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Partial response to erlotinib in a patient with imatinib-refractory sacral chordoma Clin. Sarcoma Res. Pub Date : 2020-12-12 Saurav Verma, Surya Prakash Vadlamani, Shamim Ahmed Shamim, Adarsh Barwad, Sameer Rastogi, S. T. Arun Raj
Chordoma is a rare, slow growing and locally aggressive mesenchymal neoplasm with uncommon distant metastases. It is a chemo-resistant disease with surgery and radiotherapy being the mainstay in treatment of localized disease. In advanced disease imatinib has a role. We report a case of metastatic sacral chordoma with symptomatic and radiological response to erlotinib post-progression on imatinib.
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Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response Clin. Sarcoma Res. Pub Date : 2020-12-11 Luca Improta, Sergio Valeri, Rossana Alloni, Chiara Pagnoni, Francesco Mallozzi Santa Maria, Beniamino Brunetti, Carlo Greco, Irene Aprile, Mirella Maselli, Bruno Vincenzi, Alessandro Gronchi
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue tumor that typically affects the lower limbs of men between the ages of 50 and 60. EMC of the shoulder is rare with a high risk of local recurrence and distant metastasis. A planned surgical excision in sarcoma referral centers (SRCs) is mandatory to obtain the best outcome. The role of chemotherapy (CHT) and Radiotherapy (RT) on soft
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The role of prognostic nutritional index in the management of pulmonary sarcomatoid carcinoma Clin. Sarcoma Res. Pub Date : 2020-12-07 Yan Wang, Yu Cao, Junfeng Liu
Pulmonary sarcomatoid carcinoma is characterized by poor survival rates compared with other non-small cell lung cancer. Prognostic nutritional index has significant prognostic value in many malignant tumors. We conducted this retrospective study to investigate the role of prognostic nutritional index in patients with pulmonary sarcomatoid carcinoma and to determine prognostic factors. Of 8176 patients
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Ifosfamide-induced encephalopathy: the EEG with frontal intermittent delta activity, and rapid resolution with methylene blue: A case report Clin. Sarcoma Res. Pub Date : 2020-11-28 Juliette E. Hamilton, Michael Alexander, Fergal C. Kelleher
Encephalopathy is an established side effect of the chemotherapeutic agent, ifosfamide, occurring in 10–30% of cases. The EEG commonly shows non-specific features of encephalopathy, and rarely shows frontal intermittent rhythmic delta activity (FIRDA). This is a case report of a 71 year old woman with pleomorphic sarcoma, who developed ifosfamide-induced encephalopathy with her second dose of ifosfamide
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Simultaneous diagnosis of liver PEComa in a family with known Li–Fraumeni syndrome: a case report Clin. Sarcoma Res. Pub Date : 2020-11-24 María del Mar Galera López, Iván Márquez Rodas, Carolina Agra Pujol, Ángela García Pérez, Enrique Velasco Sánchez, Rosa Álvarez Álvarez
Li–Fraumeni syndrome (LFS) is an autosomal dominant hereditary disease. It is associated with the loss of function of the p53 protein and an increased risk of malignant tumor development at early age. The most frequently detected tumors include breast cancer, sarcomas, leukemia, brain tumors, and adrenocortical carcinomas. While sarcomas account for only 1% of solid tumors, they are more frequently
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Solitary extrapleural fibrous tumor with hepatic bilobar metastases: multimodal approach treatment Clin. Sarcoma Res. Pub Date : 2020-11-18 Maitane I. Orue-Echebarria, Laura Garciafília, Luis Rodriguez-Bachiller, Benjamín Díaz-Zorita, Enrique Velasco, Enrique Ramón, Carolina Agra, Arturo Colón Rodríguez
Solitary fibrous tumor is an unusual fibroblastic mesenchymal neoplasm typically described in the pleura. It may appear anywhere with a varied anatomic distribution and essentially it can develop from any soft tissue or visceral location. Its course is usually indolent and it rarely causes distant metastases, so it has a prolonged survival rate. It sometimes presents itself as a disseminate disease
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Preoperative accelerated radiotherapy combined with chemotherapy in a defined cohort of patients with high risk soft tissue sarcoma: a Scandinavian Sarcoma Group study Clin. Sarcoma Res. Pub Date : 2020-11-17 Kirsten Sundby Hall, Øyvind S. Bruland, Bodil Bjerkehagen, Elisabet Lidbrink, Nina Jebsen, Hans Hagberg, Karin Papworth, Oskar Hagberg, Clement Trovik, Henrik Bauer, Mikael Eriksson
We recently reported outcomes from a Scandinavian Sarcoma Group adjuvant study (SSG XX group A) conducted on localized and operable high risk soft tissue sarcoma (STS) of the extremities and trunk wall. SSG XX, group B, comprised of patients in a defined cohort with locally advanced STS considered at high risk for intralesional surgery. These patients received preoperative accelerated radiotherapy
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Seeking international consensus on approaches to primary tumour treatment in Ewing sarcoma Clin. Sarcoma Res. Pub Date : 2020-11-17 Craig Gerrand, Jessica Bate, Beatrice Seddon, Uta Dirksen, R. Lor Randall, Michiel van de Sande, Paul O’Donnell, John Tuckett, David Peake, Lee Jeys, Asif Saifuddin, Mel Grainger, Jeremy Whelan
The local treatment of Ewing sarcoma of bone involves surgery, radiotherapy or both. The selection of treatment depends on the anatomical extent of the tumour, the effectiveness of the proposed treatment, its morbidity, and the expectation of cure. However, not only are there variations in the approach to local treatment between individual patients, but also between treatment centres and countries
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Glomus tumors with malignant features of the extremities: a case series Clin. Sarcoma Res. Pub Date : 2020-10-30 Taylor R. Wood, Jonathan B. McHugh, Geoffrey W. Siegel
Malignant glomus tumors are exceedingly rare, however they can be locally aggressive and have the potential to metastasize. There is limited information available in the literature regarding treatment and outcomes, therefore we present a case series of 5 patients with glomus tumors with malignant features confined to the extremities that have presented to our tertiary sarcoma center within the last
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When does a new sarcoma exist? Clin. Sarcoma Res. Pub Date : 2020-09-13 Paolo G Casali,Angelo Paolo Dei Tos,Alessandro Gronchi
Pathologists diagnose cancers by looking at how their morphological, histochemical and molecular characteristics cluster in a single case. Almost always this results in a high probability of a known pathologic diagnosis. Occasionally, clustering falls so far from any known paradigm that a new nosological entity is needed. If so, a new diagnostic label is proposed and possibly endorsed by the medical
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Survival of soft tissue sarcoma patients after completing six cycles of first-line anthracycline containing treatment: an EORTC-STBSG database study. Clin. Sarcoma Res. Pub Date : 2020-09-09 Arie Jan Verschoor,Saskia Litière,Sandrine Marréaud,Ian Judson,Maud Toulmonde,Eva Wardelmann,Axel LeCesne,Hans Gelderblom
Doxorubicin based chemotherapy is standard first line treatment for patients with soft tissue sarcoma. Currently several options to improve survival after doxorubicin based chemotherapy are being studied. This study reports on survival after completing 6 cycles of doxorubicin containing first line treatment, which is important when designing studies trying to improve outcomes of first line treatment
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Anthracycline-based and gemcitabine-based chemotherapy in the adjuvant setting for stage I uterine leiomyosarcoma: a retrospective analysis at two reference centers. Clin. Sarcoma Res. Pub Date : 2020-08-28 Giovanni Fucà,Chiara Fabbroni,Rosanna Mancari,Sara Manglaviti,Giorgio Bogani,Elena Fumagalli,Rossella Bertulli,Carlo Morosi,Paola Collini,Francesco Raspagliesi,Nicoletta Colombo,Paolo G Casali,Roberta Sanfilippo
Radically resected early uterine leiomyosarcoma (eULMS) is still marked by a poor prognosis. Adjuvant strategies investigated up to now have not been corroborated by controlled studies. We retrospectively reviewed the clinical outcome of eULMS patients treated with adjuvant anthracycline-based or gemcitabine-based chemotherapy at two Italian reference centers. In this explorative, retrospective, cohort
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Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma. Clin. Sarcoma Res. Pub Date : 2020-08-27 Ghazal Tansir,Sameer Rastogi,Adarsh Barwad,Ekta Dhamija
Mesenchymal chondrosarcoma is an exceedingly rare malignancy, accounting for around 5% of all patients with chondrosarcoma. It is a translocation-related sarcoma that tends to have both local and distant recurrences. Surgery is the mainstay of treatment in localised cases however treatment of advanced cases remains a challenge. The rarity of the disease precludes dedicated clinical trials and hence
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Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary. Clin. Sarcoma Res. Pub Date : 2020-08-25 Eve Merry,Alannah Smrke,Kapil Halai,Gulam Patel,Khin Thway,Robin L Jones,Charlotte Benson
Sarcomas are rare and heterogeneous tumours of mesenchymal origin, with over 100 histological subtypes. Paraneoplastic dermatomyositis has rarely been described in sarcoma. This is the first documented case of paraneoplastic dermatomyositis in a patient with metastatic leiomyosarcoma. A 43-year-old female diagnosed with metastatic leiomyosarcoma of unknown primary presented with a mild rash in sun-exposed
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Successful treatment of lipofibromatosis-like neural tumor of the lumbar spine with an NTRK-fusion inhibitor. Clin. Sarcoma Res. Pub Date : 2020-08-06 Megan Dupuis,Yulei Shen,Christian Curcio,Jeanne M Meis,Wei-Lien Wang,Behrang Amini,Laurence Rhines,Jacquelyn Reuther,Angshumoy Roy,Kevin E Fisher,Anthony P Conley,J Andrew Livingston
Lipofibromatosis-like neural tumors (LPF-NT) are a newly identified class of rare mesenchymal neoplasms. Current standard of care therapy is surgical resection alone; there are no chemotherapies or molecular targeted therapies that have been shown to be effective in patients who are not surgical candidates due to either tumor bulk or location. Most LPF-NT harbor NTRK fusions, although the therapeutic
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Treatment of gastrointestinal tumor (GIST) of the rectum requiring abdominoperineal resection following neoadjuvant imatinib: a cost-effectiveness analysis. Clin. Sarcoma Res. Pub Date : 2020-08-06 Mohamad Farid,Johnny Ong,Claramae Chia,Grace Tan,Melissa Teo,Richard Quek,Jonathan Teh,David Matchar
Neoadjuvant imatinib for gastrointestinal stromal tumors (GIST) of the rectum can reduce, but may not eliminate, risk of surgical morbidity from permanent bowel diversion. We sought to evaluate the cost-effectiveness of alternative strategies in rectal GIST patients requiring abdominoperineal resection following neoadjuvant imatinib. We developed a Markov model using a healthcare payers’ perspective
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A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids. Clin. Sarcoma Res. Pub Date : 2020-07-30 Saurav Verma,Ekta Dhamija,Adarsh Barwad,Venkatesan S Kumar,Sameer Rastogi
Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids. A 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence
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Chemotherapy improves distant control in localized high-grade soft tissue sarcoma of the extremity/trunk. Clin. Sarcoma Res. Pub Date : 2020-07-09 Victoria T Rizk,Arash O Naghavi,Andrew S Brohl,David M Joyce,Odion Binitie,Youngchul Kim,John P Hanna,Jennifer Swank,Ricardo J Gonzalez,Damon R Reed,Mihaela Druta
Soft tissue sarcomas (STS) are rare and heterogeneous tumors making chemotherapy use controversial. Our goal was to identify a subset of patients with primary STS that benefit with the addition of chemotherapy. A retrospective chart review included intermediate to high-grade localized primary STS of the extremity/trunk, and tumor size > 5 cm. The effect of chemotherapy was evaluated for local control
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Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report. Clin. Sarcoma Res. Pub Date : 2020-07-09 Shalabh Arora,Sameer Rastogi,Shamim Ahmed Shamim,Adarsh Barwad,Maansi Sethi
Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas. A 63 year
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Real-world experience with doxorubicin and olaratumab in soft tissue sarcomas in England and Northern Ireland. Clin. Sarcoma Res. Pub Date : 2020-05-06 Spyridon Gennatas,Florence Chamberlain,Thomas Carter,Susanna Slater,Elena Cojocaru,Beth Lambourn,Anna Stansfeld,Radha Todd,Mark Verrill,Nasim Ali,Robin L Jones,Peter Simmonds,Nicola Keay,Heather McCarty,Sandra Strauss,Vassilios Karavasilis,Palma Dileo,Charlotte Benson
A randomised phase II trial demonstrated that the addition of olaratumab to doxorubicin significantly increased overall survival (OS) in patients with advanced soft tissue sarcomas (STS) compared to doxorubicin alone. The recently presented phase III study of doxorubicin and olaratumab in advanced soft tissue sarcoma was discordant with this finding. We performed a retrospective analysis of adult patients
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Evaluation of the accuracy of algorithms to identify soft tissue sarcoma (STS) in administrative claims. Clin. Sarcoma Res. Pub Date : 2020-05-05 Nicole Princic,Donna McMorrow,Philip Chan,Lisa Hess
Lack of using a validated algorithm to select patients is a source of selection bias in oncology studies using administrative claims. The objective of this study to evaluate published algorithms to identify patients with soft tissue sarcoma (STS) in administrative claims and to evaluate new algorithms to improved performance. Two cancer populations including STS cases and non-STS controls were selected
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The anti-neoplastic effect of doxycycline in osteosarcoma as a metalloproteinase (MMP) inhibitor: a systematic review. Clin. Sarcoma Res. Pub Date : 2020-04-30 Argyris C Hadjimichael,Athanasios F Foukas,Olga D Savvidou,Andreas F Mavrogenis,Amanda K Psyrri,Panayiotis J Papagelopoulos
Osteosarcoma is a very aggressive primary bone tumour, affecting mainly young populations. Most cases diagnosed have distant macro- and micro-metastases at the time of diagnosis. Surgical resection with neoadjuvant and adjuvant therapies improves the overall and disease-free survival of patients. Doxycycline, a synthetic tetracycline, has been found to act either as an antibiotic drug or as a chemotherapeutic
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Dedifferentiated soft tissue leiomyosarcoma with heterologous osteosarcoma component: case report and review of the literature Clin. Sarcoma Res. Pub Date : 2020-04-05 Raffaele Gaeta, Davide Matera, Francesco Muratori, Giuliana Roselli, Giacomo Baldi, Domenico Andrea Campanacci, Alessandro Franchi
Soft tissue dedifferentiated leiomyosarcoma with heterologous osteosarcomatous component is an extremely rare entity described in only few cases in the literature. We report the case of a 65-year-old male patient who, after initial inadequate surgery of a tumor of the left forearm, developed local recurrence that was treated with neoadjuvant chemotherapy, surgery and postoperative radiation therapy
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The immune microenvironment of uterine adenosarcomas Clin. Sarcoma Res. Pub Date : 2020-03-28 Ali Mohammed Refaat Ali, Jen-Wei Tsai, Cheuk Hong Leung, Heather Lin, Vinod Ravi, Anthony P. Conley, Alexander J. Lazar, Wei-Lien Wang, Michael J. Nathenson
Uterine adenosarcoma (UA) is an extremely rare sarcoma subtype. There has been limited evaluation of the immune microenvironment in these tumors. The objective of this study is to examine and describe the immune infiltrate and PD-1/PD-L1 expression in UA and to correlate these changes in the tumor micro-environment with the overall survival status or the disease-free survival status (DFSS), respectively
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Accurate 3-gene-signature for early diagnosis of liposarcoma progression Clin. Sarcoma Res. Pub Date : 2020-03-05 Anastassia Serguienko, Peder Braadland, Leonardo A. Meza-Zepeda, Bodil Bjerkehagen, Ola Myklebost
Well- and dedifferentiated liposarcoma (WD/DDLPS) are rare mesenchymal malignant tumors that account for 20% of all sarcomas in adults. The WD form is a low-grade malignancy with a favourable prognosis which may progress to DDLPS, a high-grade aggressive counterpart. WDLPS is referred to as atypical lipomatous tumour (ALT) when localised in extremities, due to its better prognosis. Currently the final
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Cancer/testis antigens expression during cultivation of melanoma and soft tissue sarcoma cells. Clin. Sarcoma Res. Pub Date : 2020-02-04 Anna Danilova,Vsevolod Misyurin,Aleksei Novik,Dmitry Girdyuk,Natalia Avdonkina,Tatiana Nekhaeva,Natalia Emelyanova,Nino Pipia,Andrey Misyurin,Irina Baldueva
Autologous dendritic cells (DC) loaded with tumor-associated antigens (TAAs) are a promising approach for anticancer immunotherapy. Polyantigen lysates appear to be an excellent source of TAAs for loading onto the patient’s dendritic cells. Cancer/testis antigens (CTA) are expressed by a wide range of tumors, but are minimally expressed on normal tissues, and could serve as a universal target for immunotherapy
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Use of a simple form to facilitate communication on long-term consequences of treatment in sarcoma survivors. Clin. Sarcoma Res. Pub Date : 2020-01-16 Ivar Hompland,Lena Fauske,Geir Fagerjord Lorem,Øyvind S Bruland
To report on our experience using a simple optional form to facilitate communication on late effects between the patients and the oncologists during outpatient follow-up and to detail on the spectrum of challenges reported by sarcoma survivors. The form was presented for the patients to complete before their consultation and covered topics related to late effects and unmet needs that the patient wished
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Toxicity management of regorafenib in patients with gastro-intestinal stromal tumour (GIST) in a tertiary cancer centre Clin. Sarcoma Res. Pub Date : 2020-01-04 Florence Chamberlain, Sheima Farag, Constance Williams-Sharkey, Cecilia Collingwood, Lucia Chen, Sonia Mansukhani, Bodil Engelmann, Omar Al-Muderis, Dharmisha Chauhan, Khin Thway, Cyril Fisher, Robin L. Jones, Spyridon Gennatas, Charlotte Benson
Regorafenib is a multi-kinase inhibitor approved as third line treatment for metastatic GIST. Dose limiting toxicities are frequently seen and many patients require dose reductions. This study aimed to evaluate regorafenib toxicities and their management in a real-world GIST population. Retrospective review of a prospectively maintained database identified 50 patients with GIST treated with regorafenib
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Activity of anthracycline- and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma. Clin. Sarcoma Res. Pub Date : 2017-08-31 Vittoria Colia,Marco Fiore,Salvatore Provenzano,Elena Fumagalli,Rossella Bertulli,Carlo Morosi,Angelo P Dei Tos,Marta Barisella,Alessandro Gronchi,Paolo G Casali,Roberta Sanfilippo
BACKGROUND We report on the activity of anthracycline-based and high-dose prolonged-infusion ifosfamide chemotherapy in a retrospective series of patients affected by advanced myxofibrosarcoma treated at Istituto Nazionale Tumori in Milan, Italy, and within the Italian Rare Cancer Network (RTR). METHODS Advanced myxofibrosarcoma patients treated with anthracycline + ifosfamide and high-dose prolonged-infusion
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Radiation induced angiosarcoma of the breast: outcomes from a retrospective case series. Clin. Sarcoma Res. Pub Date : 2017-08-11 R B Cohen-Hallaleh,H G Smith,R C Smith,G F Stamp,O Al-Muderis,K Thway,A Miah,K Khabra,I Judson,R Jones,C Benson,A J Hayes
BACKGROUND Radiation induced angiosarcoma (RIAS) of the breast is a rare and aggressive complication of radiotherapy. Due to the rarity of this disease, much of the evidence for its management is based on case reports or small retrospective series. We sought to describe the management and outcomes of RIAS in a large single-institution series. METHODS All patients diagnosed with RIAS between January
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Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy. Clin. Sarcoma Res. Pub Date : 2017-07-21 Samuel Marcrom,Jennifer F De Los Santos,Robert M Conry
BACKGROUND Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of the melanocyte master transcription factor and drives the same down-stream pathways in CCS and melanoma leading to significant clinical parallels between these malignancies. Striking
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Primary pulmonary artery sarcoma masquerading as pulmonary thromboembolism: a rare diagnosis unveiled. Clin. Sarcoma Res. Pub Date : 2017-07-07 Abhishek Mahajan,Bharat Rekhi,Siddhartha Laskar,Jyoti Bajpai,Lekshmy Jayasree,Meenakshi H Thakur
BACKGROUND Primary pulmonary artery sarcomas are rare malignant vascular tumors and carry a very poor prognosis. Due to overlapping clinical and radiological features, the differentiation between pulmonary artery thromboembolism and pulmonary artery sarcoma can be challenging. CASE PRESENTATION We herein present clinical, radiological and pathological features of primary pulmonary artery high grade
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Overexpressed PRAME is a potential immunotherapy target in sarcoma subtypes. Clin. Sarcoma Res. Pub Date : 2017-06-21 Jason Roszik,Wei-Lien Wang,John A Livingston,Christina L Roland,Vinod Ravi,Cassian Yee,Patrick Hwu,Andrew Futreal,Alexander J Lazar,Shreyaskumar R Patel,Anthony P Conley
BACKGROUND PRAME (preferentially expressed antigen in melanoma), a member of the cancer-testis antigen family, has been shown to have increased expression in solid tumors, including sarcoma, and PRAME-specific therapies are currently in development for other cancers such as melanoma. METHODS To map the landscape of PRAME expression in sarcoma, we used publicly available data from The Cancer Genome
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Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease. Clin. Sarcoma Res. Pub Date : 2017-06-09 Enrico Pinotti,Marta Sandini,Simone Famularo,Marta Jaconi,Fabrizio Romano,Luca Nespoli,Luca Gianotti
BACKGROUND Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recommendations are specifically endorsed for leiomyosarcoma of the head and neck, due to the rarity of its
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Antiangiogenic effects in patients with progressive desmoplastic small round cell tumor: data from the French national registry dedicated to the use of off-labeled targeted therapy in sarcoma (OUTC's). Clin. Sarcoma Res. Pub Date : 2017-05-12 Sarah Bétrian,Christophe Bergeron,Jean-Yves Blay,Emmanuelle Bompas,Philippe A Cassier,Laure Chevallier,Jérome Fayette,Magali Girodet,Cécile Guillemet,Axel Le Cesne,Perrine Marec-Berard,Isabelle Ray-Coquard,Christine Chevreau
BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a very rare mesenchymal tumor that mainly affects teenagers and young adults with a mean age at diagnosis around 20-25 years. Although initial management still needs standardization, many centers will use multimodal treatment including intensive chemotherapy, extensive surgical resection followed by radiotherapy. Despite this, prognosis remains
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Histone 3.3 hotspot mutations in conventional osteosarcomas: a comprehensive clinical and molecular characterization of six H3F3A mutated cases. Clin. Sarcoma Res. Pub Date : 2017-05-10 Christian Koelsche,Daniel Schrimpf,Lars Tharun,Eva Roth,Dominik Sturm,David T W Jones,Eva-Kristin Renker,Martin Sill,Annika Baude,Felix Sahm,David Capper,Melanie Bewerunge-Hudler,Wolfgang Hartmann,Andreas E Kulozik,Iver Petersen,Uta Flucke,Hendrik W B Schreuder,Reinhard Büttner,Marc-André Weber,Peter Schirmacher,Christoph Plass,Stefan M Pfister,Andreas von Deimling,Gunhild Mechtersheimer
BACKGROUND Histone 3.3 (H3.3) hotspot mutations in bone tumors occur in the vast majority of giant cell tumors of bone (GCTBs; 96%), chondroblastomas (95%) and in a few cases of osteosarcomas. However, clinical presentation, histopathological features, and additional molecular characteristics of H3.3 mutant osteosarcomas are largely unknown. METHODS In this multicentre, retrospective study, a total
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IDH1 or -2 mutations do not predict outcome and do not cause loss of 5-hydroxymethylcytosine or altered histone modifications in central chondrosarcomas. Clin. Sarcoma Res. Pub Date : 2017-05-10 Arjen H G Cleven,Johnny Suijker,Georgios Agrogiannis,Inge H Briaire-de Bruijn,Norma Frizzell,Attje S Hoekstra,Pauline M Wijers-Koster,Anne-Marie Cleton-Jansen,Judith V M G Bovée
BACKGROUND Mutations in isocitrate dehydrogenase (IDH)1 or -2 are found in ~50% of conventional central chondrosarcomas and in up to 87% of their assumed benign precursors enchondromas. The mutant enzyme acquires the activity to convert α-ketoglutarate into the oncometabolite d-2-hydroxyglutarate (d-2-HG), which competitively inhibits α-ketoglutarate dependent enzymes such as histone- and DNA demethylases
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Erratum to: Osteosarcoma follow-up: chest X-ray or computed tomography? Clin. Sarcoma Res. Pub Date : 2017-05-06 Anna Paioli,Michele Rocca,Luca Cevolani,Eugenio Rimondi,Daniel Vanel,Emanuela Palmerini,Marilena Cesari,Alessandra Longhi,Massimo Eraldo Abate,Emanuela Marchesi,Piero Picci,Stefano Ferrari
[This corrects the article DOI: 10.1186/s13569-017-0067-5.].
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UK clinical practice guidelines for the management of gastrointestinal stromal tumours (GIST). Clin. Sarcoma Res. Pub Date : 2017-05-04 Ian Judson,Ramesh Bulusu,Beatrice Seddon,Adam Dangoor,Newton Wong,Satvinder Mudan
BACKGROUND Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues. Gastrointestinal stromal tumour (GIST) is the commonest STS and arises within the wall of the gastrointestinal (GI) tract. While most GISTs occur in the stomach they do occur in all parts of the GI tract. As with other STS, it is important that GISTs are managed by expert teams, to ensure consistent and optimal treatment
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Two years survival of primary cardiac leiomyosarcoma managed by surgical and adjuvant therapy. Clin. Sarcoma Res. Pub Date : 2017-03-14 K Behi,M Ayadi,E Mezni,K Meddeb,A Mokrani,Y Yahyaoui,F Ksontini,H Rais,N Chrait,A Mezlini
BACKGROUND Cardiac tumors are a very rare entity. Leiomyosarcoma represents less than 1% of cases. CASE PRESENTATION a 51-year-old woman diagnosed with primary left atrium leiomyosarcoma. She was treated by optimal surgery and adjuvant chemotherapy. She is still alive after a follow-up of 24 months without evidence of local or distant recurrence. CONCLUSIONS Cardiac leiomyosarcoma is a rare tumor with
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How a Clinical Trial Unit can improve independent clinical research in rare tumors: the Italian Sarcoma Group experience. Clin. Sarcoma Res. Pub Date : 2017-03-07 Emanuela Marchesi,Celeste Cagnazzo,Irene Quattrini,Martina Piccinni Leopardi,Chiara Villa,Giovanni Grignani,Lorenzo D'Ambrosio,Silvia Stacchiotti,Paolo Giovanni Casali,Piero Picci
BACKGROUND The Italian Sarcoma Group (ISG) is a nonprofit group of professionals established in 1997 aimed to improve the quality of care and promote the independent research in sarcomas. The increased regulatory requirements, the chance to increase the number of trials with other cooperative groups and an interest from pharmaceutical companies in supporting independent research, generated the need
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Osteosarcoma follow-up: chest X-ray or computed tomography? Clin. Sarcoma Res. Pub Date : 2017-02-24 Anna Paioli,Michele Rocca,Luca Cevolani,Eugenio Rimondi,Daniel Vanel,Emanuela Palmerini,Marilena Cesari,Alessandra Longhi,Abate Massimo Eraldo,Emanuela Marchesi,Piero Picci,Stefano Ferrari
BACKGROUND In patients with relapsed osteosarcoma, the surgical excision of all metastases, defined as second complete remission (CR-2), is the factor that mainly influences post-relapse survival (PRS). Currently a validated follow-up policy for osteosarcoma is not available, both chest X-ray and computed tomography (CT) are suggested for lung surveillance. The purpose of this study is to evaluate
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Central venous access related adverse events after trabectedin infusions in soft tissue sarcoma patients; experience and management in a nationwide multi-center study. Clin. Sarcoma Res. Pub Date : 2017-02-07 Michiel C Verboom,Jan Ouwerkerk,Neeltje Steeghs,Jacob Lutjeboer,J Martijn Kerst,Winette T A van der Graaf,Anna K L Reyners,Stefan Sleijfer,Hans Gelderblom
BACKGROUND Trabectedin has shown efficacy against soft tissue sarcomas (STS) and has manageable toxicity. Trabectedin is administered through central venous access devices (VAD), such as subcutaneous ports with tunneled catheters, Hickman catheters and PICC lines. Venous access related adverse events are common, but have not yet been reported in detail. METHODS A retrospective analysis of patient files
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Radiotherapy in the management of gist: state of the art and new potential scenarios. Clin. Sarcoma Res. Pub Date : 2017-01-13 L Gatto,M Nannini,M Saponara,V Di Scioscio,G Beltramo,G P Frezza,G Ercolani,A D Pinna,A Astolfi,M Urbini,G Brandi,G Biasco,M A Pantaleo
BACKGROUND Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. The main treatment for localized gastrointestinal stromal tumors is surgical resection. Unresectable or advanced GIST are poorly responsive to conventional cytotoxic chemotherapy but the introduction of tyrosine kinase inhibitors (TKIs) marked a revolutionary step in the treatment
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Metastatic meningioma presenting as a malignant soft tissue tumour. Clin. Sarcoma Res. Pub Date : 2017-01-04 Catherine McCarthy,Monika Hofer,Marianna Vlychou,Robar Khundkar,Paul Critchley,Simon Cudlip,Olaf Ansorge,Nick A Athanasou
BACKGROUND Extracranial metastasis of malignant meningioma to soft tissues is extremely rare and its clinical, radiological and pathological features are not well-characterised. CASE PRESENTATION We report a case of a 58 year old man who presented with a mobile mass within the left trapezius muscle. The patient had previously undergone surgery for a right frontal lobe high grade anaplastic meningioma
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Response to anti-PD1 therapy with nivolumab in metastatic sarcomas. Clin. Sarcoma Res. Pub Date : 2017-01-04 L Paoluzzi,A Cacavio,M Ghesani,A Karambelkar,A Rapkiewicz,J Weber,G Rosen
BACKGROUND Manipulation of immune checkpoints such as CTLA4 or PD-1 with targeted antibodies has recently emerged as an effective anticancer strategy in multiple malignancies. Sarcomas are a heterogeneous group of diseases in need of more effective treatments. Different subtypes of soft tissue and bone sarcomas have been shown to express PD-1 ligand. METHODS We retrospectively analyzed a cohort of
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Clinical implications of repeated drug monitoring of imatinib in patients with metastatic gastrointestinal stromal tumour. Clin. Sarcoma Res. Pub Date : 2016-12-22 Ivar Hompland,Øyvind Sverre Bruland,Kumari Ubhayasekhera,Jonas Bergquist,Kjetil Boye
BACKGROUND Imatinib mesylate (IM) is the preferred treatment for the majority of patients with metastatic gastrointestinal stromal tumour (GIST). Low trough IM concentration (Cmin) values have been associated with poor clinical outcomes in GIST patients. However, there are few studies of repeated measurements of IM levels, and therapeutic drug monitoring is not yet a part of routine clinical practice
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Pazopanib: a novel treatment option for aggressive fibromatosis. Clin. Sarcoma Res. Pub Date : 2016-12-14 Gulcan Bulut,Anil Ozluk,Atike Pınar Erdogan,Ruchan Uslu,Nevra Elmas,Burcak Karaca
BACKGROUND Aggressive fibromatosis (AF), also known as desmoid tumor, is an uncommon soft tissue neoplasm. AF does not metastasize, but it is locally invasive and its propensity for recurrence after conservative resection is well documented. No effective cytotoxic treatment has been reported, hence there is a need for novel treatment strategies. CASE PRESENTATION We present the case of an AF successfully
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UK guidelines for the management of soft tissue sarcomas. Clin. Sarcoma Res. Pub Date : 2016-11-15 Adam Dangoor,Beatrice Seddon,Craig Gerrand,Robert Grimer,Jeremy Whelan,Ian Judson
Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal
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Efficacy of pazopanib and sunitinib in advanced axial chordoma: a single reference centre case series. Clin. Sarcoma Res. Pub Date : 2016-11-09 Astrid Lipplaa,Sander Dijkstra,Hans Gelderblom
BACKGROUND Chordomas are rare malignant tumours of the axial skeleton and skull base supposed to arise from cellular remnants of the notochord. These tumours have the potential to metastasize (30-40 %), usually in the later course of the disease. However, the greatest morbidity is usually a result of loco-regional recurrence with infiltration and destruction of surrounding bone and soft tissue. Patients
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Outcome and the effect of age and socioeconomic status in 1318 patients with synovial sarcoma in the English National Cancer Registry: 1985-2009. Clin. Sarcoma Res. Pub Date : 2016-10-26 Bernadette Brennan,Charles Stiller,Robert Grimer,Nicola Dennis,John Broggio,Matthew Francis
BACKGROUND The role of age as a prognostic factor has been examined in single institutional studies and in larger data sets from the SEER database, showing a survival advantage for younger versus adult patients with synovial sarcoma (SS). To further assess the role of age, socioeconomic status and other prognostic factors on outcome for SS, we analysed a contemporary all-age population-based cohort
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High nuclear expression of proteasome activator complex subunit 1 predicts poor survival in soft tissue leiomyosarcomas. Clin. Sarcoma Res. Pub Date : 2016-10-14 Sha Lou,Arjen H G Cleven,Benjamin Balluff,Marieke de Graaff,Marie Kostine,Inge Briaire-de Bruijn,Liam A McDonnell,Judith V M G Bovée
BACKGROUND Previous studies on high grade sarcomas using mass spectrometry imaging showed proteasome activator complex subunit 1 (PSME1) to be associated with poor survival in soft tissue sarcoma patients. PSME1 is involved in immunoproteasome assembly for generating tumor antigens presented by MHC class I molecules. In this study, we aimed to validate PSME1 as a prognostic biomarker in an independent
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Current status and unanswered questions on the use of Denosumab in giant cell tumor of bone. Clin. Sarcoma Res. Pub Date : 2016-09-22 Czar Louie Gaston,Robert J Grimer,Michael Parry,Silvia Stacchiotti,Angelo Paolo Dei Tos,Hans Gelderblom,Stefano Ferrari,Giacomo G Baldi,Robin L Jones,Sant Chawla,Paolo Casali,Axel LeCesne,Jean-Yves Blay,Sander P D Dijkstra,David M Thomas,Piotr Rutkowski
Denosumab is a monoclonal antibody to RANK ligand approved for use in giant cell tumour (GCT) of bone. Due to its efficacy, Denosumab is recommended as the first option in inoperable or metastatic GCT. Denosumab has also been used pre-operatively to downstage tumours with large soft tissue extension to allow for less morbid surgery. The role of Denosumab for conventional limb GCT of bone is yet to
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Solitary fibrous tumour presenting with a single bone metastasis: report of six cases and literature review. Clin. Sarcoma Res. Pub Date : 2016-09-03 Vittoria Colia,Salvatore Provenzano,Carlo Morosi,Paola Collini,Salvatore Lorenzo Renne,Paolo G Dagrada,Claudia Sangalli,Angelo Paolo Dei Tos,Andrea Marrari,Paolo G Casali,Silvia Stacchiotti
BACKGROUND Solitary fibrous tumour (SFT) is a rare soft tissue sarcoma with a low metastatic potential. A higher metastatic rate is observed in the high-grade/dedifferentiated variant. The most common expected site of distant spread are the lungs and the liver. Bone involvement is generally viewed as a late stage of disease spread. We report on a retrospective series of SFT patients relapsing with
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Primary intraosseous meningioma: an osteosclerotic bone tumour mimicking malignancy. Clin. Sarcoma Res. Pub Date : 2016-08-16 M Vlychou,Y Inagaki,R Stacey,N A Athanasou
BACKGROUND Sclerotic tumours of the calvarial bones are rare and may be due to primary and secondary bone tumours as well as extradural tumours of meningeal origin. CASE PRESENTATION We report a case of primary intraosseous meningioma (PIM) which arose in the frontal bone of a 63 year old woman who complained of progressive pain and thickening of the right skull. Radiology showed a large osteosclerotic
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Dendritic and mast cell involvement in the inflammatory response to primary malignant bone tumours. Clin. Sarcoma Res. Pub Date : 2016-08-01 Y Inagaki,E Hookway,K A Williams,A B Hassan,U Oppermann,Y Tanaka,E Soilleux,N A Athanasou
BACKGROUND A chronic inflammatory cell infiltrate is commonly seen in response to primary malignant tumours of bone. This is known to contain tumour-associated macrophages (TAMs) and lymphocytes; dendritic cells (DCs) and mast cells (MCs) have also been identified but whether these and other inflammatory cells are seen commonly in specific types of bone sarcoma is uncertain. METHODS In this study we
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Predictors of survival in patients with sarcoma admitted to the intensive care unit. Clin. Sarcoma Res. Pub Date : 2016-07-21 Rohan Gupta,Neda Heshami,Chouhan Jay,Naveen Ramesh,Juhee Song,Xiudong Lei,Erfe Jean Rose,Kristen Carter,Dejka M Araujo,Robert S Benjamin,Shreyaskumar Patel,Joseph L Nates,Vinod Ravi
BACKGROUND Advances in treatment of sarcoma patients has prolonged survival but has led to increased disease- or treatment-related complications resulting in greater number of admissions to the intensive care unit (ICU). Survival and long-term outcome information about such critically ill patients with sarcoma is unknown. METHODS The primary objective of the study was to determine the ICU and post-ICU
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Patterns of care for patients with advanced soft tissue sarcoma: experience from Australian sarcoma services. Clin. Sarcoma Res. Pub Date : 2016-07-13 Susie Bae,Philip Crowe,Raghu Gowda,Warren Joubert,Richard Carey-Smith,Paul Stalley,Jayesh Desai
BACKGROUND There is a paucity of data on the current management of patients with advanced soft tissue sarcoma (STS) in the Australian health care setting. This study utilised the Australian sarcoma database to evaluate the patterns of care delivered to patients with advanced STS at Australian sarcoma services. METHODS Prospectively collected data from six sarcoma centres in Australia were sourced to
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Phosphorylated-insulin growth factor I receptor (p-IGF1R) and metalloproteinase-3 (MMP3) expression in advanced gastrointestinal stromal tumors (GIST). A GEIS 19 study. Clin. Sarcoma Res. Pub Date : 2016-07-01 Joan Maurel,Antonio López-Pousa,Silvia Calabuig,Silvia Bagué,Xavier Garcia Del Muro,Xavier Sanjuan,Jordi Rubió-Casadevall,Miriam Cuatrecasas,Javier Martinez-Trufero,Carlos Horndler,Joaquin Fra,Claudia Valverde,Andrés Redondo,Andrés Poveda,Isabel Sevilla,Nuria Lainez,Michele Rubini,Xabier García-Albéniz,Javier Martín-Broto,Enrique de Alava
BACKGROUND Most GISTs have mutations in KIT or PDGFRA. Patients with advanced GIST with KIT exon 9, PDGFRA mutation or WT for KIT and PDGFRA have a worse progression-free survival (PFS) compared to patients with KIT exon 11 mutated tumors. We evaluated the immunohistochemical (IHC) expression of p-IGF1R (Y1316) and MMP3 as predictors of PFS or overall survival (OS). METHODS Ninety-two advanced GIST
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The ENCCA-WP7/EuroSarc/EEC/PROVABES/EURAMOS 3rd European Bone Sarcoma Networking Meeting/Joint Workshop of EU Bone Sarcoma Translational Research Networks; Vienna, Austria, September 24-25, 2015. Workshop Report. Clin. Sarcoma Res. Pub Date : 2016-06-18 Leo Kager,Jeremy Whelan,Uta Dirksen,Bass Hassan,Jakob Anninga,Lindsey Bennister,Judith V M G Bovée,Bernadette Brennan,Javier M Broto,Laurence Brugières,Anne-Marie Cleton-Jansen,Christopher Copland,Aurélie Dutour,Franca Fagioli,Stefano Ferrari,Marta Fiocco,Emmy Fleuren,Nathalie Gaspar,Hans Gelderblom,Craig Gerrand,Joachim Gerß,Ornella Gonzato,Winette van der Graaf,Stefanie Hecker-Nolting,David Herrero-Martín
This report summarizes the results of the 3rd Joint ENCCA-WP7, EuroSarc, EEC, PROVABES, and EURAMOS European Bone Sarcoma Network Meeting, which was held at the Children's Cancer Research Institute in Vienna, Austria on September 24-25, 2015. The joint bone sarcoma network meetings bring together European bone sarcoma researchers to present and discuss current knowledge on bone sarcoma biology, genetics