Case Report

Epithelioid Malignant Peripheral Nerve Sheath Tumor in the Liver: Report of a Rare Unusual Case with Rhabdoid Morphology and Review of the Literature

Table 1

Histopathologic comparison of MPNST and EMPNST.

MPNSTEMPNST

Morphology:Spindle cell predominateEpithelioid cell predominant
Immunohistochemistry:
 SOX10Patchy positiveDiffusely positive
 S100Negative to scattered positivityDiffusely positive
 H3K27me3Lost in 50%Retained
 SMARCB1/INI1RetainedLost in 50-70%
Inactivating mutations:
SMARCB1/INI1AbsentPresent in 75%
NF1FrequentOccasionally present
NF2RareOccasionally present
CDKN2AFrequentOccasionally present
TP53FrequentOccasionally present
SUZ12 and EEDFrequentAbsent

Abbreviations: MPNST: malignant peripheral nerve sheath tumor; EMPNST: epithelioid malignant peripheral nerve sheath tumor.