Embryonal Rhabdomyosarcoma of Upper Lid in 15-Year-Old Patient

Rhabdomyosarcoma is the most common childhood primary malignant tumor of orbit. Most of patients present between the ages of 7 and 8 years. Pure eyelid rhabdomyosarcoma is a very rare tumor with only a few reported cases in the literature. We introduce a pure embryonal rhabdomyosarcoma of upper lid in 15-year-old patient and demonstrate successful management of it. 1. Case Report A 15-year-old male patient was referred to oculoplastic service of one of teaching eye hospitals in Mashhad, Iran, due to right upper lid swelling since two months before visit. On external eye exam, the eye had nontender, nonmobile, firm, subcutaneous mass with ecchymosis of right upper lid (Figure 1). Mechanical ptosis of right upper eyelid was present with sparing of the visual axis. Eyelid margin architecture was normal. There was no history of recent trauma or family history of malignancy. Patient’s right upper lid appeared normal (according to previous photograph) and swelling on right upper lid was developed 2 months before visit. The swelling increased and ecchymosis developed on lesion 1 month before visit. The lesion had been suspected of allergic reaction or insect bite and treated with topical medication but eyelid swelling did not resolve. Figure 1: Right upper lid mass with ecchymosis led to mechanical ptosis. Other part of ophthalmic examination includes visual acuity, pupillary reflex; biomicroscopic and fundoscopic exam were normal. There was no proptosis of right eye. The left eyelids, adnexa, and globe were unremarkable. No limitation of eye movements was found. Due to chronic nature of swelling suspicious to tumoral lesion, orbital CT scan with axial and coronal view was requested, which showed well-defined, homogenous preseptal soft tissue mass without intraorbital extension or adjacent bony erosion (Figure 2). Figure 2: Axial and coronal view of orbital CT scan that showed well-circumscribed, uniform preseptal mass without orbital extension. The next step was excisional biopsy of lesion. Under general anesthesia, after preparation, via lid crease incision, complete excision of mass was performed and 3 * 3 * 2.5？cm firm, fleshy mass was excised (Figure 3). Figure 3: Gross view of excised tumor. Histopathologic examination showed diffuse, spindle-shaped cells proliferation which were entirely rhabdomyoblasts (Figure 4). Imunohistochemical staining for desmin was highly positive (Figure 5). The diagnosis of embryonal rhabdomyosarcoma was confirmed with immunohistochemistry. Figure 4: Embryonal rhabdomyosarcoma consisting almost entirely of differentiated