Table I.
Clinical features of patients with orbital alveolar soft part sarcoma.
| Patient | Age, years/sex | Eye affected | Course of disease | Clinical features | Tumour location | Tumour size, cm | Management | Follow-up |
|---|---|---|---|---|---|---|---|---|
| 1 | 19/M | Right | 1 mh | Proptosis, diplopia, ophthalmalgia, anorthopia, ocular motility restriction, conjunctival congestion, papilloedema | Medial part of the orbit, adhering to medial rectus muscle, extending to apex | 2.5×1.5×1.5 | S+R | 6 mh, no Rec or M, lost |
| 2 | 51/F | Right | 1 mh | Proptosis, ophthalmalgia, anorthopia, lid swelling, palpable mass, papilloedema | Medial optic nerve, indistinguishable from medial rectus muscle | 3.5×3.5×1.2 | S+E+R | Lost |
| 3 | 2/F | Right | 1 mh | Proptosis, ophthalmalgia, anorthopia | Inside inferior rectus muscle throughout its length to apex | 5.0×3.6×0.8 | S | Gave up therapy, 2 mh Rec, 1 yr succumbed |
| 4 | 6/M | Left | 15 days | Proptosis, ptosis, lid swelling, ocular motility restriction | Inside superior rectus muscle | 2.0×1.5×1.2 | S+R | 61 mh, no Rec or M |
| 5 | 2/M | Left | 1 mh | Proptosis, tearing, anorthopia, ocular motility restriction | Between superior rectus muscle and levator palpebrae superioris muscle | 2.0×2.0×2.0 | S+R | 49 mh, no Rec or M |
| 6 | 9/F | Left | 9 days | Proptosis, lid swelling, ocular motility restriction | Inside medial rectus muscle | 4.5×3.0×1.0 | E+R+C | 13 mh, no Rec or M |
| 7 | 10/F | Left | 20 days | Proptosis, lid swelling, diplopia, pain, vision impaired, anorthopia, ocular motility restriction, papilloedema | Inferolateral, indistinguishable from inferior and lateral rectus muscle and optic nerve | 4.5×3.0×1.5 | B+E | 13 mh, no Rec or M |
| 8 | 32/F | Left | 1 mh | Vision impaired, diplopia, proptosis, ophthalmalgia | Indistinguishable from lateral rectus muscle | 5.0×3.0×2.0 | S+R+C | 3 mh, no Rec or M |
B, biopsy; S, surgical excision; E, exenteration; R, radiotherapy; C, chemotherapy; Rec, recurrence; M, metastasis; mh, months; yr, years.