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PURPOSE We retrospectively analyzed our institutional incidence of hemorrhagic cystitis, identified risk factors, and examined associations of risk factors with disease severity and genitourinary complication rates. MATERIALS AND METHODS We reviewed charts of all consecutive pediatric patients treated from 1986 to 2010. We analyzed demographics,(More)
Fibrolamellar hepatocellular carcinoma is a rare, malignant liver tumor that often arises in the otherwise normal liver of adolescents and young adults. Previous studies have focused on biomarkers and comparisons to traditional hepatocellular carcinoma, and have yielded little data on the underlying pathophysiology. We performed whole genome sequencing on(More)
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare liver tumor affecting adolescents and young adults with no history of primary liver disease or cirrhosis. We identified a chimeric transcript that is expressed in FL-HCC but not in adjacent normal liver and that arises as the result of a ~400-kilobase deletion on chromosome 19. The chimeric RNA is(More)
PURPOSE Desmoid fibromatosis is associated with frequent recurrence and significant morbidity, but no metastases. To examine the impact of initial non-operative management on event-free survival (EFS) in children, we reviewed our institutional experience with this tumor. METHODS We retrospectively reviewed our institutional database for pediatric cases of(More)
Fibrolamellar hepatocellular carcinoma (FLHCC) tumors all carry a deletion of ∼ 400 kb in chromosome 19, resulting in a fusion of the genes for the heat shock protein, DNAJ (Hsp40) homolog, subfamily B, member 1, DNAJB1, and the catalytic subunit of protein kinase A, PRKACA. The resulting chimeric transcript produces a fusion protein that retains kinase(More)
Malignant tumors of the liver comprise a relatively small fraction of the total number of pediatric malignancies. However, these tumors can be a significant cause of morbidity and mortality, and there have been significant therapeutic gains during the past few decades through advances in systemic therapy and surgical treatment. Even in patients with(More)
INTRODUCTION Retroperitoneal partial nephrectomy has not been studied as a surgical approach for children with bilateral Wilms tumor. There are advantages to this technique, including isolation of urine leaks to the retroperitoneum, decreased risk of bowel injury, and decreased time to resuming a diet. Presently, all bilateral Wilms tumors are treated with(More)
Chest wall tumors in the pediatric population can have a variety of etiologies, malignancy being the most worrisome. Hodgkin lymphoma (HL) rarely presents as a chest wall mass in the pediatric population. In this report, we describe 3 male pediatric patients, all of whom had chest wall masses present at the initial diagnosis of HL. We also discuss the(More)
The study of cancer immunology has provided diagnostic and therapeutic instruments through serum autoantibody biomarkers and exogenous monoclonal antibodies. While some endogenous antibodies are found within or surrounding transformed tissue, the extent to which this exists has not been entirely characterized. We find that in transgenic and xenograft mouse(More)