Accelerated Unilateral Radiographic Huntingtonian Changes Following Neoadjuvant Chemotherapy for a Nongerminomatous Germ Cell Tumor Leading to Identification of Occult Disease in the Dorsal Striatum.

Basal ganglia nongerminomatous germ cell tumors comprise 10% to 15% of germ cell tumor and have substantial morbidity at the time of local failure. In this submitted image we present a case where neoadjuvant chemotherapy unmasked a unilateral caudate head loss consistent with Huntingtonian changes. Careful review of the patient's imaging identified disease within the dorsal striatum that was not previously identified at the time of diagnosis. Review of the diffusion tensor fractional anisotropy imaging identified progressive white matter likely secondary to the occult disease within the dorsal striatum. Although this patient was asymptomatic and had no signs of a movement disorder, similar findings have been noted to be a prelude to such findings several months later. The occult disease was incorporated into the patient's radiotherapy planning target volume as oversight of these changes would have led to a marginal miss and potential early disease relapse.

Case Report: Insulin hypersensitivity in youth with type 1 diabetes.

Objective: Immediate type I, type III, and delayed type IV hypersensitivity reactions to insulin are rare, but potentially serious complications of exogenous insulin administration required for the treatment of type 1 diabetes (T1D). Methods: We present four cases of insulin hypersensitivity reactions occurring in youth with T1D and a literature review of this topic. Results: Insulin hypersensitivity reactions included types I, III, and IV with presentations ranging from localized urticaria, erythematous nodules, and eczematous plaques to anaphylaxis with respiratory distress. Reactions occurred in youth with newly diagnosed T1D and in those with long-standing T1D who were using both injection and insulin pump therapy. Multidisciplinary care involving pediatric endocrinology and allergy/immunology utilizing trials of many adjunct therapies yielded minimal improvement. Despite the use of various treatments, including antihistamines, topical therapies, immunosuppressant medications, desensitization trials, and intravenous immune globulin, cutaneous reactions, elevated hemoglobin A1c levels, and negative effects on quality of life remain persistent challenges. One patient became one of the youngest pancreas transplant recipients in the world at age 12 years due to uncontrollable symptoms and intolerable adverse effects of attempted therapies. Conclusion: Although rare, insulin hypersensitivity reactions negatively affect glycemic control and quality of life. These cases demonstrate the varying severity and presentation of insulin hypersensitivity reactions along with the limited success of various treatment approaches. Given the life-sustaining nature of insulin therapy, further studies are needed to better understand the underlying pathophysiology of insulin hypersensitivity and to develop targeted treatment approaches.

Pediatric genitourinary tumors.

PURPOSE OF REVIEW: We will review the 2010/2011 literature on pediatric genitourinary tumors and highlight the most significant publications. RECENT FINDINGS: New techniques such as gene expression profiling, PET, and nephron-sparing surgery are being incorporated into contemporary treatments for pediatric patients with genitourinary tumors. Biologic markers are increasingly being used to help with risk stratification of patients and to identify new targets for therapy. WT1 mutation and 11p15 loss of heterozygosity have been associated with relapse in very low-risk Wilms tumors treated with surgery alone and may help reduce the use of chemotherapy in some children. Meta-analysis of data on the use of high-dose chemotherapy with autologous hematopoietic stem cell rescue in patients with relapsed Wilms tumor and rhabdomyosarcoma suggests that some patients may benefit more from conventional salvage chemotherapy. New agents are needed for patients with high-risk and relapsed disease to improve outcomes. SUMMARY: In general, the prognosis for patients with pediatric genitourinary tumors is favorable. Further understanding of the biology in these tumors is helping to determine risk stratification, treatment strategies, and candidates for new drug development.

Pediatric genitourinary tumors.

PURPOSE OF REVIEW: To review the 2009/2010 literature on pediatric genitourinary tumors and highlight the most significant publications. RECENT FINDINGS: New techniques such as gene expression profiling, PET, nephron-sparing surgery, and stem cell transplantation are being incorporated into contemporary treatments for pediatric patients with genitourinary tumors. Biologic markers are increasingly being used to help with risk stratification of patients. WT1 mutation and 11p15 loss of heterozygosity have been associated with relapse in very low-risk Wilms tumors treated with surgery alone and may help reduce the use of chemotherapy in some children. Discussion continues on the use of fusion gene status to risk stratify alveolar rhabdomyosarcoma. Meta-analysis of the use of high-dose chemotherapy with autologous hematopoetic stem cell rescue in patients with relapsed Wilms tumor and rhabdomyosarcoma suggests that some patients may benefit more from conventional salvage chemotherapy. New agents are needed for patients with high-risk and relapsed disease to improve outcomes. SUMMARY: In general, the prognosis for patients with pediatric genitourinary tumors is favorable. The elucidation of the molecular abnormalities in these tumors is determining risk stratification, treatment strategies, and candidates for new drug development.

Pediatric genitourinary tumors.

PURPOSE OF REVIEW: To review the 2008-2009 literature on pediatric genitourinary tumors and highlight the most significant publications. RECENT FINDINGS: New techniques such as gene expression profiling, PET, nephron-sparing surgery, and stem cell transplantation are being incorporated into contemporary treatments for pediatric patients with genitourinary tumors. The WTX gene is the most commonly mutated gene in Wilms tumor, and its product enhances Wilms tumor gene 1-mediated transcription. Germline WTX mutations cause an X-linked sclerosing bone dysplasia but do not appear to predispose to Wilms tumor formation. Protocadherin gene clusters on chromosome 5q31 may act as tumor suppressors. In rhabdomyosarcoma, ILK and platelet-derived growth factor receptor-A join the paired box gene 7 and 3-forkhead box O1 fusions as potential therapeutic targets, and muscle-specific microRNAs offer promise as adjuvant therapy. Despite the high cure rate of Wilms tumor, long-term survivors remain at risk of death from various causes. SUMMARY: In general, the prognosis for patients with pediatric genitourinary tumors is favorable. The elucidation of the molecular abnormalities in these tumors is determining risk stratification, treatment strategies, and candidates for new drug development.

Patient size measured on CT images as a function of age at a tertiary care children's hospital.

OBJECTIVE: The purpose of our study was to measure patient size on CT images as a function of age at a large tertiary care children's hospital to develop current patient size data for modeling optimal x-ray exposure factors in children. MATERIALS AND METHODS: Anteroposterior and transverse dimensions of the head, thorax, abdomen, and pelvis were measured on CT examinations of pediatric patients less than 21 years old performed between June and November 2007. Patients with diseases that could affect measurements were excluded. From 1,009 patients, 336 examinations of each of four body regions were selected; 2,688 measurements were made and separated into 21 groups. Statistical model building and prediction equations were established for each region and 95% prediction intervals were used for analyses. RESULTS: Rapid growth of the head occurred from birth to approximately 2 years followed by a gradual plateau until 21 years. The thoracic, abdominal, and pelvic regions showed a linear relationship between age and size. Fitted equations showed transverse trunk measurements increased more rapidly than anteroposterior measurements. The anteroposterior trunk size growth rate was relatively region independent; transverse pelvic dimensions grew more rapidly than thoracic or abdominal regions. There was a broad overlap of predicted patient size ranges as a function of age within each region. Excellent interobserver agreement was measured by Pearson's correlation coefficient (r) (all p < 0.0001). CONCLUSION: Fitted average patient sizes are age dependent; however, predicted individual patient size does not correlate well with age. Our study suggests that pediatric patient body size should be determined for individual patients before performing diagnostic imaging procedures that entail radiation risks.