Evaluation of high-risk features of primary enucleation of patients with retinoblastoma in a tertiary center of a developing country in the era of intra-arterial chemotherapy.

PURPOSE: To evaluate the frequency of high-risk histopathologic factors in Turkish children enucleated for retinoblastoma and to analyze the association between growth pattern, rosetta formation, tumor thickness, presence of necrosis, calcification, neovascularization, rate of mitosis, and high-risk histopathologic factors. METHODS: Pathology reports of 59 eyes who had received enucleation for retinoblastoma were reviewed retrospectively. The histopathologic data included presence of choroidal invasion, optic nerve invasion, scleral extension, tumor thickness, presence of necrosis, calcification, neovascularization, rosetta formation and lymphocyte infiltration, rate of mitosis, and growth pattern. RESULTS: This study included 59 eyes from 30 (50.8%) male and 29 (49.2%) female patients. The mean age was 22.87 ± 18.99 months. There were 30 (50.8%) eyes with choroidal invasion, 30 (50.8%) eyes with optic nerve invasion, and 5 (8.5%) eyes with scleral invasion. Endophytic growth pattern was seen in 27 (45.8%) eyes, exophytic growth pattern was seen in 2 (3.4%) eyes, and combined growth pattern was seen in 30 (50.8%) eyes. Exophytic growth pattern was found statistically related to choroidal invasion (p = 0.00). Although tumor with greater thickness tended to have more choroidal invasion (p = 0.02), there was no relation between tumor thickness and optic nerve invasion (p = 0.09). CONCLUSIONS: Incidences of choroidal and optic nerve invasion showed similarity with other developing countries. Because of higher incidence of high-risk factors, intra-arterial chemotherapy with its targeted effect should be preferred carefully.

Combined excision, cryotherapy, and intraoperative mitomycin C (EXCRIM) for localized intraepithelial and squamous cell carcinoma of the conjunctiva.

BACKGROUND: To report the results of patients undergoing combined excision, cryotherapy, and intraoperative mitomycin-C (EXCRIM) for primary ocular surface squamous neoplasia (OSSN) METHODS: A retrospective review of a non-comparative interventional case series. Histopathologically confirmed primary localized (less than four clock hours) OSSN treated with EXCRIM using adjuvant 0.02 % mitomycin-C (MMC) were included in the study. The main outcome measures were recurrence and complications related to MMC. RESULTS: The study enrolled 28 eyes of 28 patients with OSSN with a median age of 64.5 (range 43 to 84) years. The mean tumor size was 6.9 × 4.35 mm. There was corneal involvement in 23 of 28 (82 %). Seven patients (21 %) had delayed epithelial healing. Two of eight patients (25 %) with squamous cell carcinoma (SCC) had positive lateral margins. There were no recurrences over a mean follow-up of 49 months (range 24 to 96). CONCLUSION: The excision of OSSN combined with cryotherapy and intraoperative MMC is effective with a low recurrence rate. Long-term follow-up yielded favorable results.

[Malignant rhabdoid tumor of the orbit]. / Orbital Malign Rabdoid Tümör.

Malignant rhabdoid tumor is a rare and malignant tumor of childhood. Generally it originates from the kidney and central nervous system, but occasionally it may arise from the orbit. Differential diagnosis from other childhood soft tissue sarcomas should be done. We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery. The 8 week-old girl was referred to our hospital with a history of right proptosis first noted at birth. Physical and laboratory evaluation of the patient was normal except for right proptosis. The mass was removed surgically. Histopathologic examination and immunohistochemical findings of the specimen were evaluated as malignant rhabdoid tumor. Chemotherapy was administered. While in clinical remission, she succumbed during a febrile episode. Malignant rhabdoid tumor can rarely originate from the orbit. Malignant rhabdoid tumor should be kept in mind in the differential diagnosis of orbital masses, and surgery, chemotherapy and local radiotherapy should be used as combined therapy due to the poor prognosis.

Gamma-knife-based stereotactic radiosurgery for medium- and large-sized posterior uveal melanoma.

BACKGROUND: This study investigated the use of gamma-knife-based stereotactic radiosurgery (GKRS) for medium- and large-sized posterior uveal melanoma treatment. METHODS: We assessed 50 eyes from 50 consecutive patients with uveal melanoma who were treated with GKRS. All tumors met the criteria for medium- and large-sized uveal melanomas. Patients underwent a single session treatment under local anesthesia; the prescribed radiation dose at the tumor periphery was standardized to 30 Gy at the 50 % isodose field for all patients. The main outcomes were local tumor control, eye retention, and survival rates. RESULTS: The median follow-up time was 40 months (16-78 months). The baseline mean tumor diameter was 10.3 mm (7.1-15.7 mm) and the apical tumor height was 8.7 mm (4.1-16.8 mm). After treatment, the mean tumor diameter was 8.7 mm (5.5-12.0 mm) and the tumor height was 6.2 mm (0.5-11.2 mm). Changes in both tumor height and diameter were statistically significant (p < 0.001). The tumor control rate was 90 %, and the eye retention rate was 82 %. A total of nine patients (18 %) developed metastasis, and seven (14 %) died due to metastasis during follow-up. Cataracts (34 %) and radiation maculopathy (30 %) were the most frequent complications, and 14 % of patients developed neovascular glaucoma. Visual acuity (VA) decreased significantly after treatment (p < 0.0001). CONCLUSION: Treatment using low doses of GKRS, (30 Gy) is an eye-sparing outpatient option for patients with medium- or large-sized posterior uveal melanomas who are not eligible for brachytherapy or particle therapy. Complications, particularly impaired VA, should be taken into consideration.

Gamma knife stereotactic radiosurgery yields good long-term outcomes for low-volume uveal melanomas without intraocular complications.

We present the outcomes of 35 uveal melanoma patients treated with gamma knife stereotactic radiosurgery. All cases were previously untreated. During follow-up, regular MRI examinations were used to detect any changes in tumor size and estimate the local long-term tumor control rate. Treatment-related complications were also recorded. During follow-up, systemic dissemination was observed in two patients, one of whom died of metastases. The most frequent complication was retinal detachment (17.1%). Three patients required enucleation. Cumulative 1-year and 3-year local tumor growth control rates were 97% and 83%, respectively. The mean and median times to local tumor progression were 48.0 and 51.7 months, respectively. Gamma knife surgery may be a suitable alternative for the treatment of low-volume uveal tumors without intraocular complications, as the control rate and long-term outcomes compare favorably with those of surgical excision and brachytherapy.

Retinoblastoma in Turkey: survival and clinical characteristics 1981-2004.

BACKGROUND: In this study, the authors aim to describe the survival and clinical characteristics of 141 retinoblastoma cases treated at Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, between 1981 and 2004. METHOD: The authors retrospectively analyzed the clinical records of 141 children (177 eyes) diagnosed with retinoblastoma and treated between 1981 and 2004. Information on gender, laterality, age at diagnosis, presenting signs, spread of tumor, treatment modality, survival rate, and family history were collected. RESULTS: A total of 105 cases (74.5%) were unilateral and 36 cases (25.5%) were bilateral. The mean age overall at the time of diagnosis was 25 months; in unilateral cases, 29 months; and in bilateral cases, 16 months. The most common presenting signs were leukocoria (116 cases, 82%), strabismus (14 cases, 10%) and proptosis (11 cases, 8%). A total of 28 cases had orbital extension, nine patients had central nervous system invasion, and five cases exhibited bone marrow involvement. In total, 16 patients (11%) had a family history of retinoblastoma. One case developed a secondary neoplasm. The 3 year cumulative survival rate of 141 patients was 89.69% (unilateral, 90.74%; bilateral 87.35% P = 0.9371, P > 0.05, log rank test). CONCLUSION: The study's survival rate was similar to developed countries. The success in higher survival rates is based on the authors multidisciplinary team approach done by the same group and the support of the authors' clinic and government in sponsoring the medical insurance of all patients.

Three-wall orbital decompression superiority to 2-wall orbital decompression in thyroid-associated ophthalmopathy.

PURPOSE: We evaluated the results of the 2-wall and 3-wall orbital decompression in patients with Graves' disease. PATIENTS AND METHODS: In this study, we present a consecutive series of 12 patients (18 orbits) who were submitted to orbital decompression by endoscopic transnasal medial wall combined with transantral inferior wall approach and 7 patients (8 orbits) who were submitted to orbital decompression by endoscopic transnasal medial wall, transantral inferior wall combined with transcutaneous lateral wall approach. The degree of exophthalmos was evaluated with the Hertel exophthalmometer preoperatively and postoperatively in the 24th hour, and first, third, and ninth months. RESULTS: At the end of the third month, the exophthalmos decreased by a mean of 4.38 mm (range, 3 to 7 mm) with the 2-wall decompression and 7.75 mm (range, 5 to 12 mm) with the 3-wall decompression. Visual acuity maintained or improved during the follow-up period. Ocular motility disturbance occurred in 1 patient after 2-wall decompression and in 1 patient after 3-wall orbital decompression. Postoperatively, new-onset diplopia was seen in only 1 patient after 2-wall orbital decompression. CONCLUSION: The 3-wall (endoscopic transnasal medial wall, transantral inferior wall combined with transcutaneous lateral wall) approach is as safe as the 2-wall approach. Proptosis reduction is much better with the 3-wall orbital decompression.

Immunohistochemical analysis of orbital connective tissue specimens of patients with active Graves ophthalmopathy.

PURPOSE: To explore the immune mechanism of Graves ophthalmopathy (GO) by analyzing infiltrating cells in orbital connective tissue (OCT) specimens of patients with active GO using immunohistochemical methods. METHODS: Five OCT specimens obtained from patients with active GO and five control specimens obtained from forensic cadavers who died from nonmedical reasons were stained with anti-CD3, CD4, CD8, CD45RO, HLA-Dr, CD25, and TNF-alpha monoclonal antibodies. Positively stained cells were counted and results were interpreted as cell counts/mm2. Four of five GO patients had never been treated with any immunomodulating therapy. Only one had received oral prednisolone prior to tissue sampling, but this treatment had ceased 5 months before surgery. RESULTS: The retro-orbital tissue specimens obtained from forensic cadavers did not show any significant positive staining for any monoclonal antibody tested. However, the specimens from GO patients showed positively stained means of 36.66 +/- 4.61 HLA-Dr+, 12.8 +/- 3.42 CD8+, 11.8 +/- 1.78 CD4+, 16.6 +/- 1.81 CD3+, 21.2 +/- 3.12 CD45RO+, 10.4 +/- 2.07 TNF-alpha+, 7.2 +/- 1.48 CD25+, 3.2 +/- 1.09 CD4+CD8+, 4.6 +/- 1.67 CD4+CD45RO+, 2.8 +/- 0.83 CD8+CD45RO+, 1.6 +/- 0.89 CD4+CD25+, and 1.8 +/- 1 0.83 CD8+CD25+ cells/mm2. CONCLUSIONS: Our study supports that most of the infiltrating lymphocytic cells in the active stage of GO are T cells, and a significant proportion of them are CD45RO+ cells. Infiltration of OCT by HLA-Dr+, CD25+, and TNF-alpha cells suggests that Th1-type immune reaction with the interference of proinflammatory cytokine(s) (TNF-alpha) may be important in the pathogenesis of disease. Further studies are needed to understand the disease pathogenesis and may provide a scientific basis for future treatment alternatives for the disease (e.g., anti-cytokine treatment).

Early surgery for dysthyroid orbitomyopathy based on magnetic resonance imaging findings.

PURPOSE: To investigate the value of magnetic resonance imaging (MRI) in the determination of the fibrotic phase in dysthyroid orbitopathy, which may be an indication for early surgery. PATIENTS AND METHODS: Thirteen patients with dysthyroid orbitopathy who had vertical deviation in excess of 20 PD and associated diplopia in the primary position that did not respond to medical treatment were included in the study. On MRI, the absence of high-intensity foci in the T2 sequence, especially in the coronal planes, indicated that the extraocular muscles were not edematous. These muscles were evaluated as being in the fibrotic phase, and surgery was performed without waiting the recommended 6 months for the stabilization of the angle of deviation. RESULTS: The complaint of diplopia disappeared after a single operation in 8 patients, and after a second operation in 4. Thus, success was obtained in 12 of the 13 patients. One patient with persistent vertical diplopia refused a second operation. CONCLUSION: Our results indicate that MRI is an effective and reliable method for the determination of the fibrotic phase in patients with dysthyroid orbitopathy who have diplopia in the primary position, and that MRI results have an important role in determining whether early surgery is appropriate.

[Orbital decompression by endoscopic transnasal and transantral approach in thyroid-associated ophthalmopathy]. / Tiroit oftalmopatisinde transantral ve endoskopik transnazal yolla orbital dekompresyon.

OBJECTIVES: We evaluated the results of orbital decompression in patients with Graves' disease. PATIENTS AND METHODS: Orbital decompression was performed by endoscopic transnasal medial wall approach and transantral inferior wall approach in 18 eyes of 12 patients (8 males, 4 females; mean age 41 years; range 24 to 65 years). The degree of exophthalmos was evaluated with the use of the Hertel exophthalmometer preoperatively, and postoperatively in the 24th hour, first and third months. RESULTS: At the end of the third month, exophthalmos decreased by a mean of 4.4 mm (range 3 to 7 mm). Visual acuity and field defects improved considerably in all patients. Complications occurred in three cases (25%) including diplopia, displacement of one eye inferiorly, and maxillary sinusitis caused by the encroachment of the adipose tissue upon the ostium, respectively. CONCLUSION: With the increasing ease of surgical procedures and enhanced experience on the part of surgeons, successful treatment of exophthalmos has become possible with decreased complication rates.

Extraocular muscle surgery in dysthyroid orbitomyopathy: influence of previous conditions on surgical results.

PURPOSE: To determine the influence of previous treatments other than the surgical treatment of restrictive myopathy resulting from dysthyroid orbitomyopathy. METHODS: The outcome of 23 cases with dysthyroid orbitomyopathy was evaluated after extraocular muscle surgery. RESULTS: It has been observed that the duration of the orbitomyopathy, severity of myopathy of the deviation angle, and the modality of treatment prior to surgery (radiotherapy and corticotherapy) did not influence surgical outcome. Binocular single vision was achieved in primary and reading positions in 14 of 23 cases (60.9%) after surgery while prisms were required to achieve the same result in 5 cases (21.7%). CONCLUSION: It is concluded that previous treatment does not influence the results of extraocular muscle surgery in patients with dysthyroid orbitomyopathy.