Abstract
The vast majority of childhood renal tumors are Wilms tumors (nephroblastoma). All other pediatric kidney tumors comprise less than 15 % of childhood renal tumors and are therefore considered rare malignancies of childhood. The most frequently observed non-Wilms renal tumors include clear cell sarcoma of the kidney (CCSK), renal cell carcinoma (RCC), malignant rhabdoid tumor of the kidney (MRTK), and congenital mesoblastic nephroma (CMN). These tumor types are markedly heterogeneous in their clinical characteristics. CMN occurs mainly in early infancy, MRTK occurs in infants and young toddlers, CCSK occurs in toddlers and young school-age children, and RCC is most common in adolescents. The outcome of CMN is excellent, with overall survival rates over 95 % without adjuvant chemotherapy. By contrast, patients with MRTK have overall survival rates of only 20–25 %, even with intensive multimodality treatment regimens. The outcomes of CCSK and RCC are intermediate between CMN and MRTK. The non-Wilms renal tumors also have markedly different biological characteristics, each bearing distinct genetic mutations and translocations. This chapter provides an overview of the major non-Wilms renal tumors of childhood, including the ultra-rare entities of renal medullary carcinoma, metanephric tumors, and renal sarcomas.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Adnani A, Latib R, Bouklata S et al (2006) [Clear cell sarcoma of the kidney in an adult: a case report] Sarcome a cellules claires du rein chez l’adulte: a propos d’un cas. J Radiol 87:136–138
Ahmed HU, Arya M, Levitt G et al (2007) Part I: Primary malignant non-Wilms’ renal tumours in children. Lancet 8:730–737
Akhtar M, Ali MA, Sackey K et al (1989) Fine-needle aspiration biopsy of clear-cell sarcoma of the kidney: light and electron microscopic features. Diagn Cytopathol 5:181–187
Ali AA, Finlay JL, Gerald WL et al (1994) Congenital mesoblastic nephroma with metastasis to the brain: a case report. Am J Pediatr Hematol Oncol 16:361–364
Amar AM, Tomlinson G, Green DM et al (2001) Clinical presentation of rhabdoid tumors of the kidney. J Pediatr Hematol Oncol 23:105–108
Amin MB, de Peralta-Venturina MN, Ro JY et al (1999) Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis. Am J Surg Pathol 23:1455–1463
Argani P (2005) Metanephric neoplasms: the hyperdifferentiated, benign end of the Wilms tumor spectrum? Clin Lab Med 25:379–392
Argani P, Beckwith JB (2000) Metanephric stromal tumor: report of 31 cases of a distinctive pediatric renal neoplasm. Am J Surg Pathol 24:917–926
Argani P, Ladanyi M (2005) Translocation carcinomas of the kidney. Clin Lab Med 25:363–378
Argani P, Faria PA, Epstein JI et al (2000a) Primary renal synovial sarcoma: molecular and morphologic delineation of an entity previously included among embryonal sarcomas of the kidney. Am J Surg Pathol 24:1087–1096
Argani P, Perlman EJ, Breslow NE et al (2000b) Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 24:4–18
Argani P, Antonescu CR, Illei PB et al (2001a) Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am J Pathol 159:179–192
Argani P, Hawkins A, Griffin CA et al (2001b) A distinctive pediatric renal neoplasm characterized by epithelioid morphology, basement membrane production, focal HMB45 immunoreactivity, and t(6;11)(p21.1;q12) chromosome translocation. Am J Pathol 158:2089–2096
Argani P, Lae M, Ballard ET et al (2006) Translocation carcinomas of the kidney after chemotherapy in childhood. J Clin Oncol 24:1529–1534
Argani P, Hicks J, De Marzo AM et al (2010) Xp11 translocation renal cell carcinoma (RCC): extended immunohistochemical profile emphasizing novel RCC markers. Am J Surg Pathol 34:1295–1303
Arroyo MR, Green DM, Perlman EJ et al (2001) The spectrum of metanephric adenofibroma and related lesions: clinicopathologic study of 25 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 25:433–444
Barnard M, Bayani J, Grant R et al (2000) Comparative genomic hybridization analysis of clear cell sarcoma of the kidney. Med Pediatr Oncol 34:113–116
Beckwith JB (1983) Wilms’ tumor and other renal tumors of childhood: a selective review from the National Wilms’ Tumor Study Pathology Center. Hum Pathol 14:481–492
Beckwith JB, Palmer NF (1978) Histopathology and prognosis of Wilms tumors: results from the First National Wilms’ Tumor Study. Cancer 41:1937–1948
Bell MD (2006) Response to paclitaxel, gemcitabine, and cisplatin in renal medullary carcinoma. Pediatr Blood Cancer 47:228
Benchekroun A, Ghadouane M, Zannoud M et al (2002) Clear cell sarcoma of the kidney in an adult. A case report. Ann Urol (Paris) 36:33–35
Betz BL, Strobeck MW, Reisman DN et al (2002) Re-expression of hSNF5/INI1/BAF47 in pediatric tumor cells leads to G1 arrest associated with induction of p16ink4a and activation of RB. Oncogene 21:5193–5203
Bhayani SB, Liapis H, Kibel AS (2001) Adult clear cell sarcoma of the kidney with atrial tumor thrombus. J Urol 165:896–897
Biegel JA, Zhou JY, Rorke LB et al (1999) Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 59:74–79
Biegel JA, Tan L, Zhang F et al (2002) Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extrarenal rhabdoid tumors. Clin Cancer Res 8:3461–3467
Bjornsson J, Short MP, Kwiatkowski DJ et al (1996) Tuberous sclerosis-associated renal cell carcinoma. Clinical, pathological, and genetic features. Am J Pathol 149:1201–1208
Bourdeaut F, Lequin D, Brugieres L et al (2011) Frequent hSNF5/INI1 germline mutations in patients with rhabdoid tumor. Clin Cancer Res 17:31–38
Brennan BM, Foot AB, Stiller C et al (2004) Where to next with extracranial rhabdoid tumours in children. Eur J Cancer 40:624–626
Brownlee NA, Perkins LA, Stewart W et al (2007) Recurring translocation (10;17) and deletion (14q) in clear cell sarcoma of the kidney. Arch Pathol Lab Med 131:446–451
Bruder E, Passera O, Harms D et al (2004) Morphologic and molecular characterization of renal cell carcinoma in children and young adults. Am J Surg Pathol 28:1117–1132
Carcao MD, Taylor GP, Greenberg ML et al (1998) Renal-cell carcinoma in children: a different disorder from its adult counterpart. Med Pediatr Oncol 31:153–158
Castellanos RD, Aron BS, Evans AT (1974) Renal adenocarcinoma in children: incidence, therapy and prognosis. J Urol 111:534–537
Chan HS, Cheng MY, Mancer K et al (1987) Congenital mesoblastic nephroma: a clinicoradiologic study of 17 cases representing the pathologic spectrum of the disease. J Pediatr 111:64–70
Charafe E, Penault-Llorca F, Mathoulin-Portier MP et al (1997) [Clear cell sarcoma of the kidney relapsing after 10 years of asymptomatic evolution] Sarcome a cellules claires du rein recidivant apres 10 ans d’evolution asymptomatique. Ann Pathol 17:400–402
Chen S, Bhuiya T, Liatsikos EN et al (2001) Primary synovial sarcoma of the kidney: a case report with literature review. Int J Surg Pathol 9:335–339
Cheng JX, Tretiakova M, Gong C et al (2008) Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. Mod Pathol 21:647–652
Chi SN, Zimmerman MA, Yao X et al (2009) Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27:385–389
Choueiri TK, Lim ZD, Hirsch MS et al (2010) Vascular endothelial growth factor-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma. Cancer 116:5219–5225
Cutcliffe C, Kersey D, Huang CC et al (2005) Clear cell sarcoma of the kidney: up-regulation of neural markers with activation of the sonic hedgehog and Akt pathways. Clin Cancer Res 11:7986–7994
Dalfior D, Eccher A, Gobbo S et al (2008) Primary pleomorphic rhabdomyosarcoma of the kidney in an adult. Ann Diagn Pathol 12:301–303
Dassi V, Das K, Singh BP et al (2009) Primary synovial sarcoma of kidney: a rare tumor with an atypical presentation. Indian J Urol 25:269–271
Davis CJ Jr, Mostofi FK, Sesterhenn IA (1995) Renal medullary carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol 19:1–11
Davis IJ, His BL, Arroyo JD et al (2003) Cloning of a novel Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q12) chromosome translocation. Proc Natl Acad Sci U S A 100:6051–6056
Dehner LP, Leestma JE, Price EB Jr (1970) Renal cell carcinoma in children: a clinicopathologic study of 15 cases and review of the literature. J Pediatr 76:358–368
Drut R, Pomar M (1991) Cytologic characteristics of clear-cell sarcoma of the kidney (CCSK) in fine-needle aspiration biopsy (FNAB): a report of 4 cases. Diagn Cytopathol 7:611–614
Eaton KW, Tooke LS, Wainwright LM et al (2011) Spectrum of SMARCB1/INI1 mutations in familial and sporadic rhabdoid tumors. Pediatr Blood Cancer 56:7–15
Eble JN, Sauter G, Epstein JI, Sesternhenn IA (2004) World Health Organization Classification of Tumours. Pathology and genetics of tumours of the urinary system and male genital organs. IARC Press, Lyon
Edge S, Byrd D, Compton C et al (2010) AJCC cancer staging handbook, 7th edn. Springer, New York
El Kababri M, Khattab M, El Khorassani M et al (2004) [Clear cell sarcoma of the kidney. A study of 13 cases] Sarcome renal a cellules claires. A propos d’une serie de 13 cas. Arch Pediatr 11:794–799
Furtwaengler R, Reinhard H, Leuschner I et al (2006) Mesoblastic nephroma–a report from the Gesellschaft fur Padiatrische Onkologie und Hamatologie (GPOH). Cancer 106:2275–2283
Furtwangler R, Reinhard H, Beier R et al (2005) Clear-cell sarcoma (CCSK) of the kidney – results of the SIOP 93-01/GPOH trial. Pediatr Blood Cancer 45:423
Furtwangler R, Gooskens SL, van Tinteren H, et al (2013) Clear cell sarcomas of the kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and 2001 protocols: a report of the SIOP Renal Tumour Study Group. Eur J Cancer 49:3497–3506
Gadd S, Sredni ST, Huang CC et al (2010) Rhabdoid tumor: gene expression clues to pathogenesis and potential therapeutic targets. Lab Invest 90:724–738
Gadd S, Beezhold P, Jennings L et al (2012) Mediators of Receptor Tyrosine Kinase activation in infantile fibrosarcoma: a Children’s Oncology Group study. J Pathol 228:119–130
Geller JI, Dome JS (2004) Local lymph node involvement does not predict poor outcome in pediatric renal cell carcinoma. Cancer 101:1575–1583
Geller JI, Dome JS (2009) Retroperitoneal lymph node dissection for pediatric renal cell carcinoma. Pediatr Blood Cancer 52:430
Geller JI, Argani P, Adeniran A et al (2008) Translocation renal cell carcinoma: lack of negative impact due to lymph node spread. Cancer 112:1607–1616
Gooskens SL, Furtwangler R, Spreafico et al (2014) Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney: a combined SIOP and AIEOP study. Br J Cancer 111:227–233
Graf N, Tournade MF, de Kraker J (2000) The role of preoperative chemotherapy in the management of Wilms’ tumor. The SIOP studies. International Society of Pediatric Oncology. Urol Clin North Am 27:443–454
Green DM, Beckwith JB, Breslow NE et al (1994) Treatment of children with stages II to IV anaplastic Wilms’ tumor: a report from the National Wilms’ Tumor Study Group. J Clin Oncol 12:2126–2131
Grier HE, Perez-Atayde AR, Weinstein HJ (1985) Chemotherapy for inoperable infantile fibrosarcoma. Cancer 56:1507–1510
Guidi CJ, Sands AT, Zambrowicz BP et al (2001) Disruption of Ini1 leads to peri-implantation lethality and tumorigenesis in mice. Mol Cell Biol 21:3598–3603
Haas JE, Palmer NF, Weinberg AG et al (1981) Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol 12:646–657
Hasselblatt M, Gesk S, Oyen F et al (2011) Nonsense mutation and inactivation of SMARCA4 (BRG1) in an atypical teratoid/rhabdoid tumor showing retained SMARCB1 (INI1) expression. Am J Surg Pathol 35:933–935
Heidelberger KP, Ritchey ML, Dauser RC et al (1993) Congenital mesoblastic nephroma metastatic to the brain. Cancer 72:2499–2502
Hoot AC, Russo P, Judkins AR et al (2004) Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors. Am J Surg Pathol 28:1485–1491
Howell CG, Othersen HB, Kiviat NE et al (1982) Therapy and outcome in 51 children with mesoblastic nephroma: a report of the National Wilms’ Tumor Study. J Pediatr Surg 17:826–831
Howlader N, Noone AM, Krapcho M, Neyman N, Aminou R, Waldron W, Altekruse SF, Kosary CL, Ruhl J, Tatalovich Z, Cho H, Mariotto A, Eisner MP, Lewis DR, Chen HS, Feuer EJ, Cronin KA, Edwards BK (2011) SEER cancer statistics review, 1975–2008. National Cancer Institute, Bethesda, http://seer.cancer.gov/csr/1975_2008/</csr/1975_2008/>
Hung NA (2005) Congenital “clear cell sarcoma of the kidney”. Virchows Arch 446:566–568
Iacovelli R, Altavilla A, Ciardi A et al (2012) Clinical and pathological features of primary renal synovial sarcoma: analysis of 64 cases from 11 years of medical literature. BJU Int 110(10):1449–54
Indolfi P, Terenziani M, Casale F et al (2003) Renal cell carcinoma in children: a clinicopathologic study. J Clin Oncol 21:530–535
Isakoff MS, Sansam CG, Tamayo P et al (2005) Inactivation of the Snf5 tumor suppressor stimulates cell cycle progression and cooperates with p53 loss in oncogenic transformation. Proc Natl Acad Sci U S A 102:17745–17750
Iyer VK, Kapila K, Verma K (2003) Fine needle aspiration cytology of clear cell sarcoma of the kidney with spindle cell pattern. Cytopathology 14:160–164
Iyer VK, Agarwala S, Verma K (2005) Fine-needle aspiration cytology of clear-cell sarcoma of the kidney: study of eight cases. Diagn Cytopathol 33:83–89
Jackson EM, Sievert AJ, Gai X et al (2009) Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors. Clin Cancer Res 15:1923–1930
Jagani Z, Mora-Blanco EL, Sansam CG et al (2010) Loss of the tumor suppressor Snf5 leads to aberrant activation of the Hedgehog-Gli pathway. Nat Med 16:1429–1433
Jones C, Rodriguez-Pinilla M, Lambros M et al (2007) c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours. J Clin Pathol 60:1226–1231
Joshi VV, Kasznica J, Walters TR (1986) Atypical mesoblastic nephroma. Pathologic characterization of a potentially aggressive variant of conventional congenital mesoblastic nephroma. Arch Pathol Lab Med 110:100–106
Kagan AR, Steckel RJ (1986) Clear cell sarcoma of the kidney: a renal tumor of childhood that metastasized to bone. AJR Am J Roentgenol 146:64–66
Kalapurakal JA, Perlman EJ, Seibel NL et al (2012) Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5. Int J Radiat Oncol Biol Phys 85(2):428–31
Karlsson J, Holmquist Mengelbier L et al (2014). Clear cell sarcoma of the kidney demonstrates an embryonic signature indicative of a primitive nephrogenic origin. Genes Chromosomes Cancer 53(5):381–391
Katsumi Y, Iehara T, Miyachi M et al (2011) Sensitivity of malignant rhabdoid tumor cell lines to PD 0332991 is inversely correlated with p16 expression. Biochem Biophys Res Commun 413:62–68
Kidd JM (1970) Exclusion of certain renal neoplasms from the category of Wilms tumor. Am J Pathol 59:16a
Kinoshita Y, Tamiya S, Oda Y et al (2001) Establishment and characterization of malignant rhabdoid tumor of the kidney. Oncol Rep 8:43–48
Klochendler-Yeivin A, Fiette L, Barra J et al (2000) The murine SNF5/INI1 chromatin remodeling factor is essential for embryonic development and tumor suppression. EMBO Rep 1:500–506
Knezevich SR, Garnett MJ, Pysher TJ et al (1998a) ETV6-NTRK3 gene fusions and trisomy 11 establish a histogenetic link between mesoblastic nephroma and congenital fibrosarcoma. Cancer Res 58:5046–5048
Knezevich SR, McFadden DE, Tao W et al (1998b) A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma. Nat Genet 18:184–187
Knutson SK, Warholic NM, Wigle TJ et al (2013) Durabloe tumor regression in genetically altered malignant rhabdoid tumors by inhibition of methyltransferase EZH2. Proc Natl Acad Scie USA 100: 7922–7927
Koga Y, Matsuzaki A, Suminoe A et al (2009) Long-term survival after autologous peripheral blood stem cell transplantation in two patients with malignant rhabdoid tumor of the kidney. Pediatr Blood Cancer 52:888–890
Krishnamurthy S, Bharadwaj R (1998) Fine needle aspiration cytology of clear cell sarcoma of the kidney. A case report. Acta Cytol 42:1444–1446
Kural AR, Onal B, Ozkara H et al (2006) Adult clear cell sarcoma of the kidney: a case report. BMC Urol 6:11
Kurkchubasche AG, Halvorson EG, Forman EN et al (2000) The role of preoperative chemotherapy in the treatment of infantile fibrosarcoma. J Pediatr Surg 35:880–883
Kusumakumary P, Chellam VG, Rojymon J et al (1997) Late recurrence of clear cell sarcoma of the kidney. Med Pediatr Oncol 28:355–357
Lack EE, Cassady JR, Sallan SE (1985) Renal cell carcinoma in childhood and adolescence: a clinical and pathological study of 17 cases. J Urol 133:822–828
Lalwani N, Prasad SR, Vikram R et al (2011) Pediatric and adult primary sarcomas of the kidney: a cross-sectional imaging review. Acta Radiol 52:448–457
Lee S, Cimica V, Ramachandra N et al (2011) Aurora A is a repressed effector target of the chromatin remodeling protein INI1/hSNF5 required for rhabdoid tumor cell survival. Cancer Res 71:3225–3235
Linehan WM, Srinivasan R, Schmidt LS (2010) The genetic basis of kidney cancer: a metabolic disease. Nat Rev Urol 7:277–285
Little SE, Bax DA, Rodriguez-Pinilla M et al (2007) Multifaceted dysregulation of the epidermal growth factor receptor pathway in clear cell sarcoma of the kidney. Clin Cancer Res 13:4360–4364
Loeb DM, Hill DA, Dome JS (2002) Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy. J Pediatr Hematol Oncol 24:478–481
MacArthur CA, Isaacs H Jr, Miller JH et al (1994) Pediatric renal cell carcinoma: a complete response to recombinant interleukin-2 in a child with metastatic disease at diagnosis. Med Pediatr Oncol 23:365–371
Maccioni F, Della Rocca C, Salvi PF et al (2000) Malignant peripheral neuroectodermal tumor (MPNET) of the kidney. Abdom Imaging 25:103–106
Madigan CE, Armenian SH, Malogolowkin MH et al (2007) Extracranial malignant rhabdoid tumors in childhood: the Childrens Hospital Los Angeles experience. Cancer 110:2061–2066
Malouf GG, Camparo P, Oudard S et al (2010) Targeted agents in metastatic Xp11 translocation/TFE3 gene fusion renal cell carcinoma (RCC): a report from the Juvenile RCC Network. Ann Oncol 21:1834–1838
Malouf GG, Camparo P, Molinie V et al (2011) Transcription factor E3 and transcription factor EB renal cell carcinomas: clinical features, biological behavior and prognostic factors. J Urol 185:24–29
Marino-Enriquez A, Ou WB, Weldon CB et al (2011) ALK rearrangement in sickle cell trait-associated renal medullary carcinoma. Genes Chromosomes Cancer 50:146–153
Marsden HB, Lawler W (1978) Bone-metastasizing renal tumour of childhood. Br J Cancer 38:437–441
Mazzoleni S, Vecchiato L, Alaggio R et al (2003) Clear cell sarcoma of the kidney in a newborn. Med Pediatr Oncol 41:153–155
Medeiros LJ, Palmedo G, Krigman HR et al (1999) Oncocytoid renal cell carcinoma after neuroblastoma: a report of four cases of a distinct clinicopathologic entity. Am J Surg Pathol 23:772–780
Metzger ML, Dome JS (2005) Current therapy for Wilms’ tumor. Oncologist 10:815–826
Miser JS, Kinsella TJ, Triche TJ et al (1987) Treatment of peripheral neuroepithelioma in children and young adults. J Clin Oncol 5:1752–1758
Mishra VK, Krishnani N, Bhandari M (1993) Clear cell sarcoma of kidney in an adult. Br J Urol 72:118
Mitchell C, Jones PM, Kelsey A et al (2000) The treatment of Wilms’ tumour: results of the United Kingdom Children’s cancer study group (UKCCSG) second Wilms’ tumour study. Br J Cancer 83:602–608
Mitchell C, Pritchard-Jones K, Shannon R et al (2006) Immediate nephrectomy versus preoperative chemotherapy in the management of non-metastatic Wilms’ tumour: results of a randomised trial (UKW3) by the UK Children’s Cancer Study Group. Eur J Cancer 42:2554–2562
Morgan E, Kidd JM (1978) Undifferentiated sarcoma of the kidney: a tumor of childhood with histopathologic and clinical characteristics distinct from Wilms’ tumor. Cancer 42:1916–1921
Muslin AJ, Tanner JW, Allen PM et al (1996) Interaction of 14-3-3 with signaling proteins is mediated by the recognition of phosphoserine. Cell 84:889–897
Neumann HP, Pawlu C, Peczkowska M et al (2004) Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 292:943–951
Newbould MJ, Kelsey AM (1993) Clear cell sarcoma of the kidney in a 4-month-old infant: a case report. Med Pediatr Oncol 21:525–528
Nickerson ML, Warren MB, Toro JR et al (2002) Mutations in a novel gene lead to kidney tumors, lung wall defects, and benign tumors of the hair follicle in patients with the Birt-Hogg-Dube syndrome. Cancer Cell 2:157–164
O’Meara E, Stack D, Lee CH et al (2012) Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney. J Pathol 227:72–80
Oda H, Shiga J, Machinami R (1993) Clear cell sarcoma of kidney. Two cases in adults. Cancer 71:2286–2291
Orbach D, Rey A, Cecchetto G et al (2010) Infantile fibrosarcoma: management based on the European experience. J Clin Oncol 28:318–323
Parikh SH, Chintagumpala M, Hicks MJ et al (1998) Clear cell sarcoma of the kidney: an unusual presentation and review of the literature. J Pediatr Hematol Oncol 20:165–168
Park DY, Kim YM, Chi JG (1997) Intracranial metastasis from clear cell sarcoma of the kidney–a case report. J Korean Med Sci 12:473–476
Pettinato G, Manivel JC, Wick MR et al (1989) Classical and cellular (atypical) congenital mesoblastic nephroma: a clinicopathologic, ultrastructural, immunohistochemical, and flow cytometric study. Hum Pathol 20:682–690
Pirich LM, Chou P, Walterhouse DO (1999) Prolonged survival of a patient with sickle cell trait and metastatic renal medullary carcinoma. J Pediatr Hematol Oncol 21:67–69
Pomara G, Cappello F, Cuttano MG et al (2004) Primitive Neuroectodermal Tumor (PNET) of the kidney: a case report. BMC Cancer 4:3
Punnett HH, Halligan GE, Zaeri N et al (1989) Translocation 10;17 in clear cell sarcoma of the kidney. A first report. Cancer Genet Cytogenet 41:123–128
Radhika S, Bakshi A, Rajwanshi A et al (2005) Cytopathology of uncommon malignant renal neoplasms in the pediatric age group. Diagn Cytopathol 32:281–286
Radulescu VC, Gerrard M, Moertel C et al (2008) Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer 50:246–249
Rakheja D, Weinberg AG, Tomlinson GE et al (2004) Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney. Cancer Genet Cytogenet 154:175–179
Ramphal R, Pappo A, Zielenska M et al (2006) Pediatric renal cell carcinoma: clinical, pathologic, and molecular abnormalities associated with the members of the mit transcription factor family. Am J Clin Pathol 126:349–364
Raney B, Anderson J, Arndt C et al (2008) Primary renal sarcomas in the Intergroup Rhabdomyosarcoma Study Group (IRSG) experience, 1972-2005: a report from the Children’s Oncology Group. Pediatr Blood Cancer 51:339–343
Reinhard H, Reinert J, Beier R et al (2008) Rhabdoid tumors in children: prognostic factors in 70 patients diagnosed in Germany. Oncol Rep 19:819–823
Renshaw AA (1999) Renal cell carcinomas in children and young adults: Increased incidence of papillary architecture and unique subtypes. Am J Surg Pathol 23:795–802
Roberts CW, Galusha SA, McMenamin ME et al (2000) Haploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice. Proc Natl Acad Sci U S A 97:13796–13800
Ronnen EA, Kondagunta GV, Motzer RJ (2006) Medullary renal cell carcinoma and response to therapy with bortezomib. J Clin Oncol 24:e14
Rosso D, Ghignone GP, Bernardi D et al (2003) Clear cell sarcoma of the kidney with invasion of the inferior vena cava. Urol Int 70:251–252
Rubin BP, Chen CJ, Morgan TW et al (1998) Congenital mesoblastic nephroma t(12;15) is associated with ETV6-NTRK3 gene fusion: cytogenetic and molecular relationship to congenital (infantile) fibrosarcoma. Am J Pathol 153:1451–1458
Sandstedt BE, Delemarre JF, Harms D et al (1987) Sarcomatous Wilms’ tumour with clear cells and hyalinization. A study of 38 tumours in children from the SIOP nephroblastoma file. Histopathology 11:273–285
Schmidt L, Duh FM, Chen F et al (1997) Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas. Nat Genet 16:68–73
Schuster AE, Schneider DT, Fritsch MK et al (2003) Genetic and genetic expression analyses of clear cell sarcoma of the kidney. Lab Invest 83:1293–1299
Seibel NL, Li S, Breslow NE et al (2004) Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms’ Tumor Study Group. J Clin Oncol 22:468–473
Seibel NL, Sun J, Anderson JR et al (2006) Outcome of clear cell sarcoma of the kidney (CCSK) treated on the National Wilms Tumor Study-5 (NWTS). J Clin Oncol 24:A9000
Selle B, Furtwangler R, Graf N et al (2006) Population-based study of renal cell carcinoma in children in Germany, 1980-2005: more frequently localized tumors and underlying disorders compared with adult counterparts. Cancer 107:2906–2914
Sharma SC, Menon PA (2001) Clear cell sarcoma of the kidney. J Postgrad Med 47:206–207
Simpson L, He X, Pins M et al (2005) Renal medullary carcinoma and ABL gene amplification. J Urol 173:1883–1888
Smith ME, Cimica V, Chinni S et al (2011) Therapeutically targeting cyclin D1 in primary tumors arising from loss of Ini1. Proc Natl Acad Sci U S A 108:319–324
Sohda T, Soejima H, Matsumoto T et al (1997) Insulin-like growth factor 2 gene imprinting in clear cell sarcoma of the kidney. Hum Pathol 28:1315–1318
Sotelo-Avila C, Gonzalez-Crussi F, Sadowinski S et al (1985) Clear cell sarcoma of the kidney: a clinicopathologic study of 21 patients with long-term follow-up evaluation. Hum Pathol 16:1219–1230
Stahlschmidt J, Cullinane C, Roberts P et al (1999) Renal medullary carcinoma: prolonged remission with chemotherapy, immunohistochemical characterisation and evidence of bcr/abl rearrangement. Med Pediatr Oncol 33:551–557
Stoneham S, Kheder M, Moroz V et al (2009) Clear Cell Sarcoma of the Kidney (CCSK) – combined 20 year experience of therapeutic outcomes from United Kingdom (UK) and France. Pediatr Blood Cancer 53:753
Strouse JJ, Spevak M, Mack AK et al (2005) Significant responses to platinum-based chemotherapy in renal medullary carcinoma. Pediatr Blood Cancer 44:407–411
Suzuki H, Honzumi M, Itoh Y et al (1983) Clear-cell sarcoma of the kidney seen in a 3-day-old newborn. Z Kinderchir 38:422–424
Taguchi K, Okada A, Kamiya H et al (2008) Clear cell sarcoma of the kidney: a case-report of an 11-year-old boy and a review of 11 cases in Japan. J Rural Med 3:19–22
Tomlinson IP, Alam NA, Rowan AJ et al (2002) Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer. Nat Genet 30:406–410
Tomlinson GE, Breslow NE, Dome J et al (2005) Rhabdoid tumor of the kidney in the National Wilms’ Tumor Study: age at diagnosis as a prognostic factor. J Clin Oncol 23:7641–7645
Tournade MF, Com-Nougue C, de Kraker J et al (2001) Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms’ tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms’ Tumor Trial and Study. J Clin Oncol 19:488–500
Toyoda Y, Yamashita C, Sugimoto T et al (1998) Clear cell sarcoma of kidney with tumor extension into the right atrium. J Cardiovasc Surg (Torino) 39:489–491
Tsuda M, Davis IJ, Argani P et al (2007) TFE3 fusions activate MET signaling by transcriptional up-regulation, defining another class of tumors as candidates for therapeutic MET inhibition. Cancer Res 67:919–929
Tzivion G, Luo Z, Avruch J (1998) A dimeric 14-3-3 protein is an essential cofactor for Raf kinase activity. Nature 394:88–92
Ueno H, Okita H, Akimoto S, et al (2013) DNA methylation profile distinguishes clear cell sarcoma of the kidney from other pediatric renal tumors. PLoS One 8:e62233
Upton MP, Parker RA, Youmans A et al (2005) Histologic predictors of renal cell carcinoma response to interleukin-2-based therapy. J Immunother 28:488–495
van den Heuvel-Eibrink MM, Grundy P, Graf N et al (2008) Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: a collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer 50:1130–1134
van den Heuvel-Eibrink MM, van Tinteren H, Rehorst H et al (2011) Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatr Blood Cancer 56:733–737
Vanharanta S, Buchta M, McWhinney SR et al (2004) Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHB-associated heritable paraganglioma. Am J Hum Genet 74:153–159
Varan A, Akyuz C, Sari N et al (2007) Renal cell carcinoma in children: experience of a single center. Nephron Clin Pract 105:c58–61
Venkatramani R, Shoureshi P, Malvar J, et al (2014) High dose alkylator therapy for extracranial malignant rhabdoid tumors in children. Pediatr Blood Cancer 61:1357–1361
Versteege I, Sevenet N, Lange J et al (1998) Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer. Nature 394:203–206
Versteege I, Medjkane S, Rouillard D et al (2002) A key role of the hSNF5/INI1 tumour suppressor in the control of the G1-S transition of the cell cycle. Oncogene 21:6403–6412
Vogelzang NJ, Fremgen AM, Guinan PD et al (1993) Primary renal sarcoma in adults. A natural history and management study by the American Cancer Society, Illinois Division. Cancer 71:804–810
Vujanic GM, Sandstedt B, Harms D et al (1996) Rhabdoid tumour of the kidney: a clinicopathological study of 22 patients from the International Society of Paediatric Oncology (SIOP) nephroblastoma file. Histopathology 28:333–340
Vujanic GM, Kelsey A, Perlman EJ et al (2007) Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. Am J Surg Pathol 31:1459–1468
Wagner L, Hill DA, Fuller C et al (2002) Treatment of metastatic rhabdoid tumor of the kidney. J Pediatr Hematol Oncol 24:385–388
Wai DH, Knezevich SR, Lucas T et al (2000) The ETV6-NTRK3 gene fusion encodes a chimeric protein tyrosine kinase that transforms NIH3T3 cells. Oncogene 19:906–915
Waldron PE, Rodgers BM, Kelly MD et al (1999) Successful treatment of a patient with stage IV rhabdoid tumor of the kidney: case report and review. J Pediatr Hematol Oncol 21:53–57
Walsh A, Kelly DR, Vaid YN et al (2010) Complete response to carboplatin, gemcitabine, and paclitaxel in a patient with advanced metastatic renal medullary carcinoma. Pediatr Blood Cancer 55:1217–1220
Wood DP Jr, Kay R, Norris D (1990) Renal sarcomas of childhood. Urology 36:73–78
Yamamoto M, Suzuki N, Hatakeyama N et al (2006) Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report. J Pediatr Hematol Oncol 28:286–289
Yanagisawa S, Kadouchi I, Yokomori K et al (2009) Identification and metastatic potential of tumor-initiating cells in malignant rhabdoid tumor of the kidney. Clin Cancer Res 15:3014–3022
Yumura-Yagi K, Inoue M, Wakabayashi R et al (1998) Successful double autografts for patients with relapsed clear cell sarcoma of the kidney. Bone Marrow Transplant 22:381–383
Yun K (1993) Clear cell sarcoma of the kidney expresses insulin-like growth factor-II but not WT1 transcripts. Am J Pathol 142:39–47
Zuo S, Xue Y, Tang S et al (2010) 14-3-3 epsilon dynamically interacts with key components of mitogen-activated protein kinase signal module for selective modulation of the TNF-alpha-induced time course-dependent NF-kappaB activity. J Proteome Res 9:3465–3478
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Dome, J.S., Gooskens, S.L., van den Heuvel-Eibrink, M.M. (2014). Non-Wilms Pediatric Renal Tumors. In: Pritchard-Jones, K., Dome, J. (eds) Renal Tumors of Childhood. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44003-2_14
Download citation
DOI: https://doi.org/10.1007/978-3-662-44003-2_14
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-44002-5
Online ISBN: 978-3-662-44003-2
eBook Packages: MedicineMedicine (R0)