Incidental diagnosis of thoracic ganglioneuroblastoma in a3 years old female with wheezing

Ganglioneuroblastoma (GNB) is a cancer of the peripheral sympathetic nervous system and represents the 30% of cases of Neuroblastoma.When originates from thoracic ganglia it may appear very late, with severe symptoms like respiratory distress or neurological dysfunctions.We present an incidental diagnosis of thoracic GNB, discovered by a chest radiography during a recurrent wheezing unresponsive to bronchodilators. The early diagnosis allowed a precocious treatment probably improving the outcome

Markers of Inflammation and Endothelial Dysfunction in Young Survivors from Acute Lymphoblastic Leukemia

Purpose: To assess subclinical markers of endothelial inflammation in young survivors from acute lymphoblastic leukemia (ALL) treated with chemotherapy without cranial irradiation. Methods: Anthropometric parameters [height (H), body mass index (BMI), waist circumference (WC), hip circumference (HC), WC/H, and WC/HC ratio], blood pressure, lipid profile, serum markers of inflammation and endothelial dysfunction [Interleukin 6 (IL-6), vascular cell adhesion molecule, intercellular adhesion molecule, tumor necrosis factor-alfa (TNF-α), Endogenous secretory Receptor for Advanced Glycation Endproducts (Es-RAGE)], and carotid intima-media thickness (c-IMT) were assessed in a group of young ALL survivors and in matched controls. Local Ethics Committee approved the study (code 56/13) on June 24, 2013. Results: 28 ALL survivors (71% male, 18% prepubertal, aged 15.98 ± 4.41 years, mean follow-up 8.57 ± 3.14 years) exhibited lower levels of Es-RAGE than controls (0.18 ± 0.07 vs. 0.27 ± 0.08 ng/mL, p < 0.001). Among survivors, Es-RAGE values significantly correlated with BMI-SD off-therapy (R2 -0.42), WC/H ratio (R2 -0.41), WC/HC ratio (R2 -0.38), and low-density-lipoprotein cholesterol (LDL-C; R2 -0.43). Most of the ALL survivors (78%) presented c-IMT above the 95th centile if compared with gender and age standard. Mean c-IMT value correlated with blood pressure (R2 0.56) and with LDL-C levels (R2 0.56). Metabolic syndrome (MetS) was fully detected only in one ALL survivor. Nevertheless, 18% ALL survivors presented more than one MetS diagnostic criteria: 14% insulin resistance, 25% dyslipidemia, and 17.8% hypertension. Conclusions: We demonstrated an initial functional vascular alteration in young ALL survivors even when treated with standard risk protocols. Our data already support the activation at endothelial level of glycosylation and oxidation processes that are persistent long after the end of the treatment