Case Reports in Pathology / 2016 / Article / Tab 1

Case Report

Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature

Table 1

Summary of main features of intraorbital PEComas.

AuthorSexAgeDimensionsHistologyIHCOutcome

Iyengar et al. 2005 [5]F91.2 × 1.0 × 0.8 cmSolid architecture with nests and trabeculae of epithelioid cells with abundant clear to eosinophilic cytoplasm. The uniform nuclei were oval to round with small nucleolus and dispersed chromatin. Within the lesion, a few cells had fine melanin pigment granules. There were infrequent mitoses. No evidence of necrosisHMB45+ + +
MART1− − − 
S100− − 
H&LMW CK− −
EMA− −
α-actin+ + −
Desmin− −
Calponin+ +
VIM−
CgA− −
No recurrence after 7 months of follow-up

Guthoff et al. 2008 [6]M541.5 × 1.0 × 1.0 cmSolid trabecular tumour cells show intimate association with ramified vascular network. The tumour cells are large with a clear to eosinophilic granular cytoplasm and round to oval nuclei. Few cells contained melanin. Proliferation index (Ki 67) was very low (<1%)HMB45+ + + 
MART1+ + + 
S100− − 
HMW CK− − 
α-actin− − 
Desmin− − 
VIM− 
CD 31− − 
CgA− − 
MiTF− −
No recurrence after 17 months of follow-up

Our caseM472.0 × 1.5 cmThe neoplasm showed a well-circumscribed, capsulated, expansive growth, formed by a cell proliferation arranged in vascular trabecular features or in solid cords. The cells were plump and large and showed an epithelioid phenotype with vesicular round nuclei, small nucleoli, and a low mitotic activity (2 mitoses in 10 HPF). The cells showed a clear or finely granular cytoplasm, sometimes containing a granular brown melanin pigment. No necrosis was observedHMB45+ + +
MART1− − 
S100− − 
H&LMW CK− − 
CK8/18− − 
α-actin+ 
Desmin− − 
Calponin+ 
VIM− − 
CD 31− − 
CD 34− − 
CgA− − 
MiTF− − 
TFE3− −
No recurrence after 40 months of follow-up