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Surgical Pathology Criteria

Succinate Dehydrogenase-Deficient Renal Cell Carcinoma

Definition

Alternate/Historical Names
SDHB-negative renal cell carcinoma
SDH-deficient renal oncocytoma

Diagnostic Criteria

Supplemental studies

Grading/Staging

Use WHO/ISUP grading system for clear cell and papillary renal cell carcinomas

Grade 1

Nucleoli are absent or inconspicuous and basophilic at 400x magnification

Grade 2

Nucleoli are conspicuous and eosinophilic at 400x and visible but not prominent at 100x

Grade 3

Nucleoli are conspicuous and eosinophilic at 100x

Grade 4

Extreme nuclear pleomorphism, multinucleate giant cells, and/or rhabdoid and/or sarcomatoid differentiation

 

Clinical

Differential diagnosis

SDH-Deficient Renal Cell Carcinoma

Chromophobe Renal Cell Carcinoma

SDHB Negative

SDHB Positive

CK7 and CD117 negative

Commonly CK7 and CD117 Positive

Neuroendocrine-like nuclei

Rasinoid or Koilocytic nuclei

Cytoplasmic inclusions present

Cytoplasmic inclusions absent

Plant-like, prominent cell borders absent

Plant-like, prominent cell borders present

Perinuclear clearing absent

Prominent perinuclear clearing

Entrapped non-neoplastic tubules common

Entrapped non-neoplastic tubules rare

 

SDH-Deficient Renal Cell Carcinoma

Renal Oncocytoma

SDHB Negative

SDHB Positive

CK7 and CD117 negative

CD117 Positive, CK7 typically negative

Varied architecture, rarely has hypocellular stroma

Nests floating in hypocellular stroma

Intracytoplasmic inclusions present

Intracytoplasmic inclusions absent

Flocculent eosinophilic cytoplasm

Granular eosinophilic cytoplasm

May show dedifferentiation

May show degenerative atypia (enlarged nuclei with smudged chromatin)

 

SDH-Deficient Renal Cell Carcinoma

Acquired cystic disease-associated renal cell carcinoma

SDHB Negative

SDHB Positive

Typically young patients with a germline mutation

Occurs in patients with end-stage renal disease and acquired cystic disease (often on dialysis)

Neuroendocrine-like nuclei

Large, irregular nuclei with prominent nucleoli

Although often has intracytoplasmic inclusions and vacuoles, lumina are absent

Intra and Intercytoplasmic lumina bestowing a cribriform appearance

No intratumoral crystals

Intratumoral calcium oxalate crystals

Lists

Renal epithelial neoplasms

Bibliography

Kurt Schaberg MD
John P Higgins MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting 11/22/16

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