Case PresentationAdenoid Cystic Carcinomas (ACC) represents the most frequent carcinoma of the lacrimal gland with a percentage of 32% [1]. Usual Symptoms comprise, propoptosis, loss of vision, diplopia, epiphora, eye lid swelling and pain [2,3]. Local therapy as surgery or radiation therapy is complicated by the orbital anatomy involving the second, third and sixth cranial nerve and essential structures for ocular function as the lacrimal gland, external and internal muscles, lens and the retina [4]. The following case illustrates the interdisciplinary approach to a rare tumor entity and the considerations made for preserving the orbital structures functionally.
PatientA 53 yrs -lady was evaluated for left eye proptosis for 6 months. Ophthalmic examination showed normal vision and a prominent tumor in the superior-temporal quadrant of the left orbit. MRI revealed, a left bulb protrusion due to a sharp demarcated tumor in the left upper quadrant (1.8* 0.7* 2.0 cm) with posterior extension to lateral rectus and left lateral wall of orbit was noticed and thought to be a pleomorphic adenoma (Figure 1). The patient underwent resection of mass by left lateral orbitotomy with osteotomy of left lateral orbital wall . Intraoperatively, the tumor was found to be superficial with extracapsular extension and was consequently removed, supposedly completely. Figure 1: MRI image showing left orbital mass lesion.Pathological examination revealed an ACC of the lacrimal gland . No evidence of perineural invasion and the resection margins were free. Subsequently, the carcinoma was staged as pT2, pNx, cMx [5]. C Kit -Positive in Immunohistochemistry