A new insight into retinal vein occlusion pathogenesis.

AIM: To evaluate (ex vivo) the characteristic of fibrin clotting in patients with retinal vein occlusion. MATERIAL AND METHODS: Fifty nine patients with a history of retinal vein occlusion were enrolled in the study. The diagnosis of retinal vein occlusion was based on the typical fundus appearance, supplemented by digital photography, fluorescein angiography, and optical coherence tomography. The control group consisted of 59 subjects matched for age, sex, body mass index, medications, and cardiovascular risk factors. The ex vivo fibrin clots obtained from citrate plasma samples from all patients were used for the measurement of clot permeation, expressed as the permeability coefficient, Ks (Darcy constant). The turbidity of fibrin clot formation, reflected by the "lag phase" of the turbidity curve and maximum absorbance at plateau (deltaAb(max)), tissue-plasminogen activator (t-PA) - induced fibrinolysis characterized by maximum rates of increase in D-dimer levels (D-Drate) and maximum D-dimer concentrations (D-Dmax) were evaluated. The time required for 50% decrease in maximum clot absorption (t50%) was chosen as an additional marker of clot susceptibility to fibrinolysis. RESULTS: Patients with retinal vein occlusion were characterized by the unfavourable plasma fibrin clot properties. Clot permeability was 30% lower, as compared to the controls (p < 0.0001), the "lag phase" was 11% shorter (p < 0.0001) indicating faster fibrin formation, and the deltaAb(max) was 19% higher (p < 0.0001), indicating thicker fibrin fibers. The D-Dmax indicating thrombotic mass available for fibrinolytic agents was 22% higher in the RVO group (p < 0.0001) and the t50% was 29% longer (p < 0.0001) compared with controls. Only the D-Drate was similar in both groups (p = 0.223). The differences remained statistically significant after adjustment for fibrinogen, glucose, and platelet count. CONCLUSION: The results indicate that in patients with retinal vein occlusion, less porous plasma fibrin clots composed of thicker fibrils with the reduced permeability and susceptibility to lysis are found, as compared to controls. Plasma fibrinogen and C-reactive protein levels are recognized as the most important modulators of fibrin function. retinal vein occlusion, pathogenesis.

[Proton beam radiotherapy of uveal melanoma--preliminary results]. / Radioterapia protonowa czerniaka blony naczyniowej--wstepne wyniki leczenia.

PURPOSE: Presentation of the proton beam radiotherapy (PBR) as a method of treatment of the uveal melanoma patients in the Department of Ophthalmology and Ocular Oncology and Nuclear Physics Institute in Cracow, Poland and their preliminary results. MATERIAL AND METHODS: Nine patients with only choroidal melanoma were treated using PBR between January and April 2011. There were 4 women (44%) and 5 men (56%), a mean age 56 years (38-72). The mean tumor thickness using ultrasounds was 4.14 mm (1.4-9.6 mm). RESULTS: The mean follow-up was 6 months (5-7). The mean tumor thickness after PBR was 2.47 mm (0-9.3 mm). In 2 patients endoresection of irradiated tumor mass was performed with final flat scar. After exclusion of these patients from analysis, the mean thickness was 3.17 mm (1.5-9.3 mm). In 6 patients the visual function was stable. CONCLUSIONS: The preliminary results show that PBR is highly precise method of uveal melanoma treatment achieving high rates of local control. This method necessitates a close co-operation between ophthalmologist, oncologist, radiotherapists, and medical physicist as well as an elaboration of own procedures of planning and treatment. uveal melanoma, radiotherapy, proton-beam radiotherapy.

[Transpupillary thermotherapy (TTT) for intraocular metastases in choroid]. / Przezzreniczna termoterapia (TTT) w leczeniu nowotworów przerzutowych rosnacych w naczyniówce.

UNLABELLED: Uveal metastases are the most common intraocular malignant tumors. Most patients who develop metastatic carcinoma to the choroid are managed by radiation or chemotherapy. Since TTT is an optional treatment for choroidal melanomas and hemangiomas, we ought to determine whether TIT is suitable for treatment of solitary choroidal metastasis at the posterior pole. PURPOSE: To evaluate effectivenes of TTT treatment for intraocular metastases. MATERIAL AND METHODS: 45 patients (59 eyes) with intraocular metastases were treated in the Ophthalmological Department of Jagiellonian University in Kraków. There were 30 women and 15 man, at the age 31-84 years (av. 57.5). The primary tumor was a breast cancer in 22 women, lung cancer in 5 men and 2 women, kidney (3), colon (2), uterus (1), larynx (1), testicle (1), esophagus (1). 10 patients had also metastases in other organs. TTT was performed in all treated eyes, in 11 combined with 106Ru brachytherapy (BT). Chemotherapy as adjuvant treatment was performed in 18 patients. The results of treatment were evaluated in mean 14.5 months (1-61) follow-up. RESULTS: After TTT, tumor shrinking was observed in most treated tumors (in 37 eyes, 62.7%), inhibition of tumor growth in 5 (8.4%), and in 4 cases (6.7%) progression of tumor growth. In 3 cases (5.1%) with flat scar and intraocular tumor shrinking, extrascleral extension located close to the tumor base appeared after treatment (TTT in 2 eyes, TTT combined with BT in one case). CONCLUSIONS: TTT is an effective treatment method for small choroidal metastases located in the posterior pole. TTT combined with 106Ru brachytherapy can be useful treatment in medium sized choroidal metastases. Efficient results encourage further application of thermotherapy in the treatment of intraocular metastases. It allows for the conservative treatment of the eyeball and also useful visual acuity. Extraocular extension appearing after TT needs further study.

[Malherbe's calcifying epithelioma (pilomatrixoma)--own experience]. / Nabloniak wapniejacy Malherbe'a (pilomatrixoma)--obserwacje wlasne.

PURPOSE: Malherbe's calcifying epithelioma is a rare hair matrix tumor. The objective is to present clinical cases and to analyze the variability of its image. MATERIAL AND METHODS: Among nearly 3500 patients with ocular tumors operated during the last 10 years in the Department of Ophthalmology and Ocular Oncology in Krakow we identified a group of 7 patients with histologically confirmed pilomatrixoma. A retrospective evaluation of their medical history was performed, paying particular attention to the co-existence of other diseases, form of growth and location of tumors, as well as treatment methods and their effectiveness. The results were compared with other clinical studies. RESULTS: Malherbe's calcifying epithelioma is characterized by clinical polymorphism and similarity to other much more frequent eyelid tumors. It usually has the form of a single, small, movable, slowly growing nodule covered by intact skin. In addition to the classic form described there are pediculated tumors, huge, fast-growing, perforating, as well as tumors in unusual locations and multiple lesions. CONCLUSIONS: The consequences of variation pilomatrixoma are clinical diagnostic errors. The one, sure and reliable criterion is a histopathology evaluation.

[Malherbe's calcifying epithelioma (pilomatrixoma)]. / Nabloniak wapniejacy Malherbe'a (pilomatrixoma).

UNLABELLED: Malherbe's calcifying epithelioma (pilomatrixoma)--is a rare, benign tumor of hair matrix. PURPOSE: Of this study is to present the clinical variability of epithelioma, pathomorfological appearance of the tumor and the views on its pathogenesis. RESULTS: Malherbe's calcifying epithelioma has a very different, sometimes unusual appearance, similar to other, more frequent laesions. Due to this fact and the rarity of the tumor it is sometimes overlooked in diagnostic considerations, or confused with more common tumors. Genetic analysis at this stage, allows to establish a probable causal relationship between mutations in the beta-catenins coding gene and development of pilomatrixoma. Extremely rare epithelioma may undergo transformation into pilomatrix carcinoma. CONCLUSIONS: The only reliable criterion of diagnosis remains pathological evaluation which despite the deceptive macroscopic appearance of the tumor provides correct diagnosis.

A case of choroidal metastasis in a male breast cancer.

PURPOSE: We present a case of male patient under therapy due to breast cancer with choroid metastasis and exudative retinal detachment in right eye. MATERIAL AND METHODS: A 59 years old male patient was referred with diagnosis of intraocular tumor in right eye Three years ago radical right-side mastectomy was performed (T2N1M0). The medical history revealed that the patient was under chemo- and hormontherapy due to right breast cancer with lungs, liver and mediastinum metastases. Based on the ophthalmological examination the diagnosis of intraocular tumor was established--choroidal metastasis with exudative retinal detachment. Due to tumor size and general dissemination external beam irradiation of tumor was performed. CONCLUSIONS: The majority of choroidal metastases in males are secondary to lung carcinoma, however in differential diagnosis it is necessary to consider also rare cases of breast carcinoma metastases. Treatment strategy should be considered individual depending on size, localization, presence of metastases to other organs and general condition of the patient.

[Mesectodermal leiomyoma of the ciliary body--case report]. / Mezektodermalny miesniak gladkokomórkowy--opis przypadku.

PURPOSE: The aim of this report is to present a case of mesectodermal leiomyoma of the ciliary body. MATERIAL AND METHODS: In the left eye of the 15 years old female patient presenting 2 months history of deterioration of visual acuity a medium-pigmented, dome-shaped tumor at the inferotemporal side was detected. It measured 21.6 mm in base diameter 11.2 mm in thickness and was revealed with imaging techniques such as ultrasound, MRI and orbital radiography. Taking into consideration all clinical features, despite patients' young age, malignant melanoma of the ciliary body was suspected. Due to the size of the tumor and the impossibility of conducting conservative treatment the globe was eventually enucleated. RESULTS: The final diagnosis was established on the basis of histopathological microscopic examination and immunohistochemical stains. The co-expression of muscular (SMA, caldesmon, desmin), as well as neural markers (S-100 protein and synaptophysin), has been proved. CONCLUSIONS: Mesectodermal leiomyoma of the ciliary body is an extremely rare benign tumor, which originates from neural crests. It exhibits features of both muscular and neural differentiation.

Lacrimal caruncle nevus associated with Rubinstein-Taybi syndrome.

We present a 28-year-old man with diagnosed Rubinstein-Taybi syndrome (RSTS), known as "Broad thumb-Hallux syndrome" with co-existing lacrimal caruncle tumor. Because of the documented enlargement of the lacrimal caruncle mass and known increased risk to develop malignancies in RSTS patients we decided to perform excisional biopsy, which revealed caruncle nevus. To our knowledge this is the first description of such an association.

[Retinoblastoma update--management and classification (Part I)]. / Siatkówczak--aktualne zalecenia dotyczace postepowania i klasyfikacji (Czesc I).

The aim of this paper is to present updated data on diagnosis, management and classification of retinoblastoma. The authors describe the growth patterns of retinoblastoma including the diffuse infiltrating type, the features of sponatneously regressed and sponatneously arrested retinoblastoma. In addition, retinoblastoma genetics including 13 q deletion syndrome, are discussed. Finally, they present the International Intraocular Retinoblastoma Classification (IIRC) and make recommendations regarding chemotherapy after enucleation based on histopathology findings.

[Retinoblastoma update--therapy (Part II)]. / Siatkówczak--aktualne zalecenia dotyczace leczenia (Czesc II).

The aim of this paper is to present updated data on the treatment of retinoblastoma. The authors describe current management based on the International Intraocular Retinoblastoma Classification (IIRC). They discuss the different methods of treatment, including chemoreduction, subconjunctival carboplatin, transpupillar thermotherapy, cryotherapy, laser photocoagulation, episcleral plaque radiotherapy, external-beem radiotherapy and enucleation.

Uveal melanoma in young patients.

INTRODUCTION: This study aimed to investigate the clinical profile of young patients with uveal melanoma treated in 1995-2002 in the Department of Ophthalmology, Jagiellonian University, Krakow, Poland. METHODS: Retrospective case-control series. RESULTS: Out of 800 patients with uveal melanoma treated between 1995 and 2002, there were 11 patients (1.4%) aged 20 or younger. The mean age of those patients was 17.9 (12-20, median 19) years. There were five men (45%) and six women (55%). In all cases, the tumor was unilateral. In five (45%) cases the tumor affected the right eye and in six (55%) cases the left eye. Eight (72%) patients were treated with local resection. Among these, eight patients, in two (18%) with iris or ciliary body melanoma we used adjuvant Ru-106 episcleral plaque brachytherapy (EPB). In two (18%) cases of choroidal melanoma, we performed I-125 EPB: one case ended with regression of the tumor, while the second did not respond to EPB and we had to enucleate the eye. In one (9%) case we performed enucleation as the primary treatment. The mean follow-up was 61.4 months, ranging 30-125 months. All those patients are still alive and no metastases were detected. CONCLUSIONS: Uveal melanoma is a rare malignancy in children and teenagers. In the present series, it occurred in the iris in more than 50% of cases.

[Radiation optic neuropathy after the brachytherapy of the uveal melanoma]. / Neuropatia popromienna po brachyterapii czerniaka naczyniówki.

PURPOSE: The evaluate the frequency of radiation of the optic neuropathy (RON) and its influence on visual acuity in patients with choroidal melanoma treated by brachytherapy. MATERIAL AND METHODS: We analyzed 583 patients with choroidal melanoma, treated with ruthenium (106Ru) or iodine (125I) plaques in Ophthalmology Department of Jagiellonian University, between 1996 and 2004 year. The frequency of RON, its influence on loss of vision, according to a kind of the used isotope, tumor size and location were analyzed. Diagnosis of RON was established on the base of characteristic symptoms in the eye fundus. RESULTS: The frequency of RON was significantly higher in cases treated by 125I vs 106Ru (14% vs 8.2%, p = 0.0014), in cases of tumors located in the posterior pole (31.3%, p = 0.012), and in cases of tumor thickness 3-8 mm (27.5%, p = 0.033). In most of patients we observed major deterioration of visual acuity. CONCLUSIONS: The radiation of the optic neuropathy was seen often after iodine BT, tumors located in the posterior pole of fundus, with thickness 3-8 mm.

[The analysis of I125 brachytherapy complications in cases of uveal melanoma]. / Analiza powiklan brachyterapii 125I w czerniakach naczyniówki.

PURPOSE: Analysis of type and frequency of complications after brachytherapy 1125 between 1-12 months after treatment and during the following controls. MATERIALS AND METHODS: 147 persons (76 men and 71 women) aged 23-86 years (mean 57 years), were treated in our clinic in 1998-2001. In 43% of the cases the thickness of the tumor was more than 8 mm and in 94% the maximum diameter was bigger than 8.1 mm. The irradiation dose was 80-120 Gy, mean 100 Gy. RESULTS: Regression and stabilization of the tumor was achieved in 84.3% of all cases. Complications of different type and intensity associated with brachytherapy I125 were observed in majority of the cases. The complications observed were: radiation retinopathy (52%), radiation maculopathy (33.6%), radiation optic neuropathy (25.3%), vitreous hemorrhage (8.8%), glaucoma (20.5%) and cataract (18.5%). CONCLUSIONS: 125I brachytherapy yields good results in cases of uveal melanoma, however it's associated with post radiation complications.

[Optic disc melanocytoma]. / Melanocytoma tarczy nerwu wzrokowego.

PURPOSE: Presentation of the cases of melanocytoma observed in the latest time in our clinic. MATERIAL AND METHODS: We present 2 cases - one in a 41 years old women S.M. and in a 77 years old women P.J. CONCLUSIONS: In case of melanocytoma there should be performed specialistic ancillary tests and the patients should be observed with photographic documentation.

[Epidemiology of choroidal melanoma in the material of the Department of Ophthalmology CM UJ in Cracow]. / Epidemiologia czerniaka naczyniówki w materiale Kliniki Okulistyki CM UJ w Krakowie.

PURPOSE: Epidemiological analysis of the choroidal melanoma cases-sent from all over the Poland and treated in our clinic between January 1998 and December 2002. MATERIAL AND METHODS: The study included 687 patients with choroidal melanoma 348 women and 339 men. RESULTS: The age of patients was 14-97 years (mean 56 years). The most numerous group consisted of patients between 60-70 years of age (22.7%). Most of the cases came from the regions of Malopolska, Silesia, Lower-Silesia and Mazovia. The time between first symptoms and the first visit in our clinic was between 1 week to 2 years. According to the COMS classification we found small melanoma in 13% of cases, medium in 51% and large in 36% of cases. We found the presence of other malignancies besides the choroidal melanoma in 4.3% patients. We analyzed the demography and positive family history of ocular and general malignancies in the patients.