Pathology features and the results of treatment of two cases of posterior choroidal leiomyoma

A 34-year-old Asian woman presented with a 3-month history suffering of burred vison of the right eye. The woman had a history of small uterine fibroids and was not treated surgically. Dilated fundus examination showed a large amelanotic mushroom-shaped choroidal mass located the superotemporal quadrant with peripheral exudative retinal detachment involving the macula, and the tumor surrounded by yellowish intraretinal exudation (Fig. 1A). Color doppler imaging (CDI) demonstrated pedunculated mass with inconsistent reflectivity of moderate intensity and no choroidal excavation, and arterial blood signals in the tumor. The size of the elevated lesion was 10.1× 5.9× 10.7 mm (Fig. 1B). PET/CT scan was performed and excluded metastases. The clinical diagnoses included amelanotic choroidal melanoma, RPE adenoma, and hemangioma. Fluorescein fundus angiography (FFA) revealed the neoplasm hypofluorescence in the early stage and hyperfluorescence with intense leakage on the surface in the late stage. Indocyanine green angiography (ICGA) showed the neoplasm hypofluorescence in the early phase and hyperfluorescence with prominent leakage in the late phase (Fig. 1C). Considering the age of the patient, a benign diagnosis was favorable, and local resection by the 23-gauge mircoinvasive vitrectomy for excision of intraocular tumors was performed. As for the neoplasm was located in the posterior pole, it was difficult to perform transscleral transillumination, therefore, before the vitrectomy, we use endo-light source to conduct transillumination and find the neoplasm was transmitted light. The neoplasm was analyzed by pathology and immunohistochemistry. A well-defined pink choroidal tumor was found on the gross examination. The section of the tumor was white and soft. Under the light microscope (LM), the tumor is composed of spindle cells, which are arranged in fascicles, with a small amount of collagen deposition between cells. The nuclei were slightly pleomorphic and hyperchromatic, with occasional nucleoli and no atypical mitosis (Fig. 1D a). Immunohistochemical studies revealed that tumor cells show positive immunoreactivity for Actin and SMA (Fig. 1D b-c), negative immunoreactivity for Desmin (d), Vimentin (e), S-100 (Fig. 1D d-f) and HMB45/Melan-A. The diagnosis of CL was established, and the best corrected visual acuity was 20/66 after the vitrectomy. There was no found recurrence or metastases after 3 years follow-up.

A 33-year-old Asian woman presented complaining of blurry vision for 2 weeks. The woman had no family history of systemic (uterine) fibroids or any subtle clinical signs associated with the disease. Fundus examination demonstrated a yellowish-white choroidal neoplasm located the inferior nasal quadrant of the right eye (Fig. 2A). Exudative retinal detachment and yellowish intraretinal exudation were also obverted. CDI was performed and showed a 12.7× 5.8× 11.6 mm tumoral mass without choroidal excavation (Fig. 2B). On FFA, the tumor showed hypofluorescence in the early stage and hyperfluorescence with strong leakage on the surface in the late stage. Moreover, the bottom of the tumor is still hypofluorescent. ICGA revealed hypofluorescence in the early stage and hyperfluorescence with obvious leakage in the late stage. Dual circulation of tumor vessels can be seen (Fig. 2C). 18F-FDG PET/CT has been underwent and no positive uptake in the other body parts. The diagnosis was not consistent with uveal melanoma or metastasis and the patient processed local resection by the 25-gauge mircoinvasive vitrectomy. The tumor exhibited marked translucency on transillumination. The tumor was sent for histopathological analysis, and the result demonstrated the tumor with same characteristics as case 1. Light microscopy showed a well-defined tumor in the posterior choroid, did not penetrate the sclera. The tumor consists of spindle cells arranged in bundles without mitosis. The background is fibrous, similar to glial tissue, and the cell outline is fuzzy. There were obvious capillary distribution between cell bundles (Fig. 2D. a). Immunohistochemistry showed that smooth muscle specific actin, SMA and Desmin were positive (Fig. 2D. b-d), while Vimentin, S-100 protein and Melan-A/HMB45 were negative (Fig. 2D. e-f). The diagnosis was choroidal leiomyoma. There was no signs of proliferative vitreoretinopathy or tumor recurrence after the surgery for 6 months of follow-up, and the best corrected visual acuity was 20/100.