Table 1.

Summary of main features of intraorbital PEComas.

Author	Sex	Age	Dimensions	Histology	IHC	Outcome
Iyengar et al. 2005 [5]	F	9	1.2 × 1.0 × 0.8 cm	Solid 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 necrosis	HMB45+ + + 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]	M	54	1.5 × 1.0 × 1.0 cm	Solid 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 case	M	47	2.0 × 1.5 cm	The 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 observed	HMB45+ + + 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