Acute Presentation of Mesectodermal Leiomyoma of the Ciliary Body

Abstract

Purpose: We report a case of acutely presenting mesectodermal leiomyoma of the ciliary body in a 29-year-old female who reported waking up with swollen eyelids of the right eye and light-perception vision. The affected eye had elevated intraocular pressure, a flat anterior chamber, and a pale, round mass arising from the nasal ciliary body, invading the angle and protruding into the visual axis posterior to the lens. Within days, the visual acuity decreased to no light perception. The eye was enucleated.

Methods: The enucleated eye harbored a tumor arising from the ciliary body, measuring 18 mm in the greatest dimension. Spindled cells with fibrillary cytoplasmic processes suggested a neural origin though negative for S-100, Melan-A, and HMB-45. The cells stained strongly positive for smooth muscle actin and vimentin, leading to the diagnosis of mesectodermal leiomyoma of the ciliary body.

Results: We review the literature to expand upon the clinical findings, diagnostic methods, and histopathologic and immunohistochemistry characteristics of mesectodermal leiomyoma.

Conclusion: Leiomyoma must be in the differential diagnosis for ciliary body mass, especially in women of reproductive age. Diagnosis relies on histopathology and immunohistochemistry. The mechanism of acute symptom onset may be multifactorial. This case emphasizes the possibility of acute presentation of a rare, benign intraocular tumor.

Keywords: Benign tumor; Enucleation; Immunohistochemistry; Leiomyoma; Melanoma.

Fig. 1

Clinical presentation of the ciliary body mesectodermal leiomyoma. a Slit-lamp photograph of the right eye. The eye is notable for diffuse conjunctival chemosis and nasal sentinel vessels associated with a mass in the nasal angle. The anterior chamber is flat secondary to the ciliary body mass causing anterior shift of the iris. Note the posterior synechiae that suggest chronic inflammation in the anterior chamber. b B-scan ocular ultrasound performed at presentation. The acoustically solid mass arising from the ciliary body has a central anechoic area that corresponds histologically with necrosis. c CT of the orbit without contrast. This imaging modality confirms the presence of a large intraocular mass arising from the nasal ciliary body that temporally displaces the crystalline lens and expands the total volume of the globe compared to the fellow eye. Proptosis is appreciated on the image.

Fig. 2

Gross and histopathologic appearance of the mesectodermal leiomyoma. a Gross section. A yellowish, solid tumor measuring 18 mm in the greatest dimension arising from the ciliary body. b Hematoxylin and eosin stain. The mass consists of cells with indistinct cell borders and round to fusiform nuclei; the cytoplasm has a fibrillary appearance that conferred a neuroid morphology. There is minimal mitotic activity. c Immunohistochemistry for smooth muscle actin; the tissue diffusely stains positive. d Immunohistochemistry for vimentin. The staining is focal, restricted to the periphery, and with an unusual perinuclear pattern. e Immunohistochemistry for Ki-67 (Mib-1). The staining index is very low at 0% in the center of the tumor, focally rising to about 5% in the periphery. Overall, it is <1%. f The neurosensory retina is diffusely detached with obvious atrophy of the outer retinal layers and associated serous subretinal fluid. HE. ×20.