Contrast Functions of αA- and αB-Crystallins in Cancer Development.

α-Crystallins, initially identified as the structural proteins of the ocular lens, belong to the small heat shock protein family. They play significant roles in maintaining the lens transparency and preventing protein aggregation. α-Crystallins exist in two isoforms: αA and αB, and they display differential tissue distribution. Their mutations are implicated in several human diseases including cardiac myopathies, neurodegenerative diseases, cataracts and various types of cancers. Increased αB expression was detected in retinoblastoma, breast cancer, glioblastoma, prostate and renal cell carcinomas, indicating its role in promoting tumor growth. A complex picture emerges for αA. Although earlier studies suggest that αA may promote cancer development, recent studies from our laboratory demonstrate that αA can act as a tumor suppressor inhibiting cell transformation and retarding cell migration through modulating MAP kinase activity. In this review, we summarize the recent progress about the functions of αA and αB in cancer development.

Sumoylation in Lens Differentiation and Pathogenesis.

Sumoylation, a post-translational modification discovered over a decade ago, turns out to be a very important regulatory mechanism mediating multiple cellular processes. Recent studies from our laboratory and others also revealed that it plays a crucial role in regulating both differentiation and pathogenesis of the ocular lens. This review will summarize these progresses.

Endoscopic repair of posttraumatic enophthalmos using medial transconjunctival approach: a case report.

A blowout fracture of the medial orbital wall should be suspected when periorbital trauma results in epistaxis, orbital hemorrhage, horizontal dysmotility or dystopia of the globe, and/or orbital emphysema. Large medial orbital wall blowout fractures are frequently complicated by posttraumatic enophthalmos. Clinicians should consider a medial transconjunctival approach for repair of these fractures when surgical repair is indicated by a comprehensive clinical and radiologic orbital evaluation. Excellent cosmetic and functional results can be achieved through the use of an extended transcaruncular incision, rigid endoscope, and high-density porous polyethylene implant placement. The technique can be used in the early, delayed, and late stages of medial orbital wall blowout fracture repair. The technique can be used alone in isolated medial orbital wall fractures or combined with other craniofacial approaches.

In vitro susceptibilities of ocular Bacillus cereus isolates to clindamycin, gentamicin, and vancomycin alone or in combination.

A broth dilution assay was used to determine the in vitro susceptibilities of 10 ocular isolates of Bacillus cereus to clindamycin, gentamicin, and vancomycin both alone and in combination. The checkerboard technique was used to determine fractional inhibitory and bactericidal concentration indices for combinations of clindamycin-gentamicin and vancomycin-gentamicin.

Blepharoptosis and central nervous system abnormalities in combined valproate and hydantoin embryopathy.

PURPOSE: To report a case of intrauterine anticonvulsant exposure with subsequent ocular adnexal manifestations. METHODS: Case report. RESULTS: An 18-month-old child with known anticonvulsant embryopathy was referred for the management of bilateral congenital blepharoptosis. Physical examination confirmed ocular and nonocular external manifestations of valproate and hydantoin embryopathies. Cavum septum pellucidum, mild sulcation defects, and cerebellar atrophy were identified on neuroimaging. CONCLUSIONS: To our knowledge, our patient represents the second reported case of anomalous septum pellucidum after intrauterine valproate exposure. Clinicians evaluating patients with craniofacial features associated with intrauterine valproate exposure should recognize that concomitant anomalies of the central nervous system, including the septum pellucidum, might exist.

Localized neurofibroma of the orbit: a radiographic and histopathologic study.

PURPOSE: To demonstrate the clinical, radiographic, intraoperative and histopathologic presentation of a localized neurofibroma of the orbit. METHODS: Clinicopathologic case report. RESULTS: A 25-year-old man was examined for inferior displacement of the right globe. Physical examination and neuro-imaging identified a localized solid tissue mass of the superior orbit. Histopathologic evaluation after complete surgical resection proved the lesion to be a localized neurofibroma. CONCLUSIONS: The patient's history, clinical presentation, and imaging studies were strongly characteristic for this infrequently encountered orbital lesion. Several imaging features, including multilobulation, multiplicity, ring-configured contrast enhancement, and magnetic resonance imaging (MRI) signal intensity heterogeneity, when present in combination, may be reasonably diagnostic of this tumor.

Bilateral orbital metastases from breast carcinoma. A case of false pseudotumor.

OBJECTIVE: To inform clinicians of a metastatic orbital neoplasm that frequently masquerades as an orbital inflammatory syndrome and to stimulate thought regarding this neoplasm's fundamental pathobiology specific to its orbital involvement. DESIGN: Interventional case report and literature review. PARTICIPANTS: A 47-year-old woman with an acquired, bilateral, diffusely infiltrative orbital process is described. INTERVENTION: The clinical and radiographic features of the patient's orbital process are reported. The review of two prior fine-needle aspiration biopsy specimens failed to reveal a diagnosis. Histopathologic examination of an open surgical biopsy specimen included standard light microscopy of frozen and formaldehyde-fixed, paraffin-embedded tissue. RESULTS: Characteristics light microscopy findings suggested the diagnosis of metastatic lobular carcinoma of the breast. The patient refused all therapeutic intervention and died 9 months after orbitotomy. CONCLUSION: The presentation of breast carcinoma metastatic to the orbit as a bilateral infiltrative process is far more common than appreciated previously. Its rate of bilateral orbital involvement is 20%, whereas this rate for other adult neoplasms is much lower. The authors report an additional case, review the clinical features of previous case reports and series, and discuss aspects of the metastatic process. Breast carcinoma must be included in the differential diagnosis of infiltrative orbital processes encountered in adult female patients. When "orbital pseudotumor" is detected in an adult female patient, a thorough medical history must be obtained and physical examination performed. If a possible primary breast carcinoma is indicated, one must be wary of metastatic disease, and a histologic orbital diagnosis is warranted. The frequency of orbital metastasis and bilateral orbital involvement of this neoplasm reinforces the possibility for organ-specific metastases.

Recurrent orbital inflammation from metastatic orbital carcinoid tumor.

PURPOSE: To report an unusual clinical presentation for carcinoid tumor metastatic to the orbit. METHODS: Two adult patients with metastatic carcinoid tumor and unilateral orbital masses are described. RESULTS: Both patients sought treatment for acute unilateral orbital inflammation. Neuroimaging revealed orbital metastases adjacent to the inflamed adnexal tissue. Based on each patient's description of similar, prior, untreated episodes, minimal medical management was initiated. Resolution of the inflammatory signs was spontaneous in one case and assisted by pulsed, systemic corticosteroids in the second case. Compressive neuropathic vision loss occurred 11 months later in the second case. CONCLUSIONS: Carcinoid tumor metastatic to the orbit may manifest as recurrent orbital and ocular adnexal inflammation. These signs differ from systemic carcinoid syndrome in that they are unilateral, limited to only the orbital and ocular adnexal soft tissues, and resolve over days. Clinicians must carefully differentiate this manifestation from that of tumor necrosis, adnexal infection, or orbital outlet obstruction. This presentation may result from the spontaneous release of local inflammatory mediators intrinsic to the orbital tumor.

Retinal vascular alterations associated with dome-shaped and mushroom-shaped choroidal melanomas.

PURPOSE: Retinal vascular abnormalities are associated with choroidal melanoma. Although tumor ischemia, resulting in soluble angiogenic factor production, is a proposed etiology of these abnormalities, other sources of ischemia may also contribute. Mushroom-shaped choroidal melanomas have increased loss of the overlying choriocapillaris and increased subretinal fluid when compared to dome-shaped tumors; factors that may result in outer retinal ischemia, angiogenic factor production and resultant retinal vascular abnormalities. The differing amounts of retinal vascular abnormalities overlying dome-shaped compared to mushroom-shaped melanomas were determined to evaluate this hypothesis. METHODS: Retinal vascular abnormalities observed on fluorescein angiograms of eyes with choroidal melanoma were compared to the tumor configuration and presence of subretinal fluid. RESULTS: 23 eyes of 22 patients were included in the study. Retinal vascular abnormalities observed included dilated capillaries, telangiectatic capillaries, capillary nonperfusion, microaneurysms, neovascularization, lipid exudation and late staining of dye within the retina. Retinal vascular abnormalities were present in 4 of 14 eyes with dome-shaped tumors (29%) and in 8 of 9 eyes with mushroom-shaped tumors (89%). (Fisher's Exact Test, p = 0.009). Retinal vascular abnormalities were present in 2 of 10 eyes without subretinal fluid (20%) and in 10 of 13 eyes with subretinal fluid (77%). (Fisher's Exact Test, p = 0.012). CONCLUSIONS: Retinal vascular abnormalities are associated with mushroom-shaped choroidal melanomas more commonly than dome-shaped tumors. Outer retinal ischemia may result from choriocapillaris loss or the presence of subretinal fluid, contributing to soluble angiogenic factor production and resultant retinal vascular abnormalities in these eyes. SUMMARY STATEMENT: Retinal vascular abnormalities are associated with mushroom-shaped choroidal melanomas more commonly than dome-shaped tumors. These retinal vascular abnormalities may be related to an increase in outer retinal ischemia associated with mushroom-shaped tumors.

Orbital solitary fibrous tumor: radiographic and histopathologic correlations.

OBJECTIVE: To correlate the clinicopathologic and radiographic features characteristic of orbital solitary fibrous tumor (SFT). METHODS: The diagnostic features and clinical outcome of seven adults with orbital SFT are retrospectively outlined. Orbital imaging was performed by ultrasonography, computed tomography, or magnetic resonance imaging. Some cases were imaged by multiple modalities. Histopathologic examination of each tumor specimen included standard light and immunohistochemical stains. RESULTS: Heterogeneous internal composition was better appreciated on magnetic resonance imaging than on computed tomography. All cases undergoing magnetic resonance imaging showed T1 isointensity and T2 hypointensity relative to gray matter. Strong, generalized immunohistochemical reactivity to vimentin and CD34 validated the diagnosis of SFT and differentiated the specimens from other spindle cell neoplasms. After complete tumor resection, our patients remain tumor free with postoperative intervals of 15 to 45 months. CONCLUSIONS: Solitary fibrous tumor has now been reported in 26 orbits. No physical finding is pathognomonic, but several imaging traits are highly characteristic. Intralesional image heterogeneity and a predominantly low T2 signal intensity are distinctive of SFT. Complete tumor resection and immunohistologic specimen evaluation are emphasized. Clinicians should consider the diagnosis of SFT when confronted with an adult patient having an orbital soft tissue mass demonstrating the distinctive magnetic resonance imaging findings.