Renal cell carcinoma in children and adolescents: a retrospective study of a French–Italian series of 93 cases

Abstract

will impact patients' and family's management and genetic counselling. A precise RCC subtype is also mandatory for the clinical management of the patients and inclusion in new prospective clinical trials.

Bilateral Native Kidney Papillary Renal Cell Carcinomas in a 11-Year-Old Renal Transplant Patient

“…In children, the median age at RCC diagnosis is 11 years which is higher in respect to Wilms' tumor. 3 Our preoperative diagnosis was in favor of RCC because of the age of the presented patient, but Wilms' tumor could only be excluded after pathological examination. Under normal circumstances, bilateralism of a renal tumor is a challenge for the surgeon and partial nephrectomy is an integral part of the treatment plan.…”

“… 4 In a large pediatric RCC series which included 93 children <18 years of age, translocation type RCC accounted for 52% of the tumors and papillary for 13%. 3 Among these, two (2%) were bilateral at the time of diagnosis.…”

“… 2 Most adults have clear-cell RCC type of the tumor and most children have translocation RCC followed by papillary morphology. 1 2 3 8 Translocation variant can be screened by TFE3 immunohistochemistry with a 100% sensitivity. 3 8 In our patient, the histopathology of the resected specimens was typical for papillary RCC with cells arranged in tubular and papillary configurations with abundant foamy macrophages and besides, TFE3 expression was negative.…”

“… 1 2 3 8 Translocation variant can be screened by TFE3 immunohistochemistry with a 100% sensitivity. 3 8 In our patient, the histopathology of the resected specimens was typical for papillary RCC with cells arranged in tubular and papillary configurations with abundant foamy macrophages and besides, TFE3 expression was negative. In general, RCC can be bilateral in 1 to 4% of all patients and bilateralism is more common in the hereditary forms of the disease than in the sporadic.…”

“… 1 RCC is rarely encountered in childhood and accounts for only approximately 5% of all pediatric renal tumors. 2 3 Of all patients, 1 to 4% have bilateral tumors and bilateralism is more common in the hereditary forms of the disease. 4 RCC developing in children are generally indistinguishable by preoperative imaging from more commonly occurring primary pediatric renal tumors, especially Wilms' tumor.…”