Primary Diffuse Large B-Cell Lymphoma of the Ciliary Body.

Purpose: To report an unusual case of an eye with primary ciliary body lymphoma which came to enucleation allowing detailed histopathological examination. Methods: A 50-year-old man presented with a painful loss of vision in the left eye. The clinical, imaging, and immunohistopathological features of this case were reviewed. Results: The vision in the left eye was light perception. There were keratic precipitates, an irregular and thickened iris with neovascularization. Imaging studies disclosed a ciliary body mass extending into the anterior chamber. The eye was enucleated and immunohistopathological examination showed positive staining with CD20, BCL-2, MUM1, and CD10. Staining with BCL-6 was weak and S100 and HMB45 expressions were negative. Occasional CD3+ reactive T cells were present. The Ki-67 index was 80-90%. All these results suggested diffuse large B-cell lymphoma. Conclusions: Diffuse large B-cell lymphoma may primarily arise from the ciliary body and can develop without systemic or central nervous system disease.

Dedifferentiation of Retinal Pigment Epithelium in a Patient With Chronic Retinal Detachment.

A 34-year-old woman presented with vision loss in her right eye, which had persisted for approximately 12 months. Funduscopy showed horseshoe retinal tear at the 8-o'clock position and retinal detachment in inferior, nasal, and temporal quadrants, with a pigmented demarcation line in the right eye. Diffuse punctate accumulations were noted on the detached retinal surface and in the vitreous. Fundus autofluorescence imaging detected diffuse punctate accumulations, which produced hyperautofluorescence spots. Cytological examination of a vitreous sample detected CD68-positive and cytokeratin- and CD45-negative macrophages. These findings indicate that epithelial-to-mesenchymal transition occurred in the retinal pigment epithelium of our patient. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:716-720.].

Mesenchymal Chondrosarcoma of the Lacrimal Gland.

A 23-year-old woman presented with right-sided painless proptosis that developed in 12 months. MRI studies demonstrated a well-delineated tumorous enlargement of the right lacrimal gland with homogenous signal intensity and compressing the globe. The tumor was removed totally and in 1 piece with the tentative diagnosis of a pleomorphic adenoma. Pathologic examination revealed biphasic neoplastic elements, which were composed of the cartilaginous matrix and small round cell component. Immunohistopathological examination showed positive CD99 staining and negative reaction to S100, panCK, and CD15. The patient then received a total of 64 Gy orbital radiotherapy in 32 fractionations. There has been no recurrence or metastasis during 14 months of follow up. This case showed that mesenchymal chondrosarcoma may arise from the lacrimal gland and must be considered in the differential diagnosis of lacrimal gland tumors in young adults.

Primary sclerosing epithelioid fibrosarcoma of kidney with variant histomorphologic features: report of 2 cases and review of the literature.

The authors present two cases of primary sclerosing epithelioid fibrosarcoma (SEF) of the kidney. Both patients had a mass in the upper part of the left kidney without any primary extrarenal neoplastic lesions. Grossly, the tumors were solid masses both measuring 7.5 cm in the greatest diameter. Histologically, one of the lesions exhibited a predominantly lobular growth of round or oval small uniform epithelioid cells in variable cellularity. Circular zones of crowded tumor cells alternating with hypocellular collagenous tissue in a concentric fashion around entrapped native renal tubules were distinctive. The second case was distinctive with significant cytological atypia in the neoplastic cells and prominent reactive proliferations in the trapped renal tubules. Immunohistochemically, vimentin, bcl-2 and MUC4 were diffusely positive in both. They were negative for S-100 protein, CD34, and desmin, whereas CD99 were positive in one lesion. Fluorescence in situ hybridization assay using dual staining probes detected EWSR1-CREB3L1 fusion in each lesion, which is characteristic molecular findings of SEF. One patient presented widespread distant metastases at the time of diagnosis. In the other, no tumor deposits were detected other than primary. Both patients have been alive with 30 and 10 month follow-ups, respectively. These tumors are 6th and 7th cases of primary renal SEF in the literature confirmed by FISH study, which exhibit unique and remarkable histomorphologic features.