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Intralobar nephrogenic rest

MedGen UID:
452959
•Concept ID:
C1319016
•
Congenital Abnormality
Synonyms: ILNR-Intralobar nephrogenic rest; Intralobar Nephrogenic Rest; Intralobar nephrogenic rests; Nephrogenic rest, intralobar
SNOMED CT: Nephrogenic rest, intralobar (405934001); ILNR-Intralobar nephrogenic rest (405934001)
 
HPO: HP:0012783

Definition

A type of nephrogenic rest usually representing single lesions within the renal lobe, renal sinus, or calyceal walls. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntralobar nephrogenic rest

Recent clinical studies

Etiology

Nephron Sparing Surgery in Bilateral Wilms Tumors With Botryoid Growth Pattern.
Mrad C, Audry G, Tabone MD, Le Pointe HD, Coulomb A, Irtan S
J Pediatr Hematol Oncol 2022 Apr 1;44(3):e740-e742. doi: 10.1097/MPH.0000000000002337. PMID: 34561400
"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Loss of heterozygosity at 11p13 and 11p15 in Wilms tumor: a study of 22 cases from India.
Sigamani E, Wari MN, Iyer VK, Agarwala S, Sharma A, Bakhshi S, Dinda A
Pediatr Surg Int 2013 Mar;29(3):223-7. Epub 2013 Jan 5 doi: 10.1007/s00383-012-3254-8. PMID: 23292539
Synchronous bilateral Wilm's tumor with complete radiographic response managed without surgical resection: a report from the National Wilm's Tumor Study 4.
Hamilton TE, Ritchey ML, Argani P, Beckwith JB, Perlman EJ, Cotton CA, Shamberger RC
J Pediatr Surg 2008 Nov;43(11):1982-4. doi: 10.1016/j.jpedsurg.2008.05.037. PMID: 18970928Free PMC Article
Nephrogenic rests in Wilms tumor patients with the Drash syndrome.
Heppe RK, Koyle MA, Beckwith JB
J Urol 1991 Jun;145(6):1225-8. doi: 10.1016/s0022-5347(17)38582-8. PMID: 1851891

Diagnosis

"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Nephrogenic rests in Wilms tumors treated with preoperative chemotherapy: The UK SIOP Wilms Tumor 2001 Trial experience.
Vujanić GM, Apps JR, Moroz V, Ceroni F, Williams RD, Sebire NJ, Pritchard-Jones K
Pediatr Blood Cancer 2017 Nov;64(11) Epub 2017 Apr 6 doi: 10.1002/pbc.26547. PMID: 28383760
Clinical features and long-term outcomes of bilateral Wilms tumor treated with Taiwan Pediatric Oncology Group protocols: A single center report.
Chen SH, Hung IJ, Yang CP, Jaing TH, Wang CJ, Hsueh C, Lai JY
Asia Pac J Clin Oncol 2016 Sep;12(3):300-7. Epub 2016 May 26 doi: 10.1111/ajco.12501. PMID: 27230630
Single-nucleotide polymorphism in WT1 gene in a hyperplastic intralobar nephrogenic rest with botryoid protrusion.
Mizuno K, Hayashi Y, Tozawa K, Iwatsuki S, Kojima Y, Kohri K
Urology 2010 Jul;76(1):149-52. Epub 2009 Nov 14 doi: 10.1016/j.urology.2009.09.012. PMID: 19914700
Noninvasive botryoid extension of Wilms' tumor into the bladder.
Mitchell CS, Yeo TA
Pediatr Radiol 1997 Oct;27(10):818-20. doi: 10.1007/s002470050243. PMID: 9323251

Therapy

Nephrogenic rests in Wilms tumors treated with preoperative chemotherapy: The UK SIOP Wilms Tumor 2001 Trial experience.
Vujanić GM, Apps JR, Moroz V, Ceroni F, Williams RD, Sebire NJ, Pritchard-Jones K
Pediatr Blood Cancer 2017 Nov;64(11) Epub 2017 Apr 6 doi: 10.1002/pbc.26547. PMID: 28383760
Synchronous bilateral Wilm's tumor with complete radiographic response managed without surgical resection: a report from the National Wilm's Tumor Study 4.
Hamilton TE, Ritchey ML, Argani P, Beckwith JB, Perlman EJ, Cotton CA, Shamberger RC
J Pediatr Surg 2008 Nov;43(11):1982-4. doi: 10.1016/j.jpedsurg.2008.05.037. PMID: 18970928Free PMC Article

Prognosis

"Teratoid" Wilms Tumor: The Extreme End of Heterologous Element Differentiation, Not a Separate Entity.
D'Hooghe E, Mifsud W, Vujanić GM
Am J Surg Pathol 2019 Nov;43(11):1583-1590. doi: 10.1097/PAS.0000000000001335. PMID: 31600178
Nephrogenic rests in Wilms tumors treated with preoperative chemotherapy: The UK SIOP Wilms Tumor 2001 Trial experience.
Vujanić GM, Apps JR, Moroz V, Ceroni F, Williams RD, Sebire NJ, Pritchard-Jones K
Pediatr Blood Cancer 2017 Nov;64(11) Epub 2017 Apr 6 doi: 10.1002/pbc.26547. PMID: 28383760
Synchronous bilateral Wilm's tumor with complete radiographic response managed without surgical resection: a report from the National Wilm's Tumor Study 4.
Hamilton TE, Ritchey ML, Argani P, Beckwith JB, Perlman EJ, Cotton CA, Shamberger RC
J Pediatr Surg 2008 Nov;43(11):1982-4. doi: 10.1016/j.jpedsurg.2008.05.037. PMID: 18970928Free PMC Article

Clinical prediction guides

Nephrogenic rests in Wilms tumors treated with preoperative chemotherapy: The UK SIOP Wilms Tumor 2001 Trial experience.
Vujanić GM, Apps JR, Moroz V, Ceroni F, Williams RD, Sebire NJ, Pritchard-Jones K
Pediatr Blood Cancer 2017 Nov;64(11) Epub 2017 Apr 6 doi: 10.1002/pbc.26547. PMID: 28383760

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