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A Righi, M Sbaraglia, M Gambarotti, S Cocchi, G Drago, R Casadei, P Picci, D Vanel, A P Dei Tos
Extra-axial chordoma is an exceedingly rare tumor, with only 28 cases reported in the literature to date. Axial and extra-axial chordoma exhibits complete morphologic and immunophenotypic (expression of brachyury) overlap. However, in consideration of the non-canonical presentation, extra-axial chordoma is under-recognized and often misdiagnosed, most often as extraskeletal myxoid chondrosarcoma or myoepithelioma. To increase our understanding of the clinicopathologic features of extra-axial chordoma, six cases have been retrieved from the files of the Istituto Ortopedico Rizzoli and of the General Hospital of Treviso...
March 20, 2018: Virchows Archiv: An International Journal of Pathology
Eric Wallace, Zachary Stewart, David Theriot, William Shaffer, Shane Guillory, Michael Hanemann, Raman Danrad, Bradley Spieler
Background: Plasmacytomas are monoclonal proliferations of plasma cells that typically affect the intramedullary axial skeleton. Imaging findings of an extramedullary plasmacytoma on radiograph and computed tomography can be nonspecific and can resemble other entities such as lymphoma, metastases, chondrosarcomas, or giant cell tumors. Case Report: A 60-year-old female with a medical history of partial complex seizures, hypertension, diabetes, glaucoma, and hyperlipidemia presented with complaints of superficial abdominal pain associated with erythema and swelling for 3 weeks...
2018: Ochsner Journal
Andrew L Folpe, Rondell P Graham, Anthony Martinez, David Schembri-Wismayer, Jennifer Boland, Karen J Fritchie
The diagnosis of mesenchymal chondrosarcoma, a distinctive biphasic malignant neoplasm harboring the HEY1-NCOA2 gene fusion and consisting of primitive round to spindled cells admixed with foci of relatively mature hyaline cartilage, is usually straightforward by morphological evaluation alone. However, in the setting of a limited biopsy, specimens lacking cartilage generate a broad differential diagnosis, encompassing a variety of other primitive sarcomas, including spindle cell/ sclerosing rhabdomyosarcoma...
March 17, 2018: Human Pathology
Onoufrios Tsavaris, Panagiota Economopoulou, Ioannis Kotsantis, Lazaros Reppas, Chrysanthi Avgerinou, Nikolaos Spathas, Maria Prevezanou, Amanda Psyrri
Chondrosarcoma is a rare malignancy characterized by the production of cartilage matrix, displaying heterogeneous histopathology and clinical behavior. Due to lack of effective treatment for advanced disease, the clinical management of metastatic chondrosarcoma is exceptionally challenging. Chondrosarcomas harbor molecular abnormalities, such as overexpression of platelet-derived growth factor receptor (PDGFR)-alpha and PDGFR-beta, which are required for cancer development, progression, and metastasis. Pazopanib is a potent and selective multitargeted tyrosine kinase inhibitor, which co-inhibits stem cell growth factor receptor (c-KIT), fibroblast growth factor receptor (FGFR), PDGFR, and vascular endothelial growth factor receptor (VEGFR) and has demonstrated clinical activity in patients with advanced previously treated soft tissue sarcoma...
2018: Frontiers in Oncology
Virginia Piombo, Katja Jochmann, Daniel Hoffmann, Manuela Wuelling, Andrea Vortkamp
Multiple osteochondromas (MO) syndrome is a dominant autosomal bone disorder characterized by the formation of cartilage-capped bony outgrowths that develop at the juxtaposition of the growth plate of endochondral bones. MO has been linked to mutations in either EXT1 or EXT2, two glycosyltransferases required for the synthesis of heparan sulfate (HS). The establishment of mouse mutants demonstrated that a clonal, homozygous loss of Ext1 in a wild type background leads to the development of osteochondromas. Here we investigate mechanisms that might contribute to the variation in the severity of the disease observed in human patients...
March 12, 2018: Bone
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
Katharine M Hinchcliff, Jessica Crockett, Steven W Thorpe, Christopher O Bayne
Long ischemia times adversely affect free flap survival, and large muscle flaps are particularly vulnerable. Hypothermic machine perfusion (HMP) is a well-established method of organ preservation, and recent literature has detailed the use of HMP to extend free flap ischemia times, predominantly in the laboratory setting. One limitation in the study and adoption of free flap HMP has been the availability of standardized perfusion machinery, as thus far institutions have built their own devices. We present a case of a 75-year-old woman with dedifferentiated chondrosarcoma of her right proximal femur...
March 14, 2018: Microsurgery
Christina Enciso Holm, Christian Bardram, Anja Falk Riecke, Peter Horstmann, Michael Mørk Petersen
PURPOSE: Previous studies reported variable outcome and failure rates after mega-prosthetic reconstructions in the lower extremities. The purpose of this study was to make a long-term single-center evaluation of patients treated with limb-sparing surgery and reconstruction with mega-prostheses in the lower extremities. METHODS: We identified 50 patients (osteosarcoma (n = 30), chondrosarcoma (n = 9), osteoclastoma (n = 6), Ewing sarcoma (n = 4), angiosarcoma (n = 1)), who underwent limb-sparing reconstruction of the lower extremities (proximal femur (n = 9), distal femur (n = 29), proximal tibia (n = 9), and the entire femur (n = 3)) between 1985 and 2005...
March 12, 2018: International Orthopaedics
Germán L Farfalli, Jose I Albergo, Nicolas S Piuzzi, Miguel A Ayerza, D Luis Muscolo, Lucas E Ritacco, Luis A Aponte-Tinao
BACKGROUND: The treatment of locally aggressive bone tumors is a balance between achieving local tumor control and surgical morbidity. Wide resection decreases the likelihood of local recurrence, although wide resection may result in more complications than would happen after curettage. Navigation-assisted surgery may allow more precise resection, perhaps making it possible to expand the procedure's indications and decrease the likelihood of recurrence; however, to our knowledge, comparative studies have not been performed...
March 2018: Clinical Orthopaedics and related Research
Alexandre Bossi Todeschini, Alaa S Montaser, Douglas A Hardesty, Ricardo L Carrau, Daniel M Prevedello
Endoscopic endonasal approaches (EEA) have gained popularity and acceptance in skull base surgery over the last two decades. So-called expanded EEA allow access in the sagittal plane from the frontal sinus to the odontoid process. The endoscopic endonasal transclival approach allows a unique trajectory into the midline clivus and skull base that is unachievable from traditional craniotomy approaches to lesions such as chondrosarcomas, chordomas, and posterior fossa meningiomas. In this review, we will assess the benefits and drawbacks to using an endoscopic endonasal approach versus transcranial approach to these challenging lesions, assess the anatomical limits of endoscopic endonasal transclival surgery, and discuss the published literature on the topic...
March 8, 2018: Journal of Neurosurgical Sciences
İsmail Burak Atalay, Selçuk Yılmaz, Mehmet Akif Şimşek, Mehmet Fatih Ekşioğlu, Bedii Şafak Güngör
OBJECTIVES: This study aims to evaluate the diagnosis and treatment approaches of the rarely seen chondrosarcomas of the phalanges of the hand. PATIENTS AND METHODS: Fifty-two patients (27 males, 25 females; mean age 41.2 years; range 12 to 70 years) with chondroid lesions localized in hand phalanges who were performed surgical treatment between December 2012 and September 2016 were retrospectively reviewed. The study included 62 phalangeal chondroid lesions. Patients' mean follow-up duration was 60...
April 2018: Eklem Hastalıkları Ve Cerrahisi, Joint Diseases & related Surgery
Daniele Marchioni, Angelo Musumeci, Cristoforo Fabbris, Stefano De Rossi, Davide Soloperto
BACKGROUND: The clivus is a region characterized by complex anatomy, with vascular and neural structures that are located in close proximity. Different pathologies can affect this area, and traditional surgical approaches were open approaches. Recently, the endoscopic transnasal technique has been introduced, and currently represents a good alternative for the surgical management of these lesions. This is a preliminary report on patients treated endoscopically for clival lesions by the authors' Skull Base Team...
March 8, 2018: European Archives of Oto-rhino-laryngology
Ashley J Guthrie, Raymond L Chai
IMPORTANCE: Transoral robotic surgery has revolutionized the practice of head and neck surgery over the past decade, with indications now expanding to include laryngeal pathology. Although laryngeal chondrosarcoma is a rare entity, trends in otolaryngology literature suggest that it can frequently be managed with conservative approaches. We hope to inspire other head and neck surgeons to consider transoral robotic surgery for the treatment of such tumors so that outcomes data can be collected and studied...
March 1, 2018: American Journal of Otolaryngology
Kiyoto Kanbara, Yoshinori Otsuki, Masahito Watanabe, Syunichi Yokoe, Yoshiaki Mori, Michio Asahi, Masashi Neo
BACKGROUND: High-grade chondrosarcoma, which has a high incidence of local recurrence and pulmonary metastasis despite surgical resection, is associated with poor prognosis. Therefore, new and effective adjuvant therapies are urgently required for this disease. Gamma-aminobutyric acid (GABA), which acts as a neurotrophic factor during nervous system development, is related to the proliferation and migration of certain cancer cells. The GABAergic system, which is composed of GABA, the GABA-synthesizing enzyme glutamic acid decarboxylase (GAD), and GABA receptors, has an important function in nerve growth and development of neural crest...
March 7, 2018: BMC Cancer
Marilla Dickfos, Stephanie B M Tan, Andrew R L Stevenson, Craig A Harris, Rachel Esler, Matthew Peters, David G Taylor
BACKGROUND: Over one-third of primary rectal cancers are locally advanced at diagnosis, and local recurrence of rectal cancer occurs at a rate of 3-10% following primary curative resection. Extended resectional surgery, including pelvic exenteration, is the only proven therapy with curative potential in the treatment of these cancers along with many other pelvic malignancies. A microscopically clear resection margin (R0 resection) is the predominant prognostic factor affecting overall and disease-free survival...
March 6, 2018: ANZ Journal of Surgery
Mohamed Amine Bani, Besma Laabidi, Faten Gargouri, Nada Mansouri, Ammar Bouziani, Issam Msakni
No abstract text is available yet for this article.
May 2017: La Tunisie Médicale
Abdelfettah Zidane, Adil Arsalane, Mohammed Lahkim, Issam Lalya, Abderrahim Ktaibi, Ismail Essadi
BACKGROUND: Radiotherapy associated with chemotherapy is a well-established treatment modality for locally advanced non-small cell lung cancers. Radiation-induced second malignancies, particularly radiation-induced sarcomas, are rare. Some authors reported a recent increase in the incidence of this rare complication, especially because of the improved prognosis and survival of patients after radiotherapy. Pathogenic mechanisms of radiation-induced sarcomas are poorly understood. However, diagnosis criteria are well established...
March 5, 2018: Journal of Medical Case Reports
Marta Karpik, Joanna Reszeć
We present the case of a 43-year-old patient with a radionegative tumor of the distal third of the femur. Work-up following a knee injury without any abnormalities on x-ray was extended to include an MRI study, which revealed an osteolytic lesion in the distal third of the femur. Extended work-up including an open biopsy identified a low-grade chondrosarcoma. Considering the patient's clinical status and the diagnostic findings, tumour resection and placement of a resection knee endoprosthesis appeared to be the best solution...
December 30, 2017: Ortopedia, Traumatologia, Rehabilitacja
T Grieser
CLINICAL ISSUE: Both benign and malignant tumours are encountered in the foot and ankle. Due to their rarity, however, diagnosis is often uncertain. Usual criteria such as tumour size, invasiveness or pain fail to differentiate benign from malignant neoplasias. STANDARD RADIOLOGICAL METHODS: Plain radiography and-due to the complex foot anatomy-CT are important in the diagnostic evaluation of primary bone tumours. In the case of soft tissue tumours, ultrasonography is used to identify ganglion cysts...
February 28, 2018: Der Radiologe
Akın Akakın, Kamran Urgun, Murat Şakir Ekşi, Baran Yılmaz, Özlem Yapıcıer, Mert Mestanoğlu, Zafer Orkun Toktaş, Mustafa Kemal Demir, Türker Kılıç
Chondrosarcoma is the second most common primary malignancy of bone after osteosarcoma. Cranial primary chondrosarcomas mostly originate from the skull base cartilage formation zones. Parasagittal falcine origin is very rare for primary extra-skeletal intracranial chondrosarcomas. We report a rare case of primary myxoid chondrosarcoma at falx cerebri. The patient was a 35-year-old lady with right arm and leg weakness. Her brain magnetic resonance imaging depicted a left parasagittal mass lesion attached to the falx cerebri...
January 2018: Asian Journal of Neurosurgery
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