Embryonal tumor with abundant neuropil and true rosettes: a report of three cases of a rare tumor,with an unusual case showing rhabdomyoblastic and melanocytic differentiation

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is an increasingly recognized entity that belongs to the family of embryonal tumors of the CNS. The authors present three cases of this rare tumor that were encountered at King Hussein Cancer Center, Amman, Jordan. Discussion of the clinicopathological findings is presented along with a recent literature review. Sixteen‐, 57‐ and 30‐month‐old children presented with tumors located in the pineal gland, the right fronto‐ parieto‐temporal region and the cerebellum, respectively. The findings of hypocellular neuropil as well as the characteristic ependymoblastic rosettes were seen. In addition the third case showed an abnormal combination of patterns including melanocytic and rhabdomyoblastic differentiation. The tumors stained positively for synaptophysin in the neuropil and small cell component, while the ependymoblastic rosettes stained for vimentin only. Epithelial membrane antigen and CD99 were negative in all components. One of the cases showed tetraploidy of chromosome 2. All cases exhibited an aggressive course. This is a rare and recently recognized tumor with dismal outcome, and reporting of additional new cases should help in gaining more knowledge about it.     

Cystic tumor in a 4 month old male

Cystic choroid plexus tumor is a rare variant of choroid plexus papilloma (CPP), reported mostly in infants. It is associated with the development of acute hydrocephalus in many cases. The presence of atypical CPP has recently been recognized by the WHO as a grade II tumor with increased mitotic activity. We are reporting a case of a 3.5 month infant who presented with seizures and features of increased intracranial pressure. He was found to have cystic atypical choroid plexus papilloma.     

Stereotactic radiotherapy in three weekly fractions for the management of vestibular schwannomas

Purpose

This study evaluates the rates of tumor control, hearing preservation and cranial nerve toxicity with the use of CyberKnife stereotactic radiotherapy consisting of 2100?cGy to the 80% isodose line delivered in three weekly fractions to treat vestibular schwannomas.

Detrimental Effect of Silicon Nanoparticles on P3HT:PCBM‐Based OPV Devices

The effect of incorporating 2.85 nm red luminescent silicon nanoparticles as photoluminescence down‐shifters on the efficiency of organic solar cells based on regioregular poly(3‐hexylthiophene‐2,5‐diyl) and [6,6]‐phenyl‐C71‐butyric acid methyl ester is investigated. The silicon nanoparticles are deposited by spin coating and inkjet printing on different layers of the devices. The presence of the silicon nanoparticles in the organic solar cells does not result in any performance improvement and causes a significant degradation of the power conversion efficiency.

     

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Background

The care of rhabdomyosarcoma (RMS) is complex due to its multimodal nature. By following standard protocols with acceptable toxicity and building local expertise, better outcome should be achievable.

Procedure

A retrospective study was conducted of records of patients (n = 45; 31 males; median age 26 months) with RMS treated at King Hussein Cancer Center in Jordan from January 2004 to December 2008. Patient demographics, tumor characteristics, risk stratification, treatment plan, and outcomes were studied. In June 2006, the cyclophosphamide dose was lowered from 2.2 g/m² to 1.2 g/m² per cycle because of the significant toxicity with higher dose. Survival rates, hematological toxicities, period of hospitalization due to febrile neutropenia (FN), and response rate at week 12 of treatment were compared between low‐ and high‐dose cyclophosphamide groups.

Results

Four‐year progression‐free survival (PFS) and overall survival (OS) rates were 61% ± 7.5% and 72% ± 6.9%, respectively. There was a significant difference in outcome by risk group in 4‐year PFS (low‐risk, 88% ± 12%; intermediate‐risk 63% ± 9.3%; high‐risk, 14% ± 13%; P = 0.0001) and OS (low‐risk, 88% ± 12%; intermediate‐risk 79% ± 7.5%; high‐risk, 17% ± 15%; P = 0.0011). There was significant reduction in hematological toxicities, incidence of FN, and period of hospitalization for FN in patients given low‐dose cyclophosphamide but no significant difference in PFS between low‐ and high‐dose cyclophosphamide groups.

Conclusions

Survival rates of patients with RMS in some developing countries can be improved by following or modifying evidence‐based approaches successful in developed countries and establishing multidisciplinary strategies. Therapy intensity should be increased in developing countries only when evidence supports its utility. Pediatr Blood Cancer 2013; 60: 53–58. © 2012 Wiley Periodicals, Inc.     

Butterfly cartilage tympanoplasty outcomes: A single-institution experience and literature review

Purpose

In 1998, Dr. Eavey described the trans-canal inlay butterfly cartilage tympanoplasty technique, also known as cartilage button tympanoplasty. Many retrospective studies have since demonstrated its efficacy and decreased operative time when compared to underlay and overlay tympanoplasty techniques. The butterfly cartilage tympanoplasty approach uses only a cartilage graft to repair tympanic membrane perforations. The aim of this study was to review the literature for studies that examined butterfly cartilage tympanoplasty success rates and outcomes and compare them to outcomes from our cohort.