Congenital Stage 1 Neuroblastoma Evolved Into Stage 4s

A newborn with a prenatally detected adrenal mass underwent complete resection of it Stage 1 favorable histology neuroblastoma (NB) without MYC-N amplification. Two months later. the infant presented with a local recurrence and multiple hepatic metastases. Close follow-up without therapy was adopted for stage 4s NB. Enlarging tumor lesions were seen until the child was S months old. followed by later decrease in size. At 36 months of follow-up. the child is alive and disease-free. We describe this case of NB and its abnormally short evolution from stage 1 to stage 4s. despite initial surgery. Its Spontaneous regression May help us understand the natural history of congenital NB

Late Relapse of Botryoid Embryonal Rhabdomyosarcoma of the Vagina in Prepubertal Age

[No abstract available

Barrett Esophagus in Long-term Survivors of Childhood Solid Tumors

We report on 2 cases of long-term survivors of childhood solid tumors, who developed Barrett esophagus (BE) after treatment for neuroblastoma and Hodgkin lymphoma, respectively. Case 1: A stage 3 neuroblastoma was treated with surgery, carboplatin/etoposide chemotherapy, and supradiaphragmatic radiotherapy (30 Gy). Twelve years later, based on endoscopic and histologic findings, BE was diagnosed on the middle segment. Case 2: A stage IIIB Hodgkin lymphoma received mechloretamine, oncovin, procarbazine, prednisone/adriamycin, bleomycin, vinblastine, dacarbazine chemotherapy and supra/subdiaphragmatic radiotherapy (25 Gy). Nineteen years later, BE was diagnosed associated with an esophageal stricture. In long-term survivors of childhood tumors who had received chest/neck radiotherapy and chemotherapy, the risk of BE may be increased, therefore the diagnosis should be considered in the presence of gastroesophageal symptoms

Venous thrombosis and procoagulant factors in high-risk neuroblastoma

Aim: The mechanism of increased thrombin production has been investigated in children with high-risk neuroblastoma (NB), to detect any possible association between catheter- related venous thrombosis (VT) and prothrombotic factors. Methods: Consecutive children with high-risk NB were studied by color-doppler ultrasonography of the upper vein system and thrombophilia factors assessment. Plasma levels of Tissue Factor (TF), Vascular Endothelial Growth Factor (VEGF), Prothrombin Activation Fragment 1+2, and Thrombin-Antithrombin Complex were evaluated. Moreover, inherited thrombophilia factors (homocystein, antithrombin, protein C, protein S, factor V Leiden, activated protein C resistence, mutation H1299R and G1691A of factor V, mutation G20210A of prothrombin, mutation T677 and A1298C of methylenetetrahydrofolate reductase, and allele 4G of plasminogen activator inhibitor-1) were tested to exclude congenital disorders. Results: Six patients with mean age: 48.8 months - were studied. Five patients were affected by stage 4 NB and another one by stage 3 NB with Myc-N amplification. All children had a central venous line (mean duration: 8.5 mos). Four patients (67%) had asymptomatic catheter-related VT visualized by color-doppler ultrasonography. No patient had major inherited thrombophilia factors. The levels of plasma TF and plasma VEGF were found elevated in all patients. Mean value of TF (nv 20.3±6.6) was 82 pg/mL with a range of 39 to 131 pg/mL. Mean value of VEGF (nv 24.3 pg/mL) was 78.5 pg/mL with a range of 31 to 142 pg/mL. Conclusion: The increased risk of catheter-related VT detected in our small series of high-risk NB patients, was associated with elevated levels of circulating TF and VEGF. Further studies are needed to evaluate if elevated levels of TF/VEGF are involved both in the hypercoagulable state and in advanced childhood cancer