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undifferentiated pleomorphic sarcoma

P Sargos, T Charleux, R L Haas, A Michot, C Llacer, L Moureau-Zabotto, G Vogin, C Le Péchoux, C Verry, A Ducassou, M Delannes, A Mervoyer, N Wiazzane, J Thariat, M P Sunyach, M Benchalal, J D Laredo, M Kind, P Gillon, G Kantor
PURPOSE: The purpose of this study was to evaluate, during a national workshop, the inter-observer variability in target volume delineation for primary extremity soft tissue sarcoma radiation therapy. METHODS AND MATERIALS: Six expert sarcoma radiation oncologists (members of French Sarcoma Group) received two extremity soft tissue sarcoma radiation therapy cases 1: one preoperative and one postoperative. They were distributed with instructions for contouring gross tumour volume or reconstructed gross tumour volume, clinical target volume and to propose a planning target volume...
March 12, 2018: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
Kelly J Butnor, Elizabeth N Pavlisko, Thomas A Sporn, Victor L Roggli
CONTEXT: - Malignant mesothelioma (MM) is a component of the BAP1 tumor predisposition syndrome. Other than in BAP1 familial studies, nonmesothelial neoplasms in individuals with MM has not been comprehensively assessed. OBJECTIVE: - To assess the spectrum and prevalence of nonmesothelial neoplasms in individuals with MM. DESIGN: - Individuals with MM and second neoplasms were identified from a database of 3900 MM cases. The expected prevalence of each type of neoplasm was calculated and compared with the actual prevalence in the study population using available Surveillance, Epidemiology, and End Results data and other published data...
March 12, 2018: Archives of Pathology & Laboratory Medicine
Shuai Ye, Matthew A Lawlor, Adrian Rivera-Reyes, Shaun Egolf, Susan Chor, Koreana Pak, Gabrielle E Ciotti, Avery C Lee, Gloria Marino, Jennifer Shah, David Niedzwicki, Kristy Weber, Paul M C Park, Md Zahidul Alam, Alison Grazioli, Malay Haldar, Mousheng Xu, Jennifer A Perry, Jun Qi, T S Karin Eisinger-Mathason
To date, no consistent oncogenic driver mutations have been identified in most adult soft tissue sarcomas; these tumors are thus generally insensitive to existing targeted therapies. Here we investigated alternate mechanisms underlying sarcomagenesis to identify potential therapeutic interventions. Undifferentiated pleomorphic sarcoma (UPS) is an aggressive tumor frequently found in skeletal muscle where deregulation of the Hippo pathway and aberrant stabilization of its transcriptional effector yes-associated protein 1 (YAP1) increases proliferation and tumorigenesis...
February 28, 2018: Cancer Research
Kentaro Igarashi, Kei Kawaguchi, Tasuku Kiyuna, Kentaro Miyake, Masuyo Miyake, Arun S Singh, Mark A Eckardt, Scott D Nelson, Tara A Russell, Sarah M Dry, Yunfeng Li, Norio Yamamoto, Katsuhiro Hayashi, Hiroaki Kimura, Shinji Miwa, Hiroyuki Tsuchiya, Shree Ram Singh, Fritz C Eilber, Robert M Hoffman
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...
February 23, 2018: Biochemical and Biophysical Research Communications
R Badanale, B Rekhi, N A Jambhekar, A Gulia, J Bajpai, S Laskar, N Khanna, G Chinnaswamy, A Puri
AIMS: To review various pathologic parameters in diagnosed cases of trunk and extremity-based soft tissue tumors (STTs), in order to identify concordance rate between initial biopsy and resection specimen and discrepancies between initial and review diagnosis, by a specialist pathologist. MATERIALS AND METHODS: Over a 2-year-period, 400 retrospectively diagnosed STTs (553 specimens) including referral and "in-house" cases were studied. The reviewing specialist pathologist was blinded to the initial diagnoses...
April 2017: Indian Journal of Cancer
Bin Li, Zhiping Deng, Xiaohui Niu
PURPOSE: Resection length should be designed before limb salvage surgery of bone tumours. The aim of this study was to evaluate the accuracy of free hand resections. METHODS: Two hundred forty-eight cases were enrolled, including 173 osteosarcomas, 24 giant cell tumours, 16 chondrosarcomas, seven spindle cell sarcomas, 14 bone metastases, three undifferentiated pleomorphic sarcomas, three Ewing sarcomas, two angiosarcomas, and six other bone and soft tissue tumours...
February 21, 2018: International Orthopaedics
Xiaona Li, Zekun Zhang, Mahrukh Latif, Wei Chen, Jianling Cui, Zhigang Peng
RATIONALE: Undifferentiated high-grade pleomorphic sarcoma (UPS), originated from bone, is a rare tumor, accounting for 2% to 5% of all primary maligment bone neoplasms. Skip lesion can be found in undifferentiated high-grade pleomorphic sarcoma of bone (UPS-B). However, the direct invasion across the articular synovium to bone has not been reported previously. PATIENT CONCERNS: We report an unusual case of a 65-year-old man complained of a year history of pain, swelling, and limitation of activity in the left knee joint...
February 2018: Medicine (Baltimore)
Varun Monga, Umang Swami, Munir Tanas, Aaron Bossler, Sarah L Mott, Brian J Smith, Mohammed Milhem
Epigenetic events and genetic alterations under the control of the tumor microenvironment potentially mediate tumor induced angiogenesis involved in soft tissue sarcoma (STS) metastasis. Addition of antiangiogenic agent, such as bevacizumab, to standard chemotherapy in treatment of sarcoma has been studied in clinical trials, but most of the findings have not supported its use. We hypothesized the existence of an epigenetically mediated "angiogenic switch", and the tumor microenvironment, prevents bevacizumab from truly blocking angiogenesis...
February 17, 2018: Cancers
Qiang Chen, Qing Huang, Jia-Xin Yan, Chao Li, Jin-Yi Lang
RATIONALE: Primary undifferentiated pleomorphic sarcoma is extremely rare in the thyroid, and can be easily misdiagnosed as anaplastic thyroid cancer. PATIENT CONCERNS: We present a case of a 71-year-old woman who presented with a rapidly growing painless mass in the neck. DIAGNOSES-INTERVENTIONS-OUTCOMES: Computed tomography showed a large hypointense mass with hyperdense areas involving whole of the right lobe of thyroid gland and fine-needle aspiration cytology found a few atypical cells...
February 2018: Medicine (Baltimore)
Stjepan Hmelik, Margareta Dobrenić, Dražen Huić
Pulmonary artery sarcomas (PAS's) are extremely rare malignant tumors that arise from the endothelial lining of the pulmonary arteries. On CT scans PAS's appear as intraluminal filling defects in the pulmonary arteries, mimicking pulmonary embolism (PE). Due to the similarities in radiographic features as well as in clinical presentation, PAS's are usually misdiagnosed as pulmonary embolism. Since PASs are F-18 FDG avid, F-18 FDG PET/CT scan is a useful imaging tool for differentiating between these two conditions, as shown in this case report...
2018: Nuclear Medicine Review. Central & Eastern Europe
M Sun, J G Liu, Q Y Weng, L Yu, J Wang
Objective: To investigate the clinicopathologic features, differential diagnosis and biological behavior of pleomorphic leiomyosarcoma (PLMS) and dedifferentiated leiomyosarcoma (DLMS). Methods: Forty-nine cases were collected from November 2007 to December 2016, including eight that diagnosed at Fudan University Shanghai Cancer Center, and 41 consultation cases. The clinical findings and pathologic features were reviewed. Immunophenotype was obtained in 33 cases and follow-up information was available in 38 cases...
February 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Ji-Gang Wang, Baolei Wang, Yanjiao Hu, Ji-Hua Liu, Bing Liu, Hui Liu, Peng Zhao, Lijuan Zhang, Yu-Jun Li
Primary cardiac tumors are uncommon, and the majority of them are benign which are curable but can cause significant morbidity if not diagnosed and treated in a timely fashion. The objective of this study was to review the clinicopathologic features and surgical outcomes of patients with primary cardiac tumors in a single medical center in China. We have retrospectively reviewed 212 consecutive adult patients who underwent surgical resection of primary cardiac tumors at our center from January of 2001 to June of 2017...
January 6, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Dasen Li, Lu Xie, Wei Guo, Xiaodong Tang, Tao Ji, Rongli Yang
INTRODUCTION: With the development of surgical techniques and improvements in hemi-pelvic prosthesis systems, extra-articular resection can be performed as a limb-salvage procedure in selected patients whose hip joint is invaded by a sarcoma. The aim of this study was to describe the indications for and the technical details, post-operative complications, and oncologic and functional outcomes of this procedure. METHODS: Eighteen patients with Enneking IIB or IB sarcoma who underwent extra-articular resection of the hip joint were enrolled...
February 5, 2018: International Orthopaedics
Melanie Boxberg, Katja Steiger, Ulrich Lenze, Hans Rechl, Rüdiger von Eisenhart-Rothe, Klaus Wörtler, Wilko Weichert, Rupert Langer, Katja Specht
Therapies targeting programmed death 1-(PD-1) or its ligand (PD-L1), promoting antitumor T-cell activity have been successfully introduced into clinical practice. Clinical response correlates with PD-L1 expression by tumor cells or immune cells within the tumor microenvironment. The PD-L1/PD-1 axis and tumor microenvironment has been rarely studied in high-grade sarcomas of soft tissue (hSTS), a group of rare, genetically heterogenous and clinically aggressive tumors. We examined PD-L1 protein and CD274/PD-L1 gene copy number variations in 128 primary resected, therapy-naive hSTS using immunohistochemistry and fluorescence-in-situ hybridization...
2018: Oncoimmunology
Austin Nguyen, Adam Vaudreuil, Paul Haun, Gabriel Caponetti, Christopher Huerter
Introduction  Benign fibrous histiocytomas are common lesions of the skin that rarely affect the tongue. Such cases are available in the literature exclusively as case reports. Similarly, malignant fibrous histiocytoma, now classified as undifferentiated pleomorphic sarcoma, is exceedingly rare in the tongue and not fully understood. Objectives  This study systematically reviews the available literature discussing the clinical and pathological features of malignant and benign fibrous histiocytomas. Data Synthesis  A total of 20 cases were included in this review...
January 2018: International Archives of Otorhinolaryngology
Nusret Can Çilesiz, Nilsen Erdoğan, Arif Özkan, Arif Kalkanlı, Aydın İsmet Hazar, Mustafa Bahadır Can Balcı, Memduh Aydın, Enver Özdemir
No abstract text is available yet for this article.
March 2018: Urology Case Reports
Jens Strohäker, Robert Bachmann, Thorsten Biegner, Bernhard Klumpp, Alfred Königsrainer, Ruth Ladurner
No abstract text is available yet for this article.
January 23, 2018: Minerva Chirurgica
Rieko Oyama, Fusako Kito, Marimu Sakumoto, Kumiko Shiozawa, Shunichi Toki, Akihiko Yoshida, Akira Kawai, Tadashi Kondo
Undifferentiated pleomorphic sarcoma (UPS) is an aggressive mesenchymal malignancy requiring novel therapeutic approaches to improve clinical outcome. Patient-derived cancer cell lines are an essential tool for investigating molecular mechanisms underlying cancer initiation and development; however, there is a lack of patient-derived cell lines of UPS available for research. The objective of this study was to develop a patient-derived cell model of UPS. A cell line designated NCC-UPS2-C1 was established from the primary tumor tissue of an 84-yr-old female patient with UPS...
January 22, 2018: In Vitro Cellular & Developmental Biology. Animal
Linda J Szymanski, Maria E Sibug Saber, Jonathan W Kim, John L Go, Gabriel Zada, Narsing Rao, Kyle M Hurth
Purpose: The aim of this study was to describe a 34-year-old male with hereditary bilateral retinoblastoma treated with radiotherapy as a child who developed 4 distinct tumors within the radiation field. Methods: A 34-year-old male with bilateral retinoblastoma status postradiation therapy and recurrence requiring enucleation presented with left-eye visual acuity changes. Magnetic resonance imaging demonstrated a left orbital mass and a right parasellar complex lobulated mass (right sphenoid and right cavernous sinus)...
December 2017: Ocular Oncology and Pathology
Alexander K Tsai, Melissa A L Vyfhuis, Martha Francis, Fikru Merechi, Allen P Burke, William F Regine
No abstract text is available yet for this article.
November 4, 2017: Practical Radiation Oncology
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