Rhabdomyoblastic pulmonary lesions in a patient with Wilms Tumor: choristomas or metastases?

New lesions that occur while patients are receiving treatment for malignant tumors may represent not only difficulties in arriving at the correct diagnosis, but also impact on subsequent therapeutic options. We encountered a patient developing new pulmonary lesions during and after receiving treatment for Stage II Wilms tumor (WT). The presence of mature rhabdomyoblasts in multiple biopsy specimens allowed rational decisions to be made regarding subsequent therapy. This and other published experiences suggest that patients with WT may develop choristomas or have tumors, which undergo cytodifferentiation. Methodologies are now available allowing differentiation of these two processes.     

P-Glycoprotein Expression,Tumor Weight,Age, and Relapse in Patients with Stage I and II Favorable-Histology Wilms’ Tumor

Fifteen percent of patients with Wilms’ tumor (WT) experience relapse. It has been suggested that weight and age may affect the chances of relapse. Few studies have investigated the role, if any, between P-glycoprotein (P-gp) and relapse. The authors assessed the prognostic value of tumor weight and age at diagnosis and asked whether some other potential biological markers, specifically P-gp protein expression, had a prognostic value in favorable-histology WT. No association between age and relapse could be found. Patients with tumor weight ≥550 g were 6 times more likely to relapse, whereas P-gp expression was positive in 18/40 (45%) of the patients, of which 10/12 (83.3%) relapsed and 8/28 (28.6%) did not. Further studies are necessary to elucidate whether or not P-gp is related to relapse in patients with histologically favorable Wilms’ tumor. If confirmed, the protein may be used in the future as a target for new drugs and treatments for this group of patients.     

Is Pre-operative Chemotherapy Desirable in all Patients of Wilms’ Tumor?

The timing and role of chemotherapy in the management of Wilms’ tumor has long been the matter of debate, with different groups showing equally comparable and encouraging results. Over the last decade, however, both the ideol-ogies seem to be converging and the attempt has been to identify groups benefitting with pre-operative chemotherapy, as well as those, where upfront resection should be attempted. In this article authors intend to discuss pros and cons of both the strategies and their applicability in a resource poor setting in developing countries like India.     

Anaplastic histology Wilms’ tumors registered to the Japan Wilms’ Tumor Study Group are less aggressive than that in the National Wilms’ Tumor Study 5

Purpose

To evaluate the clinical features and treatment results of anaplastic histology (AH) Wilms’ tumor (WT) patients registered in the Japan Wilms’ Tumor Study (JWiTS) group to elucidate the clinical characteristics of AH in the Japanese population.

Patients and methods

Of 344 WT patients who were enrolled in JWiTS between 1995 and 2013, 17 had AH. Treatment using the JWiTS protocols was similar to the fifth National Wilms’ Tumor Study 5 (NWTS-5) protocols. Clinical characteristics and mutation status of TP53 gene were evaluated and compared with those in NWST-5 study.

Results

AH incidences in JWiTS were 4.9 %, lower than that in NWTS-5. Seven tumors had focal AH and 10 had diffuse AH. Clinical stages of AH patients were stage I in seven, stage II in three, stage III in five, stage IV in one and unknown in one. Four-year event-free survival and overall survival rates were 90.9 and 86.7 %, respectively. Two patients with diffuse AH and none with focal AH had TP53 mutation.

Conclusion

Japanese patients presented with higher incidence, earlier stages and may have better outcomes than American patients, indicating a possible biological heterogeneity of AH WT. Further analysis is necessary to elucidate the different characteristic of AH WT between Japanese and American populations.

     

Severe intrarenal fibrosis,infundibular stenosis,renal cysts,and persistent perilobar nephrogenic rests in a patient with Beckwith-Wiedemann syndrome 27 years after diffuse nephroblastomatosis and Wilms tumor: natural progression or a consequence of treatment?

A27-year-old woman presented with back and abdominal pain. She was diagnosed in infancy with Beckwith-Wiedemann syndrome and bilateral multifocal perilobar nephrogenic rests that progressed to diffuse nephroblastomatosis with neoplastic nephroblastomatous rests at 14 months of age and subsequently to a right Wilms tumor at 5 years of age. Computed tomography of the abdomen during the current admission showed multiple obstructed calices. Ureteroscopic inspection of the left kidney revealed severe intrarenal scarring with multiple infundibular stenosis, hydrocalices, and nephrocalcinosis. Renal biopsy showed sclerotic glomeruli with calcification and scarring and persistent subcapsular nodular renal blastema. Electrocautery incision and balloon dilatation provided temporary pain relief. After discharge, the patient has had two or three episodes of recurrent pain associated with new areas of infundibular stenoses and renal cysts. Bilateral nephrectomy and renal transplantation is being considered for management of progressive disease and relief of intractable pain. The potential causes of progressive and severe intrarenal fibrosis, infundibular stenosis and nephrocalcinosis, and renal cysts in this patient may include abnormal renal development secondary to Beckwith-Wiedemann syndrome itself, radiation or chemotherapy damage, or a combination.     

Maternal and umbilical cord serum leptin concentrations in small-for-gestational-age and in appropriate-for-gestational-age neonates: a maternal,fetal, or placental contribution?

Leptin is secreted during pregnancy by the placenta and by the maternal and fetal adipose tissues. The leptin levels mainly reflect the amount of fat stored and thus are indicative of the energy balance, i.e., small-for-gestational-age (SGA) neonates represent the negative metabolic balance of in utero starved babies. We chose to compare maternal and umbilical cord leptin levels in pregnancies complicated by asymmetrical SGA versus those with appropriate-for-gestational-age (AGA) neonates as well as a model of multifetal growth concordant gestations in order to establish through the 'leptin link' the relative contributions of mother, fetus, and placenta to fetal weight. We found that the maternal leptin levels at delivery correlated poorly with the maternal weight gain/body mass index and with neonatal birth weight. Furthermore, the umbilical cord leptin levels correlated well with neonatal and placental weights in the AGA group but not in the SGA group. As in AGA singleton pregnancies, in multifetal uncomplicated pregnancies, the umbilical cord leptin levels correlated well with the birth weight of individuals, regardless of the status of the twin or triplet in the set. Thus, we speculated that in SGA neonates the birth weight represents the lean body weight and the low adipose tissue content (as opposed to the AGA neonates who have a substantial adipose tissue content) and, therefore, reflects mainly the basic placental contribution.     

Wilms’ Tumor: A 24-year Retrospective Study from a Single Center

Medical records of 71 children with Wilms’ tumor at Sisli Etfal Education and Research Hospital between 1990 and 2014 were reviewed. Mean age at diagnosis was 3.11 years (2 days–7 years). Male to female ratio was M/F = 6/10. The incidence of associated anomaly was 16.9%. Clinical manifestations included abdominal mass (89%), hematuria (30%), hypertansion (25%), abdominal pain (15%), fever (5%), restlessness (2%), weight loss (2%), varicocele (1%). Ultrasound (USG) was the most often initial study in a child presenting with abdominal mass. Doppler USG was also made to evaluate the inferior vena cava (IVC) for the presence of tumor extension in children with renal mass. The left kidney was affected in 33 patients (46.5%), the right was affected in 31 patients (43.7%). Two patients was extrarenal (2.8%). And 5 patients (7.04%) were bilateral on the presentation. Preoperative chemotheraphy was done in 14 cases. In 63 patients with unilateral Wilm tm, unilateral radical nefrectomy is performed. In one patient with solitary kidney, nephron sparing surgery (NSS) is performed. In 3 patients with bilateral tm NSS is performed and in 2 patients with bilateral Wilms’ tm NSS is performed in one side and nefrectomy on the other side. Out of 71 Wilms tumor (WT) patients, 17 of them has been out of our follow. And 4 of them are died. Ten of them has metastases. Forty children are under follow with no metastases. Patients with WT needs a multimodal, multidisiplinary treatment with the cooperation of pediatric oncologist and pediatric surgeon and needs close follow-up.     

What's new in paediatric sleep?

The major recent advance in our understanding of paediatric sleep is the publication of reference values for sleep in children aged 3.2-8.6 years. These data show developmental changes reflecting a subtle process of the maturation of the central nervous system with regard to sleep in childhood. In infants, a significant negative correlation has been observed between a snore-associated arousal index and an infant development scale, underlining that snoring is less innocent than has been suggested. A link between obstructive sleep apnoea (OSA) and airway inflammation has been demonstrated, with children with OSA having significantly higher expression of the leukotriene (LT) 1 and 2 receptors and higher concentrations of LT C4/D4/E4 and LT B4 in adenotonsillar tissues than children with recurrent rhinitis who have no OSA. This explains the efficacy of treatment for OSA with montelukast, a LT receptor antagonist, alone or in combination with corticosteroids. By using peripheral arterial tonometry, a noninvasive technique that allows the moment-to-moment measurement of sympathetic tone, persistent waking-associated autonomic nervous system dysfunction has been demonstrated in young children with sleep-disordered breathing (SDB). As such, SDB in childhood may represent a cardiovascular risk factor in adulthood.     

What's new in respiratory allergy?

The past 10 years have seen important advances in our understanding of allergic respiratory disease and the targets for potential therapies. Sensitisation and triggering of allergic reactions now appear to be better understood at a clinical and molecular level. Environmental intervention studies are underway attempting to reduce the sensitisation and the triggering of symptoms. Therapeutic intervention studies targeting key pathways in the allergic cascade are also taking place. This paper will assess both of these aspects of respiratory allergy, updating readers on the new evidence in our quest to understand how and when sensitisation occurs and also how we might be able to control triggered reactions using targeted therapeutics against specific elements of the allergic cascade.