Details of Disease

General Information of Disease (ID: DISUDA2P)

• Disease Name: Fibrolamellar liver cancer
• Synonyms: eosinophilic hepatocellular carcinoma with lamellar fibrosis; fibrolamellar oncocytic hepatoma; hepatocellular carcinoma (fibrolamellar variant); hepatocellular carcinoma with increased stromal fibrosis; polygonal cell hepatocellular carcinoma with fibrous stroma; FL-HCC; fibrolamellar variant of hepatocellular carcinoma; eosinophilic glassy cell hepatoma; fibrolamellar carcinoma of the liver cells; oncocytic hepatocellular tumour; FHCC; fibrolamellar cancer; fibrolamellar hepatocellular carcinoma; hepatocellular carcinoma, fibrolamellar; fibrolamellar carcinoma; hepatocellular fibrolamellar carcinoma; fibrolamellar carcinoma of liver cells; FLC; oncocytic hepatocellular tumor; fibrolamellar hepatocarcinoma; polygonal cell type hepatocellular carcinoma with fibrous Stroma; liver cell fibrolamellar carcinoma
• Disease Class: 2C12: Liver cancer
• Definition: A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and is seen predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma. The prognosis is similar to classical hepatocellular carcinoma that arises in non-cirrhotic livers, and better than hepatocellular carcinoma that arises in cirrhotic livers.
• Disease Hierarchy:
  DIS0J828: Hepatocellular carcinoma
  DISUDA2P: Fibrolamellar liver cancer
• ICD Code:
  ICD-11: ICD-11: 2C12.02
  ICD-10: ICD-10: C22
  Expand ICD-11: 2C12.02
  Expand ICD-10: C22
• Disease Identifiers:
  MONDO ID: MONDO_0006210
  MESH ID: C537258
  UMLS CUI: C0334287
  MedGen ID: 83122
  Orphanet ID: 401920
  SNOMED CT ID: 15619004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
ENMD-2076	DMJZVPB	Phase 2	Small molecular drug	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 3 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
ARAF	TT5TURO	Strong	Altered Expression	[2]
DNAJB1	TTPXAWS	Strong	Biomarker	[3]
PRKACA	TT5U49F	Strong	Biomarker	[3]

This Disease Is Related to 9 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CBFA2T3	OTOJ10S1	Strong	Biomarker	[4]
DNAJA2	OTKF1VBT	Strong	Altered Expression	[2]
DNAJB6	OTMHIIAN	Strong	Altered Expression	[2]
DNAJC14	OT7DPIVN	Strong	Altered Expression	[2]
GLIS1	OTBDNB26	Strong	Biomarker	[4]
GLIS2	OTOUUV1X	Strong	Genetic Variation	[4]
GLIS3	OTBC960E	Strong	Biomarker	[4]
NFYA	OTWFFOVH	Strong	Altered Expression	[5]
NTS	OTPGDNQS	Strong	Biomarker	[6]

References

• [1] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [2] An acquired scaffolding function of the DNAJ-PKAc fusion contributes to oncogenic signaling in fibrolamellar carcinoma.Elife. 2019 May 7;8:e44187. doi: 10.7554/eLife.44187.
• [3] DNAJB1-PRKACA-positive metastatic fibrolamellar carcinoma with unknown primary in a pediatric patient.Pediatr Blood Cancer. 2020 Feb;67(2):e28060. doi: 10.1002/pbc.28060. Epub 2019 Nov 17.
• [4] Emerging Roles of GLI-Similar Krppel-like Zinc Finger Transcription Factors in Leukemia and Other Cancers.Trends Cancer. 2019 Sep;5(9):547-557. doi: 10.1016/j.trecan.2019.07.005. Epub 2019 Aug 20.
• [5] Transcriptional regulation of the ferritin heavy-chain gene: the activity of the CCAAT binding factor NF-Y is modulated in heme-treated Friend leukemia cells and during monocyte-to-macrophage differentiation.Mol Cell Biol. 1997 Mar;17(3):1387-95. doi: 10.1128/MCB.17.3.1387.
• [6] Neurotensin as a source of cyclic AMP and co-mitogen in fibrolamellar hepatocellular carcinoma.Oncotarget. 2019 Aug 20;10(49):5092-5102. doi: 10.18632/oncotarget.27149. eCollection 2019 Aug 20.