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Multilocular clear cell renal cell carcinoma

MedGen UID:
87547
Concept ID:
C0346249
Neoplastic Process
Synonyms: Cystadenocarcinoma of kidney; cystadenocarcinoma of kidney; MCRCC; Multilocular clear cell adenocarcinoma; multilocular clear cell adenocarcinoma; Multilocular clear cell carcinoma; multilocular clear cell carcinoma; multilocular clear cell renal cell adenocarcinoma; Multilocular clear cell renal cell adenocarcinoma; multilocular clear cell renal cell carcinoma; multilocular cystic clear cell renal cell neoplasm of low malignant potential; Multilocular cystic renal cell adenocarcinoma; multilocular cystic renal cell adenocarcinoma; multilocular cystic renal cell cancer; Multilocular cystic renal cell carcinoma; multilocular cystic renal cell carcinoma; Multilocular Cystic Renal Neoplasm of Low Malignant Potential; multilocular cystic renal neoplasm of low malignant potential; Multilocular cystic renal neoplasm of low malignant potential; renal cystadenocarcinoma
SNOMED CT: Cystadenocarcinoma of kidney (254916002)
 
Monarch Initiative: MONDO:0003010
Orphanet: ORPHA319287

Definition

Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features of cysts lined by occasionally flattened cuboidal clear cells and septa containing aggregates of epithelial cells with clear cytoplasm, and excellent prognosis. The tumor usually presents as an asymptomatic, unilateral, solitary lesion, macroscopically consisting of numerous, fluid-filled, septated cysts of variable size. Rarely, the symptoms typically associated with renal tumors (flank pain, hematuria, palpable mass) may be present. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMultilocular clear cell renal cell carcinoma

Professional guidelines

PubMed

Filipović J, Bosić M, Ćirović S, Životić M, Dunđerović D, Đorđević D, Živković-Perišić S, Lipkovski A, Marković-Lipkovski J
Diagn Pathol 2019 Oct 26;14(1):120. doi: 10.1186/s13000-019-0901-6. PMID: 31655611Free PMC Article
Tay SY, Tiu CM, Hu B, Wang HK, Chiou HJ, Lin YH, Lai YC, Chan WP, Chou YH
J Chin Med Assoc 2018 Dec;81(12):1017-1026. Epub 2018 Jul 13 doi: 10.1016/j.jcma.2018.04.008. PMID: 30017809
Honda Y, Nakamura Y, Goto K, Terada H, Sentani K, Yasui W, Sekino Y, Hayashi T, Teishima J, Matsubara A, Akagi M, Fuji T, Baba Y, Iida M, Awai K
Abdom Radiol (NY) 2018 Jul;43(7):1540-1545. doi: 10.1007/s00261-018-1643-8. PMID: 29796844

Recent clinical studies

Etiology

Sheenu VS, Paul RS, Pushpa M, Ami EM, Rashmi R, Iona ML, Elizabeth M
Indian J Pathol Microbiol 2023 Apr-Jun;66(2):307-313. doi: 10.4103/ijpm.ijpm_53_21. PMID: 37077073
Wang Y, Niu X, Wang L, Li Y, Qiao B
Sci Rep 2022 Jul 27;12(1):12865. doi: 10.1038/s41598-022-16856-2. PMID: 35896562Free PMC Article
Pecoraro A, Palumbo C, Knipper S, Mistretta FA, Rosiello G, Tian Z, St-Hilaire PA, Shariat SF, Saad F, Lavallée L, Briganti A, Kapoor A, Fiori C, Porpiglia F, Karakiewicz PI
Eur Urol Focus 2021 Jul;7(4):818-826. Epub 2020 Mar 10 doi: 10.1016/j.euf.2020.02.011. PMID: 32169361
Katabathina VS, Garg D, Prasad SR, Vikram R
J Comput Assist Tomogr 2012 Nov-Dec;36(6):659-68. doi: 10.1097/RCT.0b013e31826dba18. PMID: 23192202
Hindman NM, Bosniak MA, Rosenkrantz AB, Lee-Felker S, Melamed J
AJR Am J Roentgenol 2012 Jan;198(1):W20-6. doi: 10.2214/AJR.11.6762. PMID: 22194510

Diagnosis

Stanescu AL, Acharya PT, Lee EY, Phillips GS
Magn Reson Imaging Clin N Am 2019 May;27(2):279-290. doi: 10.1016/j.mric.2019.01.006. PMID: 30910098
Honda Y, Nakamura Y, Goto K, Terada H, Sentani K, Yasui W, Sekino Y, Hayashi T, Teishima J, Matsubara A, Akagi M, Fuji T, Baba Y, Iida M, Awai K
Abdom Radiol (NY) 2018 Jul;43(7):1540-1545. doi: 10.1007/s00261-018-1643-8. PMID: 29796844
Gurel S, Narra V, Elsayes KM, Siegel CL, Chen ZE, Brown JJ
Diagn Interv Radiol 2013 Jul-Aug;19(4):304-11. doi: 10.5152/dir.2013.147. PMID: 23439256
Kuroda N, Ohe C, Mikami S, Inoue K, Nagashima Y, Cohen RJ, Pan CC, Michal M, Hes O
Histol Histopathol 2012 Aug;27(8):969-74. doi: 10.14670/HH-27.969. PMID: 22763870
Raman SP, Hruban RH, Fishman EK
Abdom Imaging 2012 Oct;37(5):873-84. doi: 10.1007/s00261-012-9903-5. PMID: 22581271

Therapy

Xu B, Wang JJ, Mi Y, Zhou LQ, Jin J, Zhang Q
Urol Int 2015;94(1):83-7. Epub 2014 Aug 7 doi: 10.1159/000363606. PMID: 25115167
Xu Y, Zhang S, Wei X, Pan Y, Hao J
Clin Hemorheol Microcirc 2014;58(3):429-38. doi: 10.3233/CH-131799. PMID: 24165574
Aubert S, Zini L, Delomez J, Biserte J, Lemaitre L, Leroy X
J Urol 2005 Dec;174(6):2115-9. doi: 10.1097/01.ju.0000181210.72528.ab. PMID: 16280739

Prognosis

Tretiakova M, Kwon JW, Paner GP
Adv Anat Pathol 2024 May 1;31(3):157-168. Epub 2024 Mar 25 doi: 10.1097/PAP.0000000000000443. PMID: 38525552
Wang Y, Niu X, Wang L, Li Y, Qiao B
Sci Rep 2022 Jul 27;12(1):12865. doi: 10.1038/s41598-022-16856-2. PMID: 35896562Free PMC Article
Sun Y, Argani P, Tickoo SK, Epstein JI
Am J Surg Pathol 2018 Oct;42(10):1396-1401. doi: 10.1097/PAS.0000000000001124. PMID: 30001236
Kuroda N, Ohe C, Mikami S, Inoue K, Nagashima Y, Cohen RJ, Pan CC, Michal M, Hes O
Histol Histopathol 2012 Aug;27(8):969-74. doi: 10.14670/HH-27.969. PMID: 22763870
Grignon DJ, Che M
Clin Lab Med 2005 Jun;25(2):305-16. doi: 10.1016/j.cll.2005.01.012. PMID: 15848738

Clinical prediction guides

Wang Y, Niu X, Wang L, Li Y, Qiao B
Sci Rep 2022 Jul 27;12(1):12865. doi: 10.1038/s41598-022-16856-2. PMID: 35896562Free PMC Article
Pecoraro A, Palumbo C, Knipper S, Mistretta FA, Rosiello G, Tian Z, St-Hilaire PA, Shariat SF, Saad F, Lavallée L, Briganti A, Kapoor A, Fiori C, Porpiglia F, Karakiewicz PI
Eur Urol Focus 2021 Jul;7(4):818-826. Epub 2020 Mar 10 doi: 10.1016/j.euf.2020.02.011. PMID: 32169361
Filipović J, Bosić M, Ćirović S, Životić M, Dunđerović D, Đorđević D, Živković-Perišić S, Lipkovski A, Marković-Lipkovski J
Diagn Pathol 2019 Oct 26;14(1):120. doi: 10.1186/s13000-019-0901-6. PMID: 31655611Free PMC Article
Sun Y, Argani P, Tickoo SK, Epstein JI
Am J Surg Pathol 2018 Oct;42(10):1396-1401. doi: 10.1097/PAS.0000000000001124. PMID: 30001236
You D, Shim M, Jeong IG, Song C, Kim JK, Ro JY, Hong JH, Ahn H, Kim CS
BJU Int 2011 Nov;108(9):1444-9. Epub 2011 Jul 1 doi: 10.1111/j.1464-410X.2011.10247.x. PMID: 21722289

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