Liver & intrahepatic bile ducts

Hepatocellular carcinoma

HCC - sarcomatoid variant



Last author update: 22 September 2022
Last staff update: 22 September 2022

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PubMed Search: Sarcomatoid hepatocellular carcinoma

Daffolyn Rachael Fels Elliott, M.D., Ph.D.
Ryan M. Gill, M.D., Ph.D.
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Cite this page: Fels Elliott DR, Gill RM. HCC - sarcomatoid variant. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorsarcomatoid.html. Accessed December 9th, 2022.
Definition / general
  • Hepatocellular carcinoma (HCC) variant, partially or entirely composed of malignant spindle cells
Essential features
  • Component of spindle cell morphology
  • Area of conventional HCC usually present to support the diagnosis
  • Worse prognosis than classic HCC
  • Sarcomatoid transformation associated with repeated chemotherapy / chemoembolization
Terminology
ICD coding
  • ICD-10: C22.7 - other specified carcinomas of liver
Epidemiology
Sites
  • Liver
Pathophysiology
Etiology
Clinical features
Diagnosis
  • Imaging modalities for diagnosis of HCC: multiphasic computed tomography (CT) or magnetic resonance imaging (MRI)
  • Tissue biopsy is indicated if imaging is not diagnostic of HCC
Laboratory
Radiology description
Radiology images

Images hosted on other servers:

Multiphase abdominal CT scan

T1 weighted MRI

T2 weighted MRI

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 with hemorrhage and necrosis

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
  • Hepatocellular markers (HepPar1, arginase1, glypican 3) typically negative in the sarcomatoid component
  • Sarcomatoid markers (e.g., vimentin, actin): typically negative in the carcinomatous component
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
  • Undifferentiated primary liver carcinoma:
  • 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 style question #1

Which of the following histologic features is characteristic of the sarcomatoid variant of hepatocellular carcinoma?

  1. Contains components of conventional HCC and spindle cell morphology
  2. Cytokeratin is always negative in the sarcomatoid component
  3. Hepatocellular markers are usually positive in the sarcomatoid component
  4. Must be entirely composed of malignant spindle cells
  5. Necrosis is not a common feature
Board review style answer #1
A. Contains components of conventional HCC and spindle cell morphology

Comment Here

Reference: Sarcomatoid hepatocellular carcinoma
Board review style 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 style answer #2
C. More common in patients undergoing repeated chemotherapy or transarterial chemoembolization

Comment Here

Reference: Sarcomatoid hepatocellular carcinoma
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