Liver and intrahepatic bile ducts - tumor
Hepatocellular carcinoma
Sarcomatoid variant


Topic Completed: 9 May 2019

Revised: 13 May 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Sarcomatoid hepatocellular carcinoma[TIAB]

Daffolyn Rachael Fels Elliott, M.D., Ph.D.
Ryan M. Gill, M.D., Ph.D.
Page views in 2018: 1,069
Page views in 2019 to date: 1,161
Cite this page: Fels Elliott DR, Gill RM. Sarcomatoid variant of hepatocellular carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/livertumorsarcomatoid.html. Accessed September 23rd, 2019.
Definition / general
  • Hepatocellular carcinoma (HCC) variant partially or entirely composed of malignant spindle cells with or without heterologous differentiation
Essential features
  • Tumor partially or entirely composed of malignant spindle cells
  • May have extensive necrosis and heterologous elements
  • Older, predominantly male patients
  • Worse prognosis than classic HCC
  • Sarcomatoid transformation has been associated with repeated chemotherapy / chemoembolization
Terminology
  • Also called spindle cell HCC, pseudosarcomatous HCC
ICD coding
  • ICD-10: C22.7 - Other specified carcinomas of liver
Epidemiology
Clinical features
Prognostic factors
Case reports
Treatment
Gross description
  • Poorly circumscribed, lobulated mass; may contain areas of necrosis and possibly heterologous elements
Gross images

Images hosted on other servers:

Well defined pink yellow mass

Whiteish tumor

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Ryan M. Gill, M.D., Ph.D.

Variable cellularity

Malignant spindle cells

Mitotic figures

Cytokeratin MNF

HepPar1


Mixed sarcomatoid HCC and cholangiocarcinoma

Sarcomatoid area

Mitotic figures

Arginase

Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Liver, mass, core biopsy:
    • Hepatocellular carcinoma, sarcomatoid variant (see comment)
    • Comment: This tumor shows two distinct morphologies. One component shows features of classic hepatocellular carcinoma, while the second component shows sarcomatoid features including pleomorphic spindle cells. Cytokeratin is variably positive and hepatocellular markers (HepPar1, arginase1 and glypican 3) are negative in the sarcomatoid component, as is typical for this variant.
Differential diagnosis
  • Sarcoma: undifferentiated pleomorphic sarcoma and fibrosarcoma
    • Clinical history of sarcoma
    • Will not have area of classic HCC
    • Immunohistochemistry (entirely negative for pancytokeratin) and potentially molecular testing (e.g. next generation sequencing cancer gene panel) are necessary for diagnosis
  • Metastatic sarcomatoid carcinoma:
    • Will not have area of classic HCC
    • Clinical history of carcinoma
    • Usually multiple nodules
    • Immunohistochemistry (pancytokeratin+) and potentially molecular testing (e.g. next generation sequencing cancer gene panel) are necessary for diagnosis
    • Negative for hepatocellular markers, positive keratin staining (usually patchy, perhaps only for high molecular weight keratins, e.g. as detected on MNF116)
Board review question #1
Which of the following histologic features is characteristic of this variant of hepatocellular carcinoma?



  1. Cytokeratin is always negative in the sarcomatoid component
  2. Hepatocellular markers are usually positive in the sarcomatoid component
  3. May show rhabdoid, chondroid or osteoid differentiation
  4. Must be entirely composed of malignant spindle cells
  5. Necrosis is not a common feature
Board review answer #1
C. May show rhabdoid, chondroid or osteoid differentiation

Reference: Sarcomatoid hepatocellular carcinoma

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Board review question #2
Which of the following is true regarding the sarcomatoid variant of hepatocellular carcinoma (HCC)?

  1. Associated with a better prognosis than classic HCC
  2. Higher serum AFP in comparison to classic HCC
  3. More common in patients undergoing repeated chemotherapy or transarterial chemoembolization
  4. Patients are predominantly female
  5. There is a lower incidence of extrahepatic spread in comparison to classic HCC
Board review answer #2
C. More common in patients undergoing repeated chemotherapy or transarterial chemoembolization

Reference: Sarcomatoid hepatocellular carcinoma

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