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Renal tumours
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Clear cell sarcoma of the kidney, has a peak incidence between 1 and 4 years of age. Boys are again more commonly affected than girls (2 : 1). Clear cell sarcoma of the kidney is also known as the bone metastasising tumour of childhood, because of its high metastatic potential. Metastases occur in 15%–60% of patients, and 4% at presentation will have distant spread. No cases of bilateral disease have been reported and there is no known familial or syndromic association.
Paediatric oncology
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2014
Stephen Lowis, Rachel Cox, John Moppett, Antony Ng
The SIOP WT 2001 study, which closed in the United Kingdom in December 2011, was based on the results of the previous SIOP, the United Kingdom and NWTS studies. It recommended the use of 4 weeks (two drugs for localized disease) to 6 weeks (three drugs for metastatic disease) preoperative chemotherapy because of the efficacy in reducing tumour rupture in surgery, inducing favourable stage distribution requiring less post-operative treatment and identifying good responders in the metastatic patients. It used the pathological staging of excised tumours, an important prognostic factor, and adopted a new histological risk-stratification to guide post-operative management. The aim was to reduce treatment for patients with low- and intermediate-risk tumours (including stromal and epithelial histology), and intensify chemotherapy and radiotherapy for high-risk patients with inadequate response to preoperative treatment. The roles of tumour volume reduction following preoperative chemotherapy and tumour genetics were further studied in the trial. Guidance was also provided for the management of clear cell sarcoma of the kidney, nephroblastomatosis, bilateral WT and other low-risk renal tumours. Current clinical management in the United Kingdom is still based on the recommendation in SIOP WT 2001 while the data are being analysed.
Metastatic TFE3-overexpressing renal clear cell carcinoma with dense granules: a histological, immunohistochemical, and ultrastructural study
Published in Ultrastructural Pathology, 2018
Shoujun Chen, Elba A. Turbat-Herrera, Guillermo A. Herrera, Meghna Chadha, Rodney E. Shackelford, Eric X. Wei
Clear cell RCC is the most common histologic variant of RCC. The clear cytoplasm is created by the rich lipid and glycogen content due to the deficient glycogenolysis and lipolysis.5 The lipid content is comparable to that of the proximal convoluted tubules,6 which is considered the cell origin or line of differentiation of the clear cell RCC variant. Accordingly, the tumor cells of the RCC exhibit tubular differentiation with tumor cells arranged around microlumens demarcated by basal lamina. Tumor cells often harbor abundant lipid vacuoles and mono-particulate glycogen, characteristic features as in this reported case. Adult clear cell sarcoma of the kidney may be suggested based on morphological and immunostaining patterns. However, in the clear cell sarcoma of the kidney, the electron microscopy normally demonstrates that the “clear” cell appearance is due to the extracellular matrix instead of cytoplasmic glycogen or droplets as shown in this case.
Delayed Metastasis of Clear Cell Sarcoma of Kidney to Bladder After 7 Disease-Free Years
Published in Fetal and Pediatric Pathology, 2018
Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood accounting for 2%–5% of all cases in this age group (7,8). With the current intensive treatment schedules, the outcome for the patient with a CCSK has significantly improved with a 5-year event-free survival of 75%–85% and a 5 year overall survival of 85%–90% compared to an estimated 25% 6-year recurrence-free survival in the early 1990s (7,9,42). It is recognized that younger patients and those with advanced stage disease have an increased relapse rate; relapses presently occur in about 15% of patients, but historically, it was reported that up to 30% of CCSKs relapsed more than 2 years after diagnosis (9). The median time from initial diagnosis to relapse is now approximately 17 months with very few reported beyond 5 years. In one study, 35 of 37 relapses (95%) of CCSK were metastatic and the most common sites were the brain, lungs, and bone (10). At the time of recurrence in our patient, the bone marrow was free of tumor and imaging studies revealed no other sites of metastatic disease. To the best of our knowledge, metastasis to the urinary bladder has not yet been reported in CCSK and this case also illustrates the relatively bland, myxomatous appearance of the metastasis which was observed earlier and reported in a case of metastatic CCSK to the mandible (5). Our case also demonstrates the somewhat unpredictable nature of metastatic sites of CCSK as in the case of an infant with a soft tissue metastasis to the leg at the initial presentation (11).
Successful use of frameless stereotactic radiosurgery for treatment of recurrent brain metastases in an 18-month-old child
Published in International Journal of Neuroscience, 2019
Elham Rahimy, Cynthia Chuang, Sheri L. Spunt, Kelly Mahaney, Sarah S. Donaldson, Iris C. Gibbs, Scott G. Soltys, Erqi L. Pollom, Susan M. Hiniker
Undifferentiated sarcomas are among the most common soft tissue sarcomas in childhood and are often characterized by oncogenic fusions [1]. The round cell phenotype is uncommon in undifferentiated sarcomas presenting in infancy, but is associated with either recurrent BCOR internal tandem duplication or a YWHAE-NUTM2B gene fusion in about half of cases [2]. These genetic findings are also found in clear cell sarcoma of the kidney (CCSK), which similarly presents in young children and has an undifferentiated round cell histologic appearance, suggesting that these tumors may be different manifestations of the same disease entity. The brain is a common site of metastasis in CCSK [3].