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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
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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
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
Omar AM, Khattak AQ, Lee JA
Int Braz J Urol 2006 Mar-Apr;32(2):187-9; discussion 189. doi: 10.1590/s1677-55382006000200009. PMID: 16650296

Diagnosis

Kumar V, Misra V, Chaurasiya D, Verma N
Indian J Pathol Microbiol 2018 Jul-Sep;61(3):410-413. doi: 10.4103/IJPM.IJPM_849_16. PMID: 30004068
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
Vandercruysse D, Oyen R, Baert L, Baert AL
J Belge Radiol 1996 Dec;79(6):280. PMID: 9031555
Feldberg MA, van Waes PF
AJR Am J Roentgenol 1982 May;138(5):953-5. doi: 10.2214/ajr.138.5.953. PMID: 6979186

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
Kumar V, Misra V, Chaurasiya D, Verma N
Indian J Pathol Microbiol 2018 Jul-Sep;61(3):410-413. doi: 10.4103/IJPM.IJPM_849_16. PMID: 30004068
Williamson SR, Cheng L
Urol Oncol 2016 Jul;34(7):292.e17-22. Epub 2016 Mar 14 doi: 10.1016/j.urolonc.2016.01.001. PMID: 26988177
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

Rotterova P, Alaghehbandan R, Skopal J, Rogala J, Slisarenko M, Strakova Peterikova A, Michalova K, Montiel DP, Farcas M, Ulamec M, Stransky P Jr, Fiala O, Pitra T, Hora M, Michal M, Pivovarcikova K, Hes O
Ann Diagn Pathol 2024 Aug;71:152297. Epub 2024 Mar 24 doi: 10.1016/j.anndiagpath.2024.152297. PMID: 38579443
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
Kumar V, Misra V, Chaurasiya D, Verma N
Indian J Pathol Microbiol 2018 Jul-Sep;61(3):410-413. doi: 10.4103/IJPM.IJPM_849_16. PMID: 30004068
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

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