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pediatric germ cell tumor

Faten Fedhila, Samar Rhayem, Habiba Hafsi, Wiem Douira, Raoudha Doghri, Monia Khemiri, Karima Mrad, Ibtissem Bellagha, Bechir Zouari, Sihem Barsaoui
Background Extracranial Germ cell tumors (GCT) are a rare and a heterogeneous group of pediatric cancers but highly curable. Aim We aimed to review management, outcome and prognostic factors that influence overall survival (OS) in a pediatric Tunisian oncologic unit. Methods We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit. Results Patients had a mean age of 57 months (ranges: 1 day-13 years)...
April 2016: La Tunisie Médicale
Manuel Nistal, Ricardo Paniagua, Pilar Gonzalez-Peramato, Miguel Reyes-Múgica
Testicular tumors in the prepubertal age are relatively rare, representing only 9.4% of the total testicular and paratesticular specimens from a 20 year review performed at a large pediatric hospital 1. They account for 1 to 2 % of all solid tumors in the pediatric age group, with an annual incidence between 0.5 and 2/100,000 boys according to Coppes et al. 2 and data from the Prepubertal Testicular Tumor Registry 3. Similar to other neoplasms afflicting children, a bimodal age distribution is observed. The first peak is between birth and 3 years of age, and a second one occurs at the onset of puberty,extending to the fourth decade...
September 14, 2016: Pediatric and Developmental Pathology
Vinodh Pillai, David M Dorfman
Many epithelial neoplasms can be analyzed by flow cytometry (FC), particularly from serous cavity effusion samples, using EpCAM, a cell adhesion molecule expressed on most normal epithelial cells and expressed at a higher level in most epithelial neoplasms. A simple 3-color flow cytometric panel can provide a high sensitivity and specificity compared to cytomorphology. FC provides more rapid immunophenotyping than conventional immunohistochemical staining, can identify rare malignant cells that could be missed by a cytological exam alone, and can be utilized to evaluate limited samples such as cerebrospinal fluid or fine-needle aspiration samples...
2016: Acta Cytologica
David M Gershenson, A Lindsay Frazier
One of the most extraordinary stories in the chronicles of gynecologic cancers has been that of malignant ovarian germ cell tumors. Prior to the mid-1960s, most patients died of disease. Fifty years later, most survive. Precisely because high cure rates are achievable, the concentration over the past decade has been on minimizing toxicity and late effects. The present review focuses on five areas of interest related to the management of malignant ovarian germ cell tumors that highlight the different therapeutic strategies practiced by pediatric and gynecologic oncologists: 1) primary surgery, 2) surgery alone (surveillance) for patients with FIGO stage IA disease, 3) postoperative management of FIGO stage IC-III disease, 4) postoperative management of pure immature teratoma, and 5) postoperative management of metastatic pure dysgerminoma...
August 25, 2016: Gynecologic Oncology
Si Zhang, Guopeng Liang, Yan Ju, Chao You
BACKGROUND: and object: Pediatric basal ganglia germ cell tumors (GCTs) represent a rare subset of tumors with poor understandings. We aimed to summarize the clinical features and radiological findings of this special subgroup of GCTs. METHODS: From January 2010 to January 2015, twelve pediatric patients suffered from basal ganglia GCTs were treated in our hospital. The clinical features, radiological findings, diagnosis, treatment and outcome of these patients were retrospectively analyzed...
August 24, 2016: World Neurosurgery
Ashley S Plant, Susan N Chi, Lindsay Frazier
Malignant germ cell tumors (GCT) arise from abnormal migration of primordial germ cells and are histologically identical whether they occur inside or outside the central nervous system (CNS). However, the treatment strategy for GCTs varies greatly depending on the location of the tumor. These differences are in part due to the increased morbidity of surgery in the CNS but may also reflect differential sensitivity of the tumors to chemotherapy and radiation therapy (RT) or not-yet-understood biologic differences between these tumors...
August 24, 2016: Pediatric Blood & Cancer
Robert E Emerson, Chia-Sui Kao, John N Eble, David J Grignon, Mingsheng Wang, Shaobo Zhang, Xiaoyan Wang, Rong Fan, Timothy A Masterson, Lawrence M Roth, Liang Cheng
AIMS: Sacrococcygeal teratomas are rare tumours that occur most frequently in neonates, although adult cases also occur. The molecular pathogenesis of these tumours and their long-term prognosis is uncertain. We investigated the i(12p) status of a large number of primary sacrococcygeal teratomas in both children and adults, including cases with malignant germ cell tumour elements. METHODS AND RESULTS: Fifty-four sacrococcygeal teratoma specimens from 52 patients were identified, and available follow-up information was obtained...
August 13, 2016: Histopathology
Shaoguang Feng, Songsong Huang, Yulong Tong, Zhongliang Chen, Delei Shen, Dazhou Wu, Xin-He Lai, Xiaoming Chen
BACKGROUND: RNA-binding protein LIN28 is involved in maintaining the pluripotency of embryonic stem cells. It has been detected in different types of testicular and ovarian germ cell tumors (GCTs), but its status in pediatric YSTs (yolk sac tumors) is still unknown. The aim of this study was to determine the immunohistochemical profile of LIN28 in pediatric YSTs. METHODS AND RESULTS: Immunohistochemistry detection of LIN28 was performed in 22 cases of pediatric YSTs and 10 mature teratomas...
August 2016: Pediatric Surgery International
Xiang Huang, Rong Zhang, Ying Mao, Liang-Fu Zhou, Chao Zhang
BACKGROUND: Intracranial germ cell tumors (IGCTs) are a group of rare pediatric brain tumors which include various subtypes. The current understanding of the etiology of the tumors and their optimal management strategies remain controversial. DATA SOURCES: The data on IGCTs were collected from articles published in the past 20 years, and the origin and etiology of IGCTs at molecular level as well as the relative roles of varied treatment strategies in different prognosis groups according to Matsutani's classification were reviewed...
August 2016: World Journal of Pediatrics: WJP
Grainne H Butler, Michael Boyle, Sally Ann Lynch, Stephanie Ryan, Michael McDermott, Michael Capra
A male preterm infant was born with dysmorphic features consistent with Rubinstein-Taybi syndrome (RTS). An undescended right testicle was noted on examination. At 5 months of age he developed a palpable right-sided abdominal mass and an elevated alpha-fetoprotein. Histology revealed a malignant germ cell neoplasm arising within the undescended testis. This is the first reported case of a germ cell tumor occurring in a pediatric patient with RTS. Urologic abnormalities occur in approximately 52% of RTS patients, of which cryptorchidism is the commonest...
August 2016: Journal of Pediatric Hematology/oncology
Douglas A Canning
No abstract text is available yet for this article.
July 2016: Journal of Urology
F Malbari, T R Gershon, J H Garvin, J C Allen, Y Khakoo, A S Levy, I J Dunkel
BACKGROUND: Central nervous system (CNS) germ cell tumors account for 3 % of all pediatric brain tumors in the USA. Presenting symptoms are typically location based with pineal tumors presenting with obstructive hydrocephalus and suprasellar tumors with hypothalamic/pituitary dysfunction and ophthalmologic abnormalities. Psychiatric manifestations such as psychosis and behavioral changes are atypical presentations of CNS germ cell tumors, with only 11 previously reported cases. METHODS: This is a retrospective case series describing patients with CNS germ cell tumors with an atypical presentation including psychiatric manifestations...
August 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Roumina Hasan, Vidya Monappa, Sandeep Kumar, Vijay Kumar
Amongst the varied, diverse causes of intraabdominal masses in infancy and early childhood, gastric teratomas (GTs) account for a very small proportion. A worldwide literature search reveals only around one hundred cases of GT and also supports the fact that its preoperative diagnosis remains elusive. Here we report the case of a two-month-old male who presented to the pediatric surgery outpatient department of Kasturba Medical College and Hospital, Karnataka, India, with progressive distension of abdomen since birth...
May 2016: Oman Medical Journal
Takahiro Aoki, Moeko Hino, Katsuyoshi Koh, Masashi Kyushiki, Hiroshi Kishimoto, Yuki Arakawa, Ryoji Hanada, Hiroshi Kawashima, Jun Kurihara, Naoki Shimojo, Shinichiro Motohashi
Programmed death 1 (PD-1)/programmed death ligand 1 (PD-L1) pathway blockade has become a promising therapeutic target in adult cancers. We evaluated PD-L1 expression and tumor-infiltrating CD8(+) T cells in formalin-fixed, paraffin-embedded tumor specimens from 53 untreated pediatric patients with eight cancer types: neuroblastoma, extracranial malignant germ cell tumor, hepatoblastoma, germinoma, medulloblastoma, renal tumor, rhabdomyosarcoma, and atypical teratoid/rhabdoid tumor. One rhabdomyosarcoma with the shortest survival exhibited membranous PD-L1 expression and germinoma contained abundant tumor-infiltrating CD8(+) T cells and PD-L1-positive macrophages...
August 2016: Pediatric Blood & Cancer
Arin L Madenci, Bat-Sheva Levine, Marc R Laufer, Theonia K Boyd, Stephan D Voss, A Lindsay Frazier, Christopher B Weldon
PURPOSE: Historically, surgical staging practices for pediatric malignant ovarian tumors mirrored adult guidelines. In 2004, the Children's Oncology Group (COG) developed guidelines specific to pediatric patients with ovarian germ cell tumors. We sought to characterize the operative management of pediatric ovarian lesions and adherence to COG surgical staging guidelines. METHODS: We conducted a single institution, retrospective study of pediatric patients who underwent surgical intervention for ovarian lesions between 1995 and 2012...
September 2016: Journal of Pediatric Surgery
Zeynep Atay, Ediz Yesilkaya, Senay Savas Erdeve, Serap Turan, Leyla Akin, Erdal Eren, Esra Doger, Zehra Aycan, Zehra Yavas Abali, Aysşehan Akinci, Zeynep Siklar, Samim Ozen, Cengiz Kara, Meltem Tayfun, Erkan Sari, Filiz Tutunculer, Gulcan Seymen Karabulut, Gulay Karaguzel, Semra Cetinkaya, Halil Saglam, Aysun Bideci, Selim Kurtoglu, Tulay Guran, Abdullah Bereket
AIM: The causes of gonadotropin-independent precocious puberty are diverse, and often have overlapping clinical and biochemical features. With the exception of congenital adrenal hyperplasia (CAH), disorders that cause gonadotropin-independent precocious puberty (GIPP) are uncommon. The literature is devoid of any large-scale studies on the etiologic distribution of GIPP. The aim of this study was to determine the frequency of each etiology in a cohort of patients with GIPP (excluding those with CAH), and to evaluate the clinical and laboratory features of these patients...
May 2016: Journal of Clinical Endocrinology and Metabolism
Anna Raciborska, Katarzyna Bilska, Jadwiga Węcławek-Tompol, Marek Ussowicz, Monika Pogorzała, Joanna Janowska, Magdalena Rychłowska-Pruszyńska, Carlos Rodriguez-Galindo, Ewa Helwich
BACKGROUND: Advances in multidisciplinary care for pediatric cancer have resulted in significant improvement in cure rates over the last decades; however, these advances have not been uniform across all age groups. Cancer is an important cause of perinatal mortality, yet the full spectrum of malignant neoplasms in newborns is not well defined. METHODS: The authors have reviewed the clinical features and outcomes of 37 newborns with congenital malignant tumors treated at three referral centers in North, Central, and South Poland between 1980 and 2014...
August 2016: Pediatrics and Neonatology
Shabnam Parvin, Moumita Sengupta, Prafulla Kumar Mishra, Uttara Chatterjee, Sugato Banerjee, Manoj Kumar Chaudhuri
BACKGROUND: Gastric teratoma is a rare entity comprising less than 1% of germ cell tumors of childhood. We present a series of seven gastric teratomas with a review of literature. OBJECTIVE: To study the demographic profile, clinicopathological features and follow-up data of gastric teratomas. METHODS: We did a retrospective analysis of 7 cases of gastric teratomas more than 15years and studied their demographic profiles, clinicopathological features and follow-up data...
July 2016: Journal of Pediatric Surgery
Andrea Ferrari, Salvatore Lo Vullo, Daniele Giardiello, Laura Veneroni, Chiara Magni, Carlo Alfredo Clerici, Stefano Chiaravalli, Michela Casanova, Roberto Luksch, Monica Terenziani, Filippo Spreafico, Cristina Meazza, Serena Catania, Elisabetta Schiavello, Veronica Biassoni, Marta Podda, Luca Bergamaschi, Nadia Puma, Maura Massimino, Luigi Mariani
BACKGROUND: The potential impact of diagnostic delays on patients' outcomes is a debated issue in pediatric oncology and discordant results have been published so far. We attempted to tackle this issue by analyzing a prospective series of 351 consecutive children and adolescents with solid malignancies using innovative statistical tools. METHODS: To address the nonlinear complexity of the association between symptom interval and overall survival (OS), a regression tree algorithm was constructed with sequential binary splitting rules and used to identify homogeneous patient groups vis-à-vis functional relationship between diagnostic delay and OS...
March 2016: Pediatric Blood & Cancer
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