Pediatric Oncology

·         Specific genetic conditions are believed to account for <5% of all pediatric malignancies.

·         The clinical features of the childhood leukemias are similar, because all involve severe disruption of bone marrow function. There is marked variability in response to therapy and in the prognosis.

·         About 20-30% of neonates with Down syndrome who develop a transient leukemia or myeloproliferative syndrome will develop typical leukemia within the first few years of life.

·         More than two thirds of cases of infant leukemia demonstrate rearrangements of the MLL gene, classically a translocation involving the q23 band of chromosome 11, and it is this subset of patients that largely accounts for the very high relapse rate.

·         Children with neuroblastoma with early-stage disease without amplified MYCN or chromosome 1p deletion can usually be cured with surgery alone. Children <1 yr of age have a 95% 3-yr survival rate, compared with 25-50% for children 1-5 yr of age.

·         Opsomyoclonus ("dancing eyes and dancing feet") is a paraneoplastic syndrome of autoimmune origin that is associated with neuroblastoma.

·         The patients at highest risk of Tumor Lysis Syndrome those with the most rapidly dividing tumors. The highest risk is with Burkitt's lymphoma/leukemia, followed by T-lineage acute lymphoblastic leukemia and T-cell lymphoblastic lymphoma.

·         Children who require repeated blood draws or intravenous medications often have a semipermanent central venous catheter placed.

A Broviac catheter is tunneled through the subcutaneous tissues of the chest and emerges as a thin plastic tube, usually at the level of the second or third rib.

A Port-A-Cath contains a subcutaneous reservoir and is implanted under the skin of the chest. It is not visible, but it must be accessed by inserting a small needle through the skin and into the reservoir.

·         Diencephalic syndrome is the constellation of symptoms that result from the presence of a hypothalamic tumor: euphoria, emaciation, and emesis.

·         Parinaud's syndrome is the result of increased intracranial pressure at the dorsal midbrain, causing downgaze, papillary dilation, and nystagmus.

·         Bilateral Wilms' tumor is known as a stage V tumor. Each tumor is staged independently; prognosis with bilateral disease is not necessarily poor.

·         Conditioning is the preparative process, performed by either chemotherapy or chemoradiotherapy, that is designed to destroy residual malignant cells, to provide immunosuppression to minimize the chance of rejection, and to create space in the marrow itself for the transplantation (bone-marrow ablation).