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Clinical Sarcoma Research

Nicholas Calvert, Jiansha Wu, Sophie Sneddon, Jennifer Woodhouse, Richard Carey-Smith, David Wood, Evan Ingley
Background: Soft tissue and bone sarcoma represent a broad spectrum of different pathology and genetic variance. Current chemotherapy regimens are derived from randomised trials and represent empirical treatment. Chemosensitivity testing and whole exome sequencing (WES) may offer personalized chemotherapy treatment based on genetic mutations. Methods: A pilot, prospective, non-randomised control experimental study was conducted. Twelve patients with metastatic bone or soft tissue sarcoma that had failed first line chemotherapy treatment were enrolled for this study...
2018: Clinical Sarcoma Research
Sergio Sandrucci, Agostino Ponzetti, Claudio Gianotti, Baudolino Mussa, Patrizia Lista, Giovanni Grignani, Marinella Mistrangelo, Oscar Bertetto, Daniela Di Cuonzo, Giovannino Ciccone
Background: Retroperitoneal sarcomas (RPS) should be surgically managed in specialized sarcoma centers. However, it is not clearly demonstrated if clinical outcome is more influenced by Center Case Volume (CCV) or by Surgeon Case Volume (SCV). The aim of this study is to retrospectively explore the relationship between CCV and SCV and the quality of surgery in a wide region of Northern Italy. Methods: We retrospectively collected data about patients M0 surgically treated for RPSs in 22 different hospitals from 2006 to 2011, dividing them in two hospital groups according to sarcoma clinical activity volume (HCV, high case volume or LCV, low case volume hospitals)...
2018: Clinical Sarcoma Research
G L Tiemeier, J M Brown, S E Pratap, C McCarthy, A Kastrenopoulou, K Bradley, S Wilson, Z Orosz, C L M H Gibbons, U Oppermann, N A Athanasou
Background: Liposarcoma is an extremely rare primary bone sarcoma. Case presentation: We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour predominantly isointense on T1- and high-signal on T2-weighted sequences with focal areas of increased T1 signal that suppressed with fat saturation. PET/CT showed marked FDG uptake (SUV = 17...
2018: Clinical Sarcoma Research
Marco Gambarotti, Alberto Righi, Marta Sbaraglia, Giuseppe Bianchi, Piero Picci, Daniel Vanel, Angelo Paolo Dei Tos
Background: Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone. Case presentation: We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery...
2018: Clinical Sarcoma Research
Salvatore Provenzano, Alessandra Raimondi, Rossella M Bertulli, Vittoria Colia, Salvatore L Renne, Paola Collini, Gianpaolo Dagrada, Dario Callegaro, Marco Fiore, Francesca G Greco, Paolo G Casali
Background: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain lineage and intermediate biological potential. It is more common in middle-aged men, usually arising from the deep tissues of the extremities. It is now established that it is a translocation related tumor, most often marked by translocation of PHF1 gene. Surgery is the mainstay of treatment and proves usually curative, although, in rarer cases the disease shows malignant features and tendency to recur both locally and at distant sites...
2017: Clinical Sarcoma Research
Catherine L McCarthy, Christopher L M H Gibbons, Kevin M Bradley, A Bass Hassan, Henk Giele, Nicholas A Athanasou
Background: Treatment of giant cell tumour of bone (GCTB) of the distal radius/ulna poses a surgical challenge, as complex reconstructive surgery may be required. This study evaluates the clinical, radiological and pathological findings in five cases of GCTB of the distal forearm where a 3 month course of denosumab was given prior to surgery. Methods: Patients with biopsy proven distal forearm GCTB, treated for 3 months with denosumab, followed by salvage surgery (curettage and cementation) were included...
2017: Clinical Sarcoma Research
Victor M Villalobos, Stacey DaCosta Byfield, Sameer R Ghate, Oluwakayode Adejoro
Background: Since treatment patterns in metastatic soft tissue sarcoma (mSTS) have not been studied subsequent to US approval of pazopanib in 2012, this study sought to examine mSTS treatment patterns by line of therapy, including regimen and duration of therapy. Methods: This retrospective study employed administrative claims from a large US health plan from 1/2006-9/2015. Adult mSTS patients were required to have an NCCN-recommended therapy and be continuously enrolled in the health plan during the study period...
2017: Clinical Sarcoma Research
E S Hookway, Z Orosz, Y Uchihara, A Grigoriadis, A B Hassan, U Oppermann, N A Athanasou
BACKGROUND: VS38c is a monoclonal antibody that recognises a rough endoplasmic reticulum (rER) intracellular antigen termed cytoskeleton-linking membrane protein 63. rER is typically found in viable tumour cells and is abundant in osteosarcoma cells. The aim of this study was to determine the diagnostic and prognostic utility of VS38c in the histological assessment of osteosarcoma and other bone tumours/tumour-like leisons. METHODS: Immunohistochemical staining with VS38c was carried out on formalin-fixed specimens of osteosarcoma (pre/post-chemotherapy) and a wide range of benign and malignant bone lesions...
2017: Clinical Sarcoma Research
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 ifosfamide as a single agent from November 2001 to December 2016 were retrospectively reviewed...
2017: Clinical Sarcoma Research
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 2000 and January 2014 were identified from an institutional database...
2017: Clinical Sarcoma Research
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 success of immune checkpoint blockade in melanoma has promoted interest in immunotherapy of CCS...
2017: Clinical Sarcoma Research
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 sarcoma (angiosarcoma) in a 59-year-old male. The patient presented with a history of breathlessness on exertion of 2-months duration and was misdiagnosed as massive pulmonary thromboembolism on initial CT imaging...
2017: Clinical Sarcoma Research
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 Atlas (TCGA) and the Cancer Cell Line Encyclopedia (CCLE) projects and determined which sarcoma subtypes and subsets are associated with increased PRAME expression...
2017: Clinical Sarcoma Research
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 presentation and consequently scarcity of data on long-term outcome. CASE PRESENTATION: A 50-year old woman, operated 10 years before for leiomyosarcoma of the nasal pit and with negative 5-years follow-up, was admitted to our ward for impairment of the hepatic function...
2017: Clinical Sarcoma Research
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 very poor and the median overall survival is 25 months. Recurrent disease is mainly treated by chemotherapy...
2017: Clinical Sarcoma Research
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 of 106 conventional high-grade osteosarcomas, across all age groups were re-examined for hotspot mutations in the H3...
2017: Clinical Sarcoma Research
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. METHODS: We therefore evaluated the effect of IDH1 or -2 mutations on histone modifications (H3K4me3, H3K9me3 and H3K27me3), chromatin remodeler ATRX expression, DNA modifications (5-hmC and 5-mC), and TET1 subcellular localization in a genotyped cohort (IDH, succinate dehydrogenase (SDH) and fumarate hydratase (FH)) of enchondromas and central chondrosarcomas (n = 101) using immunohistochemistry...
2017: Clinical Sarcoma Research
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.].
2017: Clinical Sarcoma Research
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, as well as recruitment to clinical trials, and the ongoing accumulation of further knowledge of the disease...
2017: Clinical Sarcoma Research
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 a dismal prognosis. Surgery is the mainstay of treatment...
2017: Clinical Sarcoma Research
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