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Hereditary renal cell carcinoma

MedGen UID:
392857
•Concept ID:
C2608055
•
Neoplastic Process
Synonyms: Familial Renal Cancer; familial renal carcinoma; hereditary renal carcinoma; hereditary renal cell cancer; Hereditary Renal Cell Carcinoma; hereditary renal cell carcinoma; hereditary renal cell carcinoma (disease)
 
Monarch Initiative: MONDO:0003008

Definition

Renal cell carcinoma that has developed in relatives of patients with history of renal cell carcinoma. [from NCI]

Professional guidelines

PubMed

Genetic risk assessment for hereditary renal cell carcinoma: Clinical consensus statement.
Bratslavsky G, Mendhiratta N, Daneshvar M, Brugarolas J, Ball MW, Metwalli A, Nathanson KL, Pierorazio PM, Boris RS, Singer EA, Carlo MI, Daly MB, Henske EP, Hyatt C, Middleton L, Morris G, Jeong A, Narayan V, Rathmell WK, Vaishampayan U, Lee BH, Battle D, Hall MJ, Hafez K, Jewett MAS, Karamboulas C, Pal SK, Hakimi AA, Kutikov A, Iliopoulos O, Linehan WM, Jonasch E, Srinivasan R, Shuch B
Cancer 2021 Nov 1;127(21):3957-3966. Epub 2021 Aug 3 doi: 10.1002/cncr.33679. PMID: 34343338Free PMC Article
Hereditary renal cell carcinoma syndromes: diagnosis, surveillance and management.
Maher ER
World J Urol 2018 Dec;36(12):1891-1898. Epub 2018 Apr 21 doi: 10.1007/s00345-018-2288-5. PMID: 29680948Free PMC Article
Management of renal cell carcinoma in young patients and patients with hereditary syndromes.
Leung C, Pan S, Shuch B
Curr Opin Urol 2016 Sep;26(5):396-404. doi: 10.1097/MOU.0000000000000322. PMID: 27387961

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Kidney Cancer, 2023

Recent clinical studies

Etiology

Hereditary Renal Cell Carcinoma Syndromes.
Carlo MI
Hematol Oncol Clin North Am 2023 Oct;37(5):841-848. Epub 2023 May 29 doi: 10.1016/j.hoc.2023.04.013. PMID: 37258351
Diseases of Hereditary Renal Cell Cancers.
Iliopoulos O
Urol Clin North Am 2023 May;50(2):205-215. doi: 10.1016/j.ucl.2023.01.010. PMID: 36948667
Hereditary renal cell carcinoma syndromes: diagnosis, surveillance and management.
Maher ER
World J Urol 2018 Dec;36(12):1891-1898. Epub 2018 Apr 21 doi: 10.1007/s00345-018-2288-5. PMID: 29680948Free PMC Article
Management of renal cell carcinoma in young patients and patients with hereditary syndromes.
Leung C, Pan S, Shuch B
Curr Opin Urol 2016 Sep;26(5):396-404. doi: 10.1097/MOU.0000000000000322. PMID: 27387961
Hereditary Renal Cell Carcinoma Syndromes: Clinical, Pathologic, and Genetic Features.
Adeniran AJ, Shuch B, Humphrey PA
Am J Surg Pathol 2015 Dec;39(12):e1-e18. doi: 10.1097/PAS.0000000000000562. PMID: 26559379

Diagnosis

Hereditary Renal Cell Carcinoma Syndromes.
Carlo MI
Hematol Oncol Clin North Am 2023 Oct;37(5):841-848. Epub 2023 May 29 doi: 10.1016/j.hoc.2023.04.013. PMID: 37258351
Diseases of Hereditary Renal Cell Cancers.
Iliopoulos O
Urol Clin North Am 2023 May;50(2):205-215. doi: 10.1016/j.ucl.2023.01.010. PMID: 36948667
Birt-Hogg-Dubé syndrome-associated renal cell carcinoma: Histopathological features and diagnostic conundrum.
Furuya M, Hasumi H, Yao M, Nagashima Y
Cancer Sci 2020 Jan;111(1):15-22. Epub 2019 Dec 17 doi: 10.1111/cas.14255. PMID: 31777168Free PMC Article
Hereditary renal cell carcinoma syndromes: diagnosis, surveillance and management.
Maher ER
World J Urol 2018 Dec;36(12):1891-1898. Epub 2018 Apr 21 doi: 10.1007/s00345-018-2288-5. PMID: 29680948Free PMC Article
Hereditary Renal Cell Carcinoma Syndromes: Clinical, Pathologic, and Genetic Features.
Adeniran AJ, Shuch B, Humphrey PA
Am J Surg Pathol 2015 Dec;39(12):e1-e18. doi: 10.1097/PAS.0000000000000562. PMID: 26559379

Therapy

Percutaneous microwave ablation on management of hereditary renal cell carcinoma in Von Hippel-Lindau disease.
Chen J, Zheng L, Zhang W, Wang Z, Yu J, Liang P
Int J Hyperthermia 2024;41(1):2308079. Epub 2024 Feb 12 doi: 10.1080/02656736.2024.2308079. PMID: 38346873
Percutaneous tumor ablation with radiofrequency.
Wood BJ, Ramkaransingh JR, Fojo T, Walther MM, Libutti SK
Cancer 2002 Jan 15;94(2):443-51. doi: 10.1002/cncr.10234. PMID: 11900230Free PMC Article
Parenchymal sparing surgery in patients with hereditary renal cell carcinoma.
Walther MM, Choyke PL, Weiss G, Manolatos C, Long J, Reiter R, Alexander RB, Linehan WM
J Urol 1995 Mar;153(3 Pt 2):913-6. PMID: 7853573
The etiology of renal-cell carcinoma.
Outzen HC, Maguire HC Jr
Semin Oncol 1983 Dec;10(4):378-84. PMID: 6320450

Prognosis

Recognizing Hereditary Renal Cancers Through the Microscope: A Pathology Update.
Peng YC, Chen YB
Surg Pathol Clin 2018 Dec;11(4):725-737. Epub 2018 Oct 17 doi: 10.1016/j.path.2018.07.010. PMID: 30447838
Hereditary Renal Cell Carcinoma Syndromes: Clinical, Pathologic, and Genetic Features.
Adeniran AJ, Shuch B, Humphrey PA
Am J Surg Pathol 2015 Dec;39(12):e1-e18. doi: 10.1097/PAS.0000000000000562. PMID: 26559379
Oncological outcomes of partial nephrectomy for multifocal renal cell carcinoma greater than 4 cm.
Gupta GN, Peterson J, Thakore KN, Pinto PA, Linehan WM, Bratslavsky G
J Urol 2010 Jul;184(1):59-63. Epub 2010 May 15 doi: 10.1016/j.juro.2010.03.035. PMID: 20478582Free PMC Article
Parenchymal sparing surgery in patients with hereditary renal cell carcinoma.
Walther MM, Choyke PL, Weiss G, Manolatos C, Long J, Reiter R, Alexander RB, Linehan WM
J Urol 1995 Mar;153(3 Pt 2):913-6. PMID: 7853573
The etiology of renal-cell carcinoma.
Outzen HC, Maguire HC Jr
Semin Oncol 1983 Dec;10(4):378-84. PMID: 6320450

Clinical prediction guides

Comprehensive characterization of Alu-mediated breakpoints in germline VHL gene deletions and rearrangements in patients from 71 VHL families.
Vocke CD, Ricketts CJ, Schmidt LS, Ball MW, Middelton LA, Zbar B, Linehan WM
Hum Mutat 2021 May;42(5):520-529. Epub 2021 Mar 19 doi: 10.1002/humu.24194. PMID: 33675279Free PMC Article
Hereditary Renal Cell Carcinoma Syndromes: Clinical, Pathologic, and Genetic Features.
Adeniran AJ, Shuch B, Humphrey PA
Am J Surg Pathol 2015 Dec;39(12):e1-e18. doi: 10.1097/PAS.0000000000000562. PMID: 26559379
Partial and Radical Nephrectomy for Unilateral Synchronous Multifocal Renal Cortical Tumors.
Mano R, Kent M, Larish Y, Winer AG, Chevinsky MS, Hakimi AA, Sternberg IA, Sjoberg DD, Russo P
Urology 2015 Jun;85(6):1404-10. Epub 2015 Apr 11 doi: 10.1016/j.urology.2015.02.032. PMID: 25872696Free PMC Article
Molecular study of a new family with hereditary renal cell carcinoma and a translocation t(3;8)(p13;q24.1).
Meléndez B, Rodríguez-Perales S, Martínez-Delgado B, Otero I, Robledo M, Martínez-Ramírez A, Ruiz-Llorente S, Urioste M, Cigudosa JC, Benítez J
Hum Genet 2003 Feb;112(2):178-85. Epub 2002 Nov 13 doi: 10.1007/s00439-002-0848-6. PMID: 12522559
Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience.
Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM
J Urol 2001 Mar;165(3):777-81. PMID: 11176466

Supplemental Content

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    Clinical resources

    Practice guidelines

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Kidney Cancer, 2023

    Reviews

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