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Pediatric bone tumor

Howard Y Park, Sara K Yang, William L Sheppard, Vishal Hegde, Stephen D Zoller, Scott D Nelson, Noah Federman, Nicholas M Bernthal
Aneurysmal bone cysts (ABCs) are benign bone lesions arising predominantly in the pediatric population that can cause local pain, swelling, and pathologic fracture. Primary lesions, which constitute roughly two thirds of all ABCs, are thought to be neoplastic in nature, with one third of ABCs arising secondary to other tumors. Diagnosis is made with various imaging modalities, which exhibit characteristic features such as "fluid-fluid levels," although biopsy is critical, as telangiectatic osteosarcoma cannot be excluded based on imaging alone...
October 24, 2016: Current Reviews in Musculoskeletal Medicine
Elisabeth Sterrer, Franz Windisch, Kurt Frey, Klaus Rettensteiner, Benjamin Loader
BACKGROUND: Middle ear adenoma with neuroendocrine differentiation (MEA-ND), also known as carcinoid tumor of the middle ear, is an extremely rare neoplasm, especially when located within the tympanic cavity. To the best of our knowledge, this represents the first pediatric case of a MEA-ND described in Europe. Excluding the present case, only three other pediatric cases of this disease have been published globally. CASE REPORT: We report on a 15-year-old female patient who presented with conductive hearing loss of the right ear...
October 19, 2016: Wiener Klinische Wochenschrift
Petra Balogh, Rita Bánusz, Monika Csóka, Zsófia Váradi, Edit Varga, Zoltán Sápi
BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin and comprises the largest category of soft-tissue sarcomas both in children and adolescents. From a pediatric oncology point of view, RMS has traditionally been classified into alveolar (ARMS) and embryonal (ERMS) subtypes. The anatomical localization of the tumor may vary, but commonly involve the head/neck regions, male and female urogenital tract or the trunk and extremities. CASE PRESENTATION: Here, we report two challenging cases involving 17- and 9-years-olds males where diffuse and multiplex bone lesions suggested either a hematological disease or a primary bone tumor (mesenchymal chondrosarcoma)...
October 18, 2016: Diagnostic Pathology
Joseph Frankl, Cassi Grotepas, Baldassare Stea, G Michael Lemole, Alexander Chiu, Rihan Khan
BACKGROUND: Chordoma is a rare invasive bone tumor that may occur anywhere along the neuraxis. A total of three primary histological varieties have been identified: conventional, chondroid, and dedifferentiated. CASE PRESENTATION: We report a case of an 8-year-old white girl who presented with conventional chordoma, was treated with surgical resection and mixed proton and photon beam therapy, and had a recurrence in the resection cavity 2.5 years later with dedifferentiated morphology...
October 12, 2016: Journal of Medical Case Reports
Ting Yang, Qiaoxian Lin, Jinhua Ren, Ping Chen, Xiaohong Yuan, Xiaofeng Luo, Tingbo Liu, Jing Zheng, Zhihong Zheng, Xiaoyun Zheng, Xinji Chen, Langhui Zhang, Hao Zheng, Zaisheng Chen, Xueling Hua, Shaohua Le, Jian Li, Zhizhe Chen, Jianda Hu
Haplo-HSCT has been used when HLA-matched siblings are not available. Conditioning regimens aim to reduce tumor burden prior to HSCT and provide sufficient immunoablation. We report the outcome of haplo-HSCT in 63 consecutive patients from 2/2013 to 12/2015 (19 females/44 males) with high-risk or relapsed/refractory hematological malignancies (n=29-AML; 8-sAML; 19-ALL; 5-advanced-MDS; 2-CML-BC). Median age was 20 years (range: 1.1-49). Twenty-one patients achieved remission prior to transplant, while 42 did not...
October 1, 2016: Oncotarget
Erich M Gauger, Lauren L Casnovsky, Erica J Gauger, Deborah C Bohn, Ann E Van Heest
This study reviewed the clinical history and management of acquired growth arrest in the upper extremity in pediatric patients. The records of all patients presenting from 1996 to 2012 with radiographically proven acquired growth arrest were reviewed. Records were examined to determine the etiology and site of growth arrest, management, and complications. Patients with tumors or hereditary etiology were excluded. A total of 44 patients (24 boys and 20 girls) with 51 physeal arrests who presented at a mean age of 10...
September 29, 2016: Orthopedics
Tymon Skadorwa, Bogdan Ciszek
BACKGROUND/AIMS: Tumors of the cranial vault occur at every age of childhood. Although they are mostly benign lesions, their symptomatology is variable and requires extended diagnostics. The choice of therapeutic strategy strongly depends on histopathological diagnosis, and therefore surgical excision is the elective treatment in such cases. Despite several published papers, the literature still lacks reliable clinical characteristics regarding this heterogeneous group of lesions in pediatric patients...
September 27, 2016: Pediatric Neurosurgery
Leo Kager, Gevorg Tamamyan, Stefan Bielack
High-grade osteosarcomas are the most common primary malignant tumors of bone. With complete surgical resection and multi-agent chemotherapy up to 70% of patients with high-grade osteosarcomas and localized extremity tumors can become long-term survivors. The prognosis, however, is poor for patients with nonresectable, primary metastatic or relapsed disease. Outcome is essentially unchanged for three decades. Herein, we describe selected novel insights into the genomics, biology and immunology of the disease and discuss selected strategies, which hold promise to overcome the current stagnation in the therapeutic success in childhood osteosarcoma...
September 21, 2016: Future Oncology
Amrut Borade, Harish Kempegowda, Meagan Fernandez, Daniel S Horwitz
Successful results of osteoarticular allografts in reconstruction of periarticular bone defect after tumor resection encouraged its utilization in post-traumatic defects. Here we describe a case of post-traumatic skeletal defect in a 4 year-old girl treated with osteoarticular allograft reconstruction. Due to severity of the associated soft tissue injury and contamination at presentation staged treatment with antibiotic spacer followed by the reconstruction was carried out. At the end of one year the patient achieved 'Musculoskeletal tumor society' functional score of 27 points and radiographic score of 93%...
September 6, 2016: Injury
Domenico Corica, Claudio Romano
The incidence of inflammatory bowel disease (IBD) has increased steadily worldwide, both in adult and in children; approximately 25% of IBD patients are diagnosed before the age of 18. The natural history of IBD is usually more severe in children than in adults, and can be associated with linear growth impairment, delayed puberty onset, reduced bone mass index, malnutrition, and the need for surgery. Biological therapies, especially blocking tumor necrosis factor-α (TNFα), have radically modified the treatment strategies and disease course of IBD in children...
September 15, 2016: Journal of Clinical Gastroenterology
Fabio Morandi, Sarah Pozzi, Barbara Carlini, Loredana Amoroso, Vito Pistoia, Maria Valeria Corrias
The role of nonclassical HLA-class Ib molecules HLA-G and HLA-E in the progression of Neuroblastoma (NB), the most common pediatric extracranial solid tumor, has been characterized in the last years. Since BM infiltration by NB cells is an adverse prognostic factor, we have here analyzed for the first time the concentration of soluble (s)HLA-G and HLA-E in bone marrow (BM) plasma samples from NB patients at diagnosis and healthy donors. sHLA-G and sHLA-E are present in BM plasma samples, and their levels were similar between NB patients and controls, thus suggesting that these molecules are physiologically released by resident or stromal BM cell populations...
2016: Journal of Immunology Research
Sue Creviston Kaste, Scott E Snyder, Monika L Metzger, John T Sandlund, Scott Howard, Matthew Krasin, Barry L Shulkin
BACKGROUND: Methionine transport across plasma membranes occurs via large amino acid transporter ( LAT1), which is overexpressed in malignant cells leading to tracer accumulation within tumors. We investigated the uptake of (11)C-methionine in children and young adults newly diagnosed with Hodgkin lymphoma (HL) or non-Hodgkin lymphoma (NHL) and compared the biodistribution of (11)C-methionine PET-CTs ((11)C- METPET) to that of (18)F-FDG PET-CT ((18)F-FDGPET). METHODS & MATERIALS: Conducted under an IND, we prospectively enrolled patients with newly diagnosed HL (N = 19) and NHL (N = 2) onto the IRB-approved investigation of (11)C-METPET...
September 8, 2016: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Anant Ramaswamy, Bharat Rekhi, Sameer Bakhshi, Sachin Hingmire, Manish Agarwal
Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS), chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH) and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation...
July 2016: South Asian Journal of Cancer
Sohum K Desai, Da'Marcus Baymon, Eric Sieloff, Kenneth Maynard, Marc Moisi, Achal P Patel, Joel T Patterson
AIMS: Lateral transtemporal approaches are useful for addressing lesions located ventral to the brainstem, especially when the pathologic diagnosis of the tumor dictates that a gross or near total resection improves outcomes. One approach, the presigmoid approach receives little attention in the pediatric population thus far. We sought to characterize morphometric changes, particularly the clival depth and the petroclival Cobb angle, that occur in the temporal bones of children and draw implications about doing a presigmoid approach in children...
April 2016: Journal of Pediatric Neurosciences
Assaf Kadar, Geffen Kleinstern, Mohamed Morsy, Endre Soreide, Steven L Moran
BACKGROUND: Multiple enchodromatosis of bone, termed Ollier's disease, or Maffucci syndrome when associated with hemangiomas, is a rare disease that can affect the pediatric hand. This condition often causes a finger mass, deformity, pain and possible pathologic fractures, and has been associated with malignant transformation to chondrosarcoma. The aim of our study is to describe the long-term sequela of multiple enchondromatosis of the hand in the pediatric population, specifically the rates of malignant transformation, tumor recurrence, rates of pathologic fracture, and phalangeal growth arrest...
September 3, 2016: Journal of Pediatric Orthopedics
Maria Nieto-Soler, Isabel Morgado-Palacin, Vanesa Lafarga, Emilio Lecona, Matilde Murga, Elsa Callen, Daniel Azorin, Javier Alonso, Andres J Lopez-Contreras, Andre Nussenzweig, Oscar Fernandez-Capetillo
Ewing sarcomas (ES) are pediatric bone tumors that arise from a driver translocation, most frequently EWS/FLI1. Current ES treatment involves DNA damaging agents, yet the basis for the sensitivity to these therapies remains unknown. Oncogene-induced replication stress (RS) is a known source of endogenous DNA damage in cancer, which is suppressed by ATR and CHK1 kinases. We here show that ES suffer from high endogenous levels of RS, rendering them particularly dependent on the ATR pathway. Accordingly, two independent ATR inhibitors show in vitro toxicity in ES cell lines as well as in vivo efficacy in ES xenografts as single agents...
August 26, 2016: Oncotarget
Romain Guiho, Kevin Biteau, Giulia Grisendi, Julien Taurelle, Mathias Chatelais, Malika Gantier, Dominique Heymann, Massimo Dominici, Françoise Redini
Ewing sarcoma (EWS) is the second most frequent pediatric malignant bone tumor. EWS patients have not seen any major therapeutic progress in the last 30 years, in particular in the case of metastatic disease, which requires new therapeutic strategies. The pro-apoptotic cytokine TNF-Related Apoptosis Inducing Ligand (TRAIL) can selectively kill tumor cells while sparing normal cells, making it a promising therapeutic tool in several types of cancer. However, certain EWS cell lines appear resistant to recombinant human (rh) TRAIL-induced apoptosis...
December 15, 2016: International Journal of Cancer. Journal International du Cancer
G Lakshmi Prasad, Kanthilatha Pai
Hemangiomas are benign, slow-growing tumors composed of sinusoidal blood vessels. Skeletal hemangiomas are uncommon and are mostly vertebral, followed by cranial in location. Cranial hemangiomas are very rarely encountered in children. Authors report a 12-year girl who presented with a painless enlarging mass over the parietal scalp for 3 months. Imaging revealed a left parietal intraosseous lytic mass with a sunburst appearance. Enbloc removal and cranioplasty was performed, and histopathology was suggestive of hemangioma...
August 25, 2016: Neurosurgical Review
Karen M Chisholm, Robert S Ohgami, Brent Tan, Robert P Hasserjian, Olga K Weinberg
Primary lymphoma of bone (PLB) accounts for 3-7% of primary neoplasms of bone and must be distinguished from more common bone tumors in the pediatric population such as osteosarcoma, Ewing sarcoma, and other small round blue cell tumors. In this study, pathology databases from four institutions were queried for PLB in individuals 1-21 years old. A total of 54 cases of PLB were identified, including 41 diffuse large B cell lymphomas (DLBCL, 76%), 8 B lymphoblastic lymphomas (BLL, 15%), 3 anaplastic large cell lymphomas (ALCL, 6%), and 2 low grade follicular lymphomas (FL, 4%)...
August 20, 2016: Human Pathology
Peter Kaatsch, Jill Strothotte, Cornelia Becker, Stefan Bielack, Uta Dirksen, Maria Blettner
BACKGROUND: Malignant bone tumors are a rare group of childhood cancer. MATERIALS AND METHODS: This study describes incidence rates, survival probabilities, and time trends for 1831 children below 15 years of age with a bone tumor registered at the population-based German Childhood Cancer Registry between 1987 and 2011. RESULTS: Overall age-standardized annual incidence rate (ASR) was 5.5 per million. Osteosarcomas (ASR 2.8) and Ewing tumors (ASR 2...
August 23, 2016: Acta Oncologica
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