PMID- 24793449 OWN - NLM STAT- MEDLINE DA - 20140610 DCOM- 20140801 IS - 1095-8673 (Electronic) IS - 0022-4804 (Linking) VI - 190 IP - 1 DP - 2014 Jul TI - Pediatric sinonasal tumors in the United States: incidence and outcomes. PG - 214-20 LID - 10.1016/j.jss.2014.04.004 [doi] LID - S0022-4804(14)00358-8 [pii] AB - BACKGROUND: Sinonasal tumors in the pediatric population are exceedingly rare. MATERIALS AND METHODS: Surveillance, Epidemiology, and End Results database was used to identify 250 cases of sinonasal malignancy in patients aged <20 y (1973-2010). Malignant histology codes were based on the International Classification of Disease for Oncology, third edition coding scheme. Incidence rates were adjusted to the 2000 U.S. population. Survival outcomes were plotted using the Kaplan-Meier method and compared with the log-rank test. All other analyses were performed using standard statistical methods. RESULTS: Overall incidence was 0.052 per 100,000. Rhabdomyosarcoma had the highest incidence among histologic groups. Regional stage was the most common at diagnosis (59%). Overall survival at 5-y follow-up was 62.5%. Patients in age groups 1-4 and 15-19 y had the worst survival rates, as median survival was 205 and 104 mo, respectively. Distant metastases at the time of diagnosis signified a poor prognosis. These were associated with a 39-mo median survival. Survival improved during the study period, P=0.003. Gender, race, site of lesion, or histology did not appear to affect mortality. CONCLUSIONS: Sinonasal tumors are rare in children and adolescents. Long-term survival is dependent on age and clinical stage at the time of diagnosis. Cancer-related surgery confers a survival advantage. Gender, race, and histologic type are not associated with mortality. CI - Copyright (c) 2014 Elsevier Inc. All rights reserved. FAU - Gerth, David J AU - Gerth DJ AD - Division of Plastic, Aesthetic, and Reconstructive Surgery, DeWitt-Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida. FAU - Tashiro, Jun AU - Tashiro J AD - Division of Plastic, Aesthetic, and Reconstructive Surgery, DeWitt-Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida. FAU - Thaller, Seth R AU - Thaller SR AD - Division of Plastic, Aesthetic, and Reconstructive Surgery, DeWitt-Daughtry Family Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida. Electronic address: SThaller@med.miami.edu. LA - eng PT - Journal Article DEP - 20140412 PL - United States TA - J Surg Res JT - The Journal of surgical research JID - 0376340 SB - IM MH - Adolescent MH - Adult MH - Child, Preschool MH - Female MH - Humans MH - Incidence MH - Infant MH - Male MH - Paranasal Sinus Neoplasms/*epidemiology/mortality/surgery MH - Rhabdomyosarcoma/epidemiology/mortality/surgery MH - SEER Program MH - Sarcoma/epidemiology/mortality/surgery MH - Treatment Outcome MH - United States/epidemiology OTO - NOTNLM OT - Cancer of head and neck OT - Epidemiology OT - Nasal cavity OT - Rhabdomyosarcoma EDAT- 2014/05/06 06:00 MHDA- 2014/08/02 06:00 CRDT- 2014/05/06 06:00 PHST- 2013/12/09 [received] PHST- 2014/03/27 [revised] PHST- 2014/04/03 [accepted] PHST- 2014/04/12 [aheadofprint] AID - S0022-4804(14)00358-8 [pii] AID - 10.1016/j.jss.2014.04.004 [doi] PST - ppublish SO - J Surg Res. 2014 Jul;190(1):214-20. doi: 10.1016/j.jss.2014.04.004. Epub 2014 Apr 12. PMID- 24326270 OWN - NLM STAT- MEDLINE DA - 20140108 DCOM- 20140825 IS - 1531-698X (Electronic) IS - 1040-8703 (Linking) VI - 26 IP - 1 DP - 2014 Feb TI - What is new in the biology and treatment of pediatric rhabdomyosarcoma? PG - 50-6 LID - 10.1097/MOP.0000000000000041 [doi] AB - PURPOSE OF REVIEW: The purpose of this review is to highlight some of the advances in the way we think about rhabdomyosarcoma (RMS). Recent outcome and biological analyses have shifted the risk stratification and treatment paradigms for pediatric RMS. RECENT FINDINGS: The presence or absence of the FOXO1 translocation is one of the most important prognostic factors in RMS. Future clinical studies will incorporate FOXO1 translocation status within risk stratification criteria. Molecular analyses have identified RAS/NF1, hedgehog, IL-4R, and ALK pathway abnormalities as potential therapeutic targets in RMS. Reductions in systemic therapy are possible, although radiation therapy remains essential to prevent local failures in most patients. SUMMARY: Although survival for RMS has not improved in recent years, refinement in risk stratification, further understanding of the biological drivers of the disease, and modifications in treatment intensity have set the stage for the next generation of studies in RMS. FAU - Hawkins, Douglas S AU - Hawkins DS AD - aDepartment of Pediatrics, Seattle Children's Hospital, Fred Hutchinson Cancer Research Center, University of Washington School of Medicine, Seattle, Washington bDivision of Hematology/Oncology, Hospital for Sick Children, University of Toronto, Toronto, Canada cDepartment of Pathology, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, USA. FAU - Gupta, Abha A AU - Gupta AA FAU - Rudzinski, Erin R AU - Rudzinski ER LA - eng GR - CA98543/CA/NCI NIH HHS/United States GR - U10 CA098543/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Opin Pediatr JT - Current opinion in pediatrics JID - 9000850 RN - 0 (FOXO1 protein, human) RN - 0 (Forkhead Transcription Factors) RN - 0 (Neoplasm Proteins) RN - 0 (Tumor Markers, Biological) SB - IM MH - Antineoplastic Combined Chemotherapy Protocols/adverse effects MH - Child MH - Forkhead Transcription Factors/genetics MH - Humans MH - Lymphatic Metastasis MH - Mutation MH - Neoplasm Proteins/genetics MH - Neoplasm Staging MH - Prognosis MH - Radiotherapy Dosage MH - Rhabdomyosarcoma/diagnosis/genetics/secondary/*therapy MH - Translational Medical Research/methods MH - Translocation, Genetic MH - Tumor Markers, Biological/genetics PMC - PMC4096484 MID - NIHMS607847 OID - NLM: NIHMS607847 [Available on 02/01/15] OID - NLM: PMC4096484 [Available on 02/01/15] EDAT- 2013/12/12 06:00 MHDA- 2014/08/26 06:00 CRDT- 2013/12/12 06:00 PMCR- 2015/02/01 00:00 AID - 10.1097/MOP.0000000000000041 [doi] PST - ppublish SO - Curr Opin Pediatr. 2014 Feb;26(1):50-6. doi: 10.1097/MOP.0000000000000041. PMID- 24314190 OWN - NLM STAT- MEDLINE DA - 20131209 DCOM- 20140821 IS - 1531-5037 (Electronic) IS - 0022-3468 (Linking) VI - 48 IP - 12 DP - 2013 Dec TI - An augmented reality navigation system for pediatric oncologic surgery based on preoperative CT and MRI images. PG - 2479-83 LID - 10.1016/j.jpedsurg.2013.08.025 [doi] LID - S0022-3468(13)00686-6 [pii] AB - PURPOSE: In pediatric endoscopic surgery, a limited view and lack of tactile sensation restrict the surgeon's abilities. Moreover, in pediatric oncology, it is sometimes difficult to detect and resect tumors due to the adhesion and degeneration of tumors treated with multimodality therapies. We developed an augmented reality (AR) navigation system based on preoperative CT and MRI imaging for use in endoscopic surgery for pediatric tumors. METHODS: The patients preoperatively underwent either CT or MRI with body surface markers. We used an optical tracking system to register the reconstructed 3D images obtained from the CT and MRI data and body surface markers during surgery. AR visualization was superimposed with the 3D images projected onto captured live images. Six patients underwent surgery using this system. RESULTS: The median age of the patients was 3.5 years. Two of the six patients underwent laparoscopic surgery, two patients underwent thoracoscopic surgery, and two patients underwent laparotomy using this system. The indications for surgery were local recurrence of a Wilms tumor in one case, metastasis of rhabdomyosarcoma in one case, undifferentiated sarcoma in one case, bronchogenic cysts in two cases, and hepatoblastoma in one case. The average tumor size was 22.0+/-14.2 mm. Four patients were treated with chemotherapy, three patients were treated with radiotherapy before surgery, and four patients underwent reoperation. All six tumors were detected using the AR navigation system and successfully resected without any complications. CONCLUSIONS: The AR navigation system is very useful for detecting the tumor location during pediatric surgery, especially for endoscopic surgery. CI - Crown Copyright (c) 2013. All rights reserved. FAU - Souzaki, Ryota AU - Souzaki R AD - Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Department of Advance Medicine and Innovative Technology, Kyushu University Hospital, Fukuoka, Japan. Electronic address: ryotas@pedsurg.med.kyushu-u.ac.jp. FAU - Ieiri, Satoshi AU - Ieiri S FAU - Uemura, Munenori AU - Uemura M FAU - Ohuchida, Kenoki AU - Ohuchida K FAU - Tomikawa, Morimasa AU - Tomikawa M FAU - Kinoshita, Yoshiaki AU - Kinoshita Y FAU - Koga, Yuhki AU - Koga Y FAU - Suminoe, Aiko AU - Suminoe A FAU - Kohashi, Kenichi AU - Kohashi K FAU - Oda, Yoshinao AU - Oda Y FAU - Hara, Toshiro AU - Hara T FAU - Hashizume, Makoto AU - Hashizume M FAU - Taguchi, Tomoaki AU - Taguchi T LA - eng PT - Evaluation Studies PT - Journal Article PL - United States TA - J Pediatr Surg JT - Journal of pediatric surgery JID - 0052631 SB - IM MH - Bronchogenic Cyst/radiography/surgery MH - Child MH - Child, Preschool MH - Hepatoblastoma/radiography/surgery MH - Humans MH - Image Processing, Computer-Assisted MH - Imaging, Three-Dimensional MH - Infant MH - Laparoscopy/*methods MH - Laparotomy/methods MH - Liver Neoplasms/radiography/surgery MH - *Magnetic Resonance Imaging MH - Neoplasm Recurrence, Local/radiography/surgery MH - Neoplasms/radiography/*surgery MH - *Preoperative Care MH - Rhabdomyosarcoma/radiography/secondary/surgery MH - Sarcoma/radiography/surgery MH - Surgery, Computer-Assisted/*methods MH - Thoracoscopy/*methods MH - *Tomography, X-Ray Computed MH - Treatment Outcome MH - Wilms Tumor/radiography/surgery OTO - NOTNLM OT - Augmented reality OT - Image-guided surgery OT - Laparoscopic surgery EDAT- 2013/12/10 06:00 MHDA- 2014/08/22 06:00 CRDT- 2013/12/10 06:00 PHST- 2013/08/25 [received] PHST- 2013/08/26 [accepted] AID - S0022-3468(13)00686-6 [pii] AID - 10.1016/j.jpedsurg.2013.08.025 [doi] PST - ppublish SO - J Pediatr Surg. 2013 Dec;48(12):2479-83. doi: 10.1016/j.jpedsurg.2013.08.025. PMID- 24173021 OWN - NLM STAT- MEDLINE DA - 20131122 DCOM- 20140818 IS - 1791-2423 (Electronic) IS - 1019-6439 (Linking) VI - 44 IP - 1 DP - 2014 Jan TI - The paternally imprinted DLK1-GTL2 locus is differentially methylated in embryonal and alveolar rhabdomyosarcomas. PG - 295-300 LID - 10.3892/ijo.2013.2153 [doi] AB - Parental imprinting of differentially methylated regions (DMRs) contributes to appropriate expression of several developmentally important genes from paternally or maternally derived chromosomes. Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and is associated with altered expression of certain parentally imprinted genes. As previously reported, RMS cells display loss of imprinting (LOI) of the DMR at the IGF2-H19 locus, resulting in insulin-like growth factor 2 (IGF2) transcription from both paternally and maternally inherited chromosomes, and overall IGF2 overexpression. As the DLK1-GTL2 locus is structurally similar to the IGF2-H19 locus, the status of parental imprinting of the DLK1-GTL2 locus was studied in RMS. We observed that while both embryonal and alveolar rhabdomyosarcomas (ERMS and ARMS, respectively) show LOI of the DMR at the IGF2-H19 locus, imprinting of the DMR at the DLK1-GTL2 locus varies in association with the histological subtype of RMS. We found that, while ERMS tumors consistently show LOI of the DMR at the DLK1-GTL2 locus, ARMS tumors have erasure of imprinting (EOI) at this locus. These changes in imprinting status of the DLK1-GTL2 locus result in a higher GTL2/DLK1 mRNA ratio in ARMS as compared to ERMS. This difference in imprinting elucidates a novel genetic difference between these two RMS subtypes and may provide a potential diagnostic tool to distinguish between these subtypes. FAU - Schneider, Gabriela AU - Schneider G AD - Stem Cell Institute at the James Graham Brown Cancer Center, University of Louisville, Louisville, KY, USA. FAU - Bowser, Mark J AU - Bowser MJ FAU - Shin, Dong-Myung AU - Shin DM FAU - Barr, Frederic G AU - Barr FG FAU - Ratajczak, Mariusz Z AU - Ratajczak MZ LA - eng GR - 2R01 DK074720/DK/NIDDK NIH HHS/United States GR - R01 DK074720/DK/NIDDK NIH HHS/United States GR - R01 HL112788/HL/NHLBI NIH HHS/United States GR - R01HL112788/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20131029 PL - Greece TA - Int J Oncol JT - International journal of oncology JID - 9306042 RN - 0 (DLK1 protein, human) RN - 0 (Intercellular Signaling Peptides and Proteins) RN - 0 (MEG3 non-coding RNA, human) RN - 0 (Membrane Proteins) RN - 0 (RNA, Long Noncoding) SB - IM MH - Alleles MH - Child MH - Chromosomes/genetics MH - DNA Methylation/genetics MH - Gene Expression Regulation, Neoplastic MH - Genomic Imprinting/genetics MH - Humans MH - Intercellular Signaling Peptides and Proteins/*genetics MH - Membrane Proteins/*genetics MH - RNA, Long Noncoding/*genetics MH - Rhabdomyosarcoma, Alveolar/*genetics/pathology MH - Rhabdomyosarcoma, Embryonal/*genetics/pathology PMC - PMC3867365 OID - NLM: PMC3867365 EDAT- 2013/11/01 06:00 MHDA- 2014/08/19 06:00 CRDT- 2013/11/01 06:00 PHST- 2013/08/16 [received] PHST- 2013/10/01 [accepted] PHST- 2013/10/29 [aheadofprint] AID - 10.3892/ijo.2013.2153 [doi] PST - ppublish SO - Int J Oncol. 2014 Jan;44(1):295-300. doi: 10.3892/ijo.2013.2153. Epub 2013 Oct 29. PMID- 23946091 OWN - NLM STAT- MEDLINE DA - 20131115 DCOM- 20140818 IS - 1439-3824 (Electronic) IS - 0300-8630 (Linking) VI - 225 IP - 6 DP - 2013 Nov TI - Response of children with stage IV soft tissue sarcoma to topotecan and carboplatin: a phase II window trial of the cooperative soft tissue sarcoma group. PG - 309-14 LID - 10.1055/s-0033-1341489 [doi] AB - To investigate antitumor activity and toxicity associated with combined topotecan and carboplatin treatment in children and adolescents with metastasized, untreated soft tissue sarcoma (STS).Patients (n=34) less than 21 years old and untreated, stage IV STS. Patients were treated with topotecan (1 mg/m(2)/d for 4 days) and carboplatin (150 mg/m(2)/d for 4 days) (TC course) during week 1 and 4 of a chemotherapy window trial, which was followed by chemotherapy and local therapy from week 6 on. We evaluated the side effects, toxicity and tumor response (using RECIST criteria) 6 weeks after starting the 2 TC chemotherapy courses.The objective response rate (ORR) was 38% (n=13 patients with a partial response (PR)), and a stable disease (SD) was reached in 11 cases. No patient showed a complete response (CR) of all metastatic lesions, although 1 patient showed a CR of the target lesion. 2 patients died of progress of disease (PD). Toxicity was mainly hematological (grade III/IV toxicity 79%), and nonhematological toxicities mainly included infection, fever, nausea,and vomiting. Regarding adverse events, 4 probable and 8 possible events related to study medication occurred among the 66 courses of TC.In conclusion, TC was potent against high-risk STS, but results and toxicity data were not superior to former published monotherapeutic topotecan therapies. CI - (c) Georg Thieme Verlag KG Stuttgart . New York. FAU - Bochennek, K AU - Bochennek K AD - Pediatric Hematology and Oncology, University Hospital of J. W. Goethe University, Frankfurt/Main, Germany. FAU - Dantonello, T AU - Dantonello T FAU - Koscielniak, E AU - Koscielniak E FAU - Claviez, A AU - Claviez A FAU - Dirksen, U AU - Dirksen U FAU - Sauerbrey, A AU - Sauerbrey A FAU - Beilken, A AU - Beilken A FAU - Klingebiel, T AU - Klingebiel T LA - eng PT - Clinical Trial, Phase II PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130814 PL - Germany TA - Klin Padiatr JT - Klinische Padiatrie JID - 0326144 RN - 7M7YKX2N15 (Topotecan) RN - BG3F62OND5 (Carboplatin) SB - IM MH - Adolescent MH - Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use MH - Carboplatin/*administration & dosage/adverse effects MH - Child MH - Child, Preschool MH - Cohort Studies MH - Combined Modality Therapy MH - Disease Progression MH - Dose-Response Relationship, Drug MH - Drug Administration Schedule MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Neoadjuvant Therapy MH - Neoplasm Staging MH - Rhabdomyosarcoma/diagnosis/drug therapy/mortality/pathology MH - Sarcoma/diagnosis/*drug therapy/mortality/pathology MH - Sarcoma, Ewing/diagnosis/drug therapy/mortality/pathology MH - Sarcoma, Synovial/diagnosis/drug therapy/mortality/pathology MH - Soft Tissue Neoplasms/diagnosis/*drug therapy/mortality/pathology MH - Survival Rate MH - Topotecan/*administration & dosage/adverse effects MH - Treatment Outcome EDAT- 2013/08/16 06:00 MHDA- 2014/08/19 06:00 CRDT- 2013/08/16 06:00 PHST- 2013/08/14 [epublish] PHST- 2013/08/14 [aheadofprint] AID - 10.1055/s-0033-1341489 [doi] PST - ppublish SO - Klin Padiatr. 2013 Nov;225(6):309-14. doi: 10.1055/s-0033-1341489. Epub 2013 Aug 14. PMID- 23577745 OWN - NLM STAT- MEDLINE DA - 20131218 DCOM- 20140821 IS - 1651-226X (Electronic) IS - 0284-186X (Linking) VI - 53 IP - 1 DP - 2014 Jan TI - Assessment of volume segmentation in radiotherapy of adolescents; a treatment planning study by the Swedish Workgroup for Paediatric Radiotherapy. PG - 126-33 LID - 10.3109/0284186X.2013.782104 [doi] AB - BACKGROUND AND PURPOSE: The variability in target delineation for similar cases between centres treating paediatric and adolescent patients, and the apparent differences in interpretation of radiotherapy guidelines in the treatment protocols encouraged us to perform a dummy-run study as a part of our quality assurance work. The aim was to identify and quantify differences in the segmentation of target volumes and organs at risk (OARs) and to analyse the treatment plans and dose distributions. MATERIALS AND METHODS: Four patient cases were selected: Wilm's tumour, Hodgkin's disease, rhabdomyosarcoma of the prostate and chordoma of the skull base. The five participating centres received the same patient-related material. They introduced the cases in their treatment planning system, delineated target volumes and OARs and created treatment plans. Dose-volume histograms were retrieved for relevant structures and volumes and dose metrics were derived and compared, e.g. target volumes and their concordance, dose homogeneity index (HI), treated and irradiated volumes, remaining volume at risk and relevant Vx and Dx values. RESULTS: We found significant differences in target segmentation in the majority of the cases. The planning target volumes (PTVs) varied two- to four-fold and conformity indices were in the range of 0.3-0.6. This resulted in large variations in dose distributions to OARs as well as in treated and irradiated volumes even though the treatment plans showed good conformity to the PTVs. Potential reasons for the differences in target delineation were analysed. CONCLUSION: Considerations of the growing child and difficulties in interpretation of the radiotherapy information in the treatment protocols were identified as reasons for the variation. As a result, clarified translated detailed radiotherapy guidelines for paediatric/adolescent patients have been recognised as a way to reduce this variation. FAU - Kristensen, Ingrid AU - Kristensen I AD - Department of Clinical Sciences, Oncology, Lund University , Lund , Sweden. FAU - Agrup, Mans AU - Agrup M FAU - Bergstrom, Per AU - Bergstrom P FAU - Engellau, Jacob AU - Engellau J FAU - Haugen, Hedda AU - Haugen H FAU - Martinsson, Ulla AU - Martinsson U FAU - Nilsson, Kristina AU - Nilsson K FAU - Taheri-Kadkhoda, Zahra AU - Taheri-Kadkhoda Z FAU - Lindh, Jack AU - Lindh J FAU - Nilsson, Per AU - Nilsson P LA - eng PT - Case Reports PT - Journal Article DEP - 20130412 PL - England TA - Acta Oncol JT - Acta oncologica (Stockholm, Sweden) JID - 8709065 SB - IM MH - Adolescent MH - Chordoma/pathology/*radiotherapy MH - Female MH - Hodgkin Disease/pathology/*radiotherapy MH - Humans MH - Kidney Neoplasms/pathology/radiotherapy MH - Male MH - Pediatrics MH - Prognosis MH - Prostatic Neoplasms/pathology/*radiotherapy MH - Radiotherapy Dosage MH - *Radiotherapy Planning, Computer-Assisted MH - Radiotherapy, Conformal MH - Rhabdomyosarcoma/pathology/*radiotherapy MH - Skull Base Neoplasms/pathology/*radiotherapy MH - Sweden MH - Wilms Tumor/pathology/*radiotherapy EDAT- 2013/04/13 06:00 MHDA- 2014/08/22 06:00 CRDT- 2013/04/13 06:00 PHST- 2013/04/12 [aheadofprint] AID - 10.3109/0284186X.2013.782104 [doi] PST - ppublish SO - Acta Oncol. 2014 Jan;53(1):126-33. doi: 10.3109/0284186X.2013.782104. Epub 2013 Apr 12.