Long-term renal function and hypertension in adult survivors of childhood sarcoma

Aim: Little data is available on long-term renal impairment in survivors from childhood sarcoma. We investigated the prevalence of renal impairment and hypertension after very long-term follow-up in survivors who reached adulthood after treatment for childhood sarcoma. Methods. A cross-sectional single center study was performed. Outcomes included estimating glomerular filtration rate (eGFR), albuminuria, glycosuria, serum phosphate and magnesium, tubular reabsorption phosphate (TRP), chronic kidney disease (CKD) according to the “Kidney Disease: Improving Global Outcomes” (KDIGO) guidelines and blood pressure (BP). Results: Out of 87>5-year sarcoma survivors, 30 adults (10F/20M, median age at diagnosis 9 years, median age at investigation 26 years, median follow-up 16 years, mean 19 years) were identified. Renal impairment was detected in 4 cases (13.3%); 3 of these fulfilled the criteria for CKD. Among the adult survivors, a subgroup of 15 cases (50%) had received ifosfamide without confounding factors such as a diagnosis of genito-urinary rhabdomyosarcoma or administration of other potentially nephrotoxic chemotherapy (platinum-based drugs or methotrexate); no renal dysfunction was detected in this subgroup. In the whole cohort of sarcoma survivors, hypertension was diagnosed in 4 cases (13.3%); BP was significantly correlated with body mass index [p 0.014] Conclusion: In our series of adult survivors treated for a diagnosis of sarcoma in their childhood, the prevalence of CKD was 10%. We found survivors treated with ifosfamide as the only nephrotoxic agent did not present glomerular or tubular toxicity at long term follow-up, but further studies including a larger number of cases are required to confirm it.

Propranolol as first-line treatment for life-threatening diffuse infantile hepatic hemangioma: a case report

[No abstract available

Surgical treatment of retrosternal extraosseous Ewing Sarcoma in a 6-years old female: a clamshell approach with hemysternectomy and application of a non-crosslinked extracellular matrix

Background Ewing Sarcoma (ES) and Neuroblastoma (NB) belong to a family of tumours of primitive neuroectodermal origin (PNET) that occurs in both bone and soft tissue. Notwithstanding ES and NB are two distinct malignant tumours, sometimes there could be a link between them. Case report We describe a case of an extraosseous ES localized in the retrosternal region and the upper lobe of the right lung, which had been previously treated for NB in a 6 years old female. We treated this case with a clamshell approach which allows, in a one-step surgery, a complete excision of the mass reconstructing the hemysternectomy with a non-crosslinked matrix. Conclusion the clamshell approach is therefore useful to achieve the retrosternal space and the lung with a single surgical access. According to our experience, we consider appropriate to use a non-crosslinked matrix for sternal reconstruction

Effectiveness of oral propranolol in a patient with neurofibromatosis type 1 and infantile hemangiomas

Effectiveness of oral propranolol in a patient with neurofibromatosis type 1 and infantile hemangiomas

Vincristine, Irinotecan, and Bevacizumab in Relapsed Wilms Tumor With Diffuse Anaplasia

The prognosis of relapsed Wilms tumor (WT) with diffuse anaplasia is dismal, therefore, novel therapeutic strategies need to be explored. We reported on 2 consecutive cases with relapsed anaplastic WT who presented a partial response after 2 courses of vincristine, irinotecan, and bevacizumab association. This regimen may have a role in the treatment of patients with anaplastic advanced WT

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

[No abstract available

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