The combination of conservative surgery, chemotherapy, and exclusive HDR to postchemotherapy tumor volume with a modest margin, avoiding EBRT, provided disease control in carefully selected young children, while preserving bone growth and organ function. The short duration of therapy and small volume irradiated allowed chemotherapy to be resumed shortly after brachytherapy. The use of HDR challenges the present philosophy of radiotherapy treatment volume, which holds that the prechemotherapy tumor volume should be treated with an acceptable margin. Brachytherapy should be included in multicentric clinical trials in young children.

Congenital leukemia is a rare disease developing within the first 4 to 6 weeks of life. We report a female infant born with facial mass and multiple subcutaneous nodules. The facial mass was discovered by ultrasound during a routine prenatal examination at the 36th week of gestation. Biopsies were consistent with the diagnosis of acute monoblastic leukemia (AML, FAB M5b). Cytogenetic studies showed 46 XX, t(11;19)(q23;p13.1), which is only found in acute monoblastic leukemia and involves the gene. The infant died at 12 days of age and autopsy revealed a large leukemic tumor burden in several body organs. The discovery of the facial mass prenatally and massive extramedullary leukemic burden support the notion of the in utero development of congenital leukemia.

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding disorder characterized by normal platelet count, but lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of the membrane glycoprotein IIb/IIIa complexes. Usually it is associated with mild bleeding but may lead to severe and potentially fatal hemorrhages. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment. However, because of the risks associated with HSCT, it is generally not recommended unless there are life threatening hemorrhages, or the patient has developed refractoriness to platelet transfusion due to antibody formation. Herein, we report an 11-year-old female from United Arab Emirates (UAE) with severe GT and anti platelet alloimmunization successfully treated with HSCT from her HLA-identical sibling.

The authors describe the development of bladder paralysis in a child with acute lymphoblastic leukemia undergoing maintenance chemotherapy. Immediately before the adverse clinical event, the child had received vincristine intravenously and triple therapy with hydrocortisone, cytosine arabinoside, and methotrexate intrathecally and had begun a 5-day pulse of prednisolone. The authors conclude that the ensuing reversible bladder paralysis was related to the vincristine. The clinical event resolved, and vincristine was deleted from the child's subsequent therapy until full recovery was achieved. The authors advise recognition of this problem and discontinuation of the vincristine if transient bladder paralysis develops until symptoms completely disappear.

Hepatic Sinusoidal Obstruction Syndrome (HSOS), the new name given to veno-occlusive disease (VOD) of the liver, is a well-known complication of high-dose chemotherapy employed with hematopoietic stem cell transplantation, but it has rarely been observed in children who receive conventional chemotherapy. HSOS following standard chemotherapy has been reported in patients receiving vincristine, actinomycin D, and cyclophosphamide for the treatment of Wilms tumor and more rarely rhabdomyosarcoma. We report a 14-year-old boy with high risk medulloblastoma treated with craniospinal radiation followed by chemotherapy, who experienced severe HSOS after only one course of chemotherapy including carboplatin, vincristine, and cyclophosphamide. To our knowledge, this is the second report of HSOS after standard dose chemotherapy for brain tumor in childhood.