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pediatric germ cell tumor

Ameer Hamza, Eleftherios Vouyoukas, Ian Jacob Anderson, Martha Jaye Higgins
Teratomas are one of the most frequent tumors in the pediatric population. They occur anywhere along the midline of the body, following the course of the embryonic germ cell ridge. In the mediastinal location, they exert space occupying effects, leading to a myriad of complications, including non-immune hydrops fetalis. We describe a fatal case of an immature thymic teratoma in a neonate presenting with hydrops fetalis. This case emphasizes the importance of early diagnosis and surgical intervention in such cases...
January 2018: Autopsy & Case Reports
Joshua L Pierce, A Lindsay Frazier, James F Amatruda
Germ cell tumors (GCTs) arising in infants, children, and adolescents present a set of special challenges. GCTs make up about 3% of malignancies in children aged 0-18 and nearly 15% of cancers in adolescents. Epidemiologic and molecular evidence suggests that GCTs in young children likely represent a distinct biologic group as compared to GCTs of older adolescents and adults. Despite this difference, pediatric GCTs are typically treated with cisplatin-based multiagent regimens similar to those used in adults...
2018: Advances in Urology
Briony K Varda, Patricia Cho, Andrew A Wagner, Richard S Lee
BACKGROUND: In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES: To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital...
March 2, 2018: Journal of Pediatric Urology
Aurore Bussat, Maia Proisy, Bertrand Bruneau, Guillaume Bouzillé, Céline Chappé, Laurent Riffaud
OBJECTIVE Tumor-related edema of the optic tract (EOT) corresponds to a preferential posterior distribution of peritumoral edema along the white matter tract of the visual system. To date, the consequences of EOT have never been evaluated specifically in the pediatric population. In this study, the authors attempted to identify clinical and radiological features associated with the development of EOT and the specific influence of this edema on visual function. METHODS A retrospective review was performed of data collected from patients younger than 18 years who underwent surgery for a tumor in the sellar region at the authors' institution between January 2005 and January 2016...
March 2, 2018: Journal of Neurosurgery. Pediatrics
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
Leanne de Kock, Dominique Geoffrion, Barbara Rivera, Rabea Wagener, Nelly Sabbaghian, Susanne Bens, Benjamin Ellezam, Dorothée Bouron-Dal Soglio, Jessica Ordóñez, Stephanie Sacharow, Jose Fernando Polo Nieto, R Paul Guillerman, Gordan M Vujanic, John R Priest, Reiner Siebert, William D Foulkes
Germ-line interstitial deletions involving the 14q32 chromosomal region, resulting in 14q32 deletion syndrome, are rare. DICER1 is a recently described cancer-predisposition gene located at 14q32.13. We report the case of a male child with an approximately 5.8 Mbp 14q32.13q32.2 germ-line deletion, which included the full DICER1 locus. We reviewed available clinical and pathological material, and conducted genetic analyses. In addition to having congenital dysmorphic features, the child developed multiple DICER1 syndrome-related tumors before age 5 years: a pediatric cystic nephroma (pCN), a ciliary body medulloepithelioma (CBME), and a small lung cyst (consistent with occult pleuropulmonary blastoma Type I/Ir cysts seen in DICER1 mutation carriers)...
January 9, 2018: Genes, Chromosomes & Cancer
Bryan J Dicken, Deborah F Billmire, Mark Krailo, Caihong Xia, Furqan Shaikh, John W Cullen, Thomas A Olson, Farzana Pashankar, Marcio H Malogolowkin, James F Amatruda, Frederick J Rescorla, Rachel A Egler, Jonathan H Ross, Carlos Rodriguez-Galindo, A Lindsay Frazier
PURPOSE: In this report, we characterize the timing and behavior of malignant ovarian germ cell tumors (GCTs) in pediatric patients with dysgenetic gonads compared to those with normal gonadal development. PATIENTS AND METHODS: Patients from the Children's Oncology Group AGCT0132 with malignant ovarian GCTs were included. Within this population, we sought to identify patients with gonadoblastoma, streak ovaries, or other evidence of gonadal dysgenesis (GD). Patients with malignant GCTs containing one or more of the following histologies-yolk sac tumor, embryonal carcinoma, or choriocarcinoma-were included...
December 29, 2017: Pediatric Blood & Cancer
Avani D Rao, Arif S Rashid, Qinyu Chen, Rosangela C Villar, Daria Kobyzeva, Kristina Nilsson, Karin Dieckmann, Alexey Nechesnyuk, Ralph Ermoian, Sara Alcorn, Shannon M MacDonald, Matthew M Ladra, Eric C Ford, Brian A Winey, Maria Luisa S Figueiredo, Michael J Chen, Stephanie A Terezakis
PURPOSE: Reirradiation has been proposed as an effective modality for recurrent central nervous system (CNS) malignancies in adults. We evaluated the toxicity and outcomes of CNS reirradiation in pediatric patients. METHODS AND MATERIALS: The data from pediatric patients <21 years of age at the initial diagnosis who developed a recurrent CNS malignancy that received repeat radiation therapy (RT) across 5 facilities in an international pediatric research consortium were retrospectively reviewed...
November 1, 2017: International Journal of Radiation Oncology, Biology, Physics
Chika Anele Ndubuisi, Samuel C Ohaegbulam, Linda U Iroegbu, Mike Ezeali Ekuma, Wilfred C Mezue, Uwadiegwu Alphonsus Erechukwu
Background: There is controversy about the global distribution of intracranial tumors (ICTs). The previous reports from Africa suggested low frequency and different pattern of distribution of brain tumors from what obtains in other continents. The limitations at that time, including paucity of diagnostic facilities and personnel, have improved. Objective: The objective of this study is to analyze the current trend and distribution of histology confirmed brain tumors managed in Enugu, in a decade...
October 2017: Journal of Neurosciences in Rural Practice
Cecile Faure Conter, Caihong Xia, David Gershenson, Jean Hurteau, Al Covens, Farzana Pashankar, Mark Krailo, Deborah Billmire, Catherine Patte, Brice Fresneau, Furqan Shaikh, Sara Stoneham, James Nicholson, Matthew Murray, Anne Lindsay Frazier
BACKGROUND: Whereas among pediatric oncologists, ovarian yolk sac tumor (O-YST) is considered a chemosensitive tumor, it is often cited as an adverse prognostic factor in adult women with ovarian germ cell tumors. METHODS: The Malignant Germ Cell International Consortium data set included 6 pediatric clinical trials (United States, United Kingdom, and France) and 2 adult gynecology clinical trials (United States). Any patient with an O-YST that was International Federation of Gynecology and Obstetrics stage IC or higher and treated with a platinum-based chemotherapy was eligible...
January 2018: International Journal of Gynecological Cancer
Giovanni Morana, Cesar Augusto Alves, Domenico Tortora, Jonathan L Finlay, Mariasavina Severino, Paolo Nozza, Marcello Ravegnani, Marco Pavanello, Claudia Milanaccio, Mohamad Maghnie, Andrea Rossi, Maria Luisa Garrè
PURPOSE: The role of T2*-based MR imaging in intracranial germ cell tumors (GCTs) has not been fully elucidated. The aim of this study was to evaluate the susceptibility-weighted imaging (SWI) or T2* gradient echo (GRE) features of germinomas and non-germinomatous germ cell tumors (NGGCTs) in midline and off-midline locations. METHODS: We retrospectively evaluated all consecutive pediatric patients referred to our institution between 2005 and 2016, for newly diagnosed, treatment-naïve intracranial GCT, who underwent MRI, including T2*-based MR imaging (T2* GRE sequences or SWI)...
January 2018: Neuroradiology
M Cools, K P Wolffenbuttel, R Hersmus, B B Mendonca, J Kaprová, S L S Drop, H Stoop, A J M Gillis, J W Oosterhuis, E M F Costa, S Domenice, M Y Nishi, L Wunsch, C A Quigley, G T'Sjoen, L H J Looijenga
STUDY QUESTION: What is the prevalence of malignant testicular germ cell tumors (TGCT) and its precursors, (pre-) germ cell neoplasia in situ (GCNIS), in late teenagers and adults who have androgen insensitivity syndrome (AIS) and the impact of an individual's genetic susceptibility to development of TGCT? SUMMARY ANSWER: No GCNIS or TGCT was diagnosed, but pre-GCNIS was identified in 14 and 10% of complete and partial AIS patients, respectively, and was associated with a higher genetic susceptibility score (GSS), with special attention for KITLG (rs995030) and ATFZIP (rs2900333)...
December 1, 2017: Human Reproduction
Paola Friedrich, Elena Itriago, Carlos Rodriguez-Galindo, Karina Ribeiro
Background: Few studies have comparatively assessed differences in the incidence of childhood cancer by race and ethnicity that could inform etiologic research. We aimed to identify disparities in the incidence of pediatric extracranial embryonal tumors by race and ethnicity in the United States using a population-based cancer registry. Methods: Cases of extracranial embryonal tumors among children age 0 to 19 years diagnosed between 2000 and 2010 were retrieved from the Surveillance, Epidemiology, and End Results Program 18 (n = 8188)...
October 1, 2017: Journal of the National Cancer Institute
Barbara Tanno, Simona Leonardi, Gabriele Babini, Paola Giardullo, Ilaria De Stefano, Emanuela Pasquali, Anna Saran, Mariateresa Mancuso
Medulloblastoma (MB) is the most common pediatric brain tumor, comprising four distinct molecular variants, one of which characterized by activation of the Sonic Hedgehog (SHH) pathway, driving 25-30% of sporadic MB. SHH-dependent MBs arise from granule cell precursors (GCPs), are fatal in 40-70% of cases and radioresistance strongly contributes to poor prognosis and tumor recurrence. Patched1 heterozygous (Ptch1 (+/-)) mice, carrying a germ-line heterozygous inactivating mutation in the Ptch1 gene, the Shh receptor and negative regulator of the pathway, are uniquely susceptible to MB development after radiation damage in neonatal cerebellum...
October 27, 2017: Scientific Reports
Mengmeng Wang, Peizhi Zhou, Shizhen Zhang, Xueyou Liu, Liang Lv, Zeming Wang, Feng Ye, Yan Ju, Shu Jiang
PURPOSE: Pediatric germ cell tumors (GCTs) involving the basal ganglia and thalamus are relatively rare neoplasms which have not been extensively described. We here summarize the clinical and radiological features of a series of such tumors and discuss optimal treatment strategies based upon our experience. METHODS: A total of 15 pediatric patients with basal ganglionic and thalamic GCTs were treated between 2011 and 2016 at West China Hospital. Epidemiological characteristics, clinical features, imaging findings, and treatment strategies were reviewed retrospectively...
October 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Yunosuke Kawaguchi, Takeshi Saito, Tetsuya Mitsunaga, Keita Terui, Mitsuyuki Nakata, Gen Matsuura, Katsunori Kouchi, Hideo Yoshida
PURPOSE: Fatality resulting from respiratory collapse (RC) during general anesthesia (GA) induction in children with mediastinal tumors has been reported. We explored potentially useful parameters for predicting the risk of RC based on objective imaging results. METHODS: We retrospectively reviewed the records of 31 patients (median age: 4years; range: 4months-15years) with mediastinal tumors treated between 2000 and 2015. Comparing those with RC under GA induction to those without (RC group vs...
October 2, 2017: Journal of Pediatric Surgery
Tsung-Han Hsieh, Yun-Ru Liu, Ting-Yu Chang, Muh-Lii Liang, Hsin-Hung Chen, Hsei-Wei Wang, Yun Yen, Tai-Tong Wong
Background: Pediatric central nervous system germ cell tumors (CNSGCTs) are rare and heterogeneous neoplasms, and they can be divided into germinomas and nongerminomatous GCTs (NGGCTs). NGGCTs are further subdivided into mature teratomas and nongerminomatous malignant GCTs (NGMGCTs). Clinical outcomes suggest that NGMGCTs have poor prognosis and survival, and that they require more extensive radiotherapy and adjuvant chemotherapy. However, the mechanisms underlying this difference are still unclear...
October 3, 2017: Neuro-oncology
Kirti Gupta, Madhivanan Karthigeyan, Ayushman Satapathy, Pravin Salunke
INTRODUCTION: In children, malignant tumors presenting as a calvarial mass that too as initial manifestation are unusual. Also, brain metastases per se are rare in pediatric solid tumors with an incidence of just 1.5%. CASE DESCRIPTION: We present a child with calvarial metastasis which was the presenting symptom of an otherwise asymptomatic large malignant mediastinal germ cell tumor (GCT). The lesion was dural-based involving both calvaria as well as the underlying brain parenchyma...
October 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Peili Liang, Xin Zhang, Zhenfeng Zhang, Guocai Xu, Xingsu Yu, Sijin Li, Yuanyuan Zhang, Lijuan Bian, Bingzhong Zhang
STUDY OBJECTIVE: Nonepithelial malignant ovarian tumors are rare in the pediatric and adolescent population. The aim of this study was to observe the spectrum of pathology, presentation, outcome, and risk factors for survival of pediatric nonepithelial malignant ovarian tumors in a Chinese pediatric population. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: This was a retrospective study of 171 girls (median age at presentation of 14 years) diagnosed with primary malignant ovarian tumors between 1990 and 2014 at the Yat-Sen Memorial Hospital and Cancer Center of Sun Yat-sen University...
October 6, 2017: Journal of Pediatric and Adolescent Gynecology
Yuichiro Yoneoka, Junichi Yoshimura, Masakazu Sano, Masayasu Okada, Akiyoshi Kakita, Yukihiko Fujii
We present a pediatric case of a rapidly expanding third ventricle germ cell tumor (GCT). A 14-year-old boy suffered from gradual-onset central diabetes insipidus (DI) and received desmopressin treatment. Magnetic resonance imaging (MRI) showed nonspecific findings of the pituitary-hypothalamic axis. Nine months after the initial DI diagnosis, he developed progressively worsening headache. MRI demonstrated a third ventricle tumor causing noncommunicating hydrocephalus, although an MRI 16 weeks before admission did not show the lesion...
2018: Pediatric Neurosurgery
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