phenotype. While much of Hippo biology has been described in epithelial cell systems, it is clear that dysregulated Hippo signaling also contributes to malignancies of mesenchymal origin. This review will summarize the known molecular alterations within the Hippo pathway in sarcomas, and highlight how several pharmacologic compounds have shown activity in modulating Hippo components, providing proof-of-principle that Hippo signaling may be harnessed for therapeutic application in sarcomas.

Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial)

Science.gov (United States)

2018-05-15

Advanced Malignant Solid Neoplasm; RB1 Positive; Recurrent Childhood Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Kidney Wilms Tumor; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma

Management of locally recurrent soft-tissue sarcoma after prior surgery and radiation therapy

International Nuclear Information System (INIS)

Torres, Mylin A.; Ballo, Matthew T.; Butler, Charles E.; Feig, Barry W.; Cormier, Janice N.; Lewis, Valerae O.; Pollock, Raphael E.; Pisters, Peter W.; Zagars, Gunar K.

2007-01-01

Purpose: The aim of this study was to evaluate outcome and treatment toxicity after wide local re-excision (WLE), with or without additional radiation therapy, for patients with isolated first local recurrence of soft-tissue sarcoma arising within a previously irradiated field. Methods: A retrospective review was performed of 62 consecutive patients. All patients underwent prior resection and external beam radiation. For recurrent disease, 25 patients were treated with WLE alone, and 37 patients were treated with WLE and additional radiation (45- 64 Gy). In 33 patients, the radiation was delivered via an afterloaded brachytherapy, single-plane implant. Results: The 5-year disease specific and distant metastasis-free survival rates were 65% and 73%, respectively. Local control (LC) at 5 years was 51%, and on multivariate analysis, a positive surgical resection margin (p< 0.001) was associated with a lower rate of LC. Reirradiation was not associated with improved LC; however complications requiring outpatient or surgical management were more common in patients who had undergone reirradiation (80% vs. 17%, p < 0.001). Amputation was also more common in the subgroup of patients who underwent extremity reirradiation (35% with radiation vs. 11% without, p = 0.05), although only one amputation was performed to resolve a treatment complication. Conclusion: Conservative surgery alone results in LC in a minority of patients who have failed locally after previous excision and external beam radiation. Although selection biases and small patient numbers confound the analysis, local treatment intensification with additional radiation does not clearly improve outcome after surgical excision alone, and is associated with an increase in complications

The role of imaging in the evaluation of extraskeletal Ewing's sarcoma

African Journals Online (AJOL)

Primary bone tumours account for 5% of all adolescent and child cancers. When Ewing's sarcoma arises in soft tissue rather than bone, it is referred to as extraskeletal Ewing's sarcoma (ESS). Computed tomography (CT) scanning and magnetic resonance imaging (MRI) features of Ewing's sarcoma are non-specific, and a ...

Positron emission tomography with fluorine-deoxyglucose in sarcomas and non-sarcoma non-epithelial tumors

OpenAIRE

Massardo, Teresa; Jofré, María Josefina; Sierralta, María Paulina; Canessa, José; Castro, Gabriel; Berrocal, Isabel; Gallegos, Iván

2012-01-01

Background: The usefulness of positron emission tomography (PET) with fluorine-deoxyglucose (FDG) in sarcomas and non-sarcoma non-epithelial (NSNE) tumors is not clearly defined. Aim: To report a Chilean experience with NSNE tumors evaluated using PET with FDG. Material and Methods: Retrospective review of the database of a PET laboratory. Demographic data, indications and metabolic findings were compared with conventional imaging in 88 adults and children with diverse bone and soft tissue sa...

Cost-Effectiveness Analysis of Intensity Modulated Radiation Therapy Versus 3-Dimensional Conformal Radiation Therapy for Preoperative Treatment of Extremity Soft Tissue Sarcomas

Energy Technology Data Exchange (ETDEWEB)

Richard, Patrick, E-mail: patrjr@uw.edu [Department of Radiation Oncology, University of Washington, Seattle, Washington (United States); Phillips, Mark; Smith, Wade [Department of Radiation Oncology, University of Washington, Seattle, Washington (United States); Davidson, Darin [Department of Orthopedic Surgery, University of Washington, Seattle, Washington (United States); Kim, Edward; Kane, Gabrielle [Department of Radiation Oncology, University of Washington, Seattle, Washington (United States)

2016-07-01

Purpose: Create a cost-effectiveness model comparing preoperative intensity modulated radiation therapy (IMRT) versus 3-dimensional conformal radiation therapy (3DCRT) for extremity soft tissue sarcomas. Methods and Materials: Input parameters included 5-year local recurrence rates, rates of acute wound adverse events, and chronic toxicities (edema, fracture, joint stiffness, and fibrosis). Health-state utilities were used to calculate quality-adjusted life years (QALYs). Overall treatment costs per QALY or incremental cost-effectiveness ratio (ICER) were calculated. Roll-back analysis was performed using average costs and utilities to determine the baseline preferred radiation technique. One-way, 2-way, and probabilistic sensitivity analyses (PSA) were performed for input parameters with the largest impact on the ICER. Results: Overall treatment costs were $17,515.58 for 3DCRT compared with $22,920.51 for IMRT. The effectiveness was higher for IMRT (3.68 QALYs) than for 3DCRT (3.35 QALYs). The baseline ICER for IMRT was $16,842.75/QALY, making it the preferable treatment. The ICER was most sensitive to the probability of local recurrence, upfront radiation costs, local recurrence costs, certain utilities (no toxicity/no recurrence, grade 1 toxicity/no local recurrence, grade 4 toxicity/no local recurrence), and life expectancy. Dominance patterns emerged when the cost of 3DCRT exceeded $15,532.05 (IMRT dominates) or the life expectancy was under 1.68 years (3DCRT dominates). Furthermore, preference patterns changed based on the rate of local recurrence (threshold: 13%). The PSA results demonstrated that IMRT was the preferred cost-effective technique for 64% of trials compared with 36% for 3DCRT. Conclusions: Based on our model, IMRT is the preferred technique by lowering rates of local recurrence, severe toxicities, and improving QALYs. From a third-party payer perspective, IMRT should be a supported approach for extremity soft tissue sarcomas.

Cost-Effectiveness Analysis of Intensity Modulated Radiation Therapy Versus 3-Dimensional Conformal Radiation Therapy for Preoperative Treatment of Extremity Soft Tissue Sarcomas

International Nuclear Information System (INIS)

Richard, Patrick; Phillips, Mark; Smith, Wade; Davidson, Darin; Kim, Edward; Kane, Gabrielle

2016-01-01

Purpose: Create a cost-effectiveness model comparing preoperative intensity modulated radiation therapy (IMRT) versus 3-dimensional conformal radiation therapy (3DCRT) for extremity soft tissue sarcomas. Methods and Materials: Input parameters included 5-year local recurrence rates, rates of acute wound adverse events, and chronic toxicities (edema, fracture, joint stiffness, and fibrosis). Health-state utilities were used to calculate quality-adjusted life years (QALYs). Overall treatment costs per QALY or incremental cost-effectiveness ratio (ICER) were calculated. Roll-back analysis was performed using average costs and utilities to determine the baseline preferred radiation technique. One-way, 2-way, and probabilistic sensitivity analyses (PSA) were performed for input parameters with the largest impact on the ICER. Results: Overall treatment costs were $17,515.58 for 3DCRT compared with $22,920.51 for IMRT. The effectiveness was higher for IMRT (3.68 QALYs) than for 3DCRT (3.35 QALYs). The baseline ICER for IMRT was $16,842.75/QALY, making it the preferable treatment. The ICER was most sensitive to the probability of local recurrence, upfront radiation costs, local recurrence costs, certain utilities (no toxicity/no recurrence, grade 1 toxicity/no local recurrence, grade 4 toxicity/no local recurrence), and life expectancy. Dominance patterns emerged when the cost of 3DCRT exceeded $15,532.05 (IMRT dominates) or the life expectancy was under 1.68 years (3DCRT dominates). Furthermore, preference patterns changed based on the rate of local recurrence (threshold: 13%). The PSA results demonstrated that IMRT was the preferred cost-effective technique for 64% of trials compared with 36% for 3DCRT. Conclusions: Based on our model, IMRT is the preferred technique by lowering rates of local recurrence, severe toxicities, and improving QALYs. From a third-party payer perspective, IMRT should be a supported approach for extremity soft tissue sarcomas.

Standardizing evaluation of sarcoma proliferation- higher Ki-67 expression in the tumor periphery than the center

DEFF Research Database (Denmark)

Fernebro, J; Engellau, J; Persson, A

2007-01-01

Soft tissue sarcomas often present as large and histopathologically heterogenous tumors. Proliferation has repeatedly been identified as a prognostic factor and immunostaining for Ki-67 represents the most commonly used proliferation marker. There is, however, a lack of consensus on how to evaluate...... of proliferation in soft tissue sarcomas should be standardized for clinical application of Ki-67 as a prognostic marker....

Ewing Sarcoma: Current Management and Future Approaches Through Collaboration

NARCIS (Netherlands)

Gaspar, Nathalie; Hawkins, Douglas S.; Dirksen, Uta; Lewis, Ian J.; Ferrari, Stefano; Le Deley, Marie-Cecile; Kovar, Heinrich; Grimer, Robert; Whelan, Jeremy; Claude, Line; Delattre, Olivier; Paulussen, Michael; Picci, Piero; Sundby Hall, Kirsten; van den Berg, Hendrik; Ladenstein, Ruth; Michon, Jean; Hjorth, Lars; Judson, Ian; Luksch, Roberto; Bernstein, Mark L.; Marec-Bérard, Perrine; Brennan, Bernadette; Craft, Alan W.; Womer, Richard B.; Juergens, Heribert; Oberlin, Odile

2015-01-01

Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or

The use of thallium-201 scintigraphy in the assessment and management of bone and soft tissue tumours

International Nuclear Information System (INIS)

Jong, I.; Schlicht, S.M.; Smith, P.; J Slavin; Powell, G.; Choong, P.F.M.

2004-01-01

Introduction: Thallium-201 scintigraphy is routinely performed on all patients presenting to the bone and soft tissue sarcoma service at St. Vincent's Hospital Melbourne. Methods: As part of the multimodality approach to patients presenting to the service, Thallium-201 planar images are obtained at 30 minutes following a resting injection of tracer with delayed planar and where possible SPECT images at 4 hours. Uptake is qualitatively assessed using adjacent muscle uptake at the time of the scan. All thallium-201 scans are read in conjunction with the correlative structural images performed. Results: High grade bone and soft tissue sarcomas typically show significant retention of tracer relative to background activity and adjacent anatomically structures. Benign lesions may show early activity but typically washout on the delayed images. However, some lesions may show marked thallium-201 activity which will be discussed. At our institution the thallium-201 scans are used for assessment of metabolic activity and tumour grade, planning of image guided pre-treatment biopsies and monitoring of treatment response particularly following radiotherapy. Conclusion: Thallium-201 scintigraphy plays a vital role in assessment and management of bone and soft tissue tumours at our institution. (authors)

Ewing's Sarcoma of the Adrenal Gland.

Science.gov (United States)

Pal, Dilip Kumar; Chandra, Vipin; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

2016-01-01

Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery.

Ewing's sarcoma of the head and neck

Directory of Open Access Journals (Sweden)

Adriano Santana Fonseca

2000-11-01

Full Text Available CONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual. CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

The Molecular Biology of Soft-Tissue Sarcomas and Current Trends in Therapy

Directory of Open Access Journals (Sweden)

Jorge Quesada

2012-01-01

Full Text Available Basic research in sarcoma models has been fundamental in the discovery of scientific milestones leading to a better understanding of the molecular biology of cancer. Yet, clinical research in sarcoma has lagged behind other cancers because of the multiple clinical and pathological entities that characterize sarcomas and their rarity. Sarcomas encompass a very heterogeneous group of tumors with diverse pathological and clinical overlapping characteristics. Molecular testing has been fundamental in the identification and better definition of more specific entities among this vast array of malignancies. A group of sarcomas are distinguished by specific molecular aberrations such as somatic mutations, intergene deletions, gene amplifications, reciprocal translocations, and complex karyotypes. These and other discoveries have led to a better understanding of the growth signals and the molecular pathways involved in the development of these tumors. These findings are leading to treatment strategies currently under intense investigation. Disruption of the growth signals is being targeted with antagonistic antibodies, tyrosine kinase inhibitors, and inhibitors of several downstream molecules in diverse molecular pathways. Preliminary clinical trials, supported by solid basic research and strong preclinical evidence, promises a new era in the clinical management of these broad spectrum of malignant tumors.

Angiographic picture of soft tissue desmoid fibromas

International Nuclear Information System (INIS)

Nikitaev, N.S.; Kuznetsova, M.A.

1987-01-01

Arteriography was performed in 35 patients with soft tissue desmoid fibromas. Angiographic semiotics of this disease was described. The frequency of detectability of symptoms in the arterial, parenchymatous and venous phases was analyzed. A tumor in the arterial phase was shown to be of normevascular or moderately hypervascular type without noticeable winding of the nutrient arteries, such features of malignancy as lacunae and ''tumor'' vessels being absent. In the parenchymatous phase desmoid tumors were shown to accumulate moderately a contrast substance, a tumor contour at a vast length was ill defined and poorly marked from surrounding tissues. The venous phase was less noticeable and the time of its appearance was usually within normal. In general by most of its parameters the angiographic picture of agressive fibromatosis corresponded to that of benign tumors and could be used for differential diagnosis of desmoid fibromas and some types of tissue sarcomas

Primary renal synovial sarcoma

Directory of Open Access Journals (Sweden)

Girish D. Bakhshi

2012-03-01

Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

A prognostic model for soft tissue sarcoma of the extremities and trunk wall based on size, vascular invasion, necrosis, and growth pattern

DEFF Research Database (Denmark)

Carneiro, Ana; Bendahl, Par-Ola; Engellau, Jacob

2011-01-01

type, necrosis, and grade. METHODS:: Whole-tumor sections from 239 soft tissue sarcomas of the extremities were reviewed for the following prognostic factors: size, vascular invasion, necrosis, and growth pattern. A new prognostic model, referred to as SING (Size, Invasion, Necrosis, Growth......), was established and compared with other clinically applied systems. RESULTS:: Size, vascular invasion, necrosis, and peripheral tumor growth pattern provided independent prognostic information with hazard ratios of 2.2-2.6 for development of metastases in multivariate analysis. When these factors were combined...... into the prognostic model SING, high risk of metastasis was predicted with a sensitivity of 74% and a specificity of 85%. Moreover, the prognostic performance of SING compared favorably with other widely used systems. CONCLUSIONS:: SING represents a promising prognostic model, and vascular invasion and tumor growth...

Primary extra-skeletal Ewing's sarcoma mimicking a disc protrusion.

Science.gov (United States)

Ruelle, A; Boccardo, M

1987-07-01

One of the rarest cases of primary epidural neoplasm is a soft tissue sarcoma histologically similar to Ewing's sarcoma of the bone. In the literature only eleven cases of such an extra-skeletal Ewing's sarcoma have been described. The authors report an additional case presenting as a disc protrusion in a young male. The authors include some diagnostic, prognostic and nosologic remarks about this condition.

Current State of Pediatric Sarcoma Biology and Opportunities for Future Discovery: A Report from the Sarcoma Translational Research Workshop

Science.gov (United States)

Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D.; Kadoch, Cigall; Mackall, Crystal L.; Khan, Javed; Shern, Jack F.; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A.; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J.; Adamson, Peter C.; Lupo, Philip J.; Mody, Rajen; DuBois, Steven G.; Parsons, D. Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S.; Randall, R. Lor; Smith, Malcolm; Sorensen, Poul H.; Plon, Sharon E.; Skapek, Stephen X.; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R.

2017-01-01

Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA on April 18, 2015 sponsored by the QuadW foundation, Children’s Oncology Group and CureSearch for Children’s Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at the current time. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop. PMID:27132463

PET-CT in the evaluation of sarcomas of soft tissues

International Nuclear Information System (INIS)

Serna M, J.A.; Quiroz C, O.; Sanchez C, N.; Diaz V, G.

2007-01-01

18 F-FDG PET-CT is an image modality of great utility in the evaluation of primary or recurrent lesions of soft tissues. It is necessary to determine the cost-benefit of the different image modalities, although one waits that by means of a better diagnostic, statification and the determination of the grade of malignancy, the PET-CT nowadays can reduce the cost and the complications of the invasive diagnostic methods. (Author)

Epidemiological Evaluation of Head and Neck Sarcomas in Iran (the Study of 105 Cases Over 13 Years).

Science.gov (United States)

Alishahi, Batoul; Kargahi, Neda; Homayouni, Solmaz

2015-08-01

Head and neck sarcomas are exceedingly rare and they include 4% - 10% of all sarcomas and less than 1% of all neoplasm of head and neck. The aim of this study is to evaluate the epidemiological characteristics of head and neck sarcomas of patients in Isfahan, Iran. In this retrospective study, from the 16000 patients whose files were evaluated, the total number of 105 head and neck sarcomas were collected. They were evaluated with due attention to age, gender of the patients and the most common location of the lesion. From the total number of 105 (0.6%) patients with sarcomas, 56 were men (53.33%) and 49 women (46.66%). The most common head and neck sarcomas among this population were Osteosarcoma (32 cases, 30.47%), Chondrosarcoma (14 cases, 13.33%), and Ewing sarcoma (11 cases, 10.47%).The most common soft tissue sarcoma was Rabdomiosarcoma. Mandible was the most common location for these lesions. In this study, the hard tissue sarcomas were more prevalent than soft tissue ones. Hence, special attention should be paid to the patients when being diagnosed.

Limb sparing approach: Adjuvant radiation therapy in adults with intermediate or high-grade limb soft tissue sarcoma

International Nuclear Information System (INIS)

Merimsky, Ofer; Soyfer, Vjacheslav; Kovner, Felix; Bickels, Jacob; Issakov, Josephine; Flusser, Gideon; Meller, Isaac; Ofer, Oded; Kollender, Yehuda

2005-01-01

Background: Limb soft tissue sarcomas (STS) are currently treated with limb sparing surgery (LSS) followed by radiation therapy (RT). Patients and methods: Between October 1994 and October 2002, 133 adult patients with intermediate or high-grade limb STS were approached by LSS+RT. Results: RT related toxicity was manageable, with a low rate of severe effects. At 4-year median follow-up, there were 48 recurrences of any type, 23 of isolated local failure, and 35 of systemic spread w/o local failure. DFS and OS were influenced by disease stage II vs I, primary site in the upper limb vs lower limb, MPNST vs other types, induction therapy vs no induction, adequate resection vs marginal resection or involved margins, and good response to induction therapy vs bad response. DFS and OS were Patient's age and sex, tumor depth, acute or late toxicity of RT, or the interval of time between the date of definitive surgery and the start of RT did not affect DFS and or OS. Conclusions: The RT protocol is applicable in the era of complicated, expensive and time-consuming 3D therapy. Our results of LSS+RT in adults with limb HG STS are satisfactory

Soft tissue changes and its stability as a sequlae to mandibular advancement

OpenAIRE

Uppada, Uday Kiran; Sinha, Ramen; Reddy, D. Sreenatha; Paul, Dushyanth

2014-01-01

Purpose of the Study: To predict the changes and evaluate the stability that occurs in the soft tissues following the skeletal movement subsequent to surgical advancement of the mandible through bilateral sagittal split osteotomy and to provide the patient reliable information with regard to esthetic changes that can be expected following the treatment. Materials and Methods: Twenty adult patients diagnosed with skeletal class II malocclusion and underwent bilateral sagittal split osteotomy f...

A Device and Procedure for Immobilization of Patients Receiving Limb-Preserving Radiotherapy for Soft Tissue Sarcoma

International Nuclear Information System (INIS)

Dickie, Colleen I.; Parent, Amy; Griffin, Anthony; Craig, Tim; Catton, Charles; Chung, Peter; Panzarella, Tony; O'Sullivan, Brian; Sharpe, Michael

2009-01-01

The purpose of this study was to determine the accuracy and efficiency of a custom-designed immobilization device for patients with extremity soft-tissue sarcoma. The custom device consisted of a thermoplastic shell, vacuum pillow, and adaptable baseplate. The study included patients treated from January 2005 to March 2007, with 92 patients immobilized with the custom device and 98 with an established standard. Setup times for these cohorts were analyzed retrospectively for conformal and intensity modulated radiotherapy techniques (IMRT). Thigh tumor setup times were analyzed independently. A subset of patients treated with IMRT was analyzed for setup error using the radiographically verified isocenter position measured daily with electronic portal imaging and cone-beam computed tomography. Mean setup time was reduced by 2.2 minutes when using the custom device for conformal treatment (p = 0.03) and by 5.8 min for IMRT of thigh tumors (p = 0.009). All other setup time comparisons were not significant. A significant systematic error reduction was seen in all directions using the custom device. Random error standard deviations favored the custom device. The custom device offers immobilization advantages. Patient setup time was reduced for conformal techniques and IMRT of thigh tumors. Positioning uncertainty was improved, permitting a reduction of the planning target volume margin by 2 to 4 mm.

Osseous Kaposi sarcoma in an HIV-positive patient

International Nuclear Information System (INIS)

Thanos, Loukas; Mylona, Sofia; Kalioras, Vasilios; Pomoni, Maria; Batakis, Nikolaos

2004-01-01

A case of osseous Kaposi sarcoma in a 35-year-old man is described. The patient (HIV-positive for 8 years) suffered from cutaneous Kaposi sarcoma and presented with right-sided chest pain. He underwent a chest CT scan that revealed three osteolytic lesions involving rib and vertebra with large soft tissue masses, without cutaneous lesions at these sites. CT-guided core needle biopsy led to a histological diagnosis of Kaposi sarcoma. (orig.)

MRI features of soft-tissue lumps and bumps

International Nuclear Information System (INIS)

Zhuang, K.D.; Tandon, A.A.; Ho, B.C.S.; Chong, B.K.

2014-01-01

Superficial soft-tissue lesions are frequently encountered by radiologists in everyday practice. Characterization of these soft-tissue lesions remains problematic, despite advances in imaging. By systematically using clinical history, anatomical location, and signal intensity characteristics on MRI images, one can determine the diagnosis for the subset of determinate lesions that have characteristic clinical and imaging features as well as narrow the differential diagnoses for lesions with non-specific or indeterminate characteristics. In this review, a spectrum of histologically proven benign and malignant superficial soft-tissue lesions from a single institution will be presented. In addition, a few tumour-like conditions will be included as they can be encountered during imaging of soft-tissue masses

Cirurgia conservadora, radioterapia externa e reforço de dose com braquiterapia de alta taxa de dose: uma nova perspectiva no tratamento de sarcomas de partes moles do adulto Limb-sparing surgery, external beam radiotherapy and boost with high-dose rate brachytherapy: a new perspective for the treatment of soft tissue sarcomas in adults

Directory of Open Access Journals (Sweden)

Antonio Cássio Assis Pellizzon

2002-03-01

Full Text Available OBJETIVO: Avaliar a influência no controle local de pacientes adultos e portadores de sarcoma de partes moles em extremidades e submetidos a cirurgia conservadora do membro, com braquiterapia de alta taxa de dose (BATD como reforço para a radioterapia externa (RT. MATERIAL E MÉTODOS: Foram avaliados 16 pacientes tratados, de 1993 até 1999. A RT foi utilizada com finalidade pré ou pós-operatória (30--55 Gy e BATD com dose de 18--36 Gy (fx 3--6 Gy BID. Com base no modelo linear quadrático calculou-se a dose efetiva biológica ("biological effective dose" - BED para o tumor e comparou-se seu valor a dados da literatura internacional, que utiliza tratamentos com RT e braquiterapia de baixa taxa de dose (BBTD. RESULTADOS: Os valores médios e medianos da BED para os sarcomas de partes moles foram de 78,5 Gy7 e 80 Gy7. A análise univariada mostrou que a BED para o tumor, quando utilizada BATD, era semelhante ao valor de 83 Gy7 quando utilizada BBTD (p = 0,008. As taxas de controle local, sobrevida livre de doença e sobrevida global atuarial em cinco anos foram de 83,2%, 75% e 93,7%, respectivamente. CONCLUSÕES: A BATD, quando utilizada como método complementar no reforço de dose da RT no tratamento conservador dos sarcomas de partes moles, apresenta taxas de controle local equiparáveis às da literatura internacional; no entanto, estudos com número maior de pacientes e período maior de seguimento são ainda necessários para determinar o verdadeiro potencial da BATD em substituir a BBTD.PURPOSE: To evaluate the influence on local control in adult patients with soft tissue sarcomas of the limbs that underwent limb-sparing surgery and high-dose rate brachytherapy (HDRB in association with teletherapy. MATERIAL AND METHODS: Sixteen patients treated from 1993 to 1999 were reviewed. Teletherapy was used pre- or postoperatively (30--55 Gy in association with HDRB in a dose range of 18--36 Gy (fx 3--6 Gy BID. The linear quadratic model was

Down-Regulation of AKT Signalling by Ursolic Acid Induces Intrinsic Apoptosis and Sensitization to Doxorubicin in Soft Tissue Sarcoma.

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Victor Hugo Villar

Full Text Available Several important biological activities have been attributed to the pentacyclic triterpene ursolic acid (UA, being its antitumoral effect extensively studied in human adenocarcinomas. In this work, we focused on the efficacy and molecular mechanisms involved in the antitumoral effects of UA, as single agent or combined with doxorubicin (DXR, in human soft tissue sarcoma cells. UA (5-50 μM strongly inhibited (up to 80% the viability of STS cells at 24 h and its proliferation in soft agar, with higher concentrations increasing apoptotic death up to 30%. UA treatment (6-9 h strongly blocked the survival AKT/GSK3β/β-catenin signalling pathway, which led to a concomitant reduction of the anti-apoptotic proteins c-Myc and p21, altogether resulting in the activation of intrinsic apoptosis. Interestingly, UA at low concentrations (10-15 μM enhanced the antitumoral effects of DXR by up to 2-fold, while in parallel inhibiting DXR-induced AKT activation and p21 expression, two proteins implicated in antitumoral drug resistance and cell survival. In conclusion, UA is able to induce intrinsic apoptosis in human STS cells and also to sensitize these cells to DXR by blocking the AKT signalling pathway. Therefore, UA may have beneficial effects, if used as nutraceutical adjuvant during standard chemotherapy treatment of STS.

An Unusual Location of Extraosseous Ewing's Sarcoma

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Lisanne Geens

2013-05-01

Full Text Available Ewing's sarcoma (ES is the second most common malignant bone tumor in children and young adults. ES also occurs as a primary soft tissue neoplasm without involvement of bone. We report the second case of extraosseous (EO ES emerging from the omentum and a review of the relevant literature. EO ES should be included in the differential diagnosis of soft tissue neoplasms in the abdomen.

Newly Characterized Murine Undifferentiated Sarcoma Models Sensitive to Virotherapy with Oncolytic HSV-1 M002

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Eric K. Ring

2017-12-01

Full Text Available Despite advances in conventional chemotherapy, surgical techniques, and radiation, outcomes for patients with relapsed, refractory, or metastatic soft tissue sarcomas are dismal. Survivors often suffer from lasting morbidity from current treatments. New targeted therapies with less toxicity, such as those that harness the immune system, and immunocompetent murine sarcoma models to test these therapies are greatly needed. We characterized two new serendipitous murine models of undifferentiated sarcoma (SARC-28 and SARC-45 and tested their sensitivity to virotherapy with oncolytic herpes simplex virus 1 (HSV-1. Both models expressed high levels of the primary HSV entry molecule nectin-1 (CD111 and were susceptible to killing by interleukin-12 (IL-12 producing HSV-1 M002 in vitro and in vivo. M002 resulted in a significant intratumoral increase in effector CD4+ and CD8+ T cells and activated monocytes, and a decrease in myeloid-derived suppressor cells (MDSCs in immunocompetent mice. Compared to parent virus R3659 (no IL-12 production, M002 resulted in higher CD8:MDSC and CD8:T regulatory cell (Treg ratios, suggesting that M002 creates a more favorable immune tumor microenvironment. These data provide support for clinical trials targeting sarcomas with oncolytic HSV-1. These models provide an exciting opportunity to explore combination therapies for soft tissue sarcomas that rely on an intact immune system to reach full therapeutic potential.

Prevalence, extension and characteristics of fluid-fluid levels in bone and soft tissue tumors

Energy Technology Data Exchange (ETDEWEB)

Dyck, P. van; Venstermans, C.; Gielen, J.; Parizel, P.M. [University Hospital Antwerp, Department of Radiology, Edegem (Belgium); Vanhoenacker, F.M. [University Hospital Antwerp, Department of Radiology, Edegem (Belgium); AZ St-Maarten, Department of Radiology, Duffel/Mechelen (Belgium); Vogel, J. [Leiden University Medical Centre, Department of Orthopedics, Leiden (Netherlands); Kroon, H.M.; Bloem, J.L. [Leiden University Medical Centre, Department of Radiology, Leiden (Netherlands); Schepper, A.M.A. de [University Hospital Antwerp, Department of Radiology, Edegem (Belgium); Leiden University Medical Centre, Department of Radiology, Leiden (Netherlands)

2006-12-15

The purpose of this study was to determine the prevalence, extension and signal characteristics of fluid-fluid levels in a large series of 700 bone and 700 soft tissue tumors. Out of a multi-institutional database, MRI of 700 consecutive patients with a bone tumor and MRI of 700 consecutive patients with a soft tissue neoplasm were retrospectively reviewed for the presence of fluid-fluid levels. Extension (single, multiple and proportion of the lesion occupied by fluid-fluid levels) and signal characteristics on magnetic resonance imaging of fluid-fluid levels were determined. In all patients, pathologic correlation was available. Of 700 patients with a bone tumor, 19 (10 male and 9 female; mean age, 29 years) presented with a fluid-fluid level (prevalence 2.7%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included aneurysmal bone cyst (ten cases), fibrous dysplasia (two cases), osteoblastoma (one case), simple bone cyst (one case), telangiectatic osteosarcoma (one case), ''brown tumor'' (one case), chondroblastoma (one case) and giant cell tumor (two cases). Of 700 patients with a soft tissue tumor, 20 (9 males and 11 females; mean age, 34 years) presented with a fluid-fluid level (prevalence 2.9%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included cavernous hemangioma (12 cases), synovial sarcoma (3 cases), angiosarcoma (1 case), aneurysmal bone cyst of soft tissue (1 case), myxofibrosarcoma (1 case) and high-grade sarcoma ''not otherwise specified'' (2 cases). In our series, the largest reported in the literature to the best of our knowledge, the presence of fluid-fluid levels is a rare finding with a prevalence of 2.7 and 2.9% in bone and soft tissue tumors, respectively. Fluid-fluid levels remain a non-specific finding and can

Ewing?s Sarcoma of the Adrenal Gland

OpenAIRE

Pal, Dilip Kumar; Chandra, Vipin; Ranjan, Kumar Rajiv; Chakrabortty, Debasis; Banerjee, Manju

2016-01-01

Ewing’s sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing’s sarcoma is very rare. Here we report a case of Ewing’s sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five mon...

Limb sparing surgery and boost with high dose rate interstitial brachytherapy in treatment of soft tissue sarcoma

International Nuclear Information System (INIS)

Koike, P.; Miziara, M.; Soares, C.; Fogaroli, R.; Baraldi, H.; Pellizoni, A.; Borba, G.

2003-01-01

Soft tissue sarcoma, a rare neoplasia with high aggressively, accounts for approximately 0,7% of the malignant tumors and occurs more often with youngest. Because of the potential risk of local recurrence, theoretically surgical resection, encompassing macroscopic tumor with a margin of macroscopically noninvolved tissue is the right way to perform, with a wide 'en bloc' resection, amputation, with bad functional results. The aim of conservative treatment is combined modality therapy as surgical resection and irradiation to obtain a local control rate as high as possible while preserving functional results. A retrospective review of 31 patients treated with high dose rate (HDR) Brachytherapy in the Radiotherapy Department Arnaldo Vieira de Carvalho Cancer Institute. Methods: Between April 1995 and August 1999, 31 patients who underwent a combine therapy; examined the results on multivariate analysis of conservative surgery and brachytherapy follow/or not by external beam radiation (EBRT). The 31 patients treated, 17 ( 54,8%) females and 14(45,2%) males have a median age of 48 years ( range,19 to 77 years). Most of the tumors was located in the lower limb (17/31 - 54,8%) . The other sites were the upper limb (10/31-32,3%), thoracic wall and abdomen (3/31-9,7%).Classification of the tumors, according to the International Union Against Cancer (UICC) staging was T1 5 patients (16%), T2 (24/31-77%). Median size of the tumors was 9,2cm ( ranged, 2,5 to 24cm). Most of the tumors being malignant fibrous histiocytomas (9/31-29%) and the histological grade II (14/31-45%). Twenty-two (71%) patients had intraoperative implants and the insertion of the radioactive source was delayed 24 to 120 hours. Eight patients (25,8%) had postoperative and received HDRB 45 to 60 days after the surgery . Guide needles were placed in the tumor bed, perpendicular to the scar, systematically in a single plane, the implant volume being defined by radiotherapist . A minimum safety margin of 2 cm

Tumour oxygenation assessed by 18F-fluoromisonidazole PET and polarographic needle electrodes in human soft tissue tumours

DEFF Research Database (Denmark)

Bentzen, L.; Keiding, S.; Nordsmark, M.

2003-01-01

Background and purpose: The aim of the study was to identify hypoxia in human soft tissue sarcomas (STS) by PET scanning using the hypoxia marker [F-18]-fluoromisonidazole ([F-18]FMISO) and invasive oxygen sensitive probes (Eppendorf pO(2) Histograph, Germany). Materials and methods: Thirteen pat...

Damage Models for Soft Tissues: A Survey.

Science.gov (United States)

Li, Wenguang

Damage to soft tissues in the human body has been investigated for applications in healthcare, sports, and biomedical engineering. This paper reviews and classifies damage models for soft tissues to summarize achievements, identify new directions, and facilitate finite element analysis. The main ideas of damage modeling methods are illustrated and interpreted. A few key issues related to damage models, such as experimental data curve-fitting, computational effort, connection between damage and fractures/cracks, damage model applications, and fracture/crack extension simulation, are discussed. Several new challenges in the field are identified and outlined. This review can be useful for developing more advanced damage models and extending damage modeling methods to a variety of soft tissues.

Anticipated Intraoperative Electron Beam Boost, External Beam Radiation Therapy, and Limb-Sparing Surgical Resection for Patients with Pediatric Soft-Tissue Sarcomas of the Extremity: A Multicentric Pooled Analysis of Long-Term Outcomes

Energy Technology Data Exchange (ETDEWEB)

Sole, Claudio V., E-mail: cvsole@uc.cl [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); Calvo, Felipe A. [Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); School of Medicine, Complutense University, Madrid (Spain); Polo, Alfredo [Service of Radiation Oncology, Hospital Universitario Ramón y Cajal, Madrid (Spain); Cambeiro, Mauricio [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain); Alvarez, Ana; Gonzalez, Carmen [Service of Radiation Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Gonzalez, Jose [Service of Pediatric Orthopedics and Traumatology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); San Julian, Mikel [Service of Orthopedics and Traumatology, Clínica Universidad de Navarra, Pamplona (Spain); Martinez-Monge, Rafael [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain)

2014-09-01

Purpose: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. Methods and Materials: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. Results: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). Conclusions: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results.

Anticipated Intraoperative Electron Beam Boost, External Beam Radiation Therapy, and Limb-Sparing Surgical Resection for Patients with Pediatric Soft-Tissue Sarcomas of the Extremity: A Multicentric Pooled Analysis of Long-Term Outcomes

International Nuclear Information System (INIS)

Sole, Claudio V.; Calvo, Felipe A.; Polo, Alfredo; Cambeiro, Mauricio; Alvarez, Ana; Gonzalez, Carmen; Gonzalez, Jose; San Julian, Mikel; Martinez-Monge, Rafael

2014-01-01

Purpose: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. Methods and Materials: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. Results: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). Conclusions: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results

Intraneural synovial sarcoma of the median nerve

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Rahul Kasukurthi

2010-06-01

Full Text Available Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathological examination of the surgical speci­men, synovial sarcoma should be kept in mind when dealing with what may seem like a benign nerve tumor.

Pedicled Gastrocnemius Flap: Clinical Application in Limb Sparing Surgical Resection of Sarcoma Around the Knee Region and Popliteal Fossa

International Nuclear Information System (INIS)

EL-SHERBINY, M.

2008-01-01

To highlight on the versatility of superiorly based pedicled gastrocnemius muscle flap in the limb-sparing surgery for bone or soft tissue sarcoma around the knee and popliteal fossa. Patients and Methods: A total of 30 patients with localized bone or soft tissue sarcoma around the knee and popliteal fossa were treated with limb-salvage procedure. The study included 5 cases with bone sarcoma of the distal femur, 15 cases having bone sarcoma of proximal tibia and 10 cases having soft tissue sarcoma around the knee region and popliteal fossa. Routine preoperative staging studies were done for every patient and included local plain radiography, local MRI, isotopic bone scan and CT chest. Local MRA or angiography was done in selected cases. According to the Enneking staging system, 19 patients had stage IIB and 11 had stage IIA. Patients having bone sarcoma of the proximal tibia were subjected to wide resection, endo prosthetic reconstruction and reconstruction of the extensor mechanism by the medial gastrocnemius muscle flap. Patients having bone sarcoma of the distal femur were subjected to wide resection, endo prosthetic reconstruction and coverage of the prosthesis and re balance of the patellar tendon by the medial gas-trocnemius flap. Patients having soft tissue sarcoma were subjected to wide resection and soft tissue coverage with either medial or lateral myocutaneous gastrocnemius flap or muscle flap with grafting. Limb function was evaluated according to MSTS functional scores. Adjuvant chemotherapy or radiotherapy was given according to nationally agreed protocols. Results: There were 18 males and 12 females with a mean age of 29 years at the time of surgery (range 11-44 years). The mean follow-up period was 52 months (range 25-72 months). Resection with a negative bony and soft tissue margins could be achieved in all cases. A total of 30 flaps were used and included medial gastrocnemius muscle flaps in 21 cases (15 cases had proximal tibia endoprothesis, 5

Cytogenetic support for primacy prostatic cancer in a patient presenting with a soft tissue mass in the leg

NARCIS (Netherlands)

Molenaar, WM; Stoepker, MEJ; deRuiter, AJ; Hoekstra, HJ; vandenBerg, E

A 65-year-old man presented with a soft tissue mass in the leg, clinically suspect of a sarcoma. Histologic examination suggested a metastatic adenocarcinoma of the prostate, which could not be confirmed by immunohistologic studies. However, cytogenetic analysis strongly supported this diagnosis. A

Alveolar Soft Part Sarcoma Presenting as Hypervascular Adrenal Metastasis

OpenAIRE

Goroshi, Manjunath; Lila, Anurag R.; Bandgar, Tushar; Shah, Nalini S.

2018-01-01

Hypervascular adrenal masses include pheochromocytoma, metastases caused by clear renal cell carcinoma/hepatocellular carcinoma. Alveolar soft part sarcoma (ASPS) causing hypervascular metastases is not described in the literature. Here, we describe the first case of ASPS presenting as hypervascular metastasis. Our case was a 23-year-old male incidentally detected right adrenal mass during the evaluation of pain in the abdomen. On computed tomography (CT), adrenal mass showed bright enhanceme...

Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy: a subgroup analysis of the European Organisation for Research and Treatment of Cancer 62012 study.

Science.gov (United States)

Young, Robin J; Litière, Saskia; Lia, Michela; Hogendoorn, Pancras C W; Fisher, Cyril; Mechtersheimer, Gunhild; Daugaard, Søren; Sciot, Raf; Collin, Françoise; Messiou, Christina; Grünwald, Viktor; Gronchi, Alessandro; van der Graaf, Winette; Wardelmann, Eva; Judson, Ian

2017-07-01

The European Organization for Research and Treatment of Cancer (EORTC) 62012 study was a Phase III trial of doxorubicin versus doxorubicin-ifosfamide chemotherapy in 455 patients with advanced soft tissue sarcoma (STS). Analysis of the main study showed that combination chemotherapy improved tumor response and progression-free survival, but differences in overall survival (OS) were not statistically significant. We analyzed factors prognostic for tumor response and OS, and assessed histological subgroup and tumor grade as predictive factors to identify patients more likely to benefit from combination chemotherapy. Central pathology review was performed by six reference pathologists. Gender, age, performance status, time from first presentation with sarcoma to starting palliative chemotherapy, tumor grade, histological subgroup, primary tumor site involvement, and sites of metastases were assessed as prognostic factors. Three hundred and ten patients were included in this study. Discordance between local and central pathology opinion of tumor histology and tumor grade was observed in 98 (32%) and 122 (39%) cases, respectively. In multivariate analysis, liposarcoma patients had improved tumor response compared to other histological subgroups, whilst patients with metastases other than lung, liver or bone had a poorer response [odds ratio (OR) 0.42, 95% confidence interval (CI) 0.23-0.78; p = 0.006]. Patients with bone metastases had reduced OS [hazard ratio (HR) 1.56, 95% CI 1.16-2.09; p = 0.003]. By central pathology review, patients with undifferentiated pleomorphic sarcoma (UPS) had improved tumor response and OS with doxorubicin-ifosfamide compared to single-agent doxorubicin (OR 9.90, 95% CI 1.93-50.7 and HR 0.44, 95% CI 0.26-0.79, respectively). Grade III tumors had improved response with combination chemotherapy but there was no interaction between chemotherapy and grade on OS. Prospective central pathology review of tumor histology should be

Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment

Directory of Open Access Journals (Sweden)

Daniel Paulino Santana

2017-07-01

Full Text Available The epithelioid sarcoma (ES is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the “proximal-type” in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with “proximal-type” ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The “proximal-type” ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task. Resumo: O sarcoma epitelióide (SE é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como “tipo proximal”. São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com

Blue Cell Tumour at Unusual Site: Retropritoneal Ewings Sarcoma

OpenAIRE

Javalgi, Anita P; Karigoudar, Mahesh H; Palur, Katyayani

2016-01-01

Ewing’s sarcoma is a highly malignant tumour of osseous or non-osseous origin, tremed as extra-skeletal Ewings sarcoma if arising from soft tissue. It is rare occurrence tumor most commonly occurring in paravertebral area, chest wall, head & neck and retroperitoneum. Reporting an interesting case of retroperitoneal Ewing’s sarcoma in 39 years old female. Patient had complains of abdominal discomfort & vague pain since 2 months, following weakness in lower limb and loss of weight. On detail hi...

A Trp53fl/flPtenfl/fl mouse model of undifferentiated pleomorphic sarcoma mediated by adeno-Cre injection and in vivo bioluminescence imaging.

Directory of Open Access Journals (Sweden)

Marisa R Buchakjian

Full Text Available Genetic mouse models of soft tissue sarcoma provide critical insights into disease pathophysiology, which are oftentimes unable to be extracted from human tumor samples or xenograft models. In this study we describe a mouse model of soft tissue sarcoma mediated by adenoviral-Cre recombinase injection into Trp53fl/fl/Ptenfl/fl lox-stop-lox luciferase mice. Injection of adenovirus expressing Cre recombinase, either subcutaneously or intramuscularly in two experimental groups, results in viral infection and gene recombination with 100% penetrance within the first 24 hours following injection. Luciferase expression measured by real-time bioluminescence imaging increases over time, with an initial robust increase following viral injection, followed by a steady rise over the next several weeks as primary tumors develop and grow. Intramuscular injections were more commonly associated with evidence of systemic viral distribution than subcutaneous injections. All mice developed soft tissue sarcomas at the primary injection site, with histological examination identifying 93% of tumors as invasive pleomorphic sarcomas based on microscopic morphology and immunohistochemical expression of sarcoma markers. A lymphocytic infiltrate was present in 64% of the sarcomas in this immunocompetent model and 71% of tumors expressed PD-L1. This is the first report of a viral-Cre mediated Trp53/Pten mouse model of undifferentiated pleomorphic sarcoma. The bioluminescence imaging feature, along with high penetrance of the model and its immunological characteristics, makes it suited for pre-clinical studies of soft tissue sarcoma.

Imaging Ewing's sarcoma

International Nuclear Information System (INIS)

Henk, C.B.; Grampp, S.; Kainberger, F.; Breitenseher, M.; Imhof, H.; Mostbeck, G.H.

1998-01-01

Ewing's sarcoma is a highly malignant neoplasm of the bone whose origin is still uncertain. A strong relationship exists between Ewing's sarcoma and tumors of neural origin (Ewing family of tumors). Ewing's sarcoma must be distinguished from other round-cell tumors like lymphoma and neuroblastoma and also must be differentiated from osteogenic sarcomas. On plain radiographs, Ewing's sarcoma appears as a lytic or mixed lytic-sclerotic, rarely as predominantly sclerotic lesion with margins Lodwick grade III. It is located primarily in the diaphyseal and metadiaphyseal regions of the long bones of the lower extremities. A large soft tissue tumor is usually present. Magnetic resonance imaging is the imaging modality of choice to evaluate the extent of the primary lesion, to monitor the response to neoadjuvant chemotherapy and to follow up non-resected Ewing's sarcomas. Bone scintigraphy is necessary to detect skeletal metastasis, and 201 thallium scanning has been shown to be sensitive in the monitoring of treatment response. Today, computed tomography is not longer used to image the tumor site; however, spiral CT of the lungs plays a central role as a staging and follow-up tool. (orig.) [de

Quality of Life and Utility in Patients with Metastatic Soft Tissue and Bone Sarcoma: The Sarcoma Treatment and Burden of Illness in North America and Europe (SABINE Study

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Peter Reichardt

2012-01-01

Full Text Available The aim of the study was to assess health-related quality of life (HRQoL among metastatic soft tissue (mSTS or bone sarcoma (mBS patients who had attained a favourable response to chemotherapy. We employed the EORTC QLQ-C30, the 3-item Cancer-Related Symptoms Questionnaire, and the EQ-5D instrument. HRQoL was evaluated overall and by health state in 120 mSTS/mBS patients enrolled in the SABINE study across nine countries in Europe and North America. Utility was estimated from responses to the EQ-5D instrument using UK population-based weights. The mean EQ-5D utility score was 0.69 for the pooled patient sample with little variation across health states. However, patients with progressive disease reported a clinically significant lower utility (0.56. Among disease symptoms, pain and respiratory symptoms are common. This study showed that mSTS/mBS is associated with reduced HRQoL and utility among patients with metastatic disease.

Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

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O'Donnell, P.; Diss, T.C.; Whelan, J.; Flanagan, A.M.

2006-01-01

Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

Radiation planning comparison for superficial tissue avoidance in radiotherapy for soft tissue sarcoma of the lower extremity

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Griffin, Anthony M.; Euler, Colleen I.; Sharpe, Michael B.; Ferguson, Peter C.; Wunder, Jay S.; Bell, Robert S.; Chung, Peter W.M.; Catton, Charles N.; O'Sullivan, Brian

2007-01-01

Purpose: Three types of preoperative radiotherapy (RT) plans for extremity soft tissue sarcoma were compared to determine the amount of dose reduction possible to the planned surgical skin flaps required for tumor resection and wound closure, without compromising target coverage. Methods and Materials: Twenty-four untreated patients with large, deep, lower extremity STS treated with preoperative RT and limb salvage surgery had their original conventional treatment plans re-created. The same clinical target volume was used for all three plans. The future surgical skin flaps were created virtually through contouring by the treating surgeon and regarded as an organ at risk. The original, conformal, and intensity-modulated RT (IMRT) plans were created to deliver 50 Gy in 25 fractions to the clinical target volume. Clinical target volume and organ-at-risk dose-volume histograms were calculated and the plans compared for conformality, target coverage, and dose sparing. Results: The mean dose to the planned skin flaps was 42.62 Gy (range, 30.24-48.65 Gy) for the original plans compared with 40.12 Gy (range, 24.24-47.26 Gy) for the conformal plans and 26.71 Gy (range, 22.31-31.91 Gy) for the IMRT plans (p = 0.0008). An average of 86.4% (range, 53.2-97.4%) of the planned skin flaps received ≥30 Gy in the original plans compared with 83.4% (range, 36.2-96.2%) in the conformal plans and only 34.0% (range, 22.5-53.3%) in the IMRT plans (p = 0.0001). IMRT improved target conformality compared with the original and conformal plans (1.27, 2.34, and 1.76, respectively, p = 0.0001). Conclusion: In a retrospective review, preoperative IMRT substantially lowered the dose to the future surgical skin flaps, sparing a greater percentage of this structure's volume without compromising target (tumor) coverage

[Clinical, pathological and imaging features of primary pelvic Ewing's sarcoma].

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Liu, J; Chen, Y; Ling, X L; Gong, Y; Ding, J P; Zhang, Z K; Wang, Y J

2016-07-19

To explore the clinical, pathological and imaging features of Ewing's sarcoma in pelvis and to improve knowledge and diagnosis of the disease. A retrospective analysis of the clinical, pathological and imaging data of pathologically confirmed 13 cases of Ewing's sarcoma in pelvis was carried out between May 2008 and March 2016 in the Affiliated Hospital of Hangzhou Normal University, the Third Hospital of Hebei Medical University and the Second Hospital of Hebei Medical University. The median age 13 cases of pelvic primary Ewing's sarcoma was 17 years old.The X-ray and CT imagings showed osteolytic and mixed bone destruction, CT showed mixed type in 10 cases, 8 cases of bone tumors as a flocculent, 10 cases of bone expansion failure, 10 cases of periosteal reaction, the layered 5 cases, radial in 5 cases.Thirteen cases showed soft tissue mass, soft tissue mass was equal or slightly lower density.Four cases showed heterogeneous contrast enhancement.The lesions showed low signal in T1WI and mixed high signal in T2WI of magnetic resonance imaging(MRI). The boundary of the lesions were obscure, and 5 cases had patchy necrosis area, and 9 cases had incomplete false capsule, surrounding soft tissue was violated.Four cases showed heterogeneous contrast enhancement after MRI enhancement scan. The age of onset of Ewing's sarcoma of the pelvis is more concentrated in about 15 years.The imaging feaures are mixed bone destruction and more bone is swelling and permeability damage, soft tissue mass is larger, bone tumor is cloudy or acicular, periosteal reaction in a layered and radial, most cases show that the false envelope is not complete.Combined with clinical and imaging examination, the diagnosis of the disease can be made.

Synovial sarcoma: a rare presentation of parapharyngeal mass.

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Shaariyah, Mohd Mokhtar; Mazita, Ami; Masaany, Mansor; Razif, Mohd Yunus; Isa, Mohamed Rose; Asma, Abdullah

2010-06-01

Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.

Efficacy of Sunitinib and Radiotherapy in Genetically Engineered Mouse Model of Soft-Tissue Sarcoma

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Yoon, Sam S.; Stangenberg, Lars; Lee, Yoon-Jin; Rothrock, Courtney; Dreyfuss, Jonathan M.; Baek, Kwan-Hyuck; Waterman, Peter R.; Nielsen, G. Petur; Weissleder, Ralph; Mahmood, Umar; Park, Peter J.; Jacks, Tyler

2009-01-01

Purpose: Sunitinib (SU) is a multitargeted receptor tyrosine kinase inhibitor of the vascular endothelial growth factor and platelet-derived growth factor receptors. The present study examined SU and radiotherapy (RT) in a genetically engineered mouse model of soft tissue sarcoma (STS). Methods and Materials: Primary extremity STSs were generated in genetically engineered mice. The mice were randomized to treatment with SU, RT (10 Gy x 2), or both (SU+RT). Changes in the tumor vasculature before and after treatment were assessed in vivo using fluorescence-mediated tomography. The control and treated tumors were harvested and extensively analyzed. Results: The mean fluorescence in the tumors was not decreased by RT but decreased 38-44% in tumors treated with SU or SU+RT. The control tumors grew to a mean of 1378 mm 3 after 12 days. SU alone or RT alone delayed tumor growth by 56% and 41%, respectively, but maximal growth inhibition (71%) was observed with the combination therapy. SU target effects were confirmed by loss of target receptor phosphorylation and alterations in SU-related gene expression. Cancer cell proliferation was decreased and apoptosis increased in the SU and RT groups, with a synergistic effect on apoptosis observed in the SU+RT group. RT had a minimal effect on the tumor microvessel density and endothelial cell-specific apoptosis, but SU alone or SU+RT decreased the microvessel density by >66% and induced significant endothelial cell apoptosis. Conclusion: SU inhibited STS growth by effects on both cancer cells and tumor vasculature. SU also augmented the efficacy of RT, suggesting that this combination strategy could improve local control of STS.

Cost-Effectiveness Analysis of Preoperative Versus Postoperative Radiation Therapy in Extremity Soft Tissue Sarcoma

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Qu, Xuanlu M.; Louie, Alexander V.; Ashman, Jonathan; Wasif, Nabil

2017-01-01

Purpose: Surgery combined with radiation therapy (RT) is the cornerstone of multidisciplinary management of extremity soft tissue sarcoma (STS). Although RT can be given in either the preoperative or the postoperative setting with similar local recurrence and survival outcomes, the side effect profiles, costs, and long-term functional outcomes are different. The aim of this study was to use decision analysis to determine optimal sequencing of RT with surgery in patients with extremity STS. Methods and Materials: A cost-effectiveness analysis was conducted using a state transition Markov model, with quality-adjusted life years (QALYs) as the primary outcome. A time horizon of 5 years, a cycle length of 3 months, and a willingness-to-pay threshold of $50,000/QALY was used. One-way deterministic sensitivity analyses were performed to determine the thresholds at which each strategy would be preferred. The robustness of the model was assessed by probabilistic sensitivity analysis. Results: Preoperative RT is a more cost-effective strategy ($26,633/3.00 QALYs) than postoperative RT ($28,028/2.86 QALYs) in our base case scenario. Preoperative RT is the superior strategy with either 3-dimensional conformal RT or intensity-modulated RT. One-way sensitivity analyses identified the relative risk of chronic adverse events as having the greatest influence on the preferred timing of RT. The likelihood of preoperative RT being the preferred strategy was 82% on probabilistic sensitivity analysis. Conclusions: Preoperative RT is more cost effective than postoperative RT in the management of resectable extremity STS, primarily because of the higher incidence of chronic adverse events with RT in the postoperative setting.

Cost-Effectiveness Analysis of Preoperative Versus Postoperative Radiation Therapy in Extremity Soft Tissue Sarcoma

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Qu, Xuanlu M. [Department of Oncology, Queen' s University, Kingston, Ontario (Canada); Louie, Alexander V. [Department of Radiation Oncology, London Health Sciences Center, London, Ontario (Canada); Ashman, Jonathan [Department of Radiation Oncology, Mayo Clinic Arizona, Phoenix, Arizona (United States); Wasif, Nabil, E-mail: wasif.nabil@mayo.edu [Department of General Surgery, Mayo Clinic Arizona, Phoenix, Arizona (United States)

2017-02-01

Purpose: Surgery combined with radiation therapy (RT) is the cornerstone of multidisciplinary management of extremity soft tissue sarcoma (STS). Although RT can be given in either the preoperative or the postoperative setting with similar local recurrence and survival outcomes, the side effect profiles, costs, and long-term functional outcomes are different. The aim of this study was to use decision analysis to determine optimal sequencing of RT with surgery in patients with extremity STS. Methods and Materials: A cost-effectiveness analysis was conducted using a state transition Markov model, with quality-adjusted life years (QALYs) as the primary outcome. A time horizon of 5 years, a cycle length of 3 months, and a willingness-to-pay threshold of $50,000/QALY was used. One-way deterministic sensitivity analyses were performed to determine the thresholds at which each strategy would be preferred. The robustness of the model was assessed by probabilistic sensitivity analysis. Results: Preoperative RT is a more cost-effective strategy ($26,633/3.00 QALYs) than postoperative RT ($28,028/2.86 QALYs) in our base case scenario. Preoperative RT is the superior strategy with either 3-dimensional conformal RT or intensity-modulated RT. One-way sensitivity analyses identified the relative risk of chronic adverse events as having the greatest influence on the preferred timing of RT. The likelihood of preoperative RT being the preferred strategy was 82% on probabilistic sensitivity analysis. Conclusions: Preoperative RT is more cost effective than postoperative RT in the management of resectable extremity STS, primarily because of the higher incidence of chronic adverse events with RT in the postoperative setting.

Radiographic appearance of Ewing sarcoma of the hands and feet: report from the Intergroup Ewing Sarcoma Study

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Reinus, W.R.; Gilula, L.A.; Shirley, S.K.; Askin, F.B.; Siegal, G.P.

1985-01-01

Review of current data from the Intergroup Ewing Sarcoma Study (IESS) shows that Ewing sarcoma is rare in bones of the hands and feet. The 12 patients from the IESS protocols with hand or foot Ewing sarcoma are comparable to those already reported in the literature. With the exception of lesions in the calcaneus, the prognosis for disease-free survival is excellent. The radiographic features of hand and foot Ewing sarcoma are generally those of classic Ewing sarcoma: permeation, soft-tissue mass, and often, associated sclerotic reaction. However, with the exception of sclerosis, features suggesting bone reaction and slow tumor growth in these patients were distinctly uncommon compared with Ewing sarcoma in general. Apparently location of the lesion is important, since in the reported cases in the literature and in this series, lesions of the calcaneus fared poorly. The importance of this set of patients therefore relates to awareness and early recognition of an unusual appearance and location of Ewing sarcoma

A novel splice variant of the stem cell marker LGR5/GPR49 is correlated with the risk of tumor-related death in soft-tissue sarcoma patients

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Rot, Swetlana; Taubert, Helge; Bache, Matthias; Greither, Thomas; Würl, Peter; Eckert, Alexander W; Schubert, Johannes; Vordermark, Dirk; Kappler, Matthias

2011-01-01

The human leucine-rich, repeat-containing G protein-coupled receptor (LGR) 5, also called GPR49, is a marker of stem cells in adult intestinal epithelium, stomach and hair follicles. LGR5/GPR49 is overexpressed in tumors of the colon, ovary and liver and in basal cell carcinomas. Moreover, an expression in skeletal muscle tissues was also detected. However, there has been no investigation regarding the expression and function of LGR5/GPR49 in soft-tissue sarcomas (STS) yet. Seventy-seven frozen tumor samples from adult STS patients were studied using quantitative real-time TaqMan™ PCR analysis. The mRNA levels of wild type LGR5/GPR49 and a newly identified splice variant of LGR5/GPR49 lacking exon 5 (that we called GPR49Δ5) were quantified. A low mRNA expression level of GPR49Δ5, but not wild type LGR5/GPR49, was significantly correlated with a poor prognosis for the disease-associated survival of STS patients (RR = 2.6; P = 0.026; multivariate Cox's regression hazard analysis). Furthermore, a low mRNA expression level of GPR49Δ5 was associated with a shorter recurrence-free survival (P = 0.043). However, tumor onset in patients with a lower expression level of GPR49Δ5 mRNA occurred 7.5 years later (P = 0.04) than in patients with a higher tumor level of GPR49Δ5 mRNA. An attenuated mRNA level of the newly identified transcript variant GPR49Δ5 is a negative prognostic marker for disease-associated and recurrence-free survival in STS patients. Additionally, a lower GPR49Δ5 mRNA level is associated with a later age of tumor onset. A putative role of GPR49Δ5 expression in tumorigenesis and tumor progression of soft tissue sarcomas is suggested

Bone and soft tissue tumors of hip and pelvis

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Bloem, Johan L., E-mail: j.l.bloem@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands); Reidsma, Inge I., E-mail: i.i.reidsma@lumc.nl [Leiden University Medical Center, Department of Radiology, PO Box 9600, 2300 RC Leiden (Netherlands)

2012-12-15

Objective is to identify epidemiologic and radiologic criteria allowing specific diagnoses of tumors and tumor-like lesions in the hip region and pelvis, and to optimize pre-operative staging. Patients with pelvic tumors are usually older, and their tumors are larger relative to patients with tumors in extremities. The majority of tumors in the pelvis are malignant (metastases, myeloma, chondrosarcoma, Ewing-, osteo-, and MFH/fibrosarcoma), while those in the proximal femur are in majority benign (fibrous dysplasia, solitary bone cyst, and osteoid osteoma). Soft tissue masses in the thigh in the elderly are typically sarcomas without tumor specific signs. Common tumor-like lesions occurring in the hip and pelvis that can mimic neoplasm are: infections (including tuberculosis), insufficiency/avulsion fractures, cysts, fibrous dysplasia, aneurysmal bone cyst, Langerhans cell histiocytosis, and Paget's disease. Local MR staging is based on the compartmental anatomy. The psoas and gluteal muscles are easily invaded by sarcoma originating in the ileum. The pectineus muscle protects the neurovascular bundle at the level of the hip. The thigh is separated into three compartments, some structures (Sartorius muscle) cross borders between compartments. Immobile joints (SI-joints, osteoarthritic hip) are relatively easily crossed by sarcoma and giant cell tumor.

PET-MR image fusion in soft tissue sarcoma: accuracy, reliability and practicality of interactive point-based and automated mutual information techniques

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Somer, Edward J.R.; Marsden, Paul K.; Benatar, Nigel A.; O'Doherty, Michael J.; Goodey, Joanne; Smith, Michael A.

2003-01-01

The fusion of functional positron emission tomography (PET) data with anatomical magnetic resonance (MR) or computed tomography images, using a variety of interactive and automated techniques, is becoming commonplace, with the technique of choice dependent on the specific application. The case of PET-MR image fusion in soft tissue is complicated by a lack of conspicuous anatomical features and deviation from the rigid-body model. Here we compare a point-based external marker technique with an automated mutual information algorithm and discuss the practicality, reliability and accuracy of each when applied to the study of soft tissue sarcoma. Ten subjects with suspected sarcoma in the knee, thigh, groin, flank or back underwent MR and PET scanning after the attachment of nine external fiducial markers. In the assessment of the point-based technique, three error measures were considered: fiducial localisation error (FLE), fiducial registration error (FRE) and target registration error (TRE). FLE, which represents the accuracy with which the fiducial points can be located, is related to the FRE minimised by the registration algorithm. The registration accuracy is best characterised by the TRE, which is the distance between corresponding points in each image space after registration. In the absence of salient features within the target volume, the TRE can be measured at fiducials excluded from the registration process. To assess the mutual information technique, PET data, acquired after physically removing the markers, were reconstructed in a variety of ways and registered with MR. Having applied the transform suggested by the algorithm to the PET scan acquired before the markers were removed, the residual distance between PET and MR marker-pairs could be measured. The manual point-based technique yielded the best results (RMS TRE =8.3 mm, max =22.4 mm, min =1.7 mm), performing better than the automated algorithm (RMS TRE =20.0 mm, max =30.5 mm, min =7.7 mm) when

Extraosseous Ewing's sarcoma, a case report on a rare diagnosis in ...

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