Bilateral clear cell sarcoma of the kidney Zekri, Wael; Yehia, Dina; Elshafie, Maged M. ...
Journal of Egyptian National Cancer Institute,
06/2015, Letnik:
27, Številka:
2
Journal Article
Recenzirano
Odprti dostop
Clear cell sarcoma of the kidney (CCSK) accounts for 2–5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with ...bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patient’s tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSK’s ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases).
Opsoclonous myoclonous ataxia syndrome (OMAS) is a rare primarily immune-mediated disease in children. The current study aim was to find out the patterns and outcome of OMAS associated with ...neuroblastoma (NBL) among Children's Cancer Hospital-Egypt patients. Data was reviewed for 15 eligible patients enrolled between 2007 and 2016. OMAS treatment included prednisolone and cyclophosphamide with/without intravenous immunoglobulin; NBL treatment was given according to risk-corresponding protocol. Patients' age ranged from 0.75 to 12 years at presentation with male/female: 1.1/1. Concurrent diagnosis of OMAS and NBL occurred in 6 patients (40%). OMAS preceded NBL within 0.25 to 2 years in 33%, while NBL preceded OMAS within 0.5 to 1.5 years in 27%. Full OMAS picture was present in 10/15 patients, while 20% presented with truncal ataxia and myoclonus, 1 with truncal ataxia and opsoclonus, and 1 had opsoclonus and myoclonus. Median time till improvement of manifestations was 5 months. The 5-year OMAS progression-free survival was 33.3%, where 10 patients needed second-line therapy due to relapse/progression of OMAS. The median time to progression was 28 months measured from OMAS diagnosis. All patients remained alive with NBL 5-year overall survival of 100% and event-free survival of 85.7% for. However, 73% of the patients showed late sequelae ranging from ocular to cognitive, behavioral and motor disorders; rarely seizures and hemolytic anemia.
Adrenocortical carcinoma (ACC) is a rare, aggressive endocrine neoplasm. Complete surgical resection is the single most important treatment. Most available information has been learned from ...experience with its more frequent adult counterpart. In this study, we assessed the features and survival outcome of patients with ACC at Children's Cancer Hospital Egypt (CCHE). Patients diagnosed at CCHE between July 2007 and November 2016 were followed up on until November 2018. Patients with stages I and II were operated upon, while stages III and IV had received combinations of doxorubicin, etoposide, platinol, and mitotane (DEPM) beside the attempt to conduct surgery when feasible. Data belonging to 18 patients (7 men and 11 women) were analyzed; median age at diagnosis was 48.5 months. Sixteen patients had presented with secreting tumors. Six patients were diagnosed with stage I disease; four with stage II; three with stage III; and five with stage IV carcinoma. By the end of this study, 10 patients have survived; five-year overall survival of 66.3%. Surviving patients were all of stage I or II diseases and were all in remission. Seven patients who did not survive died due to tumor progression, while one patient died after chemotherapy. The prognosis of ACC is essentially dependent on a successful complete resection of the tumor and thus on the initial tumor stage. The mitotane and DEP protocols may help control tumor growth in the advanced stages for only short periods.
Key points
Initial stage and resectability are the main indicators of outcomes in adrenocortical carcinoma.
Chemotherapeutic agents used in developed countries did not achieve the same outcomes.
Further molecular-pharmacology differentiation is needed for various ethnic populations.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. CCSK has a wide histologic spectrum that mimics other pediatric renal neoplasms with considerable diagnostic ...difficulty. Our aim is to show various histopathologic CCSK patterns, pitfalls, diagnostic utilities and outcome in one of the developing countries.
We included all patients diagnosed as CCSK in the period between July 2007 and May 2013 at Childrens Cancer Hospital, Egypt. Patient demographics, clinical presentation, pathology and management were reviewed. Follow up was continued until March 2013.
Thirty-two patients were found within that interval, accounting for 6.4% of all renal tumors. Median age was 28.5 months. Stages I, II, III, IV and V represented 11 (34.4%), 3 (9.4%), 13 (40.6%), 3 (9.4%) and 2 (6.3%), respectively. The majority of tumors showed classic pattern either solely or focally. Other patterns were myxoid, spindle, cellular, epithelioid, haemangiopericytomatous, palisading and anaplastic. Wide immunohistochemical panels were done including vimentin, INI-1, WT1, CK, CD99, Synaptophysin, Desmin, LCA, CD34, P53 and EGFR. Fluorescence in situ hybridization (FISH) for EWS break apart translocation was done for 5 cases (suspected PNET) and synovial sarcoma breakapart translocation in 4 cases. Overall survival and event-free survival was 93% (95% CI 92.06–93.94%) for 16 months.
CCSK has many histologic patterns that highlight the diagnostic pathologic dilemma. In addition, our institution showed increased survival of CCSK.
Financial fraud, considered as deceptive tactics for gaining financial benefits, has recently become a widespread menace in companies and organizations. Conventional techniques such as manual ...verifications and inspections are imprecise, costly, and time consuming for identifying such fraudulent activities. With the advent of artificial intelligence, machine-learning-based approaches can be used intelligently to detect fraudulent transactions by analyzing a large number of financial data. Therefore, this paper attempts to present a systematic literature review (SLR) that systematically reviews and synthesizes the existing literature on machine learning (ML)-based fraud detection. Particularly, the review employed the Kitchenham approach, which uses well-defined protocols to extract and synthesize the relevant articles; it then report the obtained results. Based on the specified search strategies from popular electronic database libraries, several studies have been gathered. After inclusion/exclusion criteria, 93 articles were chosen, synthesized, and analyzed. The review summarizes popular ML techniques used for fraud detection, the most popular fraud type, and evaluation metrics. The reviewed articles showed that support vector machine (SVM) and artificial neural network (ANN) are popular ML algorithms used for fraud detection, and credit card fraud is the most popular fraud type addressed using ML techniques. The paper finally presents main issues, gaps, and limitations in financial fraud detection areas and suggests possible areas for future research.
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