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Bone sarcomas

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https://www.readbyqxmd.com/read/28648942/dedifferentiated-chondrosarcoma-of-bone-with-prominent-rhabdoid-component
#1
Matthew Stemm, Chad Beck, Rajeev Mannem, John Neilson, Michael J Klein
Dedifferentiated chondrosarcomas (DDCS) are rare lesions, defined as tumors having a low-grade chondrosarcomatous component with an abrupt transition to a high-grade sarcoma. Although "malignant fibrous histiocytoma" (undifferentiated pleomorphic sarcoma) is the most common high grade saromatous component, many different types of sarcoma have been described. We present a case of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation harboring a prominent rhabdoid tumor component. To our knowledge, rhabdoid morphology in dedifferentiated chondrosarcoma has not been described in the English-language literature...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28643081/distraction-osteogenesis-reconstruction-of-large-segmental-bone-defects-after-primary-tumor-resection-pitfalls-and-benefits
#2
REVIEW
Jan Lesensky, Daniel E Prince
Successful cure is achieved in almost 70% of patients with primary bone sarcomas with currently available therapies. Some soft tissue sarcomas require wide bone resection in order to achieve appropriate margins for cure of disease, and patients undergoing these procedures need durable reconstruction. Biological reconstruction has been shown to provide patients with superior long-term results over other alternatives. Distraction osteogenesis is well studied in the correction of deformities as well as in addressing some congenital musculoskeletal pathologies...
June 22, 2017: European Journal of Orthopaedic Surgery & Traumatology: Orthopédie Traumatologie
https://www.readbyqxmd.com/read/28641661/-clinical-and-pathologic-features-of-myeloid-sarcoma
#3
Ya-Jun Jiang, Hong-Xia Wang, Wan-Chuan Zhuang, Hao Chen, Chang Zhang, Xiu-Mei Li, Gui-Hua Zhu, Yao He
OBJECTIVE: To explore the clinicopathologic features, differential diagnosis and therapy of myeloid sarcoma. METHODS: The clinical data including clinical manifestations, laboratorial tests, histopathologicical examination, immunohistochemistry and clinical prognosis of 10 patients with myeloid sarcoma were analyzed retrospectively. Among 10 patients, 5 male and 5 female, aged 23 to 71 years old (median = 36 years). RESULTS: 2 cases of myeloid sarcoma were secondary from chronic myeloid leukemia, and 1 cases of myeloid sarcoma occurred after the allogeneic hematopoietic stem cell transplantation due to acute myeloid leukemia, and the others lacked the anamnesis of malignancies...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28639531/outcome-of-bone-recycling-using-liquid-nitrogen-as-bone-reconstruction-procedure-in-malignant-and-recurrent-benign-aggressive-bone-tumour-of-distal-tibia-a-report-of-four-cases
#4
Eka Wiratnaya I Gede, Arrisna Artha Ida Ayu, Yudhi Setiawan I Gn, Wien Aryana Ign, Suyasa I Ketut, Siki Kawiyana I Ketut, Astawa Putu
Amputation still considered as primary choice of malignancy treatment in distal tibia. Bone recycling with liquid nitrogen for reconstruction following resection of malignant bone tumours offers many advantages. We presented four patients with osteosarcoma, Ewing sarcoma, adamantinoma and recurrent giant cell tumour over distal tibia. All of the patients underwent wide excision and bone recycling using liquid nitrogen as bone reconstruction. The mean functional Musculoskeletal Tumor Society (MSTS) score was 75% with no infection and local recurrent...
May 2017: Journal of Orthopaedic Surgery
https://www.readbyqxmd.com/read/28639517/development-of-finite-element-model-for-customized-prostheses-design-for-patient-with-pelvic-bone-tumor
#5
Taimoor Iqbal, Lei Shi, Ling Wang, Yaxiong Liu, Dichen Li, Mian Qin, Zhongmin Jin
The aim of this study was to design a hemi-pelvic prosthesis for a patient affected by pelvic sarcoma. To investigate the biomechanical functionality of the pelvis reconstructed with designed custom-made prosthesis, a patient-specific finite element model of whole pelvis with primary ligaments inclusive was constructed based on the computed tomography images of the patient. Then, a finite element analysis was performed to calculate and compare the stress distribution between the normal and implanted pelvis models when undergoing three different static conditions-both-leg standing, single-leg standing for the healthy and the affected one...
June 2017: Proceedings of the Institution of Mechanical Engineers. Part H, Journal of Engineering in Medicine
https://www.readbyqxmd.com/read/28638739/ewing-sarcoma-partial-regression-without-gvhd-by-chondromodulin-i-hla-a-02-01-specific-allorestricted-t-cell-receptor-transgenic-t-cells
#6
Uwe Thiel, Sebastian J Schober, Ingo Einspieler, Andreas Kirschner, Melanie Thiede, David Schirmer, Katja Gall, Franziska Blaeschke, Oxana Schmidt, Susanne Jabar, Andreas Ranft, Rebeca Alba Rubío, Uta Dirksen, Thomas G P Grunewald, Poul H Sorensen, Günther H S Richter, Irene Teichert von Lüttichau, Dirk H Busch, Stefan E G Burdach
Background: Chondromodulin-I (CHM1) sustains malignancy in Ewing sarcoma (ES). Refractory ES carries a dismal prognosis and patients with bone marrow (BM) metastases do not survive irrespective of therapy. We assessed HLA-A*02:01/CHM1-specific allorestricted T cell receptor (TCR) wild-type and transgenic cytotoxic (CD8(+)) T cells against ES. Patients and Methods: Three refractory HLA-A2(+) ES patients were treated with HLA-A*02:01/peptide-specific allorepertoire-derived (i.e., allorestricted) CD8(+) T cells...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28630846/chronic-recurrent-multifocal-osteomyelitis-a-case-report-with-atypical-presentation
#7
Miguel Pádua Figueiredo, Marco Pato, Fernando Amaral
INTRODUCTION: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory condition. The clinical picture consists of sterile osteomyelitis, typically with multiple-site lesions in the metaphysis of long bones and not uncommonly, symmetrical bone involvement. It is a poorly understood entity, whose prognosis, etiology and ideal treatment are still controversial. The authors report a case of unifocal presentation with an atypical location. CASE REPORT: A previously healthy 12-year-old Caucasian girl came to our institution due to progressive pain on her left thigh for the previous 3 months...
January 2017: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28630763/malignant-tumours-of-the-foot-and-ankle
#8
E Mascard, N Gaspar, L Brugières, C Glorion, S Pannier, A Gomez-Brouchet
Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients...
May 2017: EFORT open reviews
https://www.readbyqxmd.com/read/28628542/a-rare-intramedullary-spinal-cord-metastasis-from-a-retroperitoneal-leiomyosarcoma-presenting-as-a-non-traumatic-spinal-cord-injury
#9
Andrew Lamberth Parker, Terrence Pugh, Mark A Hirsch
Leiomyosarcoma (LMS) is a rare but well-recognized malignant soft tissue sarcoma of smooth muscle origin. Metastases commonly occur in the lungs, liver, kidney, brain, and bone. Cases of metastatic osseous lesions or other extradural space-occupying masses secondary to LMS leading to neurologic compromise are relatively commonplace in the literature. Conversely, cases of intramedullary spinal cord metastasis (ISCM), an unusual entity as a sequela of any cancer, are exceedingly rare as a consequence of LMS. Only 2 cases of an ISCM from LMS are currently documented in the literature, and to the best of our knowledge, no case is described in the rehabilitation literature...
July 2017: American Journal of Physical Medicine & Rehabilitation
https://www.readbyqxmd.com/read/28626593/langerhans-cell-sarcoma-a-case-report-demonstrating-morphological-and-immunophenotypical-variability-within-a-single-lesion
#10
Rasika Singh, Charles Edward Keen, Christopher Stone, Patrick Sarsfield
Langerhans cells are antigen presenting dendritic cells and tumours arising from these are rare. The tumours arising from these dendritic cells are divided into two categories according to a WHO classification: Langerhans cell histiocytosis and Langerhans cell sarcoma. It is the degree of atypia and clinical aggressiveness that distinguishes the two subtypes. Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells which can occur in skin, bone marrow, lymph nodes, spleen, liver, and lung...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28626490/soft-tissue-sarcomas-in-the-head-and-neck-25-years-of-experience
#11
Juan Francisco Liuzzi, Maribel Da Cunha, Daniuska Salas, Saul Siso, Esteban Garriga
Sarcomas are infrequent and heterogeneous tumours. They represent 1-2% of all malignant neoplasms in adults and between 4% and 10% of head and neck cancers. METHODS: The research was retrospective, descriptive, and cross-sectional. RESULTS: A study population of 62 patients with a mean age of 44 years was obtained; the most frequent location was the soft tissues of the neck (25.3%) and the mean tumour size was 7.1 cm; the most frequent diagnosis was undifferentiated pleomorphic sarcoma (25...
2017: Ecancermedicalscience
https://www.readbyqxmd.com/read/28626407/systemic-therapy-outcomes-in-adult-patients-with-ewing-sarcoma-family-of-tumors
#12
Mario Valdes, Garth Nicholas, Shailendra Verma, Timothy Asmis
BACKGROUND: The Ewing sarcoma family of tumors (ESFT) is a rare but curable bone neoplastic entity. The current standard of care involves chemotherapy and local disease control with surgery or radiation regardless of the extent of disease at presentation. Data that document the effectiveness of the current approach in the adult patient population are limited. METHODS: We performed a retrospective review including all ESFT patients older than 19 years of age who received systemic therapy between January 2002 and December 2013 at our institution...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28623757/heterotopic-mesenteric-and-abdominal-wall-ossification-two-case-reports-in-one-institution
#13
Cátia Ferreira, Carina Gomes, Ana Melo, Nádia Tenreiro, Bruno Pinto, Herculano Moreira, Artur Ribeiro, Paulo Avelar
INTRODUCTION: Heterotopic ossification occurs when bone develops in tissues which usually don't undergo ossification. Heterotopic mesenteric ossification, also known as intra-abdominal myositis ossificans, is a rare and benign form of ossification, usually related with previous abdominal surgery or trauma. PRESENTATION OF CASES: We report two cases of heterotopic ossification both after multiple abdominal surgeries, with intraoperative findings of mesenteric and abdominal wall ossification...
June 8, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28619757/cdk4-6-inhibitors-sensitize-rb-positive-sarcoma-cells-to-wee1-kinase-inhibition-through-reversible-cell-cycle-arrest
#14
Ashleigh M Francis, Angela Alexander, Yanna Liu, Smruthi Vijayaraghavan, Kwang Hui Low, Dong Yang, Tuyen Bui, Neeta Somaiah, Vinod Ravi, Khandan Keyomarsi, Kelly K Hunt
Research into the biology of soft tissue sarcomas has uncovered very few effective treatment strategies that improve upon the current standard of care which usually involves surgery, radiation, and chemotherapy. Many patients with large (>5cm), high-grade sarcomas develop recurrence, and at that point have limited treatment options available. One challenge is the heterogeneity of genetic drivers of sarcomas, and many of these are not validated targets. Even when such genes are tractable targets, the rarity of each subtype of sarcoma makes advances in research slow...
June 15, 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/28619077/what-is-the-impact-of-local-control-in-ewing-sarcoma-analysis-of-the-first-brazilian-collaborative-study-group-ewing1
#15
Ricardo G Becker, Lauro J Gregianin, Carlos R Galia, Reynaldo Jesus-Garcia Filho, Eduardo A Toller, Gerardo Badell, Suely A Nakagawa, Alexandre David, André M Baptista, Eduardo S Yonamime, Osvaldo A Serafini, Valter Penna, Julie Francine C Santos, Algemir L Brunetto
BACKGROUND: Relapse in localized Ewing sarcoma patients has been a matter of concern regarding poor prognosis. Therefore, we investigated the impact of local control modality (surgery, surgery plus radiotherapy, and radiotherapy) on clinical outcomes such as survival and recurrence in patients with non-metastatic Ewing sarcoma treated on the first Brazilian Collaborative Group Trial of the Ewing Family of Tumors (EWING1). METHODS: Seventy-three patients with localized Ewing sarcoma of bone aged < 30 years were included...
June 15, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28607872/vascularized-fibula-based-physis-transfer-a-follow-up-study-of-longitudinal-bone-growth-and-complications
#16
Ronnie L Shammas, Yash J Avashia, Alfredo E Farjat, Anthony A Catanzano, L Scott Levin, William C Eward, Brian E Brigman, Detlev Erdmann
BACKGROUND: The vascularized free fibula epiphyseal transfer provides an option for the preservation of limb lengthening after resection of the proximal humerus in pediatric sarcoma patients. The purpose of this study was to provide a long-term follow-up of longitudinal growth patterns and outcomes after free fibula epiphyseal transfer in upper extremity reconstruction. METHODS: A retrospective review of 4 patients who underwent free fibula epiphyseal transfer after oncologic resection of the proximal humerus for osteosarcoma was performed...
May 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/28607816/recurrence-case-of-rare-scalp-dermatofibrosarcoma-protuberans-two-case-reports-of-a-wide-radical-excision-craniectomy-bone-involvement-followed-by-cranioplasty-and-reconstruction
#17
Ahmad Faried, Wargian Hadisaputra, Muhammad Z Arifin
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade sarcoma of the fibroblast originating from the dermal layer of the skin, characterized by a locally aggressive growth and high rate of local recurrence. CASE DESCRIPTION: Two patients underwent a wide radical excision of recurrent scalp DFSP which was reconstructed with translational skin flap and split-thickness skin graft. We described above cases several years ago with a local excision of the tumor; recently, they developed local recurrence of DFSP with calvarial involvement...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28601416/statistics-of-soft-tissue-sarcoma-in-japan-report-from-the-bone-and-soft-tissue-tumor-registry-in-japan
#18
Koichi Ogura, Takahiro Higashi, Akira Kawai
BACKGROUND: No previous reports to date have characterized the national profiles of soft-tissue sarcomas (STSs). In the present study, we reviewed current practice for STSs in Japan using data from a nationwide organ-specific cancer registry for bone and soft-tissue tumors in Japan, the Bone and Soft Tissue Tumor (BSTT) Registry. METHODS: In the registry, we identified 8228 patients with STSs during the period 2006-2012, and extracted data on patient demographics, treatment, and outcome at the last follow-up for each patient...
June 7, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28600479/trabectedin-overrides-osteosarcoma-differentiative-block-and-reprograms-the-tumor-immune-environment-enabling-effective-combination-with-immune-checkpoint-inhibitors
#19
Chiara Ratti, Laura Botti, Valeria Cancila, Silvia Galvan, Ilaria Torselli, Cecilia Garofalo, Maria Cristina Manara, Lucia Bongiovanni, Cesare F Valenti, Alessia Burocchi, Mariella Parenza, Barbara Cappetti, Sabina Sangaletti, Claudio Tripodo, Katia Scotlandi, Mario P Colombo, Claudia Chiodoni
Purpose: Osteosarcoma (OS), the most common primary bone tumor, is characterized by an aggressive behavior with high tendency to develop lung metastases as well as by multiple genetic aberrations that have hindered the development of targeted therapies. New therapeutic approaches are urgently needed; however, novel combinations with immunotherapies and checkpoint inhibitors require suitable preclinical models with intact immune systems to be properly tested. <p>Experimental Design: We have developed immuno-competent OS models that grow orthotopically in the bone and spontaneously metastasize to the lungs, mimicking human OS...
June 9, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28593036/disseminated-and-late-metastatic-disease-from-nasal-pit-leiomyosarcoma-after-radical-surgical-resection-case-report-of-a-singular-presentation-of-a-rare-disease
#20
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
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