A 3-year-old girl presented with a distorted pupil and decrease of visual acuity. A ciliary body mass at the superior-temporal quadrant could be seen at slit-lamp examination. Ultrasound biomicroscopy revealed a lesion with lobulated surface, arising at the ciliary body, composed of compact and cystic areas. An iridocyclectomy was performed but the tumour recurred after a few months. The eye was then enucleated. Histopathologic evaluation showed sheets and cords of proliferated medullary epithelium. Cystic spaces and Flexner-Wintersteiner rosettes were also seen. Those findings correlated well with ultrasound biomicroscopic images. The knowledge of echographic characteristics can significantly assist in the diagnosis of medulloepithelioma of the ciliary body.
Although extranodal manifestations are common in RDD and the lack of lymph node involvement is rare, our study supports that whenever there is an epibulbar tumor as a manifestation of RDD, the absence of lymphadenopathy is characteristic. Only 2 of the 9 reported cases presented with lymphadenopathy. The presence of emperipolesis and S-100-positive histiocytes during histopathologic evaluation confirms the diagnosis even in the absence of lymphadenopathy.
Corneal stromal dystrophies have distinct histopathologic features, which can be diagnosed with routine and special staining, particularly for combined granular/lattice (Avellino) dystrophy. Clinically, combined granular/lattice (Avellino) dystrophy can have features of both lattice and granular dystrophies, which can be misleading for diagnosis. Lattice dystrophy was the most frequent stromal dystrophy in this particular population.
Purpose Our aim was to evaluate the potential effect of imatinib mesylate (IM), a small molecule that specifically inhibits the tyrosine quinase receptors, on the proliferation and invasive abilities of two human retinoblastoma (Rb) cell lines. Furthermore, the ability of IM to radiosensitize Rb cells was evaluated. The potential targets of IM (C-kit, PDGRF-a and -b, and c-Abl) were also investigated in these cell lines. Methods Two human Rb cell lines (WERI-RB-1 and Y79) were cultured under normal growth conditions. An MTT-based proliferation assay and a Matrigel invasion assay were performed with and without exposure to 10 lM of IM. The cells were also irradiated with graded dosages of 0, 2, 4, 6, 8, and 10 Gy with and without IM and their proliferations rates were analyzed. Western blot and immunocytochemical analysis of cytospins were performed to evaluate the expression of C-kit, PDGRF-a and -b, and c-Abl. Results When IM was added to both cell lines a statistically significant (Po0.05) reduction in proliferation and invasive ability were observed. Exposure to IM also significantly increased the radiosensitivity of both Rb cell lines. The c-Abl expression was strongly positive, PDGRF-a and -b expression were also positive but the C-kit expression was negative in both cell lines. Conclusions These results indicate that Gleevec may be useful as an adjuvant treatment in Rb patients, specially those considered for radiation therapy.
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