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https://www.readbyqxmd.com/read/28550366/minimally-invasive-resection-of-adrenal-masses-in-infants-and-children-results-of-a-european-multi-center-survey
#1
Francesco Fascetti-Leon, Giovanni Scotton, Luca Pio, Raimundo Beltrà, Paolo Caione, Ciro Esposito, Girolamo Mattioli, Amulya K Saxena, Sabine Sarnaki, Piergiorgio Gamba
BACKGROUND: Minimal access adrenal surgery (MAAS) for adrenal pathologies is the standard for many pediatric surgical centers. However, the literature offers few reports and minimal evidence from small case series. The aim of this study was to evaluate the outcomes of pediatric MAAS through a multi-center data analysis. METHOD: Pediatric patients who underwent MAAS between January 2002 and December 2013 were retrospectively included. Data analysis was conducted using Spss software (Welch's t-test, X-square, Fisher tests, multiple regression model)...
May 26, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28550026/localized-adult-ewing-sarcoma-favorable-outcomes-with-alternating-vincristine-doxorubicin-cyclophosphamide-and-ifosfamide-etoposide-vdc-ie-based-multimodality-therapy
#2
Jennifer L Pretz, Constance M Barysauskas, Suzanne George, Jason L Hornick, Chandrajit P Raut, Yen-Lin E Chen, Karen J Marcus, Edwin Choy, Francis Hornicek, John E Ready, Thomas F DeLaney, Elizabeth H Baldini
BACKGROUND: In children with localized Ewing sarcoma (ES), addition of ifosfamide and etoposide to cyclophosphamide, doxorubicin, and vincristine (VDC/IE) improved 5-year overall survival (OS) to 70%-80%. Prior to delivery of VDC/IE in adults, 5-year OS was <50%. We reviewed our institutional outcomes for adults with ES who received VDC/IE-based treatment. MATERIALS AND METHODS: Between 1997-2013, 67 adults with localized ES were treated with curative intent...
May 26, 2017: Oncologist
https://www.readbyqxmd.com/read/28549964/a-strange-collection-after-surgery-for-an-aneurysm-of-the-popliteal-artery
#3
Gian Franco Veraldi, Luca Mezzetto, Roberta La Mendola, Paolo Criscenti, Marco Macrì, Albino Eccher
The popliteal fossa is the site of several diseases that may be similar in the clinical setting but very different in aetiology, treatment and prognosis. The contemporary presentation of more than one of these conditions is a rare though potentially fatal combination that may lead to a delay in the diagnosis and therapeutic approach. In this report we describe the case of a patient that presented at our Vascular Department for persistent pain and severe swelling of the right popliteal fossa eight months after the surgical treatment of a symptomatic popliteal aneurysm by mean of a dacron prosthetic graft...
May 23, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28549419/fluorescent-cxcr4-targeting-peptide-as-alternative-for-antibody-staining-in-ewing-sarcoma
#4
Laurens G L Sand, Tessa Buckle, Fijs W B van Leeuwen, Willem E Corver, Alwine B Kruisselbrink, Aart G Jochemsen, Pancras C W Hogendoorn, Károly Szuhai
BACKGROUND: Ewing sarcoma is an aggressive, highly metastatic primary bone and soft tissue tumor most frequently occurring in the bone of young adolescents. Patients, especially those diagnosed with a metastatic disease, have a poor overall survival. Chemokine receptor CXCR4 has a key pro-tumorigenic role in the tumor microenvironment of Ewing sarcoma and has been suggested to be involved in the increased metastatic propensity. Earlier studies on CXCR4 protein expression in Ewing sarcoma yielded contradictory results when compared to CXCR4 RNA expression studies...
May 26, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28549153/chippi-a-novel-method-for-mapping-chimeric-protein-protein-interactions-uncovers-selection-principles-of-protein-fusion-events-in-cancer
#5
Milana Frenkel-Morgenstern, Alessandro Gorohovski, Somnath Tagore, Vaishnovi Sekar, Miguel Vazquez, Alfonso Valencia
Fusion proteins, comprising peptides deriving from the translation of two parental genes, are produced in cancer by chromosomal aberrations. The expressed fusion protein incorporates domains of both parental proteins. Using a methodology that treats discrete protein domains as binding sites for specific domains of interacting proteins, we have cataloged the protein interaction networks for 11 528 cancer fusions (ChiTaRS-3.1). Here, we present our novel method, chimeric protein-protein interactions (ChiPPI) that uses the domain-domain co-occurrence scores in order to identify preserved interactors of chimeric proteins...
May 26, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28548706/45-gy-is-not-sufficient-radiotherapy-dose-for-group-iii-orbital-embryonal-rhabdomyosarcoma-after-less-than-complete-response-to-12-weeks-of-arst0331-chemotherapy-a-report-from-the-soft-tissue-sarcoma-committee-of-the-children-s-oncology-group
#6
Ralph P Ermoian, John Breneman, David O Walterhouse, Yueh-Yun Chi, Jane Meza, James Anderson, Douglas S Hawkins, Andrea A Hayes-Jordan, David M Parham, Torunn I Yock, Sarah S Donaldson, Suzanne L Wolden
BACKGROUND: Recent Children's Oncology Group (COG) trials tested the efficacy of reduced therapy in an effort to lessen late effects compared to the Intergroup Rhabdomyosarcoma Study (IRS) IV regimen with associated hematologic and hepatic toxicity, and infertility. Here, we analyze the efficacy of 45 Gray (Gy) local radiotherapy (RT) in patients with Group III orbital embryonal rhabdomyosarcoma (ERMS) enrolled on the COG low-risk study ARST0331. PROCEDURE: Sixty-two patients with Group III orbital ERMS were treated on ARST0331 with four cycles of vincristine (VCR), dactinomycin (DACT), and cyclophosphamide (CPM; VAC, total cumulative CPM dose 4...
May 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28548244/primary-histiocytic-sarcoma-presenting-as-diffuse-leptomeningeal-disease-case-description-and-review-of-the-literature
#7
Magda Zanelli, Moira Ragazzi, Giovanni Marchetti, Alessandra Bisagni, Massimo Principi, Daniela Fanni, Elisabetta Froio, Silvia Serra, Eleonora Zanetti, Loredana De Marco, Felice Giangaspero, Stefano Ascani
Histiocytic sarcoma is a rare malignant neoplasm arising most commonly in lymph nodes, intestinal tract, skin and soft tissue. The incidence of primary CNS histiocytic sarcoma is even rarer with a total of just 27 cases reported in the literature so far. Herein we describe the first autopsy case of histiocytic sarcoma presenting as a diffuse leptomeningeal disease in absence of a CNS tumor-forming parenchymal lesion. The clinical, pathological and immunophenotypic features are described and an updated literature review on primary CNS histiocytic sarcoma is included...
May 26, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28548090/synergistic-il-6-and-il-8-paracrine-signalling-pathway-infers-a-strategy-to-inhibit-tumour-cell-migration
#8
Hasini Jayatilaka, Pranay Tyle, Jonathan J Chen, Minsuk Kwak, Julia Ju, Hyun Ji Kim, Jerry S H Lee, Pei-Hsun Wu, Daniele M Gilkes, Rong Fan, Denis Wirtz
Following uncontrolled proliferation, a subset of primary tumour cells acquires additional traits/mutations to trigger phenotypic changes that enhance migration and are hypothesized to be the initiators of metastasis. This study reveals an adaptive mechanism that harnesses synergistic paracrine signalling via IL-6/8, which is amplified by cell proliferation and cell density, to directly promote cell migration. This effect occurs in metastatic human sarcoma and carcinoma cells- but not in normal or non-metastatic cancer cells-, and likely involves the downstream signalling of WASF3 and Arp2/3...
May 26, 2017: Nature Communications
https://www.readbyqxmd.com/read/28547734/systemic-therapy-for-soft-tissue-sarcoma-proposals-for-the-optimal-use-of-pazopanib-trabectedin-and-eribulin
#9
REVIEW
Akira Kawai, Kan Yonemori, Shunji Takahashi, Nobuhito Araki, Takafumi Ueda
Soft tissue sarcoma (STS) is a rare tumor with more than 50 histologic subtypes. Although treatment outcomes for patients with STS have improved greatly over the past few decades owing to the adoption of a multidisciplinary approach, patients with advanced disease have a poor prognosis. The development of anticancer drugs has been directed toward improving overall survival (OS). Doxorubicin monotherapy is currently the only standard option for the first-line treatment of STS. However, there is no standard therapy for second-line and later treatment at present...
May 25, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28547562/major-amputations-for-extremity-soft-tissue-sarcoma
#10
Henry G Smith, Joseph M Thomas, Myles J F Smith, Andrew J Hayes, Dirk C Strauss
INTRODUCTION: With modern techniques facilitating limb conservation, amputation for extremity soft-tissue sarcoma (ESTS) is now rare. We sought to determine the indications and outcomes following major amputation for ESTS and whether amputation is prognostic of oncological outcomes in primary disease. PATIENTS AND METHODS: Patients undergoing major amputations for ESTS from 2004 to 2014 were identified from electronic patient records. RESULTS: The amputation rate in primary localized disease was 4...
May 25, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28546788/dmd-transcripts-in-crl-2061-rhabdomyosarcoma-cells-show-high-levels-of-intron-retention-by-intron-specific-pcr-amplification
#11
Emma Tabe Eko Niba, Ryo Yamanaka, Abdul Qawee Mahyoob Rani, Hiroyuki Awano, Masaaki Matsumoto, Hisahide Nishio, Masafumi Matsuo
BACKGROUND: The DMD gene encoding dystrophin is mutated in Duchenne muscular dystrophy, a fatal progressive muscle wasting disease. DMD has also been shown to act as a tumor suppressor gene. Rhabdomyosarcoma (RMS) is a mesodermal sarcoma that shares characteristics of skeletal muscle precursors. Products of the DMD gene in RMS have not yet been fully clarified. Here, DMD products were analyzed in CRL-2061 cells established from alveolar RMS. METHODS: The 14-kb long DMD cDNA was PCR amplified as 20 separated fragments, as were nine short intron regions...
2017: Cancer Cell International
https://www.readbyqxmd.com/read/28546782/neoadjuvant-ifosfamide-and-epirubicin-in-the-treatment-of-malignant-peripheral-nerve-sheath-tumors
#12
Angela C Hirbe, Pippa F Cosper, Sonika Dahiya, Brian A Van Tine
Background and Objectives. Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with poor overall survival. Response to chemotherapy has been debated for these tumors. Methods. We performed a retrospective analysis of the patients at our institution with a biopsy-proven diagnosis of MPNST that underwent neoadjuvant chemotherapy prior to surgery. Results. We retrospectively identified five patients who received neoadjuvant chemotherapy with epirubicin and ifosfamide that demonstrated a 30% reduction in tumor growth and a 60% response rate by RECIST criteria...
2017: Sarcoma
https://www.readbyqxmd.com/read/28546729/rare-case-report-of-alveolar-soft-part-sarcoma-of-the-orbit
#13
G Kranthi Kumar, Hemant Nemade, Krishnamohan, Daphne Fonseca, L M Chandra Sekhara Rao, T Subramanyeshwar Rao
Alveolar soft part sarcoma (ASPS) is a rare but distinct soft tissue tumor with unique histopathological and electron microscopic features. Orbital involvement is rare with only few reports published in the literature. ASPS have an indolent clinical course, but it is known to metastasize. Primary modality of treatment is surgery followed by adjuvant treatment. This case is a unique presentation with orbital mass with on and off bleeding. This is the largest orbital ASPS for which orbital exenteration was performed...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28546722/malignant-peripheral-nerve-sheath-tumour-of-the-small-bowel-presenting-with-intussusception-and-perforation-a-double-jeopardy
#14
Ananth P Abraham, Joshua Franklyn, Jagan Chandramohan, Pranay Gaikwad, John C Muthusami
Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcomas which largely occur in the extremities and the head and neck region. The tumours are aggressive with a high rate of recurrence. Radical surgical resection remains the treatment of choice with adjuvant radiation therapy and chemotherapy still failing to demonstrate a clear benefit. The gastrointestinal tract is an exceedingly rare site for these tumours. We report an unusual case of a young male with an MPNST of the small bowel who presented with an ileocolic intussusception and sigmoid perforation...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28546720/dermatofibrosarcoma-protruberans-of-the-breast-skin-simulating-mammary-carcinoma
#15
Prakriti Shukla, Hanni Vasudev Gulwani
Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma that arises from the dermis and invades deeper tissues. Usually, it behaves as an intermediate grade malignancy but in rare instances, it can metastasize. Frequent occurrences have been observed in trunk and extremities but involvement of the breast has rarely been reported. Therefore, dermatofibrosarcoma protuberans (DFSP) breast often masquerades primary breast malignancy on clinical and radiological grounds. Histomorphology and immunohistochemistry are helpful in making a definitive diagnosis...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28546706/prognostic-factors-in-indian-patients-operated-for-soft-tissue-sarcomas-a-retrospective-cross-sectional-analysis
#16
Akshay Tiwari, Swati Shah, Ashwani Kumar Sharma, Sandeep Mehta, Ullas Batra, S K Sharma, A K Dewan
Soft tissue sarcomas are a rare entity. While surgery is established as the mainstay of treatment, the exact role and sequencing of adjuvant therapy is not well defined. Literature on Indian patients with soft tissue sarcoma with respect to clinical profile and prognostic factors is scarce. We retrospectively analysed the data of 112 patients operated for soft tissue sarcoma of extremity or trunk (excluding retroperitoneal and mediastinal sarcomas, round cell histology) at our institute from 1 January 2009 to 31 December 2013...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28546431/disruption-of-mitochondrial-electron-transport-chain-function-potentiates-the-pro-apoptotic-effects-of-mapk-inhibition
#17
Andrew P Trotta, Jesse D Gelles, Madhavika N Serasinghe, Patrick Loi, Jack L Arbiser, Jerry E Chipuk
The mitochondrial network is a major site of ATP production through the coupled integration of the electron transport chain (ETC) with oxidative phosphorylation. In melanoma, arising from the Val600Glu mutation in the kinase v-RAF murine sarcoma viral oncogene homolog B (BRAFV600E), oncogenic signaling enhances glucose-dependent metabolism, while reducing mitochondrial ATP production. Likewise, when BRAFV600E is pharmacologically inhibited by targeted therapies (e.g. PLX-4032/Vemurafenib), glucose metabolism is reduced and cells increase mitochondrial ATP production to sustain survival...
May 25, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28546135/neoadjuvant-hypofractionated-radiotherapy-and-chemotherapy-in-high-grade-extremity-soft-tissue-sarcomas-phase-2-clinical-trial-protocol
#18
Ranyell Msb Spencer, Samuel Aguiar Junior, Fabio O Ferreira, Paulo R Stevanato Filho, Bruna Ec Kupper, Maria Lg Silva, Celso A Mello, Tiago S Bezerra, Ademar Lopes
BACKGROUND: Neoadjuvant radiotherapy (RT) and chemotherapy are applied to large, high-grade extremity soft tissue sarcomas to treat metastatic disease earlier and sterilize margins to perform R0 surgery. However, preoperative RT increases wound complication rates (rWC), delaying adjuvant chemotherapy or preventing it from being administered altogether. Hypofractionated neoadjuvant RT can be offered in this situation, concomitant to chemotherapy, allowing patients to receive chemotherapy as a preoperative treatment in less time with an acceptable rWC...
May 25, 2017: JMIR Research Protocols
https://www.readbyqxmd.com/read/28545570/the-feasibility-of-contrast-enhanced-ultrasonography-ceus-in-the-diagnosis-of-non-cardiac-thoracic-disorders-of-dogs-and-cats
#19
N Linta, M Baron Toaldo, G Bettini, A Cordella, M Quinci, P Pey, V Galli, M Cipone, A Diana
BACKGROUND: This study describes the feasibility of Contrast Enhanced Ultrasonography (CEUS) in the diagnostic work-up of non-cardiac thoracic disorders of small animals. The second aim is to assess the usefulness of CEUS as a direct guide for sample procedures. RESULTS: Forty animals, 28 dogs and 12 cats, were included in the study. Thoracic disorders included 23 pulmonary lesions [primary carcinoma (14), lymphoma (1), sarcoma (1), histiocytic sarcoma (1), abscess (1) and pneumonia (5)] and 17 mediastinal lesions [lymphoma (8), thymoma (3), mesothelioma (1), melanoma (1), carcinomatous lymphadenopathy (1), mixsosarcoma (1), lipoma (1), and abscess (1)]...
May 25, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28544823/fine-needle-aspiration-cytology-findings-of-myxoinflammatory-fibroblastic-sarcoma-a-case-report
#20
Satoru Ozaki, Satomi Kasashima, Atsuhiro Kawashima, Akishi Ooi
Myxoinflammatory fibroblastic sarcoma (MIFS) is rare low-grade soft-tissue tumor that occurs in extremities. Clinically it is difficult to differentiate from benign lesions, such as nodular fasciitis, and malignant tumors, such as liposarcoma. We report a case of MIFS in the forearm of a 34-year-old man. The resected tumor measured 5.3 × 2.7 × 2.5 cm(3) , had a lobular structure with indistinct boundary, and consisted of a large amount of translucent and yellow mucous-like substrate. Cytological examination of a preoperative puncture aspiration specimen showed histiocyte- and fibroblast-like tumor cells in a mucous-like matrix together with scattered lipoblast- and ganglion-like cells...
May 24, 2017: Diagnostic Cytopathology
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