Pediatric and young adult renal cell carcinoma

Abstract

Renal cell carcinoma (RCC) is rare in children but is the most common renal tumor in adults. Pediatric RCC has different clinical characteristics, histopathology, and treatment compared with adult disease. Databases were reviewed from inception to February 2020, identifying 32 publications pertaining to 350 patients under 27 years. Surgery is the cornerstone for cure in localized RCC. Lymph node dissection remains controversial. Conventional radiotherapy has no curative role in RCC; similarly, conventional chemotherapy has not proven to be effective in large cohorts. Pediatric metastatic RCC has a poor outlook. There are no published prospective studies demonstrating which adjuvant therapy could improve outcome. Sunitinib, a tyrosine kinase inhibitor, is recommended in this group despite limited evidence. This review provides an overview for pediatric RCC, including the evolving role of precision medicine. K E Y W O R D S pediatrics, renal cell carcinoma, targeted therapy, treatment 1 INTRODUCTION In Europe, around 1000 children are diagnosed with a malignant renal tumor annually. 1 Renal cell carcinomas (RCC) make up 1.9% to 6% of all kidney cancers in children. 2-5 The international incidence of childhood cancer volume 3 (IICC-3) shows that the annual incidence of pediatric RCC is increasing globally, almost doubling, in 0-to 19-year

Clear Cell Renal Cell Carcinoma, Diagnostic and Therapeutic Difficulties, Case Report and Literature Review

“…Prognosis in RCC is linked to tumour staging and can range between complete remission (CR) rate of 92.5% in those diagnosed at stage 1, where nephrectomy is the only treatment, to 12.5% in those diagnosed at stage 4 [ 1 , 2 , 3 , 11 , 12 , 13 ]. There is a study where all patients diagnosed at stage 4 (11 of 24 enrolled) died throughout the follow up [ 1 ].…”

“…Chemotherapy with gemcitabine, doxorubicin, oxaliplatin or irinotecan is less commonly used nowadays. There are no uniform treatment guidelines for unresectable and metastatic RCC as the condition is particularly rare and treatment response data are scarce [ 2 , 3 , 12 , 13 ].…”

Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

“…However, there are still some challenges in the renal tumor field. Patients with localized RCC have particularly high overall survival rates, however when presenting with distant metastases, outcome is very poor [22][23][24][25]. MRTKs characterized by (SMARCB1 (95%) and SMARCA4 (5%) aberrations [26]) represent a highly chemotherapyinsensitive tumor with a typically poor outcome.…”

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

“…In fact, PRCCs could be better subclassified into ‘type 1’ and ‘others, not otherwise specified (NOS)’, being the former category of ‘PRCC type 2’ considered as a miscellanea of heterogeneous, mainly high‐grade PRCCs. Microphthalmia transcription ( MiT ) family–translocation RCCs ( MiTF ::TRCCs) represent 40–49% of RCCs in children and 1.6–4% in adults 7,8 . In adults, compared to children and adolescents, these tumours tend to be more aggressive and have more nodal metastases 9 .…”

“…Microphthalmia transcription (MiT) family-translocation RCCs (MiTF::TRCCs) represent 40-49% of RCCs in children and 1.6-4% in adults. 7,8 In adults, compared to children and adolescents, these tumours tend to be more aggressive and have more nodal metastases. 9 The prognostic value of metastatic lymph nodes in children is unclear, with contradictory data in the literature.…”