Table 1.
Clinical features and immunohistochemical findings of all previously reported perivascular epithelioid cell tumors arising from the eye and orbit
| Reference | Age, years/sex | Clinical presentation | Location/dimensions | Treatment | Immunohistochemistry |
Status at last follow-up | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| HMB-45 | melan-A | TFE3 | SMA | desmin | S-100 | vimentin | MITF | others | CD10 | CD31 | CD68 | ||||||
| Iyengar et al., 2005 [7] | 9/F | painless progressive swelling |
right inferomedial orbit/12×10×8 mm | excision | + | - | NA | + | - | - | - | NA | calponin +, tyrosinase + | NA | NA | NA | NR |
| Guthoff et al., 2008 [2] | 54/M | painless, progressive swelling |
right inferotemporal orbit/15×10×10 mm | excision | + | + | NA | NA | - | - | - | - | - | + | - | + | NR |
| Furusato et al., 2010 [8] (case 1) | 26/F | painless, progressive swelling |
left superolateral orbit/20×17×14 mm | excision | + | + | + | + | + | - | NA | - | - | NA | NA | NA | NR |
| Furusato et al., 2010 [8] (case 2) | 7/M | NA | left ciliary body/13×10 mm | excision | + | - | - | + | + | - | NA | - | - | NA | NA | NA | NR |
| Goto et al., 2015 [1] | 13/M | reduced vision, exotropia | right ciliary body/10×11×11 mm | excision | + | + | + | + | - | - | NA | NA | NSE –, GFAP– | NA | NA | NA | NR |
| Lubo et al., 2016 [9] | 47/M | progressive diplopia |
left inferomedial orbit/20×15 mm | excision | + | - | + | + | - | - | - | + | calponin + | - | - | NA | NR |
| Lin et al., 2016 [11] | 80/F | painless, progressive swelling |
left superomedial orbit/18×18×13 mm | excision | - | - | NA | NA | NA | NA | NA | NA | CD34 +, CK +, HHF-35 + | NA | NA | NA | NR |
| Paliogiannis et al., 2017 [10] | 46/M | painful swelling |
right medial orbit/15 mm (diameter) | excision | + | - | NA | + | NA | - | - | NA | cathepsin K + | NA | NA | NA | NR |
| Alam et al., 2017 [12] | 5/M | painless, progressive swelling |
right medial orbit/»50 mm along greatest dimension | excision plus CTx1 | + | NA | NA | + | NA | - | + | - | CD34 +, CK – synaptophysin – | NA | NA | NA | NR1 |
| Current case, 2017 | 9/F | painless, progressive swelling, reduced vision, proptosis | right superior orbit/52×35×28 mm | excision | + | - | NA | - | - | - | - | - | MyoD1 –, CD34 – | - | - | NA | NR |
CD, cluster of differentiation; CK, cytokeratin; CTx, chemotherapy; GFAP, glial fibrillary acidic protein; HHF-35, muscle actin antibody; HMB-45, Human Melanoma Black-45; MITF, microphthalmia-associated transcription factor; NR, no recurrence; NSE, neuron-specific enolase; SMA, smooth muscle actin; TFE3, transcription factor E3. 1 Recurrence was seen after 5 cycles of CTx following which re-excision was done. At last follow-up, the authors reported no recurrence.