Extraskeletal conventional chondrosarcoma of genitalia in a child- An unusual pathology for a rare tumor.

INTRODUCTION AND IMPORTANCE: Chondrosarcoma is a rare malignant tumor considered as the second common sarcoma of bone following osteosarcoma. Less than 5 % of chondrosarcomas occur in children. Conventional chondrosarcoma is the most common type observed as skeletal tumors. Extraskeletal chondrosarcomas account for about 1 % of chondrosarcomas. They are almost always of myxoid or mesenchymal subtypes. CASE PRESENTATION: A 4-year-old girl was referred with pain and palpable mass in the labia majora since 2 years of age for which she had undergone twice biopsies, reported as soft tissue enchondroma. At this time, complete excision of the lesion with negative margins was carried out. The pathology was in favor of extraskeletal chondrosarcoma of conventional type without any evidence of bone involvement. CLINICAL DISCUSSION: Our case was a very young child with extraskeletal conventional chondrosarcoma, not reported yet in the literature. The vast majority of extraskeletal myxoid chondrosarcomas arise in the soft tissues of the extremities. The lesion in this case was located within the subcutaneous tissue of the labia majora; however, the tumor was not compatible with myxoid chondrosarcoma. Mesenchymal chondrosarcoma is another type of extraskeletal chondrosarcoma but the histopathologic patterns observed in our patient were not compatible even with the mesenchymal chondrosarcoma. CONCLUSION: We present a unique case of extraskeletal chondrosarcoma in a child with conventional subtype which has not been reported in the literature. In addition, the lesion was assumed to arise from a previous soft tissue enchondroma of the labia majora since infancy which makes the case distinctive.

Electrochemical measuring of reactive oxygen species levels in the blood to detect ratio of high-density neutrophils, suitable to alarm presence of cancer in suspicious cases.

Here for the first time, a real-time electrochemical assay on unprocessed blood was designed to detect the presence of cancer in patients. The system has been based on the recently approved pathway, which indicates that the abundance of immature and mature low-density neutrophils (LDNs) with reduced ROS production in peripheral blood is increased with the presence of active cancer tumors. Reduced ROS/H2O2 released from LDNs play the main role in determining the ROS/H2O2 levels of peripheral blood. In contrast, HDNs with increased levels of released ROS/H2O2 have higher concentrations than LDNs in normal cases. Hence, the reduced level of ROS species in peripheral blood recorded by our carbon nanostructure decorated sensor in less than 30 seconds showed a great pre-warning about the presence of non-treated cancer in patients with suspicious mass who have been sent for further evaluations.

Microbial contamination of home nebulizers in children with cystic fibrosis and clinical implication on the number of pulmonary exacerbations.

BACKGROUND: Early detection of pulmonary contamination in children with cystic fibrosis (CF) is essential since these children are vulnerable to Pseudomonas aeruginosa (P. aeruginosa) colonization. In Iran, home nebulization of antibiotics is a widespread practice in treatment for patients with CF and, to the best our knowledge, no bacteriological surveys have been conducted till date in this regard. METHOD: This observational, cross sectional study was conducted on 61 children with CF at Mofid Children's Hospital, Tehran, from September 2017 to march 2018. The swab sampling was performed from 61 home nebulizers used by children diagnosed with CF. Contemporaneous sputum sample or deep nasopharyngeal swab was taken from each patient for bacterial and fungal testing. Medical records of the patients were reviewed and the number of exacerbations were recorded over the last 12 months prior to the study enrollment. RESULTS: The results of study showed that, 43 (70.5%) nebulizers were contaminated; 31 (50.8%) mouthpieces, 21 (34.4%) reservoirs, and 11 (18%) connecting tubes. The most common organism to be isolated was P. aeruginosa and was recovered from 19 (31%) nebulizers, 16 of them belonged to patients chronically colonized with P. aeruginosa. The remaining three had at least one positive sputum culture for P. aeruginosa in the past 1 year before the study. There was a significant increase in the number of CF exacerbations with an average number of exacerbation being 1.5 ± 1(SD) over last 12 months in children who had pathogenic organisms recovered from their home nebulizers compared with 0.4 ± 0.7(SD) exacerbations per year in whom non-pathogenic organisms were isolated from their nebulizers (P < 0.001). CONCLUSION: The majority of domiciliary nebulizers used by children with CF were contaminated with microorganisms indicating that the nebulizers may serve as potential reservoirs of pathogens for the patients' lung. Perpetuating colonization is a possible concern in the ones recently colonized with P. aeruginosa and, therefore, decontamination of nebulizer requires more attention to prevent ongoing infection. The negative impact of contamination of nebulizer on CF exacerbation requires serious attention and further investigations.

Investigation into breast cancer and partial breast reconstruction: A review.

Growing increasingly in South America, Africa and Asia, breast cancer is known as the dominant type of cancer in women. Different treatments are available for breast cancer, among which surgery is the most widely used, but researchers are trying to develop new strategies. One of the most prominent surgical methods is referred to as oncoplastic surgery, that helps to remove segments of malignant breast tissue. This type of surgery aims to obtain vast surgical margins, while the remaining tissue is rearranged so that the better cosmetic outcome is obtained. This review will investigate the breast cancer and then discuss partial breast reconstruction. Before outlining the procedures, the different types of partial breast reconstruction will be discussed. Finally, advantages and disadvantages will be outlined. MEDLINE database was used to conduct the search. The main terms used were 'Conservation Breast Surgery Reconstruction' AND 'Oncoplastic Surgery', 'Partial Mastectomy Reconstruction' AND 'Conservative Breast Surgery Reconstruction', 'oncoplastic' [All Fields], 'breast' AND 'surgery' OR 'surgery' operative', 'oncoplastic' ('breast')'. The bibliographies of relevant papers were manually searched up to October 2018, but more recent voices are also included.

Very early onset inflammatory bowel disease: Investigation of the IL-10 signaling pathway in Iranian children.

BACKGROUND & AIM: Comparing to adult inflammatory bowel disease (IBD), those with early onset manifestations have different features in terms of the underlying molecular pathology, the course of disease and the response to therapy. We investigated the IL-10 signaling pathway previously reported as an important cause of infantile (Very Early Onset) IBD to find any possible variants. METHOD: With the next generation sequencing technique we screened IL-10, IL-10RA and IL10RB genes of 15 children affected by very early onset-GI (gastrointestinal) disorders. Additionally, we analyzed them based on Thermo Fisher immune deficiency panel for genes either having a known role in IBD pathogenesis or cause the disorders with overlapping manifestations. We performed multiple functional analyses only for the cases showing variants in IL-10- related genes. RESULT: In 3 out of 15 patients we identified variants including a homozygous and heterozygote mutations in IL-10RA and a novel homozygous mutation in IL-12RB1. Our functional studies reveal that in contrast to the IL-10RA heterozygote mutation that does not have deleterious effects, the homozygous mutation abrogates the IL-10 signaling pathway. CONCLUSION: Our study suggests we need to modify the classical diagnostic approach from functional assays followed by candidate- gene or genes sequencing to the firstly parallel genomic screening followed by functional studies.

Infantile splenorenopancreatic mucormycosis complicating neuroblastoma.

Herein is described the first case of an infant with neuroblastoma who developed isolated splenorenopancreatic mucormycosis. An 18-month-old boy with neuroblastoma who was on intensive chemotherapy was admitted with febrile neutropenia. On abdominal computed tomography, multiple hypodense lesions in the spleen with invasion to the upper pole of the left kidney were demonstrated. Enlargement of splenic lesions with a complete hypoechoic pattern replacing the whole spleen, consistent with splenic abscess were observed on serial ultrasound. On splenectomy the resected spleen appeared to be severely fragmented and necrotic. On pathology, massive infiltration of broad, non-septate hyphal fragments identified as Mucor with invasion to all blood vessels was seen. Histologically, a piece of the tail of the pancreas also showed involvement by the filamentous Mucor. The present case highlights the necessity of high index of suspicion in susceptible patients, early diagnosis and appropriate management in order to minimize the mortality rate.