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Succinate Dehydrogenase-Deficient Renal Cell Carcinoma

MedGen UID:
1662930
•Concept ID:
C4744841
•
Neoplastic Process
Synonyms: SDH Deficient Renal Cell Carcinoma; SDH-Deficient Renal Cell Carcinoma; Succinate Dehydrogenase Renal Cell Carcinoma

Definition

A very rare renal cell carcinoma that usually affects young adults. It is characterized by mutations in one of the four subunits of the SDH complex (SDHA, SDHB, SDHC, or SDHD gene). Most frequently, the mutations occur in the SDHB subunit. It has a relatively good prognosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSuccinate Dehydrogenase-Deficient Renal Cell Carcinoma

Professional guidelines

PubMed

The New WHO Category of "Molecularly Defined Renal Carcinomas": Clinical and Diagnostic Features and Management Implications.
Kanakaraj J, Chang J, Hampton LJ, Smith SC
Urol Oncol 2024 Jul;42(7):211-219. Epub 2024 Mar 21 doi: 10.1016/j.urolonc.2024.02.003. PMID: 38519377

Recent clinical studies

Etiology

Succinate Dehydrogenase Deficient Renal Cell Carcinoma With Sarcomatoid and Rhabdoid Features-A Diagnostic Dilemma.
Ajmal N, Lallas CD, McCue P, Li L
Int J Surg Pathol 2024 Dec;32(8):1508-1513. Epub 2024 Feb 4 doi: 10.1177/10668969241229333. PMID: 38311902
Clinicopathological characterisation of renal cell carcinoma in young adults: a contemporary update and review of literature.
Abdulfatah E, Kennedy JM, Hafez K, Davenport MS, Xiao H, Weizer AZ, Palapattu GS, Morgan TM, Mannan R, Wang XM, Dhanasekaran SM, Kaffenberger SD, Spratt DE, Kunju L, Wu A, Lew M, Udager AM, Chinnaiyan AM, Mehra R
Histopathology 2020 May;76(6):875-887. Epub 2020 Apr 27 doi: 10.1111/his.14051. PMID: 31872452

Diagnosis

Genomic alterations and diagnosis of renal cancer.
Zhang X, Bolck HA, Rupp NJ, Moch H
Virchows Arch 2024 Feb;484(2):323-337. Epub 2023 Nov 24 doi: 10.1007/s00428-023-03700-9. PMID: 37999735Free PMC Article
Hereditary succinate dehydrogenase-deficient renal cell carcinoma.
Rogala J, Zhou M
Semin Diagn Pathol 2024 Jan;41(1):32-41. Epub 2023 Nov 8 doi: 10.1053/j.semdp.2023.11.001. PMID: 37981479
Pink renal conundrum.
Sanders ML, Thomas DH, Varma M
J Clin Pathol 2020 Sep;73(9):610. Epub 2020 Feb 14 doi: 10.1136/jclinpath-2019-206260. PMID: 32060077
Clinicopathological characterisation of renal cell carcinoma in young adults: a contemporary update and review of literature.
Abdulfatah E, Kennedy JM, Hafez K, Davenport MS, Xiao H, Weizer AZ, Palapattu GS, Morgan TM, Mannan R, Wang XM, Dhanasekaran SM, Kaffenberger SD, Spratt DE, Kunju L, Wu A, Lew M, Udager AM, Chinnaiyan AM, Mehra R
Histopathology 2020 May;76(6):875-887. Epub 2020 Apr 27 doi: 10.1111/his.14051. PMID: 31872452
Succinate Dehydrogenase-Deficient Renal Cell Carcinoma.
Tsai TH, Lee WY
Arch Pathol Lab Med 2019 May;143(5):643-647. Epub 2018 Dec 21 doi: 10.5858/arpa.2018-0024-RS. PMID: 31026220

Prognosis

Succinate Dehydrogenase Deficient Renal Cell Carcinoma With Sarcomatoid and Rhabdoid Features-A Diagnostic Dilemma.
Ajmal N, Lallas CD, McCue P, Li L
Int J Surg Pathol 2024 Dec;32(8):1508-1513. Epub 2024 Feb 4 doi: 10.1177/10668969241229333. PMID: 38311902
Hereditary succinate dehydrogenase-deficient renal cell carcinoma.
Rogala J, Zhou M
Semin Diagn Pathol 2024 Jan;41(1):32-41. Epub 2023 Nov 8 doi: 10.1053/j.semdp.2023.11.001. PMID: 37981479
Succinate dehydrogenase deficient renal cell carcinoma: A case report of an uncommon renal cancer.
Kodare D, Menon S, Prakash G, Desai S
Indian J Cancer 2023 Oct 1;60(4):583-585. Epub 2024 Jan 4 doi: 10.4103/ijc.ijc_801_21. PMID: 38189645
Succinate Dehydrogenase-Deficient Renal Cell Carcinoma.
Tsai TH, Lee WY
Arch Pathol Lab Med 2019 May;143(5):643-647. Epub 2018 Dec 21 doi: 10.5858/arpa.2018-0024-RS. PMID: 31026220

Clinical prediction guides

Immunoexpression of SDHB, FH, and CK20 among eosinophilic renal tumors: A tissue microarray study.
Karatay H, Ozluk Y, Dogan MA, Erdem S, Kilicaslan I
Ann Diagn Pathol 2021 Oct;54:151788. Epub 2021 Jul 2 doi: 10.1016/j.anndiagpath.2021.151788. PMID: 34246177

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