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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

Low-grade Oncocytic Fumarate Hydratase-deficient Renal Cell Carcinoma: An Update on Biologic Potential, Morphologic Spectrum, and Differential Diagnosis With Other Low-grade Oncocytic Tumors.
Hamza A, Sirohi D, Smith SC, Amin MB
Adv Anat Pathol 2021 Nov 1;28(6):396-407. doi: 10.1097/PAP.0000000000000321. PMID: 34561376
Report From the International Society of Urological Pathology (ISUP) Consultation Conference on Molecular Pathology of Urogenital Cancers: III: Molecular Pathology of Kidney Cancer.
Williamson SR, Gill AJ, Argani P, Chen YB, Egevad L, Kristiansen G, Grignon DJ, Hes O
Am J Surg Pathol 2020 Jul;44(7):e47-e65. doi: 10.1097/PAS.0000000000001476. PMID: 32251007Free PMC Article
The classification of pediatric and young adult renal cell carcinomas registered on the children's oncology group (COG) protocol AREN03B2 after focused genetic testing.
Cajaiba MM, Dyer LM, Geller JI, Jennings LJ, George D, Kirschmann D, Rohan SM, Cost NG, Khanna G, Mullen EA, Dome JS, Fernandez CV, Perlman EJ
Cancer 2018 Aug;124(16):3381-3389. Epub 2018 Jun 15 doi: 10.1002/cncr.31578. PMID: 29905933Free PMC Article

Recent clinical studies

Etiology

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

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
SDH-deficient renal cell carcinoma - clinical, pathologic and genetic correlates: a case report.
Kumar R, Bonert M, Naqvi A, Zbuk K, Kapoor A
BMC Urol 2018 Nov 27;18(1):109. doi: 10.1186/s12894-018-0422-8. PMID: 30482207Free PMC Article
Succinate dehydrogenase B-deficient renal cell carcinoma: A case report with novel germline mutation.
Iwashita H, Okudela K, Matsumura M, Yamanaka S, Sawazumi T, Enaka M, Udaka N, Miyake A, Hibiya T, Miyake N, Matsumoto N, Makiyama K, Yao M, Nagashima Y, Ohashi K
Pathol Int 2017 Nov;67(11):585-589. Epub 2017 Sep 28 doi: 10.1111/pin.12587. PMID: 28960644

Prognosis

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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