Developments in the Surgical Approach to Staging and Resection of Rhabdomyosarcoma

Local treatment; Rhabdomyosarcoma; SurgeryTratamiento local; Rabdomiosarcoma; CirugíaTractament local; Rabdomiosarcoma; CirurgiaAlthough survival after rhabdosarcoma treatment has improved over the years, one third of patients still develop locoregional relapse. This review aims to highlight developments pertaining to staging and local treatment of specific RMS tumor sites, including head and neck, chest/trunk, bladder-prostate, female genito-urinary, perianal, and extremity sites

Nuclear Medicine in Pediatric Nephro-Urology: An Overview.

In the context of ante-natally diagnosed hydronephrosis, the vast majority of children with a dilated renal pelvis do not need any surgical treatment, as the dilatation resolves spontaneously with time. Slow drainage demonstrated at Tc-99m-mercaptoacetyltriglycine (MAG3) renography does not necessarily mean obstruction. Obstruction is defined as resistance to urinary outflow with urinary stasis at the level of the pelvic-ureteric junction (PUJ) which, if left untreated, will damage the kidney. Unfortunately this definition is retrospective and not clinically helpful. Therefore, the identification of the kidney at risk of losing function in an asymptomatic patient is a major research goal. In the context of renovascular hypertension a DMSA scan can be useful before and after revascularisation procedures (angioplasty or surgery) to assess for gain in kidney function. Renal calculi are increasingly frequent in children. Whilst the vast majority of patients with renal stones do not need functional imaging, DMSA scans with SPECT and a low dose limited CT can be very helpful in the case of complex renal calculi. Congenital renal anomalies such as duplex kidneys, horseshoe kidneys, crossed-fused kidneys and multi-cystic dysplastic kidneys greatly benefit from functional imaging to identify regional parenchymal function, thus directing further management. Positron emission tomography (PET) is being actively tested in genito-urinary malignancies. Encouraging initial reports suggest that F-18-fluorodeoxyglucose (FDG) PET is more sensitive than CT in the assessment of lymph nodal metastases in patients with genito-urinary sarcomas; an increased sensitivity in comparison to isotope bone scans for skeletal metastatic disease has also been reported. Further evaluation is necessary, especially with the promising advent of PET/MRI scanners. Nuclear Medicine in paediatric nephro-urology has stood the test of time and is opening up to new exciting developments

Epidemiology of paediatric renal stone disease: a 22-year single centre experience in the UK

Abstract Background Whilst still rare, the incidence of paediatric stone disease is increasing in developed countries and it is important to evaluate the aetiology. We set up a dedicated renal stone service for children combining medical and surgical expertise in 1993 and now have a large case series of children to investigate the epidemiology. Methods A retrospective hospital note review of children presenting with kidney stones during the last 22 years (1993–2015) was conducted. All patients had a comprehensive infective and metabolic screen and were classified as metabolic, infective or idiopathic stone disease. Results Five hundred eleven patients (322 male) were reviewed. The median age of presentation was 4.4y for males (1 m-16.6y) and 7.3y (1–18.5y) for females with a median height and weight on the 25th centile for male and on 10th and 25th for female, respectively. One hundred seventy five (34%) had an underlying metabolic abnormality, 112 (22%) had infective stones and 224 (44%) were classified as idiopathic. Of the 175 patients with a metabolic abnormality: 91 (52%) had hypercalciuria (76 persistent and 15 transient), 37 (21%) hyperoxaluria, 38 (22%) cystinuria, 3 (2%) abnormalities in the purine metabolism and the remainder other metabolic abnormalities. Bilateral stones occurred in 27% of the metabolic group compared to 16% in the non-metabolic group (OR 0.2, p < 0.05). Urinary tract infection was a common complication (27%) in the metabolic group. Conclusions In this paper, we present the largest cohort of paediatric stone disease reported from a developed country giving details on both, clinical and laboratory data. We show that in the majority of the patients there is an identifiable underlying metabolic and/or infective aetiology emphasizing the importance of a full work up to provide adequate treatment and prevent recurrence. Moreover, we show that stone disease in children, in contrast to the adult population, does not seem to be associated with obesity, as children have a weight below average at presentation

Developments in the Surgical Approach to Staging and Resection of Rhabdomyosarcoma

Although survival after rhabdosarcoma treatment has improved over the years, one third of patients still develop locoregional relapse. This review aims to highlight developments pertaining to staging and local treatment of specific RMS tumor sites, including head and neck, chest/trunk, bladder-prostate, female genito-urinary, perianal, and extremity sites

Developments in the Surgical Approach to Staging and Resection of Rhabdomyosarcoma

Although survival after rhabdosarcoma treatment has improved over the years, one third of patients still develop locoregional relapse. This review aims to highlight developments pertaining to staging and local treatment of specific RMS tumor sites, including head and neck, chest/trunk, bladder-prostate, female genito-urinary, perianal, and extremity sites

A Single-Center, Observational Study of 607 Children and Young People Presenting With Differences of Sex Development (DSD).

CONTEXT: Differences of sex development (DSD) represent a wide range of conditions presenting at different ages to various health professionals. Establishing a diagnosis, supporting the family, and developing a management plan are important. OBJECTIVE: We aimed to better understand the presentation and prevalence of pediatric DSD. METHODS: A retrospective, observational cohort study was undertaken in a single tertiary pediatric center of all children and young people (CYP) referred to a DSD multidisciplinary team over 25 years (1995-2019). In total, 607 CYP (520 regional referrals) were included. Data were analyzed for diagnosis, sex-assignment, age and mode of presentation, additional phenotypic features, mortality, and approximate point prevalence. RESULTS: Among the 3 major DSD categories, sex chromosome DSD was diagnosed in 11.2% (68/607) (most commonly 45,X/46,XY mosaicism), 46,XY DSD in 61.1% (371/607) (multiple diagnoses often with associated features), while 46,XX DSD occurred in 27.7% (168/607) (often 21-hydroxylase deficiency). Most children (80.1%) presented as neonates, usually with atypical genitalia, adrenal insufficiency, undescended testes or hernias. Those presenting later had diverse features. Rarely, the diagnosis was made antenatally (3.8%, n = 23) or following incidental karyotyping/family history (n = 14). Mortality was surprisingly high in 46,XY children, usually due to complex associated features (46,XY girls, 8.3%; 46,XY boys, 2.7%). The approximate point prevalence of neonatal referrals for investigation of DSD was 1 in 6347 births, and 1 in 5101 overall throughout childhood. CONCLUSION: DSD represent a diverse range of conditions that can present at different ages. Pathways for expert diagnosis and management are important to optimize care