We present a series of four newborns diagnosed with complicated congenital vascular anomalies, with different localization: Congenital lymphatic malformation (CLM) on the left hemithorax extending on the left upper limb; congenital hepatic hemangioma (CHH) with important complications in the first 7 weeks of life; Kaposiform hemangioendothelioma (KHE) of the left lower limb complicated with Kasabach Merritt phenomenon (KMM) and most probable diffuse capillary malformation with overgrowth (DCMO). All patients were treated with combined antiproliferative therapy with sirolimus and propranolol. The initial dose of sirolimus was 0.45-0.5 mg/m
2
with doses adjusted according to plasmatic levels. Therapeutic intervals of sirolimus were considered at plasmatic levels of 7-12 ng/ml. Our aim was to use the lowest therapeutic dose in order to avoid possible side effects. Propranolol was initiated in doses of 0.5-1.0 mg/kg/day and was increased up to 3.0 mg/kg/day depending on tolerability. Following two months, every patient showed a marked reduction in the size of the mass, improvement in overall appearance or even calcification in the liver vascular tumor. No patient showed life threatening side effects to the treatment. Hypertriglyceridemia was the only side effect noted in all patients. This is in accordance with several international studies, which try to demonstrate the importance of sirolimus in neonatal vascular malformations in monotherapy or combined with different drugs.
Nephroblastoma is the most common pediatric abdominal malignancy, representing approximately 30% of pediatric cancers.Our case concerns a 3-year-old girl diagnosed with bilateral nephroblastoma, who presented 4 renal tumors, of which one that originated in the left kidney was occupying almost entirely the left half of her abdomen. The patient, D. A., was hospitalized in the surgery department of „Maria Sklodowska Curie” Emergency Children’s Hospital in Bucharest on 10.10.2014 in order to continue investigations regarding a right renal mass discovered incidentally during a routine abdominal echography. An abdominal CT scan revealed 2 masses in her left kidney, the largest of which measured 12/8/9 cm, and another 2 in her right kidney.Clinical and paraclinical data was suggestive for nephroblastoma, so a 4 week combined chemotherapy regimen (Vincristine and Actinomycin D) was initiated. A second abdominal CT showed a significant reduction in the size of the masses, which prompted physicians to repeat the regimen. Afterwards, a third abdominal CT scan showed an even further reduction in size, the largest mass now measuring 49/43 mm.The patient underwent a left ureteronephrectomy on 24.12.2014, along with the excision of the 2 right renal tumors. Postoperatively, a third chemotherapy regimen was initiated, with the same medication, this time lasting 27 weeks. At the time of publishing, the patient is still under chemotherapy treatment. This case presents a few interesting features, highlighting the possibility of a very positive response to treatment of nephroblastoma, despite a dire initial prognosis.
Clinical presentation of hepatoblastoma is often insidious and its paraneoplastic manifestations are not uncommon. We report an extremely rare case of a renin-secreting hepatoblastoma inducing malignant hypertension remarked in our clinic. The hepatic malignancy classified as PRETEXT IV and a multidisciplinary approach according to SIOPEL III was made with a poor outcome. Hepatoblastoma in children should be considered as possible etiology of hypertension in children.
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