11 research outputs found
How we approach paediatric renal tumour core needle biopsy in the setting of preoperative chemotherapy: A Review from the SIOP Renal Tumour Study Group
The International Society of Paediatric Oncology Renal Tumour Study Group (SIOP-RTSG) advocate treating children with Wilms tumour (WT) with preoperative chemotherapy, whereas the Renal Tumor Committee of the Children's Oncology Group (COG) advocates primary nephrectomy (without biopsy) when feasible. Successive SIOP-RTSG trial protocols recommended pretreatment biopsy of children with unilateral tumours only where there were features to suggest an increased probability of a non-WT requiring a change in management. The UK experience in the SIOP WT 2001 trial showed that an alternate approach of performing biopsies on all children with renal tumour masses to determine histology at diagnosis rarely changes management, and can result in misdiagnosis (particularly patients in the age range typical for WT). Although a more selective approach to biopsy has been routine practice in all other countries participating in SIOP-RTSG trials, there was variation between national groups. To address this variation and provide evidence-based recommendations for the indications and recommended approach to renal tumour biopsy within the SIOP paradigm, an international, multidisciplinary working group of SIOP-RTSG members was convened. We describe the resulting recommendations of this group, which are to be incorporated in the ongoing SIOP-RTSG UMBRELLA study
Prospective study of 18F-FDG PET in pediatric mediastinal lymphoma: A single center experience
Fine-needle aspiration in desmoplastic small round cell tumor: A report of 10 new tumors in 8 patients with clinicopathological and molecular correlations with review of the literature
BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a rare round
cell sarcoma entity characterized by a specific t(11;22)(p13;q12)
translocation, usually intra-abdominal localization and an aggressive
clinical outcome. To date, only 35 DSRCT cases diagnosed by fine-needle
aspiration have been described. METHODS This study reports the
cytological diagnosis of DSRCT. Ten tumors from 8 patients were sampled
for diagnosis and analyzed to search the characteristic translocation
using fluorescence in situ hybridization or reverse transcription
polymerase chain reaction methods. RESULTS Smears were always
hypercellular and consisted of nonspecific round cell sarcoma. Nuclei
were polymorphic round, kidney-, or heart-shaped. Nuclear molding was
usually present. Paranuclear cytoplasmic densities were obvious and
noted in 7 cases. Cytonuclear atypia, mitotic figures, numerous crushed
nuclei, and apoptosis were frequently seen. Purple-stained stroma was
present in 8 cases (ranging from few connective tissue fragments to
large hyalinized deposits). Molecular studies based on cytological
aspirates were performed in 8 patients. The presence of the fusion gene
EWSR1-WT 1 transcript was identified in all, which confirmed the
diagnosis of DSRCT. CONCLUSIONS Smears showing poorly differentiated
round cells associated with cytoplasmic densities and connective stoma,
in a specific clinical context, young adult age, intra-abdominal
localization, suggestive immunocytochemical profile, and a unique
cytogenetic abnormality are highly specific and allow an accurate
diagnosis of DSRCT. Cancer (Cancer Cytopathol) 2014;122:386-393. (c)
2014 American Cancer Society
Second‐line therapy in young patients with relapsed or refractory orbital rhabdomyosarcoma
How we approach paediatric renal tumour core needle biopsy in the setting of preoperative chemotherapy: A Review from the SIOP Renal Tumour Study Group
The International Society of Paediatric Oncology Renal Tumour Study Group (SIOPRTSG) advocate treating children with Wilms tumour (WT) with preoperative
chemotherapy, whereas the Renal Tumor Committee of the Children’s Oncology Group
(COG) advocates primary nephrectomy (without biopsy) when feasible. Successive
SIOP-RTSG trial protocols recommended pretreatment biopsy of children with unilateral tumours only where there were features to suggest an increased probability of a non-WT requiring a change in management. The UK experience in the SIOP
WT 2001 trial showed that an alternate approach of performing biopsies on all
children with renal tumour masses to determine histology at diagnosis rarely changes
management, and can result in misdiagnosis (particularly patients in the age range typical for WT). Although a more selective approach to biopsy has been routine practice
in all other countries participating in SIOP-RTSG trials, there was variation between
national groups. To address this variation and provide evidence-based recommendations for the indications and recommended approach to renal tumour biopsy within the
SIOP paradigm, an international, multidisciplinary working group of SIOP-RTSG members was convened. We describe the resulting recommendations of this group, which
are to be incorporated in the ongoing SIOP-RTSG UMBRELLA study
Is It Pre-Enucleation Chemotherapy or Delayed Enucleation of Severely Involved Eyes With Intraocular Retinoblastoma That Risks Extraocular Dissemination and Death?
Cranio-facial second primary tumors in retinoblastoma patients previously treated by radiation therapy: a multicenter clinical and radiological study of 42 patients
Guidelines for Imaging and Staging of Neuroblastic Tumors: Consensus Report from the International Neuroblastoma Risk Group Project
Neuroblastoma is an enigmatic disease entity; some tumors disappear spontaneously without any therapy, while others progress with a fatal outcome despite the implementation of maximal modern therapy. However, strong prognostic factors can accurately predict whether children have good or bad disease at diagnosis, and the clinical stage is currently the most significant and clinically relevant prognostic factor. Therefore, for an individual patient, proper staging is of paramount importance for risk assessment and selection of optimal treatment. In 2009, the International Neuroblastoma Risk Group (INRG) Project proposed a new staging system designed for tumor staging before any treatment, including surgery. Compared with the focus of the International Neuroblastoma Staging System, which is currently the most used, the focus has now shifted from surgicopathologic findings to imaging findings. The new INRG Staging System includes two stages of localized disease, which are dependent on whether image-defined risk factors (IDRFs) are or are not present. IDRFs are features detected with imaging at the time of diagnosis. The present consensus report was written by the INRG Imaging Committee to optimize imaging and staging and reduce interobserver variability. The rationales for using imaging methods (ultrasonography, magnetic resonance imaging, computed tomography, and scintigraphy), as well as technical guidelines, are described. Definitions of the terms recommended for assessing IDRFs are provided with examples. It is anticipated that the use of standardized nomenclature will contribute substantially to more uniform staging and thereby facilitate comparisons of clinical trials conducted in different parts of the world. (C) RSNA, 201