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Pediatric bone tumor

Rosa Ruchlemer, Michal Amit-Kohn, Ariella Tvito, Irena Sindelovsky, Ari Zimran, David Raveh-Brawer
PURPOSE: Bone loss-osteopenia and osteoporosis-is a recognized consequence of solid tumors in adults, of pediatric hematological malignancies, and of the treatment for these diseases, but little research has been published on the adverse effects of hematological malignancies on the bone in adults. The aim of this study is to identify hematological diseases that are associated with the highest prevalence and severity of osteoporosis. METHODS: We evaluated DXA (dual-energy X-ray absorptiometry) in a cross-section of 181 adult patients with hematological neoplasms, excluding multiple myeloma...
March 16, 2018: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
Matthew J Thompson, John C Shapton, Stephanie E Punt, Christopher N Johnson, Ernest U Conrad
BACKGROUND: The quantitative accuracy of MRI in predicting the intraosseous extent of primary sarcoma of bone has not been definitively confirmed, although MRI is widely accepted as an accurate tool to plan limb salvage resections. Because inaccuracies in MRI determination of tumor extent could affect the ability of a tumor surgeon to achieve negative margins and avoid local recurrence, we thought it important to assess the accuracy of MR-determined tumor extent to the actual extent observed pathologically from resected specimens in pediatric patients treated for primary sarcomas of bone...
March 2018: Clinical Orthopaedics and related Research
Yusuf Menda, Mark T Madsen, Thomas M O'Dorisio, John Sunderland, Leonard G Watkins, Joseph S Dillon, Sarah L Mott, Michael King Schultz, Gideon K D Zamba, David L Bushnell, M Sue O'Dorisio
Pre-therapy PET imaging with86 Y-DOTATOC PET is considered the ideal dosimetry protocol for90 Y-DOTATOC therapy; however, its cost, low availability and the need for infusion of amino acids to mimic the therapy administration limit its use in the clinical setting. Objectives: To develop a dosimetric method for90 Y-DOTATOC therapy using90 Y-DOTATOC PET/CT and Bremsstrahlung SPECT/CT imaging and to determine if dosimetry-based administered activities differed significantly from standard administered dosages. Materials and Methods: This is a prospective Phase 2 trial of90 Y-DOTATOC therapy in patients with somatostatin receptor positive tumors...
March 9, 2018: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Zhenqin Zhang, Zhenzhu Zhu, Cheng Luo, Chengcheng Zhu, Changli Zhang, Zijian Guo, Xiaoyong Wang
Osteosarcoma (OS) is the most common primary pediatric bone tumor lethal to children and adolescents. Chemotherapeutic agents such as cisplatin are not effective for OS because of their poor accessibility to this cancer and severe systemic toxicity. In this study, a lipophilic platinum(II) complex bearing a bisphosphonate bone-targeting moiety, cis-[PtL(NH3 )2 Cl]NO3 {BPP; L = tetraethyl [2-(pyridin-2-yl)ethane-1,1-diyl]bisphosphonate}, was prepared and characterized by NMR, electrospray ionization mass spectrometry, and single-crystal X-ray crystallography...
March 7, 2018: Inorganic Chemistry
Suoyuan Li, Tao Zhang, Weiguo Xu, Jianxun Ding, Fei Yin, Jing Xu, Wei Sun, Hongsheng Wang, Mengxiong Sun, Zhengdong Cai, Yingqi Hua
PURPOSE: Osteosarcoma is the most common primary bone cancer and is notorious for pulmonary metastasis, representing a major threat to pediatric patients. An effective drug targeting osteosarcoma and its lung metastasis is urgently needed. DESIGN: In this study, a sarcoma-targeting peptide-decorated disulfide-crosslinked polypeptide nanogel (STP-NG) was exploited for enhanced intracellular delivery of shikonin (SHK), an extract of a medicinal herb, to inhibit osteosarcoma progression with minimal systemic toxicity...
2018: Theranostics
Monique J L Mastboom, Floortje G M Verspoor, Daniël Uittenbogaard, Gerard R Schaap, Paul C Jutte, H W Bart Schreuder, Michiel A J van de Sande
BACKGROUND: Tenosynovial giant cell tumor (TGCT) is a rare, benign, monoarticular entity. Many case-series in adults are described, whereas TGCT is only incidentally reported in children. Therefore, its incidence rate and natural history in children are unknown. QUESTIONS/PURPOSES: (1) How many cases have been reported of this condition, and what were their characteristics? (2) What is the standardized pediatric incidence rate for TGCT? (3) Is there a clinical difference in TGCT between children and adults? (4) What is the risk of recurrence after open resection in children compared with adults? METHODS: Data were derived from three sources: (1) a systematic review on TGCT in children, seeking sources published between 1990 and 2016, included 17 heterogeneous, small case-series; (2) the nationwide TGCT incidence study: the Dutch pediatric incidence rate was extracted from this nationwide study by including patients younger than 18 years of age...
February 8, 2018: Clinical Orthopaedics and related Research
Angela R Shih, Gregory M Cote, Ivan Chebib, Edwin Choy, Thomas DeLaney, Vikram Deshpande, Francis J Hornicek, Ruoyu Miao, Joseph H Schwab, G Petur Nielsen, Yen-Lin Chen
Chordoma is a rare malignant tumor of bone with high morbidity and mortality. Recently, aggressive pediatric poorly differentiated chordoma with SMARCB1 loss has been described. This study summarizes the clinicopathologic features of poorly differentiated chordoma with SMARCB1 loss in the largest series to date. A search of records between 1990-2017 at MGH identified 19 patients with poorly differentiated chordoma. Immunohistochemical stains were evaluated. Kaplan-Meier survival statistics and log-rank (Mantel Cox) tests compared survival with other subtypes...
February 26, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Jun Wu, Cui Wang, Ming Li, Cong Luo, Xing Liu, Lina Xie, Ling He, Mengjia Gong, Zujie Hu, Anthony J Bleyer, Yuanyuan Zhang
OBJECTIVE: This study aimed to investigate the application of 3D computed tomography (CT) angiography with a novel post-processing technique in diagnosis of malignant bone tumors in children. METHODS: Twenty-seven pediatric patients (15 males and 12 females; average age: 10±3.4 years old, with a range from 2 months to 14 years old) with suspected bone tumors were evaluated histopathologically using 3D CT angiography and a multislice scanner. CT angiography image data were analyzed with a novel post-processing technique that included separating, fusing opacifying false-coloring, and volume rendering...
2018: Journal of X-ray Science and Technology
Gabriela Maciel Vieira, Gabriela Molinari Roberto, Régia Caroline Lira, Edgard Eduard Engel, Luiz Gonzaga Tone, María Sol Brassesco
Ewing's sarcoma (EWS) is a highly aggressive bone cancer that affects children and adolescents. Despite advances in multimodal management, 5-year event-free survival rates for patients presenting with metastases at diagnosis remain at 25%. As key regulators of actin organization, the Rho-associated coiled-coil containing protein kinases, ROCK1 and ROCK2, have been associated with cancer dissemination and poorer prognosis. Recently, in vitro data indicating ROCK2 as a molecular target for the treatment of EWS has been presented...
February 2018: Oncology Letters
Junhua Cao, Qi An, Lei Wang
Sarcomas arise from primitive mesenchymal cells, which are classified, into two main groups: Bone and soft tissue sarcomas. We have searched all-important electronic databases including Google scholar and PubMed for the collection of latest literature pertaining to pediatric sarcomas. Latest literature confirmed that these tumors are relatively rare and represent only 1% of all malignancies but they have higher incidence in children. Pediatric sarcomas comprise about 13% of all pediatric malignancies and are ranked third in childhood cancers...
February 2018: Oncology Letters
D S Lima, R P G Lemes, D M Matos
In tumor microenvironment, immunosuppression is a common event and results from the inhibition of activated immune cells and generation of cells with immunosuppressive capacity, as some subtypes of monocytes. The aim of this study was to evaluate the presence of immunosuppressive CD14 + /HLA-DR low/- monocytes in pediatric patients with the diagnosis of B-cell acute lymphoblastic leukemia (B-ALL) and, moreover, verify whether the chemotherapeutic treatment has any effect on these cells. Peripheral blood (PB) and bone marrow (BM) samples were collected from 15 untreated pediatric patients...
February 10, 2018: Medical Oncology
Fabio Santanelli di Pompeo, Gennaro Selvaggi, Benedetto Longo, Rosaria Laporta, Vittoria Amorosi, Michail Sorotos
We present a case report of a 10-year-old girl diagnosed with Ewing sarcoma treated with intra-articular wide resection of the right femur and reconstruction with a series-connected double-barrel bilateral vascularized fibula graft (db-BVFG), including fibular head for articulation with the acetabulum of the pelvic bone and preservation of the epiphyseal growth plates for eventual limb growth. No postoperative complications were observed and bone union was achieved with fibular graft hypertrophy, allowing for full weight bearing...
February 8, 2018: Microsurgery
Reghu Kesavapillai Sukumaran, Binitha Rajeshwari, Subin Sugath, S Guruprasad Chellappan, Priyakumari Thankamony, Kusumakumary Parukuttyamma
Background: Osteosarcoma (OS) is the most common primary malignant tumor of bone. The survival of OS patients has steadily improved from <20% in the early 20th century to around 70% with current treatment. There are very few studies in pediatric OS from India analyzing various aspects of the disease. This study focuses on the clinical profile, treatment options, and their complications and survival outcomes in pediatric osteosarcoma (OS) patients. Materials and Methods: This was a retrospective observational study which included pediatric patients <14 years of age, with newly diagnosed OS confirmed by histological diagnosis...
January 2018: Indian Journal of Orthopaedics
Marguerite T Parisi, Ramesh S Iyer, A Luana Stanescu
The introduction of diphosphonates in the 1970s revolutionized not only nuclear medicine but musculoskeletal imaging as well, providing functional assessment of entities such as osteomyelitis, trauma, and osseous metastatic disease. Although rarely the first-line imaging modality used today, nuclear medicine procedures continue to play a pivotal role in the evaluation of musculoskeletal diseases in children, providing whole-body assessment of disease involvement. More recently, the introduction of technologies such as single-photon emission computed tomography/computed tomography (SPECT/CT), as well as newer positron-emitting tracers such as 18 fluorine-fluorodeoxyglucose and sodium 18 F-fluorine, particularly when combined with CT (positron emission tomography/CT), have injected new life into the older established techniques and expanded the application of nuclear medicine imaging into new arenas...
February 2018: Seminars in Musculoskeletal Radiology
Roberta Bortolozzi, Elena Mattiuzzo, Luca Trentin, Benedetta Accordi, Giuseppe Basso, Giampietro Viola
Dysregulation of the cyclin D1-CDK4/CDK6 complex is frequently observed in almost all human cancer and contributes to aberrant cell proliferation and consequent tumorigenesis. Although many reports described the importance of CDK4/CDK6 in different set of human tumors, only few studies have been performed on leukemia. By gene expression analysis performed in a cohort of childhood patients affected by B-acute lymphoblastic leukemia (B-ALL) we found that both CDK4 and CDK6 are highly expressed. Moreover, Reverse Phase Protein Array (RPPA) analysis showed that cyclin D1 levels are higher in patients undergoing relapse...
February 3, 2018: Biochemical Pharmacology
Karun V Sharma, Pavel S Yarmolenko, Avinash Eranki, Ari Partanen, Haydar Celik, AeRang Kim, Matthew Oetgen, Peter C W Kim
Magnetic resonance imaging-guided high-intensity focused ultrasound (MR-HIFU) is a novel technology that integrates magnetic resonance imaging with therapeutic ultrasound. This unique approach provides a completely noninvasive method for precise thermal ablation of targeted tissues with real-time imaging feedback. Over the past 2 decades, MR-HIFU has shown clinical success in several adult applications ranging from treatment of painful bone metastases to uterine fibroids to prostate cancer and essential tremor...
February 2018: Topics in Magnetic Resonance Imaging: TMRI
Roberto Blanco Sequeiros, Juha-Jaakko Sinikumpu, Risto Ojala, Jyri Järvinen, Jan Fritz
Minimally invasive procedures play a crucial role in the diagnosis and treatment of many pediatric musculoskeletal conditions. Although computed tomography and fluoroscopy are commonly used for image guidance, the associated exposure to ionizing radiation is especially concerning in pediatric patients. Ultrasonography may be used successfully in a subset of interventions, but it is often not useful for complex, deep, and osseous targets. Interventional magnetic resonance imaging (iMRI) facilitates targeting and treatment of musculoskeletal lesions at many locations with high accuracy due to its excellent tissue contrast...
February 2018: Topics in Magnetic Resonance Imaging: TMRI
Melissa A LoPresti, Jonathan N Sellin, Franco DeMonte
Objectives  To review developmental surgical anatomy and technical nuances related to pediatric skull base surgery. Design  Retrospective, single-center case series with literature review. Setting  MD Anderson Cancer Center. Participants  Patients undergoing pediatric skull base surgery. Main Outcome Measures  Review developmental anatomy of the pediatric skull base as it relates to technical nuance of various surgical approaches and insight gained from a 25-year institutional experience with this unique patient population...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
Andrew B Smitherman, Danielle Mohabir, Tania M Wilkins, Julie Blatt, Hazel B Nichols, Stacie B Dusetzina
OBJECTIVE: To describe the patterns of prescription drug use among child and adolescent survivors of cancer in the early post-therapy period compared with matched peers without a cancer history. STUDY DESIGN: Using the MarketScan commercial insurance claims database, we performed a retrospective cohort study identifying survivors of pediatric (0-21 years of age at diagnosis) leukemia, lymphoma, central nervous system, bone, or gonadal cancers who completed therapy from 2000 to 2011 and remained insured for 3 years post-therapy...
January 30, 2018: Journal of Pediatrics
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