Author: Nat Pernick, M.D. (see Authors page)

Revised: 20 November 2015, last major update November 2013

Copyright: (c) 2015,, Inc.

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PubMed Search: IDH1[title]

Definition / General

Role in TCA cycle

Reactions catalyzed by wild-type and mutant IDH1 and IDH2

Uses by Pathologists
  • Bone: IDH1/2 mutation may differentiate chondrosarcoma (mutation present) from chondroblastic osteosarcoma (mutation not present, Am J Surg Pathol 2013;37:787); presence of IDH1/2 mutations may confirm diagnosis of dedifferentiated chondrosarcoma if osteosarcomatous differentiation present
  • CNS - glioma and glioblastoma:
Micro Images

Images hosted on other servers:

Kidney: normal


Oligoastrocytoma, anaplastic


Images hosted on


Various images

With PNET-like foci

Granular cell glioblastoma

Giant cell glioblastoma

With oligodendroglial component

Positive Staining - Disease
Negative Staining
  • Primary glioblastoma (includes small cell, granular cell, giant cell variants)