Intrathoracic malignant peripheral nerve sheath tumor with angiosarcoma in a child with NF1.

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in children and adolescents but occur more frequently in NF1 patients. Angiosarcomatous differentiation in MPNSTs is a rare entity with poor prognosis. We report on a 13-year-old boy with intrathoracic angiosarcoma arising in MPNST associated with NF1.

Current radiological techniques used to evaluate unilateral partial ureteral obstruction: an experimental rabbit study.

AIM: The aim of this study was to evaluate functional and prognostic benefits of Doppler ultrasonography (DU), diuretic renal scintigraphy (DRS), and magnetic resonance urography (MRU) during diagnosis and follow-up of ureteropelvic junction obstruction (UPJO) and to examine apoptosis rates caused by UPJO in an experimental rabbit model. METHOD: Twenty-four rabbits were divided randomly into two groups. The left kidneys of 15 rabbits from the first group underwent Ulm-Miller surgery to create UPJO, whereas the left kidneys of nine rabbits from the second group underwent sham surgery. A pressure flow study (Whitaker's test) was done during postoperative week 6. Based on the Whitaker test, the DU, DRS, and MRU findings were compared. The number of apoptotic renal cells was counted after death. RESULT: The Whitaker test run during postoperative week 6 revealed obstructions in 15 rabbits from group 1; the nine rabbits of the sham group had no obstructions. Sensitivity and specificity of DRS were 93.3 and 88.8 %, respectively, and those of MRU were 93.3 and 88.8 %, respectively. The postoperative mean RI values were significantly higher than the preoperative values, associated with sensitivity of 86.6 % and specificity of 77.5 % for detecting UPJO. DRS, MRU, and RI could not predict UPJO in one (8 %), one (8 %), and two (16 %) kidneys, respectively. Likelihood ratio (LR) was 8.4 for MRU and scintigraphy, while for RI, LR was 3.9. Pathology specimens revealed that all kidneys with UPJO underwent apoptosis, and the number of apoptotic cells was significantly higher on the UPJO-created side than on the contralateral and in the sham group (p < 0.05). No test predicted all apoptosis related to UPJO. CONCLUSION: The RI, DRS, and DMRU results correlated with the pressure flow results for detecting UPJO. No single radiological technique predicted all initial UPJO-created kidneys that concluded with apoptosis. Further studies are required to seek with better methods for diagnosing an obstruction or to define a combination of radiological techniques aiding in the management decision.

Successful pregnancy and delivery in a patient with bladder exstrophy.

Bladder exstrophy (BE) is a rare congenital anomaly. Owing to the advanced reconstructive surgical techniques and effective antibiotics, the incidence of urinary and systemic complications in patients with BE has decreased and the life expectancy has increased. However, this brings along social, sexual, and psychological problems; particularly, successful pregnancy and delivery is extremely rare in females with BE. We present a pregnancy of an 18-year-old female with BE, who has been followed at our university hospital since birth.

Is voiding cystourethrogram necessary in all cases of antenatal hydronephrosis?

Hydronephrosis is the most common genitourinary anomaly as detected on obstetric ultrasonography and the incidence of associated vesicoureteral reflux is around 10-12%. There is inconsistency in the literature regarding which child should under go a voiding cystourethrogram (VCUG) in cases of antenatal hydronephrosis (AHN). Besides, there is a scarcity of prospective studies to demonstrate the risk of varying degree of AHN, associated reflux and their long-term impact on the kidneys. The present analysis suggests that children with AHN should undergo an ultrasound within the first month of life and further course of action should be decided on the basis of the individual case. Children with persistent moderate to severe AHN should undergo a VCUG and a functional study.