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 2

Immunophenotype of neoplasms in the differential diagnosis of epithelioid malignant peripheral nerve sheath tumor.

Neoplasm
EMPNSTMalignant rhabdoid tumorMyoepithelial carcinomaEpithelioid sarcomaMalignant melanomaMGNET

Immunostain
 INI1Absent (50-70%)Absent (98%)Absent (10-40%)Absent (90%)RetainedRetained
 KeratinRarePositivePositivePositiveNegativeNegative
 EMARarePositivePositivePositiveNegativeNegative
 SMANegativeVariableVariableNegativeNegativeNegative
 DesminNegativeVariableNegativeNegativeNegativeNegative
 MyogeninNegativeNegativeNegativeNegativeNegativeNegative
 MelanANegativeNegativeNegativeNegativePositiveNegative
 HMB45NegativeNegativeNegativeNegativePositiveNegative
 SOX10PositiveNegativePositiveNegativePositivePositive
 S100PositiveVariablePositiveNegativePositivePositive
 GFAPNegativeVariableNegativeNegativeNegativeNegative
 CD34NegativeNegativeNegativeVariableNegativeNegative

EMPNST with rhabdoid differentiation may express SMA. ^Please note that the immunophenotypes for the neoplasms listed above may deviate from what is listed. Abbreviations: EMPNST: epithelioid malignant peripheral nerve sheath tumor; MGNET: malignant gastrointestinal neuroectodermal tumor; EMA: epithelial membrane antigen; SMA: smooth muscle actin; GFAP: glial fibrillary acidic protein.