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Renal hamartoma

MedGen UID:
690113
Concept ID:
C1266142
Neoplastic Process
Synonym: Renal hamartomas
SNOMED CT: Renomedullary fibroma (128761000); Renomedullary interstitial cell tumor (128761000)
 
HPO: HP:0008696

Definition

A disordered proliferation of mature tissues that are native to the kidneys. [from HPO]

Conditions with this feature

Perlman syndrome
MedGen UID:
162909
Concept ID:
C0796113
Disease or Syndrome
Perlman syndrome (PRLMNS) is an autosomal recessive congenital overgrowth syndrome with similarities to Beckwith-Wiedemann syndrome (BWS; 130650). Affected children are large at birth, are hypotonic, and show organomegaly, characteristic facial dysmorphisms (inverted V-shaped upper lip, prominent forehead, deep-set eyes, broad and flat nasal bridge, and low-set ears), renal anomalies (nephromegaly and hydronephrosis), frequent neurodevelopmental delay, and high neonatal mortality. Perlman syndrome is associated with a high risk of Wilms tumor, with a 64% incidence in infants surviving beyond the neonatal period. The tumor is diagnosed at an earlier age in these individuals compared with sporadic cases (less than 2 years and 3-4 years of age, respectively), and there is a high frequency of bilateral tumors (55%). Histologic examination of the kidneys in children with Perlman syndrome shows frequent nephroblastomatosis, which is a precursor lesion for Wilms tumor (summary by Astuti et al., 2012).

Professional guidelines

PubMed

McCullough DL
Urology 1974 Sep;4(3):235-40. doi: 10.1016/0090-4295(74)90367-7. PMID: 4421678

Recent clinical studies

Etiology

Li H, Zhao S, Fan HY, Li Y, Wu XP, Miao YP
Clin Lab 2023 Nov 1;69(11) doi: 10.7754/Clin.Lab.2023.221126. PMID: 37948477
Yang J, Hu X, Li J, Zhang G, Ge Y, Wei W
BMC Med Imaging 2022 Feb 7;22(1):22. doi: 10.1186/s12880-022-00743-3. PMID: 35125098Free PMC Article
Xu XF, Hu XH, Zuo QM, Zhang J, Xu HY, Zhang Y
Medicine (Baltimore) 2020 May;99(20):e20167. doi: 10.1097/MD.0000000000020167. PMID: 32443333Free PMC Article
Guan W, Bai J, Hu Z, Su Y, Zhuang Q, Ye Z
J Huazhong Univ Sci Technolog Med Sci 2010 Oct;30(5):669-71. Epub 2010 Nov 10 doi: 10.1007/s11596-010-0562-x. PMID: 21063854
Valvo JR, Feinstein MJ, Wallinga H, Cockett AT
Urology 1983 Sep;22(3):286-9. doi: 10.1016/s0090-4295(83)80017-x. PMID: 6623779

Diagnosis

Li H, Zhao S, Fan HY, Li Y, Wu XP, Miao YP
Clin Lab 2023 Nov 1;69(11) doi: 10.7754/Clin.Lab.2023.221126. PMID: 37948477
Yang J, Hu X, Li J, Zhang G, Ge Y, Wei W
BMC Med Imaging 2022 Feb 7;22(1):22. doi: 10.1186/s12880-022-00743-3. PMID: 35125098Free PMC Article
Li C, Li WS
Urol J 2020 May 16;17(3):321-323. doi: 10.22037/uj.v0i0.4714. PMID: 31134609
Yashiro N, Ohtomo K, Itai Y, Iio M
Br J Radiol 1985 Jun;58(690):503-9. doi: 10.1259/0007-1285-58-690-503. PMID: 4063709
BLACKWELL JB, SAUNDERS JM, VAUGHAN BF
Aust N Z J Surg 1964 Nov;34:128-31. doi: 10.1111/j.1445-2197.1964.tb06210.x. PMID: 14218946

Therapy

Guan W, Bai J, Hu Z, Su Y, Zhuang Q, Ye Z
J Huazhong Univ Sci Technolog Med Sci 2010 Oct;30(5):669-71. Epub 2010 Nov 10 doi: 10.1007/s11596-010-0562-x. PMID: 21063854

Prognosis

Li H, Zhao S, Fan HY, Li Y, Wu XP, Miao YP
Clin Lab 2023 Nov 1;69(11) doi: 10.7754/Clin.Lab.2023.221126. PMID: 37948477
Xu XF, Hu XH, Zuo QM, Zhang J, Xu HY, Zhang Y
Medicine (Baltimore) 2020 May;99(20):e20167. doi: 10.1097/MD.0000000000020167. PMID: 32443333Free PMC Article
Guan W, Bai J, Hu Z, Su Y, Zhuang Q, Ye Z
J Huazhong Univ Sci Technolog Med Sci 2010 Oct;30(5):669-71. Epub 2010 Nov 10 doi: 10.1007/s11596-010-0562-x. PMID: 21063854

Clinical prediction guides

Li H, Zhao S, Fan HY, Li Y, Wu XP, Miao YP
Clin Lab 2023 Nov 1;69(11) doi: 10.7754/Clin.Lab.2023.221126. PMID: 37948477
Xu XF, Hu XH, Zuo QM, Zhang J, Xu HY, Zhang Y
Medicine (Baltimore) 2020 May;99(20):e20167. doi: 10.1097/MD.0000000000020167. PMID: 32443333Free PMC Article
Wang XH, Wang YJ, Lei CG
Clin Imaging 2011 Nov-Dec;35(6):447-51. doi: 10.1016/j.clinimag.2010.11.001. PMID: 22040789
Guan W, Bai J, Hu Z, Su Y, Zhuang Q, Ye Z
J Huazhong Univ Sci Technolog Med Sci 2010 Oct;30(5):669-71. Epub 2010 Nov 10 doi: 10.1007/s11596-010-0562-x. PMID: 21063854
Longa L, Scolari F, Brusco A, Carbonara C, Polidoro S, Valzorio B, Riegler P, Migone N, Maiorca R
Nephrol Dial Transplant 1997 Sep;12(9):1900-7. doi: 10.1093/ndt/12.9.1900. PMID: 9306341

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