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Pediatric sarcoma

Maha A T Elsebaie, Mohamed Amgad, Ahmed Elkashash, Ahmed Saber Elgebaly, Gehad Gamal E L Ashal, Emad Shash, Zeinab Elsayed
This is the second-largest retrospective analysis addressing the controversy of whether adult rhabdomyosarcoma (RMS) should be treated with chemotherapy regimens adopted from pediatric RMS protocols or adult soft-tissue sarcoma protocols. A comprehensive database search identified 553 adults with primary non-metastatic RMS. Increasing age, intermediate-risk disease, no chemotherapy use, anthacycline-based and poor chemotherapy response were significant predictors of poor overall and progression-free survival...
June 19, 2018: Scientific Reports
Jidi Gao, Erika Chow, Aishatu Aloma, Payal Gupta
Primitive neuroectodermal tumor is a high-grade malignant tumor originating from the neural crest and neuroectoderm, which can be subdivided into central and peripheral categories. Peripheral primitive neuroectodermal tumor is thought to be identical to Ewing's sarcoma, and falls under a broader category of Ewing's sarcoma family of tumors. Very rarely, it may present without osseous involvement, known as extraosseous Ewing's sarcoma. Here we present a case of a 38-year-old woman, who presented with several-month history of a slow-growing chest wall mass, initially thought to be a breast mass...
April 2018: Radiology Case Reports
Jagdish P Meena, Menka Yadav, Aditya K Gupta, Prashant Ramteke, Priyanka Naranje, Rachna Seth
Extramedullary leukemia is common in pediatric acute myeloid leukemia (AML) and occurs as a solid tumor (myeloid sarcoma). We report a case of a child who presented with acute onset of paraparesis and found to have intracranial and paravertebral mass; subsequently, he was diagnosed as having AML on tissue biopsy. He was started on AML treatment protocol, and later he was in remission and myeloid sarcoma got cleared from intracranial and paravertebral region. Timely diagnosis and initiation of treatment are essential to improve survival in such cases...
January 2018: Journal of Pediatric Neurosciences
Giulia Bertolini, Luca Bergamaschi, Andrea Ferrari, Salvatore L Renne, Paola Collini, Cecilia Gardelli, Marta Barisella, Giovanni Centonze, Stefano Chiaravalli, Cinzia Paolino, Massimo Milione, Maura Massimino, Michela Casanova, Patrizia Gasparini
BACKGROUND: Rhabdomyosarcomas (RMSs) are the most frequent soft tissue sarcoma in children and adolescents, defined by skeletal muscle differentiation and the status of FOXO1 fusions. In pediatric malignancies, in particular RMS, scant and controversial observations are reported about PD-L1 expression as a putative biomarker and few immune checkpoint clinical trials are conducted. METHODS: PD-L1 assessment was evaluated by immunohistochemistry (IHC) utilizing two anti-PDL1 antibodies, in a pilot cohort of 25 RMS...
June 13, 2018: BMC Cancer
Francisco Giner, José A López-Guerrero, Antonio Fernández-Serra, Isidro Machado, Empar Mayordomo-Aranda, Amando Peydró-Olaya, Antonio Llombart-Bosch
Background Ewing sarcoma (EWS) is the second most common bone cancer in pediatric patients. Angiogenesis is a major factor for tumor growth and metastasis. Our aim was to carry out a histological, immunohistochemical, and molecular characterization of the neovascularization established between xenotransplanted tumors and the host during the initial phases of growth in nude mice in three angiogenesis experiments (ES2, ES3, and ES4). Methods The original human EWS were implanted subcutaneously on the backs of three nude mice...
January 1, 2018: Pediatric and Developmental Pathology
Alejandro Manzanares, Camilo A Restrepo-Perdomo, Gaia Botteri, Helena Castillo-Ecija, Guillem Pascual-Pasto, Francesc Cano, Laura Garcia-Alvarez, Carles Monterrubio, Bonaventura Ruiz, Manuel Vazquez-Carrera, Mariona Suñol, Jaume Mora, Jose A Tornero, Alejandro Sosnik, Angel M Carcaboso
Delivery of chemotherapy in the surgical bed has shown preclinical activity to control cancer progression upon subtotal resection of pediatric solid tumors, but whether this new treatment is safe for tumor-adjacent healthy tissues remains unknown. Here, Wistar rats are used to study the anatomic and functional impact of electrospun nanofiber matrices eluting SN-38-a potent chemotherapeutic agent-on several body sites where pediatric tumors such as neuroblastoma, Ewing sarcoma, and rhabdomyosarcoma arise. Blank and SN-38-loaded matrices embracing the femoral neurovascular bundle or in direct contact with abdominal viscera (liver, kidney, urinary bladder, intestine, and uterus) are placed...
June 11, 2018: Advanced Healthcare Materials
Joana Ruivo Rodrigues, Manuel João Brito, Rui Pedro Faria Pais, Sílvia Carvalho
Myeloid sarcoma (MS), granulocytic sarcoma or chloroma, is defined as a localized extramedullary mass of blasts of granulocytic lineage with or without maturation, occurring outside the bone marrow. MS can be diagnosed concurrently with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). The authors report a case of sacral MS occurring as a relapse of myeloid leukemia in a 5-year-old girl who was taken to the emergency department with radiculopathy symptoms.
2018: Case Reports in Radiology
Y H Ma, P Huang, H Q Gao, W C Li
No abstract text is available yet for this article.
June 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Christian Koelsche, Martin Mynarek, Daniel Schrimpf, Luca Bertero, Jonathan Serrano, Felix Sahm, David E Reuss, Yanghao Hou, Daniel Baumhoer, Christian Vokuhl, Uta Flucke, Iver Petersen, Wolfgang Brück, Stefan Rutkowski, Sandro Casavilca Zambrano, Juan Luis Garcia Leon, Rosdali Yesenia Diaz Coronado, Manfred Gessler, Oscar M Tirado, Jaume Mora, Javier Alonso, Xavier Garcia Del Muro, Manel Esteller, Dominik Sturm, Jonas Ecker, Till Milde, Stefan M Pfister, Andrey Korshunov, Matija Snuderl, Gunhild Mechtersheimer, Ulrich Schüller, David T W Jones, Andreas von Deimling
Patients with DICER1 predisposition syndrome have an increased risk to develop pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, and several other rare tumor entities. In this study, we identified 22 primary intracranial sarcomas, including 18 in pediatric patients, with a distinct methylation signature detected by array-based DNA-methylation profiling. In addition, two uterine rhabdomyosarcomas sharing identical features were identified. Gene panel sequencing of the 22 intracranial sarcomas revealed the almost unifying feature of DICER1 hotspot mutations (21/22; 95%) and a high frequency of co-occurring TP53 mutations (12/22; 55%)...
June 7, 2018: Acta Neuropathologica
Clayton B Hess, Daniel J Indelicato, Arnold C Paulino, William F Hartsell, Christine E Hill-Kayser, Stephanie M Perkins, Anita Mahajan, Nadia N Laack, Ralph P Ermoian, Andrew L Chang, Suzanne L Wolden, Victor S Mangona, Young Kwok, John C Breneman, John P Perentesis, Sara L Gallotto, Elizabeth A Weyman, Benjamin V M Bajaj, Miranda P Lawell, Beow Y Yeap, Torunn I Yock
Background/objectives: The Pediatric Proton Consortium Registry (PPCR) was established to expedite proton outcomes research in the pediatric population requiring radiotherapy. Here, we introduce the PPCR as a resource to the oncology community and provide an overview of the data available for further study and collaboration. Design/methods: A multi-institutional registry of integrated clinical, dosimetric, radiographic, and patient-reported data for patients undergoing proton radiation therapy was conceived in May 2010...
2018: Frontiers in Oncology
Jacek Wachowiak, Alicja Chybicka, Jerzy R Kowalczyk, Mariusz Wysocki, Jolanta Goździk, Ewa Gorczyńska, Krzysztof Kałwak, Jan Styczyński, Katarzyna Drabko, Anna Pieczonka
The purpose of the survey was to evaluate the development and current use of hematopoietic stem cell transplantation (HSCT) in Poland between 1989-2016. The data for analysis (indication, number of performed HSCT, HSCT type, donor type, and stem cell source, year) have been collected annually using a standardized form. In Poland, between 1989-2016, the number of pediatric transplant beds grew from one to 40 and number and rate of transplants increased annually from 1/year (0.8/10 million) to 186/year (248/10 million)...
May 16, 2018: Transfusion and Apheresis Science
Yasushi Ishida, Miho Maeda, Souichi Adachi, Hiroko Inada, Hiroshi Kawaguchi, Hiroki Hori, Atsushi Ogawa, Kazuko Kudo, Chikako Kiyotani, Hiroyuki Shichino, Takeshi Rikiishi, Ryoji Kobayashi, Maho Sato, Jun Okamura, Hiroaki Goto, Atsushi Manabe, Shinji Yoshinaga, Dongmei Qiu, Junichiro Fujimoto, Tatsuo Kuroda
BACKGROUNDS: Multidisciplinary therapy has increased the risk of subsequent late effects, but detailed analyses on secondary cancers in childhood cancer survivors (CCSs) are limited in Asian countries. METHODS: This was a retrospective cohort study comprising 10,069 CCSs who were diagnosed between 1980 and 2009 across 15 Japanese hospitals. We conducted secondary analyses to estimate the incidence of secondary cancer according to each primary malignancy and to elucidate the association between primary and secondary cancers...
June 5, 2018: International Journal of Clinical Oncology
Genevieve C Kendall, Sarah Watson, Lin Xu, Collette A LaVigne, Whitney Murchison, Dinesh Rakheja, Stephen X Skapek, Franck Tirode, Olivier Delattre, James F Amatruda
Alveolar rhabdomyosarcoma is a pediatric soft-tissue sarcoma caused by PAX3/7-FOXO1 fusion oncogenes and is characterized by impaired skeletal muscle development. We developed human PAX3-FOXO1 -driven zebrafish models of tumorigenesis and found that PAX3-FOXO1 exhibits discrete cell lineage susceptibility and transformation. Tumors developed by 1.6-19 months and were primitive neuroectodermal tumors or rhabdomyosarcoma. We applied this PAX3-FOXO1 transgenic zebrafish model to study how PAX3-FOXO1 leverages early developmental pathways for oncogenesis and found that her3 is a unique target...
June 5, 2018: ELife
Jeannine Lacroix, Zoltán Kis, Rafael Josupeit, Franziska Schlund, Alexandra Stroh-Dege, Monika Frank-Stöhr, Barbara Leuchs, Jörg R Schlehofer, Jean Rommelaere, Christiane Dinsart
About 70% of all Ewing sarcoma (EWS) patients are diagnosed under the age of 20 years. Over the last decades little progress has been made towards finding effective treatment approaches for primarily metastasized or refractory Ewing sarcoma in young patients. Here, in the context of the search for novel therapeutic options, the potential of oncolytic protoparvovirus H-1 (H-1PV) to treat Ewing sarcoma was evaluated, its safety having been proven previously tested in adult cancer patients and its oncolytic efficacy demonstrated on osteosarcoma cell cultures...
June 3, 2018: Viruses
Kunal C Shah, Akshay Gadia, Uday Pawar, Abhay Nene
OBJECTIVES: Cervical kyphosis surgery poses significant challenge to the spine surgeons. Etiologies of cervical kyphosis are many, each having its own outcome and treatment challenges. Irrespective of the etiology, the treating physician should consider all options fully to improve function and prevent neurological worsening. We outline management principles and decision-making in case of rigid rounded kyphosis of cervical spine and highlight the technique of the internal gibbectomy procedure, which is never reported in cervical spine METHODS: We report a case of Ewing's sarcoma of cervical spine that presented with progressive myelopathy symptoms...
June 1, 2018: World Neurosurgery
Panjarat Sowithayasakul, Phakatip Sinlapamongkolkul, Jitsupa Treetipsatit, Nassawee Vathana, Nattee Narkbunnam, Kleebsabai Sanpakit, Jassada Buaboonnam
Mediastinal germ cell tumor (MGCT), which accounts for 1% to 3% of extragonadal germ cell tumors, has unique manifestations; it is associated with several types of hematologic malignancy, particularly myeloid neoplasm. The aim of this study was to report the 10-year incidence, clinical characteristics, and outcomes of MGCT at Thailand's national pediatric tertiary referral center. This retrospective study included patients diagnosed with MGCT at the Department of Pediatrics, Siriraj Hospital during 2005 to 2014...
June 1, 2018: Journal of Pediatric Hematology/oncology
Chu-Yi Lee, Yi-Wei Chen, Yi-Yen Lee, Feng-Chi Chang, Hsin-Hung Chen, Shih-Chieh Lin, Donald Ming-Tak Ho, Ming-Chao Huang, Sang-Hue Yen, Tai-Tong Wong, Muh-Lii Liang
PURPOSE: Complications can occur following a prolonged latency period after radiation therapy for cancer, and this is a growing concern because secondary tumors are potentially fatal. Few studies have examined secondary tumors in patients who received radiation therapy as children. METHODS AND MATERIALS: This retrospective study examined 1697 pediatric patients with central nervous system tumors who received treatment at Taipei Veterans General Hospital from January 1, 1975, to December 31, 2013...
April 24, 2018: International Journal of Radiation Oncology, Biology, Physics
Pedram Argani, Bruce Pawel, Sara Szabo, Miguel Reyes-Múgica, Charles Timmons, Cristina R Antonescu
No abstract text is available yet for this article.
May 30, 2018: American Journal of Surgical Pathology
Saadiya Javed Khan, Ather Kazmi
OBJECTIVE: To evaluate the outcomes of patients undergoing radiotherapy for primary local control of pelvic ewing sarcoma (ES). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Shaukat Khanum Memorial Cancer Hospital, Lahore, from January 2010 to October 2015. METHODOLOGY: Patients with primary pelvic ES were included in the analysis and all other primary disease sites were excluded. All of them were treated with radiotherapy and followed the EuroEwing-99 chemotherapy protocol...
June 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Zara Forbrigger, Stefan Kuhle, Mary Margaret Brown, Paul C Moorehead, Carol Digout, Ketan Kulkarni
Venous thromboembolism (VTE) is a well-recognized complication in pediatric oncology patients. Studies in adult oncology patients have suggested a potential negative association between VTE and survival, but this association has not been examined in pediatric patients yet. The aim of this study was to assess the association of VTE with survival in pediatric oncology patients. Data from all pediatric oncology patients treated at the two tertiary care centers in Atlantic Canada were pooled to create a population-based cohort...
May 30, 2018: Annals of Hematology
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