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Pediatric sarcoma

H Wilkens, S Konstantinides, I Lang, A C Bunck, M Gerges, F Gerhardt, A Grgic, C Grohé, S Guth, M Held, J Hinrichs, M M Hoeper, W Klepetko, T Kramm, U Krüger, M Lankeit, B C Meyer, K M Olsson, H-J Schäfers, M Schmidt, H J Seyfarth, S Ulrich, C B Wiedenroth, E Mayer
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH), but also other significant subgroups of pulmonary hypertension (PH). In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany to discuss open and controversial issues surrounding the practical implementation of the European Guidelines...
October 2016: Deutsche Medizinische 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
Maureen O'Sullivan
Since its foundation by remarkably talented and insightful individuals, prominently including Pepper Dehner, pediatric soft tissue tumor pathology has developed at an immense rate. The morphologic classification of tumoral entities has extensively been corroborated, but has also evolved with refinement or realignment of these classifications, through accruing molecular data, with many derivative ancillary diagnostic assays now already well-established. Tumors of unclear histogenesis, classically morphologically undifferentiated, are prominent amongst pediatric sarcomas, however, the classes of undifferentiated round- or spindle-cell-tumors-not-otherwise-specified are being dismantled gradually with the identification of their molecular underpinnings...
September 5, 2016: Seminars in Diagnostic Pathology
Alicia M Waters, Laura L Stafman, Evan F Garner, Smitha Mruthyunjayappa, Jerry E Stewart, Gregory K Friedman, Jennifer M Coleman, James M Markert, G Yancey Gillespie, Elizabeth A Beierle
Rhabdomyosarcoma (RMS), a tumor of skeletal muscle origin, is the most common sarcoma of childhood. Despite multidrug chemotherapy regimens, surgical intervention, and radiation treatment, outcomes remain poor, especially in advanced disease, and novel therapies are needed for the treatment of these aggressive malignancies. Genetically engineered oncolytic viruses, such as herpes simplex virus-1 (HSV), are currently being explored as treatments for pediatric tumors. M002, an oncolytic HSV, has both copies of the γ134...
October 2016: Translational Oncology
Brian Y Chan, Kara G Gill, Susan L Rebsamen, Jie C Nguyen
The bone marrow is one of the largest organs in the body and is visible in every magnetic resonance (MR) imaging study. It is composed of a combination of hematopoietic red marrow and fatty yellow marrow, and its composition changes throughout life in response to normal maturation (red to yellow conversion) and stress (yellow to red reconversion). MR imaging is highly sensitive for detection of altered marrow signal intensity, and the T1-weighted spin-echo sequence provides the most robust contrast between yellow marrow and disease...
October 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Joel C Thompson, Gary M Woods, Michael A Arnold, Charles Elmaraghy, Samir B Kahwash, Timothy P Cripe, Bhuvana A Setty
Pediatric soft tissue sarcomas of the oral/maxillofacial region are rare neoplasms that present significant difficulty with respect to treatment and local control measures. We report four cases of pediatric oral/maxillofacial soft tissue sarcomas from our tertiary care pediatric hospital and emphasize the rarity of these malignancies and the challenges encountered in treating these lesions, and suggest areas for further research. We conclude that multimodal therapy and interdisciplinary cooperation are paramount to successful management of these lesions...
May 2016: Case Reports in Oncology
Jason A Jarzembowski
Dr. Louis "Pepper" Dehner is an internationally renowned surgical pathologist, especially in the subspecialty of pediatric pathology. Although his clinical and academic expertise are broad, with over 400 published articles, some of his most intriguing contributions have been in the area of pediatric renal and genitourinary pathology. This review focuses on the entities in these following organ systems where he has focused his efforts: malignant rhabdoid tumor, renal medullary carcinoma, Ewing sarcoma/peripheral neuroectodermal tumor, and the DICER1-related lesions cystic nephroma, embryonal rhabdomyosarcoma of the uterine cervix, and Sertoli-Leydig cell tumor...
August 31, 2016: Seminars in Diagnostic Pathology
Michele N Edison, M Cody O'Dell, Haley P Letter, Kurt Scherer, Jennifer L Williams
An 8-year-old girl presented with bilateral breast masses and was subsequently diagnosed with juvenile myelomonocytic leukemia. Juvenile myelomonocytic leukemia is a rare myelodysplastic syndrome that typically presents in boys younger than 3 years of age with splenomegaly, lymphadenopathy and skin findings. Bilateral breast masses in a child are rare and, as such, present a diagnostic dilemma due to the relative paucity of cases in the literature. We present a case of granulocytic sarcoma of the breasts in a patient with juvenile myelomonocytic leukemia...
October 7, 2016: Pediatric Radiology
Misa Yoshida, Satoshi Hamanoue, Masafumi Seki, Mio Tanaka, Kenichi Yoshida, Hiroaki Goto, Seishi Ogawa, Junko Takita, Yukichi Tanaka
Anaplastic sarcoma of the kidney (ASK) is a tumor found in the pediatric age group and shows many histopathological similarities to pleuropulmonary blastoma (PPB). We present a 12-year-old girl diagnosed with ASK and, three years later, with thyroid follicular carcinoma (TFC) with DICER1 abnormalities. Germline insertion/deletion (p.G1809_S1814delinsA) and independent somatic mutations (p.E1705K in ASK, p.E1813D in TFC) were identified. All of these abnormalities are in the catalytic domain of RNase IIIb. Single-nucleotide polymorphism genotyping microarray revealed independent copy number alterations (trisomy 8, monosomy 10, loss of 17p and partial gain of 17q in ASK; trisomy 5 and partial loss of Xq in TFC)...
September 30, 2016: Human Pathology
Amy Sherborne, Vincent Lavergne, Katharine Yu, Leah Lee, Philip Davidson, Tali Mazor, Ivan V Smirnov, Andrew E Horvai, Mignon Loh, Steven G Dubois, Robert Goldsby, Joseph P Neglia, Sue Hammond, Leslie L Robison, Rosanna Wustrack, Joseph F Costello, Alice Nakamura, Kevin M Shannon, Smita Bhatia, Jean L Nakamura
PURPOSE: Second malignant neoplasms (SMNs) are severe late complications that occur in pediatric cancer survivors exposed to radiotherapy and other genotoxic treatments. To characterize the mutational landscape of treatment-induced sarcomas and to identify candidate SMN-predisposing variants we analyzed germline and SMN tumor samples from pediatric cancer survivors. EXPERIMENTAL DESIGN: We performed whole exome sequencing (WES) and RNA sequencing on radiation-induced sarcomas arising from two pediatric cancer survivors...
September 28, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Kiki M G J Wigny, Wendy van Dorp, Anne-Lotte L F van der Kooi, Yolanda B de Rijke, Andrica C H de Vries, Marij Smit, Saskia M F Pluijm, Erica L T van den Akker, Rob Pieters, Joop S E Laven, Marry M van den Heuvel-Eibrink
STUDY QUESTION: Are Inhibin B and testosterone levels reduced in boys with newly diagnosed cancer prior to therapy? SUMMARY ANSWER: Pretreatment serum levels of Inhibin B and testosterone are significantly reduced in boys with newly diagnosed cancer, compared to reference values. WHAT IS ALREADY KNOWN: Disease-related gonadal impairment has been demonstrated in girls and young women diagnosed with cancer, prior to therapy. STUDY DESIGN, SIZE, DURATION: We conducted a descriptive study in boys newly diagnosed with cancer between January 2006 and February 2014...
September 28, 2016: Human Reproduction
C L Tinkle, I Fernandez-Pineda, Y Li, C H Hua, M D Neel, S C Kaste, T E Merchant, L E Kun, S L Spunt, M Krasin
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Corrado Spatola, Alessandra Tocco, Roberto Milazzotto, Antonio Pagana, Ilenia Chillura, Roberta Bevilacqua, Carmelo Militello, Vincenzo Salamone, Luigi Raffaele, Marcello Migliore, Giuseppe Privitera
The management of pediatric thoracic synovial sarcoma remains a matter of debate in clinical oncology, especially as regard to the local control of the disease. Surgery remains the gold standard, while the role and timing of radiotherapy is still controversial. We report a 14-year-old male, who has not received proper treatment at the time of diagnosis and initial management. Intensity-modulated irradiation was performed only at relapse, as a salvage treatment and, at 10-month follow-up, the young patient was free from relapse, without significant acute and subacute toxicity...
September 23, 2016: Future Oncology
N H Alotaibi, Aurélie Bornand, Nicolas Dulguerov, M Becker, Pavel Dulguerov
INTRODUCTION: Synovial sarcoma (SS) is uncommon high grade soft tissue sarcoma, accounting for less than 10% of all head and neck sarcomas. Also, about 10% of SS occur within the Head & Neck. In the pediatric population, SS is an extremely rare head & neck malignancy. PRESENTATION OF CASE: We present a case of sixteen years old boy diagnosed with SS situated of the hypopharynx treated by surgical excision and post operative radio-chemotherapy. DISCUSSION: This anatomical location brings additional functional challenges (swallowing, phonation, respiration), especially in the pediatric population...
September 3, 2016: International Journal of Surgery Case Reports
Michela Casanova, Eleonora Basso, Chiara Magni, Luca Bergamaschi, Stefano Chiaravalli, Roberto Carta, Elisa Tirtei, Maura Massimino, Franca Fagioli, Andrea Ferrari
Pazopanib is an oral multikinase inhibitor that has proved effective in adults treated for relapsing soft tissue sarcoma and synovial sarcoma in particular. Two cases are reported here of pediatric patients with pretreated relapsing synovial sarcoma whose tumors showed a prolonged response to pazopanib given on compassionate grounds. These results suggest that new agents found effective in adult patients might achieve similar results in adolescents with the same disease. Facilitating the availability of new drugs for children and adolescents is a major challenge for pediatric oncologists...
August 25, 2016: Tumori
Sabine Stegmaier, Ivo Leuschner, Christopher Poremba, Ruth Ladenstein, Bernarda Kazanowska, Gustaf Ljungman, Monika Scheer, Bernd Blank, Stefan Bielack, Thomas Klingebiel, Ewa Koscielniak
BACKGROUND: The aim of our analysis was the evaluation of the prognostic impact of SYT-SSX fusion status and histological grading in synovial sarcoma (SS) of children and adolescents in the context of the consistent multimodal treatment strategy of the CWS (Cooperative Weichteilsarkom Studie; Cooperative Soft Tissue Sarcoma Study Group) and in comparison with other risk factors. PROCEDURE: Between 1986 and 2006, out of 243 patients with SS, tumor samples from 84 patients with localized disease were available for RT-PCR analysis...
September 13, 2016: Pediatric Blood & Cancer
Hernán Correa
Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by a germline mutation of the TP53 gene on chromosome 17p13.1. It has an autosomal dominant pattern of inheritance with high penetrance. These patients have a very high lifetime cumulative risk of developing multiple malignancies and have a strong family history of early-onset malignancies. The protein p53, encoded by TP53, has a complex set of genome-preserving functions initiated during episodes of cellular stress and DNA damage. In LFS, TP53 gene mutations cause the loss of function of p53, leading to downstream events permissive for development of various malignancies throughout life...
June 2016: Journal of Pediatric Genetics
Christine M Heske, Arnulfo Mendoza, Leah D Edessa, Joshua T Baumgart, Sunmin Lee, Jane Trepel, David A Proia, Len Neckers, Lee J Helman
Long-term survival in patients with metastatic, relapsed, or recurrent Ewing sarcoma and rhabdomyosarcoma is dismal. Irinotecan, a topoisomerase 1 inhibitor, has activity in these sarcomas, but due to poor bioavailability of its active metabolite (SN-38) has had limited clinical efficacy. In this study we have evaluated the efficacy and toxicity of STA-8666, a novel drug conjugate which uses an HSP90 inhibitor to facilitate intracellular, tumor-targeted delivery of the topoisomerase 1 inhibitor SN-38, thus preferentially delivering and concentrating SN-38 within tumor tissue...
September 6, 2016: Oncotarget
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
Andrea Ferrari, Uta Dirksen, Stefan Bielack
The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition...
2016: Progress in Tumor Research
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