Hayyam Kiratli

Correlation of clinicopathological parameters with HGF, c-Met, EGFR, and IGF-1R expression in uveal melanoma.

This study evaluated the expression profile of hepatocyte growth factor (HGF), c-Met, epidermal growth factor receptor (EGFR), insulin-like growth factor-1 receptor (IGF-1R), and vitreal and serum EGF, HGF, IGF-1 levels in patients with uveal melanoma and assessed their correlation with the clinicopathological parameters. Forty patients with uveal melanoma were included in the study. Clinicopathological parameters were evaluated with hematoxylin–eosin staining. HGF, c-Met, EGFR, and IGF-1R expressions were evaluated immunohistochemically. HGF, EGF, and IGF-1 levels were measured with enzyme-linked immunosorbent assay in vitreous and serum specimens taken at enucleation and 6 months after the enucleation. HGF, c-Met, IGF-1R, and EGFR expressions were detected in 57.5, 20, 20, and 12.5% of cases, respectively. IGF-1R expression was significantly correlated with the degree of pigmentation, necrosis, and lymphocyte infiltration (P=0.013, 0.04, and 0.017). EGFR expression was significantly correlated with the mitosis rate (P=0.02). Vitreal EGF and serum IGF-1 levels were significantly higher in patients with scleral invasion (15.72±29.13, 199.01±154.01 pg/ml, respectively) when compared with the patients without invasion (0.56±1.05, 33.01±36.52 pg/ml) (P=0.03 and 0.015). When the preoperative and postoperative serum growth factor levels were compared, the serum EGF level was found to be lower (125.93±62.84, 100.02±31.19 pg/ml, P=0.007) and the serum IGF-1 level (165.81±153.6, 301.35±131.24 pg/ml, P<0.001) was found to be higher in the postoperative 6-month specimens. Uveal melanomas express HGF, c-Met, EGFR, and IGF-1R. Vitreal growth factor levels and expression of EGFR and IGF-1R are correlated with some clinicopathological parameters. IGF-1 and EGF may have a role in the development and progression of uveal melanoma.

Treatment of Retinoblastoma With Indirect Ophthalmoscope Laser Photocoagulation

INTRODUCTION The indirect ophthalmoscope laser photocoagulation delivery system is relatively new and is potentially applicable for treating selected small retinoblastomas. There has been very little reported on the results achieved with this laser delivery system in the treatment of retinoblastoma. METHODS We reviewed all patients with retinoblastoma managed by indirect ophthalmoscope laser photocoagulation on the Ocular Oncology Service between July 1, 1991 and July 1, 1994. The individual tumor size, location, and proximity to the optic disc and foveola, as well as the laser power, duration, and number of sessions ,were recorded. We assessed the tumor response to treatment and the visual outcome of the patients. RESULTS There were 30 retinoblastomas in 20 eyes of 18 patients managed with indirect ophthalmoscope laser photocoagulation over the 3-year period. The mean tumor base was 2.1 mm (range: 1 mm to 8 mm); the mean tumor thickness, 1.2 mm (range: 0 mm to 3 mm). The tumor margin was a mean of 6.5 mm (range: 0 mm to 19 mm) from the foveola, and 7.7 mm (range: 0 mm to 22 mm) to the optic disc. In general, it required a power of approximately 350 mW and a continuous duration lasting 1 to 4 seconds to obtain satisfactory obliteration of feeding blood vessels. The tumor responded in one to three sessions (mean: 1.9 sessions per tumor). Complete regression occurred in 21 tumors (70%) and local recurrence in nine (30%). The recurrent tumors were successfully treated with plaque radiotherapy in seven cases and cryotherapy in two cases. The central vision was minimally distorted due to foveal traction from the laser treatment in three cases. CONCLUSION Indirect ophthalmoscope laser photocoagulation is an effective conservative method to manage selected small retinoblastomas. Tumors treated by this technique should be followed closely due to the moderate risk for local recurrence.

Percutaneous treatment of an orbital hydatid cyst: a new therapeutic approach

PURPOSE To describe the percutaneous treatment of an orbital hydatid cyst as an alternative approach to conventional surgery. METHODS In a 21-year-old man with diplopia and right proptosis, radiologic studies disclosed a 25 x 25 x 20-mm purely cystic mass in the right retrobulbar area. Based on the presumptive diagnosis of hydatid cyst, the cyst was treated percutaneously under ultrasonographic guidance with aspiration, 15% hypertonic saline injection, and reaspiration without any complication. RESULTS A substantial decrease in the size of the cyst was observed in the 3 months after treatment. Nine months after treatment, the shrunken cyst had a volume of only 0.5 ml, and the patient was asymptomatic. Twenty-one months after the procedure, the findings were consistent with those at 9 months of follow-up. CONCLUSION Percutaneous treatment of orbital hydatid cysts, which is more satisfactory to both the patient and the physician, may be a safe and effective alternative to surgical extirpation.

Multifocal fibrosclerosis: a new case report and review of the literature.

Abstract. A case of multifocal fibrosclerosis is presented with MR images. Bilateral sclerosing orbital pseudotumor invading cavernous sinuses were the presenting disorder. Magnetic resonance imaging showed involvement of paranasal sinuses bilaterally and multiple supratentorial dural masses. Retroperitoneal fibrosis was associated with the condition. Multifocal involvement should be considered in patients with sclerosing orbital pseudotumor and an imaging approach should be performed on the appropriate clinical condition to document possible coexistence of other disorders including retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, and Riedels thyroiditis

Spontaneous regression of retinal astrocytic hamartoma in a patient with tuberous sclerosis

PURPOSE To report the complete regression of a retinal astrocytic hamartoma in conjunction with tuberous sclerosis. DESIGN Observational case report. METHODS A 12-year-old boy with tuberous sclerosis, diagnosed early in life, was found to have a retinal astrocytic hamartoma in his left eye. He had bilateral optic atrophy because of long-standing increased intracranial pressure due to a cerebral giant cell astrocytoma. He was followed on a yearly basis with fundus photography and fluorescein angiography. RESULTS At the time of initial presentation, the minimally elevated intraretinal tumor measured 2 x 1.5 mm. A year later, the same tumor measured 1.5 x 1 mm. Two years after initial presentation, the tumor totally disappeared. CONCLUSION This patient has demonstrated that retinal astrocytic hamartoma may undergo spontaneous regression.

Intramuscular hydatid cyst of the medial rectus muscle

PURPOSE To describe a unique case of orbital hydatid cyst that was located solely within the medial rectus muscle. DESIGN Retrospective, interventional case report. METHODS A 20-year-old woman presented with right periocular pain induced by ocular movements. Magnetic resonance imaging studies showed a focal, well-circumscribed intramuscular cystic lesion of the right medial rectus muscle. The cyst was completely extirpated from the muscle, but some of the fluid content escaped into the surrounding tissues. Irrigation with hypertonic saline was performed. RESULTS Histopathologic examination showed an acellular cyst wall with inner germinal layer, diagnostic for hydatid cyst. Two cycles of oral albendazole were administered with no recurrence. CONCLUSIONS In the orbit, hydatid cyst may be localized primarily within an extraocular muscle and produce painful eye movements. Hydatid cyst must be considered in the differential diagnosis of solitary cystic enlargements of extraocular muscles.

PARANEOPLASTIC RETINOPATHY ASSOCIATED WITH METASTATIC CUTANEOUS MELANOMA OF UNKNOWN PRIMARY SITE

Purpose: To describe further the clinical and immunological features of cutaneous melanoma-associated retinopathy, which is an infrequent form of paraneoplastic syndrome.Methods: We studied the salient clinical and immuno-logical aspects of a 66-year-old man with metastatic cutaneous melanoma to lymph nodes of unknown primary site who developed melanoma-associated retinopathy.Results: There was gradual loss of vision in the left eye. Colour vision and night vision were not affected. Visual fields showed arcuate defects. A full-field electroretinogram demonstrated attenuation of the b-wave amplitude in the left eye. The a-wave was intact. Indirect immunofluorescence techniques showed that the antibody reactions took place mainly in the outer plexiform layer of the retina.Conclusions: Bipolar cells seem to be the target in melanoma-associated retinopathy. Contrary to previous reports, night blindness may not be a universal finding.

Poland-Moebius Syndrome: A Case Report

BACKGROUND The primary site of pathology in Moebius syndrome is still unknown, although several studies have variably localized the lesion in the extraocular muscles, cranial nerves, or central nervous system. CASE A 24-year-old man with Poland-Moebius syndrome and acquired progressive bilateral paralytic lower eyelid ectropion is described. OBSERVATIONS In this patient, magnetic resonance imaging studies revealed a barely detectable pontine hypoplasia and normal recti muscles. Nerve conduction studies of the facial nerves showed a severe demyelinating or dysmyelinating type of neuropathy. Bilateral lower eyelid ectropium of the patient was successfully corrected by canthal tightening procedures. CONCLUSION Contrary to many reported cases, this patient serves as a rare example of a progressive type of Poland-Moebius syndrome presumably resulting from a combination of a brainstem abnormality and a peripheral neural degenerative process.

Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland

PURPOSE Mucosa-associated lymphoid tissue lymphoma recently has been defined as a distinct subtype of non-Hodgkins lymphoma with characteristic clinicopathologic features. A 37-year-old woman with systemic lupus erythematosus and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrimal gland is described. METHODS The tumor was totally excised by lateral orbitotomy. Immunohistochemical studies were performed with UCHL-1, CD20 (L26), leukocyte common antigen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. RESULTS The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expressed the CD20 protein and were positive for immunoglobulin kappa light chain. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. CONCLUSION The features of this case support the association between systemic diseases and the subsequent development of mucosa-associated lymphoid tissue lymphoma.

Isolated extraocular muscle involvement as the ophthalmic manifestation of leukaemia

Background:  Clinical and imaging features of patients with orbital leukaemia primarily involving extraocular muscles were evaluated.