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BACKGROUND Rhabdoid tumours (RTs) are aggressive malignancies of childhood, mainly occurring in the kidney and brain. We describe a national multi-centre retrospective analysis of extra-renal non-cranial RTs (ERRTs). PROCEDURE Diagnosis relied on central histological review and/or on hSNF5/INI1 defect, evidenced by immunohistochemistry or molecular(More)
PURPOSE Germline hSNF5/INI1 mutations are responsible for hereditary cases of rhabdoid tumors (RT) that constitute the rhabdoid predisposition syndrome (RPS). Our study provides the first precise overview of the prevalence of RPS within a large cohort of RT. EXPERIMENTAL DESIGN hSNF5/INI1 coding exons were investigated by sequencing and by multiplex(More)
PURPOSE To determine whether the clinical and molecular biologic characteristics of the alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS) subtypes have relevance independent of the presence or absence of the PAX/FOXO1 fusion gene. PATIENTS AND METHODS The fusion gene status of 210 histopathologically reviewed, clinically annotated(More)
Ovarian transposition was the first procedure proposed to preserve fertility in girls with cancer and is indicated for patients with tumours requiring pelvic radiation at doses of 42·0-58·4 Gy, much higher doses than those that can induce loss of ovarian function (4-20 Gy). Ovarian transposition is usually done after neoadjuvant chemotherapy and is(More)
BACKGROUND Wilms Tumor (WT) can occur in association with tumor predisposition syndromes and/or with clinical malformations. These associations have not been fully characterized at a clinical and molecular genetic level. This study aims to describe clinical malformations, genetic abnormalities, and tumor predisposition syndromes in patients with WT and to(More)
PURPOSE Solid pseudo-papillary tumors (SPT) are rare pancreatic neoplasms of low-malignant potential occurring mainly in young women. The purpose of this report is to describe our experience with laparoscopic management of these tumors with 4-year follow-up. METHODS Three children with SPT were admitted to two hospitals in Paris, France, between February(More)
Hypercalcemia is a rare but potentially fatal complication during the management of childhood cancer. The treatment of severe hypercalcemia in children has not been clearly defined. The authors present a retrospective series of 16 children (11 boys and 5 girls) with severe hypercalcemia (>/=2.9 mmol/L) treated between 1997 and 2004 for malignancy. Median(More)
PURPOSE Ifosfamide is widely used in pediatric oncology but its nephrotoxicity may become a significant issue in survivors. This study is aimed at evaluating the incidence of late renal toxicity of ifosfamide and its risk factors. PATIENTS AND METHODS Of the 183 patients prospectively investigated for renal function, 77 treated for rhabdomyosarcoma, 39(More)
BACKGROUND Salivary gland tumors represent 1% of head and neck tumors, with only 5% of these occurring in patients younger than 20 years. Mucoepidermoid carcinoma (MEC) is one of the most frequent salivary gland cancers among adults and children. METHODS This survey was conducted among 34 French pediatric oncology departments. From 1980 to 2000, 18 cases(More)
BACKGROUND Infantile myofibromatosis is characterized by proliferation of benign fibrous tumors arising in skin, subcutaneous tissue, muscle, or bone. Solitary and multicentric forms are described. Few reports are available in the pediatric population. PROCEDURE To improve the knowledge of this rare tumor in infants, the authors present a series of all(More)