Orbital malignant melanoma associated with nevus of Ota
A 45-year-old male, who had a history of diabetes, presented to our department with the complaint of swelling of left eye (LE). He had noticed a gradual increase in the swelling for the last 6-8 months. On examination, LE revealed loss of perception of light, edema of lids, and bluish black pigmentation in the periorbital region and conjunctiva []. Ocular motility was restricted in all directions. An irregular mass could be felt through the upper lid, which was fixed to deeper structures. Scar of orbitotomy was seen on the left side. On retraction of the lids, the left eyeball was shrunken and pushed upward by the mass. Only a part of the cornea was visible []. He was using scleral shell prosthesis for cosmetic correction. The right eye was normal with a visual acuity of 6/9. Ophthalmoscopic examination of right eye showed few microaneurysms in the macular area. Patient revealed history of bluish black discolouration around the LE, since childhood. Approximately 13 years back, he had noticed protrusion of LE and was diagnosed elsewhere as orbital malignant melanoma. It was managed with lateral orbitotomy for excision biopsy followed by external beam radiotherapy. Later,cosmetic correction with scleral shell prosthesis was given elsewhere, over the phthisical LE. Four years back, in 2005, he attended the Oncology department of Amala Institute of Medical Sciences, for check up. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) of left orbit showed orbital lesion and he was given Cobalt-60 teletherapy. There was no bony metastasis or extraorbital extension of the lesion as per records.\nIn late 2008, he was referred to us with recurrence of left orbit swelling which gradually increased in size. Repeat CT showed recurrent malignant melanoma in left orbit with extension into the optic canal and lateral wall of orbit []. The orbit was filled by the pigmented tumor with infiltration into the orbital tissues and periorbita. Histopathology showed a mass of brownish soft tissue measuring 3.5 × 3 × 1.5 cm behind the eyeball. Serial sectioning of the eyeball showed atrophic eyeball. Microscopic examination showed cellular neoplasm arranged in a fascicular pattern composed of spindle cells having prominent nuclei and eosinophilic cytoplasm []. Mitotic figures were frequent and cells were laden with dark brown melanin pigment. The eyeball was distorted by the neoplasm. There was infiltration into the optic nerve, orbital muscles, and retrobulbar fibroadipose tissue. In view of the extensive spread of the tumor locally, a modified exenteration with frontal flap repair was done on the left side. Adjuvant chemotherapy was given after wound healing. During follow-up, prosthesis for LE was given []. After 1 year of follow-up, the patient did not have any recurrence or metastasis.