Conjunctival Stromal Tumor: Report of 4 Cases

Abstract

Purpose To describe the clinical, histopathologic, immunohistochemical, and ultrastructural features of a case series of benign stromal tumors in the bulbar conjunctiva. Design Observational case series. Participants Four patients with a conjunctival lesion that were classified histologically as low grade stromal tumors consisting of spindle-shaped cells with occasional pseudonuclear inclusion and multinucleated cells in a partly myxoid matrix. Methods Four cases of low grade conjunctival stromal tumors were retrospectively identified in an ophthalmic pathology laboratory database. Patients’ records were analyzed for demographic data, clinical appearance and the post-operative course. Formalin-fixed paraffin-embedded specimens were routinely processed and stained with hematoxylin and eosin (H&E) and periodic acid Schiff (PAS). Immunohistochemical stains for vimentin, S100, CD34, SMA (smooth muscle actin), CD68, and factor XIIIa were performed. Transmission electron microscopy (TEM) was performed on three of the cases. Main Outcome Measures Histopathologic evaluation (including immunostains and TEM) and clinical correlation. Results All four tumors occurred in the bulbar conjunctiva of patients between 41 to 53 years of age. None of the patients developed recurrence after excisional biopsy. Histologically, all tumors exhibited spindle-shaped cells with pseudonuclear inclusions and occasional multinuclear cells. Mitotic figures were not observed. The stroma appeared myxoid to collagenous. Immunohistochemical stains were positive for CD34, vimentin, and focally for CD68, but were negative for S100 and SMA. Conclusions We propose to classify these benign lesions which share distinct histopathologic features as “conjunctival stromal tumor (COST)”. A reactive/inflammatory component needs to be considered in the pathogenesis of this lesion.

Conjunctival Stromal Tumor: Expansion of Findings in a Newly Described Entity

“…The description of case 4 in Herwig et al’s initial description 2 is quite similar to our case 1 but the size of the lesion and clinical photograph were not available. Another unusual feature of case 1 is the almost completely myxoid stroma, which has not been described, while the cases in Herwig et al’s initial description 2 were of a mixed myxoid/collagenous type. Interestingly, the histology of our case 2 showed a purely collagenous stroma without myxoid changes and with multiple spindle-shaped cells.…”

“…It is important to recognize this entity since it might be associated with cardiac myxoma which can be life threatening 2 . Based on a series of 4 cases, Herwig et al recently described the new entity “conjunctival stromal tumor” (COST), with its name deduced from the well-known gastrointestinal stromal tumor (GIST) showing a partially similar immunomarker profile 2 . Positive markers for COST include CD34, Vimentin, and partially for CD68; negative markers include S100 and smooth muscle actin.…”

“…The specimens of the four COST cases published so far had a maximum size of 4×5×4 mm 2 . The description of case 4 in Herwig et al’s initial description 2 is quite similar to our case 1 but the size of the lesion and clinical photograph were not available.…”

“…The specimens of the four COST cases published so far had a maximum size of 4×5×4 mm 2 . The description of case 4 in Herwig et al’s initial description 2 is quite similar to our case 1 but the size of the lesion and clinical photograph were not available. Another unusual feature of case 1 is the almost completely myxoid stroma, which has not been described, while the cases in Herwig et al’s initial description 2 were of a mixed myxoid/collagenous type.…”

Conjunctival Myxoma: A Synopsis of a Rare Ocular Tumor

“…The ocular and physical examinations are generally unremarkable, except for minimal changes in ocular mobility and acuity. Patients gave very similar clinical histories, reporting a slow-growing, painless mass that had been present from 1 week in 2 exceptional cases 13,14 to a range from 4 to 288 months. Conjunctival myxoma was located in the temporal region of the eye in 20 cases (49%) including the authors' case, the nasal region in 11 cases (27%), and other locations (superior or unknown) in 10 cases (24%).…”