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Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht.

Whenever funduscopy reveals possible choroidal melanoma, all available information must be gathered to either confirm or exclude the diagnosis. Well-defined funduscopic criteria are available, which can already lead to a high degree of diagnostic certainty. Additional technical examinations can be used to exclude possible differential diagnoses. In cases where no clear diagnosis can be established, it is possible to take a biopsy or to watch and wait in order to observe possible growth. Whenever the diagnosis of a choroidal melanoma is established, cancer staging has to be performed in order to search for possible metastases., Competing Interests: Nein., (Georg Thieme Verlag KG Stuttgart · New York.)

Despite the diversity of techniques for documentation and diagnostics of choroidal nevi, the differential diagnosis, especially regarding small uveal melanomas, remains difficult. In many cases, frequent controls to exclude growth or for the detection of an unchanged appearance are recommended. This article reviews-under consideration of the actual literature-different diagnostic techniques for documentation and differentiation of choroidal nevi., Competing Interests: Die Autoren haben keine finanziellen Interessenkonflikte., (Georg Thieme Verlag KG Stuttgart · New York.)

We present the case of a 38-year-old Asian patient who reported vision loss of her left eye since 4 weeks. The funduscopy showed a choroidal tumor in the papillomacular bundle, which could be identified as a choroidal osteoma with secondary choroidal neovascularization (CNV). OCT angiography (OCT-A) detected abnormal flow in the choriocapillaris; the osteoma showed no flow in the OCT angiogram of the choroid level. Therefore, OCT-A can be a helpful adjuvant for diagnosis of CNV secondary to choroidal osteoma.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Background: Case report of a 6-year-old boy with bilateral choroidal osteoma., Case Report: The patient was followed up for the next 6 years and during this period bilateral tumor progression was observed. In one eye a staphyloma posticum developed in the area of the osteoma which led to secondary anisometric myopia., Conclusion: If undetected, this rare complication can lead to anisometric amblyopia; therefore, repeated cycloplegic refractometry is advisable in children with choroidal osteoma.

Choroidal lymphoma is a rare disease and can be classified into primary and secondary choroidal lymphomas. Primary choroidal lymphoma is a low-grade extranodal marginal zone B-cell lymphoma and secondary choroidal lymphomas present ocular manifestations of disseminated systemic lymphomas. Typical clinical features of choroidal lymphoma are multifocal, yellow-whitish choroidal infiltrates. The vitreous body is usually clear and cell-free. Choroidal lymphoma has a tendency to extend through the sclera. In contrast to primary choroidal lymphoma, which is more often unilateral, does not show signs of anterior segment involvement and has a slow progression, secondary choroidal lymphoma is more often bilateral, has a rapidly progressive course with anterior segment and vitreous involvement and belongs to the high-grade lymphomas. The definitive diagnosis of choroidal lymphoma can only be confirmed by histopathological examination of biopsy tissue. The choroidal biopsy is the gold standard in the diagnostics of choroidal lymphoma. To date, no standardized treatment for choroidal lymphoma has been established. The treatment modalities include external beam radiotherapy, immunotherapy with rituximab and chemotherapy. The prognosis for survival of primary choroidal lymphoma is usually good. The prognosis of secondary choroidal lymphoma depends on the malignancy grade of systemic lymphoma.

Choroidal metastasis represents the most common type of intraocular malignancy and preferably involves the posterior uveal tract. Breast and lung cancer - known or so far undiagnosed - are most frequently identified as the underlying tumor disease. The majority of patients diagnosed with uveal metastasis have additional metastatic manifestations elsewhere, so re-staging before treatment is recommended. The importance of a multidisciplinary approach is obvious. Early diagnosis and timely initiation of treatment are mandatory in case of vision-threatening situations. External beam radiation remains the therapy of choice. Overall survival of patients with uveal metastasis is limited, averaging six to twelve months. The other eye is frequently enough affected as well, justifying regular ophthalmologic follow-up during the further course of the disease.

Background: Prognosis evaluation of patients with choroidal and ciliary melanoma has experienced recent progress through tumour sampling and cytogenetic analysis of metastatic risk. By allocating tumor extension, height and linear basal diameter to defined TNM stages, an estimation of prognosis can also be made without invasive tissue sampling., Methods: Therapeutic strategies of organ preserving irradiation using different sources have clearly come to the forefront., Results: Due to microscopic haematogenous spreading of tumour cells prior to treatment, the metastatic risk following radiation of any form is not influenced in comparison to primary enucleation., Conclusion: However, metastatic disease still remains a fatal condition which currently may only be influenced by early detection and treatment of uveal melanomas., (Georg Thieme Verlag KG Stuttgart · New York.)

Choroidal osteoma is a rare benign osseous tumor of the choroid usually affecting young healthy women. The diagnosis can be confirmed by the clinical appearance and with use of fluorescein or indocyanine angiography, optical coherence tomography, computed tomography or magnetic resonance imaging. A direct therapy of the tumor is indicated only for a few cases. The main reasons for vision loss are choroidal neovascularization, subretinal fluid and changes in the photoreceptor layer or the retinal pigment epithelium. Choroidal neovascularization and subretinal fluid in particular can be treated with laser therapy, photodynamic therapy or vascular endothelial growth factor inhibitors.

We present the case of a 41-year-old female patient with acute onset of nyctalopia, xanthopsia and impairment of visual field. The ophthalmologic examination revealed peripheral choroidal elevation with accompanying retinal detachment. Due to the the presence of a nanophthalmic eye without any evidence of inflammation or malignant growth, the diagnosis uveal effusion syndrome could be made.

Immunohistochemical evaluation of primary and secondary (adeno-) carcinomas of the lung often includes utilisation of two different clones (8G7G3/1 or SPT24) of TTF-1 (thyroid transcription factor 1) antibodies. In a subgroup of adenocarcinomas with a primary site other than the lung a positive reaction of clone SPT24 and also of clone 8G7G3/1 is described. We report on a patient with TTF-1 (clone 8G7G3/1) positive adenocarcinoma of the colon with metastases to the eye and lung and discuss TTF-1 based diagnostic considerations.

The choroidal osteoma is a typically unilateral, benign ossifying tumor of the choroid. It is mostly located in the juxtapapillary area and usually found in females in the second or third decade of life. Most patients are asymptomatic. However, some can present with blurred vision, metamorphopsia, and visual field defects. Ultrasonography can show a calcified choroidal plaque with a typical shadowing posterior to the lesion. Fluorescein angiography and optical coherence tomography should be used if choroidal neovascularization is suspected as a possible complication of the choroidal osteoma.

Background: The aim of this study was to evaluate the long-term results of transpupillary thermotherapy (TTT) for small malignant choroidal melanomas., Patients and Methods: In the time period 1/1998 to 10/1999 in a prospective non-randomised analysis 26 eyes with small malignant melanomas (located posterior to the equator with base ≤ 12 and thickness ≤ 4.5 mm) were primary treated with the TTT standard protocol (follow-up over a time span of at least 10 years)., Results: Thirteen women and thirteen men (mean age: 64 years) underwent TTT. The mean preoperative tumour thickness was 2.45 mm (0.8 - 4.5 mm). Ten years postoperatively tumour regression without recurrence after 1.4 treatment sessions (mean) was achieved in 16 / 26 eyes, primary regression followed by tumour regrowth in 6 / 26 eyes, and primary failure of tumour regression in 4 / 26 eyes. Two patients died on liver metastasis. Ocular complications (with preference in posterior tumours after multiple TTT sessions) were observed in 14 eyes: macular pucker in 8, macular oedema in 6, choroidal neovascularisation in 4 and posterior synechia with iris atrophy in one eye., Conclusions: Choroidal melanomas treated with TTT as a stand-alone procedure need a close monitoring since these tumours developed a significant rate of local recurrences and ocular side-effects in the long run., (© Georg Thieme Verlag KG Stuttgart · New York.)

A solitary synchronous distant metastasis of newly diagnosed non-small cell lung cancer (NSCLC) occurs in only a small number of patients. The available literature suggests a significant rise in long-term survival for these patients when resection of the bronchial carcinoma and the metastasis are feasible and performed. To demonstrate a multidisciplinary concept, we present the case of a patient with a peripheral NSCLC and a solitary synchronous intraocular metastasis. To our knowledge, this concept with curative intention has not been published before regarding a patient with an intraocular metastasis.

Purpose: The aim of this study was to evaluate tumour regression in a large series of choroidal melanomas, which were treated with three different eye-sparing treatment modalities., Patients and Methods: Retrospective review of the treatment results in all eyes with malignant choroidal melanoma, which were treated in the University Eye Clinic of Mainz consecutively in the time span 1.1992 to 12.2000 with transpupillary thermotherapy (TTT, standard protocol Oosterhuis JA 1995), ruthenium brachytherapy (RB, tumor apex dose 150 Gy) or sandwich therapy (ST). One-step ST was defined as TTT followed by RB with 100 Gy tumor apex dose within 48 hours. The treatment of residual prominences with TTT secondary to RB after different time spans was called two-step ST. Follow-up was 2 years., Results: 131 eyes with malignant choroidal melanoma (mean tumour thickness: 4.5 mm) were treated with RB (66 eyes), TTT (26 eyes) or ST (39 eyes). Preservation of the globe was achieved in 109 eyes (81 %). Local tumour control was found in small melanomas (prominence up to 3 mm) in 89 %, in large tumors (prominence 8 mm and higher) in 50 %. In a subgroup of small posterior melanomas (n = 70 eyes, prominence up to 4.5 mm, located posterior to the equator) local tumour control was noted in 91 %. The time span to reach local tumour control (Kaplan-Meier estimates) was the shortest after TTT (median: 20 weeks), compared with RB (48 weeks) and one-step ST (29 weeks)., Conclusions: In choroidal melanomas the chance of local tumour control and preservation of the globe decreases with increase of the tumour prominence. In small choroidal melanomas with posterior location local tumour control was achieved significantly faster after TTT than after RB., (Copyright Georg Thieme Verlag KG Stuttgart . New York.)

We report the case of a 31-year-old patient who presented with unilateral acute loss of vision, severe headache, and symptoms of an acute angle-closure glaucoma. Surprisingly, a prepapillary uveal melanoma was the underlying reason; that is, a masquerade syndrome was seen. Enucleation and histopathological investigation of that eye were performed within 3 days after first presentation. Acute angle-closure glaucoma as the initial presentation of uveal melanoma is rare. Nevertheless, clinicians should be aware that patients with refractory unilateral angle-closure glaucoma and asymmetrically opaque media may harbor an occult uveal melanoma.

The treatment of large uveal melanomas poses a therapeutic challenge, due to the expected treatment-related side-effects. After sole radiotherapy the majority of patients are faced with radiogenic complications secondary to the large amount of tumour necrosis. Alternative treatment modalities addressing this issue are transscleral resection in arterial hypotension in anteriorly located tumours and endoresection via pars plana vitrectomy in posteriorly located tumours. A surgical resection treatment was applied in 292 patients with large uveal melanomas. In 150 patients the tumour was treated by transscleral resection and postoperative adjuvant (106)ruthenium brachytherapy and 142 patients were treated by primary proton beam irradiation and secondary endoresection. The mean follow-up time was 3.8 and 2.5 years, respectively. Local tumour control was achieved in 76 % and 98 %, respectively. The 5-year metastatic rates were 28 % and 21 % and eye retention was achieved in 82 % and 97 %, respectively. Surgical resection of uveal melanomas with adjuvant radiotherapy is the treatment of choice in cases of large tumours, avoiding enucleation in the vast majority of cases in the long term, without increasing the incidence of tumour-related risks., ((c) Georg Thieme Verlag KG Stuttgart-New York.)

Background: Today photodynamic therapy (PDT) with Verteporfin is the standard treatment option for symptomatic choroidal haemangiomas. Nevertheless, peripapillary or large haemangiomas pose a therapeutic dilemma, since severe adverse events have been demonstrated in cases where the optic disc was included in the treatment spot. In addition due to the size and the shape of the laser spot, peripapillary and large haemangiomas can only be undertreated or overtreated (with overlapping multiple spots). With the presented new "paint-brush technique" it is possible to treat peripapillary and large choroidal haemangiomas completely without danger for the optic disc and without overtreatment., Material and Methods: With the new technique the application of the laser spot is performed in a "paint-brush fashion" by moving he spot with constant speed excentrically around the lesion's centre over the entire tumour surface. This allows under visual control a complete and confluent PDT of the whole tumour surface without overlapping and/or missing areas or treating the optic disc. In total, 13 eyes of 13 patients (6 with peripapillary and 7 with large choroidal haemangiomas) have been treated. The mean follow-up time was 25.6, respectively, 14.5 months., Results: The PDT with the "paint-brush technique" allows an effective treatment of the leakage in both groups. The mean increase of visual acuity was 1, respectively, 1.4 ETDRS lines 3 months after therapy, which could be maintained over the whole follow-up period. At the last examination visual acuity was unchanged or better in 12 / 13 (92 %) of eyes. After 3 months the tumour height was significantly (p = 0.031 and 0.018) reduced to 1.7 mm (52 %), respectively, 1.3 mm (30 %), which was stable during the whole follow-up. No significant side effects could be detected., Conclusion: The use of the PDT "paint-brush technique" allows complete treatment of choroidal haemangiomas under visual control. Sensitive structures like the optic disc could be excluded from treatment without double- or undertreatment. The functional and anatomic results are good. The technique is a safe and effective amendment in the treatment of symptomatic choroidal haemangiomas., ((c) Georg Thieme Verlag KG Stuttgart-New York.)

Bone metastases are found in 29% of patients with metastatic malignant choroidal melanoma, which is associated with poor prognosis. However there are several reports about prolonged survival. The unusual case of a patient is described, who suffered from a melanoma with orbital invasion and survived more than 18 years. Metastases were found 12 years after initial therapy. Three palliative operations made a survival of further 7 years with high quality of life possible. Therefore moderately palliative operations are recommended in case of metastatic malignant choroidal melanoma.

Purpose: (31)P MR spectroscopy (MRS) allows the noninvasive assessment of metabolic alterations in tumors. Due to physical as well as technical limitations, mostly large and single voxels are used. We used a spatially resolved (31)P MRS technique to characterize metabolic abnormalities inside and adjacent to liver metastases of patients with uvea melanoma., Materials and Methods: Optimization of 3D chemical shift imaging (3D CSI) was performed in healthy volunteers (n = 19; voxel size 25 ml). Patients (n = 8) with liver metastases were then examined. Cross sectional imaging was available for all patients., Results: Compared to healthy volunteers, the PME/PDE ratios of patients with liver metastasis were significantly higher (0.56 +/- 0.30 vs. 0.39 +/- 0.21; p < 0.05). A trend towards increased PME/beta ATP ratios (2.07 +/- 1.83 vs. 1.02 +/- 0.45; p = 0.12) and decreased Pi/PME ratios (0.57 +/- 0.29 vs. 1.06 +/- 0.58; p = 0.06) was also observed. Patients with metastases > or = 5 cm showed significantly higher PME/PDE ratios (0.68 +/- 0.17 vs. 0.45 +/- 0.03; p < 0.05). Liver parenchyma adjacent to metastases did not show any significant changes compared to non-diseased tissue., Conclusion: 3D CSI allows the simultaneous analysis of metabolic alterations in diseased as well as in healthy human liver. Metastases show significant metabolic alterations. Thus, (31)P MRS opens new possibilities for therapeutic monitoring.

Background: Malignant melanoma of the choroid is the most common ocular primary malignancy, but is still a rare tumour. The occurrence of bilateral uveal melanoma is exceedingly rare. The probability of any one individual developing bilateral melanoma is estimated to be 1 to 50 million., Materials and Methods: A retrospective search of the photo database of patients between 1970 and 2006 with uveal melanoma was performed., Results: Four patients with bilateral melanoma were identified. The case reports of the two females and two males are presented., Conclusions: In summary, bilateral primary uveal melanoma seems to occur more frequently than expected. The estimated probability for patients with unilateral primary uveal melanoma of developing bilateral melanoma is 0.2 %. In single cases the interval between the occurrence of the second melanoma can be more than 30 years. In patients with a history of malignant melanoma of the choroid it is important to carefully observe the other eye life-long.

Background: The aim of this study was to evaluate what are the most frequent ocular diseases that were suspected for choroidal melanoma ("pseudo melanoma")., Patients and Methods: The data of all patients who were seen in the University Hospital of Mainz under the suspicion of choroidal melanoma between 1.1.1994 and 1.1.2004 and underwent ultrasound examination, fluorescein angiography or fundus photography, were analysed retrospectively. Among 458 examined eyes a choroidal melanoma was diagnosed in 212 cases. In 246 cases the diagnosis was "pseudomelanoma"., Results: The most frequent ocular diseases suspected for choroidal melanoma were "suspicious nevi" in 31 % of the cases and "disciforme macula degeneration" in 34 %. Less frequently we found hyperplasty of pigment epithelium (5 %), melanocytoma (10 %), choroidal haemangioma (6 %), choroidal detachment (7 %) and retinal macroaneurysma with epi-/subretinal haemorrhages (3 %). Rare diagnoses were orbital tumour, scleritis posterior and a combined hamartoma of retina and pigment epithelium (4 % at all). In 58 eyes without a fundus view, ultrasound revealed an intraocular tumour. In these cases vitrectomy/cataract extraction was performed and we found a choroidal melanoma in 5 eyes, a disciforme macula degeneration in 48 eyes and in 5 eyes a retinal macroaneurysma., Conclusions: Age-related macula degeneration (AMD) is the cause for one third of all pseudomelanomas. Because of its increasing incidence in the future, more attention must be paid to AMD in the differential diagnosis of chorioretinal lesions suspicious of melanoma.

Background: The aim of this study was to evaluate the course of tumor regression and visual impairment in choroidal melanomas treated by transpupillary thermotherapy (TTT) alone., Patients and Methods: In a prospective, non-randomized analysis 26 patients suffering from primary choroidal melanoma (posterior to the equator with base < or = 12 and thickness < or = 4.5 mm) were treated with the TTT standard protocol and followed for 33 - 45 months. Fluorescein and ICG angiographies were performed initially and after tumor regression clinically., Results: 22 tumors were significantly flattened after 1 - 3 TTT sessions. 4 tumors required additional therapy with ruthenium plaque. Visual acuity remained unchanged or improved in 11 eyes. In all eyes fluorescein and indocyanine green angiography revealed a sharp demarcation of the heat-treated area. Except for macular edema and gliosis in 6 eyes no vascular or retinal tissue damage was noticed outside the treatment zone. In the periphery of the treatment zone all eyes showed residual choriocapillaries remaining perfused. During follow-up 2 eyes revealed tumor regrowth at the posterior margin of the scar, which was successfully managed by TTT or a ruthenium plaque. 2 eyes developed late choroidal neovascularizations (CNV) in the heat-treated area. One patient died of liver metastases., Conclusions: Fluorescein and indocyanine green angiographic findings after TTT for small choroidal melanomas suggest that the tissue damage in the choroidal layer is less effective, which perhaps can lead to a higher rate of tumor regrowth. Development of choroidal neovascularization may be a sign of late heat-induced side effects of TTT.

Background: The aim of this study was to evaluate whether photodynamic therapy (PDT) with verteporfin is able to induce tumor cell necrosis in human uveal melanomas., Methods: On four eyes with an uveal melanoma, PDT with verteporfin was performed on the tumor 2-3 days before planned enucleation. The eyes were evaluated histologically., Results: In two melanomas with only mild pigmentation some effects after PDT were detected on tumor tissue in a depth up to 2.5 mm at light doses > or =100 J/cm(2). Histologically, vascular occlusion and thrombosis in tumor vessels were observed. In the heavily pigmented melanoma no tumor necrosis was induced with the above-mentioned parameters., Conclusion: Depending on treatment parameters and tumor pigmentation, PDT with verteporfin is able to induce tumor necrosis in human uveal melanomas. Based on these results it is possible that PDT can become an adjuvant treatment method for uveal melanoma.

Aim: Evaluation of functional and anatomical long-term results of patients with symptomatic choroidal hemangioma treated with photodynamic therapy (PDT)., Patients and Methods: All 8 patients suffered from progressive loss of vision caused by exudation into the macular area. PDT with a verteporfin dose of 6 mg/m(2) body surface area and a light dose of 100 J/cm(2) at 692 nm was carried out. Two to four treatments were applied at 6 week intervals using a single laser spot. Standardized evaluation was used before and at 6-week intervals after each treatment and at 3, 6 and 12 months. In the further follow-up control intervals were extended up to 1 year in the case of stable anatomical and functional results., Results: All patients showed complete regression of the tumor. The tumor height was reduced from an average of 3.64 mm to undetectable. One patient showed a stable visual acuity, seven patients a significant increase. Central scotometry showed withdrawal of scotomas from the macula. No patient showed any sign of recurrence during the follow-up of up to 60 months., Conclusion: The long-term results of patients with active choroidal hemangioma demonstrate that PDT is a most effective and safe treatment option. Complete tumor regression is combined with good functional recovery.

Background: Primary intraocular lymphoma is a distinct subset of primary non-Hodgkin's lymphoma of the CNS. In general, the primary non-Hodgkin's lymphoma of the CNS is rare, accounting for 1 % of all non-Hodgkin's lymphomas and less than 1 % of all intraocular tumors., History and Signs: A 70-year-old man was hospitalized in June 2002 because of acute loss of vision on his left eye. A severe vitreous hemorrhage was observed. Ultrasound showed solid subretinal lesions at the posterior fundus. Diagnostic vitreous surgery including a biopsy was performed. An intraocular malignant B-cell lymphoma was determined by immunohistochemistry. General screening revealed no further manifestations of the lymphoma., Therapy and Outcome: The patient initially refused any therapy until a painful secondary neovascular glaucoma with complete loss of visual function developed, thus prompting us to perform an enucleation. The following immunohistochemical examination confirmed the initial diagnosis. A chemotherapy with high-dose methotrexate and leucovorin rescue was initiated., Conclusions: Primary intraocular lymphoma can present as diffuse uveitis refractory to corticosteroids. Diagnosis can be difficult and is often delayed.

Background: To determine the efficacy of intravenous indocyanine green (ICG) as an adjuvant to TTT for hypopigmented choroidal melanomas., Patients and Methods: ICG kinetics in melanoma tissue were evaluated by taking standardized ICG angiograms in 28 eyes with small choroidal melanomas. In a prospective non-randomized analysis 12 eyes with hypopigmented choroidal melanomas (posterior to the equator with thickness

Background: Choroidal metastasis (CM) is the most common malignant intraocular tumor. The aim of this study was the characterization of the ophthalmoscopic aspect, the underlying tumor disease and differential diagnosis of this entity., Study Design: Retrospective observational case series., Patients and Methods: A total of 71 consecutive patients with CM were evaluated. Special attention was given to ocular symptoms, the ophthalmoscopic and sonographic aspects and the characteristics of the underlying tumor disease. All patients had undergone screening (CCT, CT of thorax and abdomen; bone scintigraphy) for the primary tumor and further organ metastases., Results: Of the patients 95% had symptomatic CM, in 5% of the patients CM was detected by chance, 60% had a single CM, 40% showed multiple (2-14) lesions and 18% ( n=13) had no history of tumor disease. In 12 of these patients lung cancer could be diagnosed. In 53% of the patients the primary tumor was breast cancer, in 26% lung cancer, in 3% kidney cancer, bowel cancer, choroidal or cutaneous melanoma and in single patients cervical, ovarian, thymus or prostate cancer. In one patient who died from disseminated metastases, no definite primary tumor could be detected. Of the patients 96% had further metastases, CM occurred with a mean interval after diagnosis of the primary of 9 months in lung cancer and 68 months in breast cancer. CM was the first clinical sign of tumor disease in 58% of patients with lung cancer and the first clinical sign of metastatic disease in 28% of patients with breast cancer., Conclusions: Choroidal metastasis occurs almost exclusively in metastatic disease. In breast cancer it typically occurs years after diagnosis of the primary tumor and may be the first sign of metastatic disease. In lung cancer choroidal metastasis may be the first sign of the tumor disease.

Background: Choroidal metastases generally respond well to radiation treatment. However, a deterioration of visual acuity after radiation must be taken into account in lesions involving the optic nerve. In these cases, alternatives to radiation, which is a palliative treatment in most cases anyway, should be considered., Material and Methods: We present the clinical and echographic findings of a case of a 39-year-old patient with choroidal metastasis from breast carcinoma., Results: We observed a regression of the metastasis and an improvement of visual acuity from 0.2 to 1.0 under systemic chemotherapy. Echography showed a decrease of tumor height and increasing reflectivity indicating progressive scarring of the lesion. Up to now, the patient has survived 9 years after initial diagnosis., Conclusions: Especially in cases in which a deterioration of visual acuity after radiation must be expected, the follow-up during systemic chemotherapy alone might be considered an alternative option, as it not only might preserve vision, but also allows an "in vivo" monitoring of the success of the chemotherapy applied.

Background: Up to now the diagnosis of choroidal tumors was mainly based on ophthalmoscopy and ultrasound examination. Indocyanine green angiography allows the identification of vascular patterns in these tumors, which could be helpful in the differential diagnosis. The aim of the study was to characterize the staining patterns of choroidal metastases., Methods: In the years 1996-2000 we performed ICG-A of 15 choroidal metastasis using the scanning laser ophthalmoscope from Rodenstock. The mean tumor height was 3.7 mm (2.0-9.0). These angiographies were compared to the angiographies of 38 choroidal malignant melanomas with a mean height of 3.6 mm (1.4-6-6)., Results: All metastases showed a blockage of the background staining and a patchy staining of the tumor surface. Intratumoral vessels could be detected in 13% of all cases of metastases. The choroidal melanomas examined showed a blockage of the background staining in all cases and tumor vessels in 89%., Conclusion: The angiographic picture of the metastatic lesions was characteristic. Intratumoral vessels could not be detected using ICG-A. Most of the metastases showed a patchy staining of the tumor surface.

Background: Transpupillary thermotherapy is a new and most promising treatment modality for up to medium-sized choroidal melanoma at the posterior pole. We analysed the results of conventional ruthenium-106 brachytherapy in these special tumour subgroup., Patients and Methods: Results of ruthenium-106 brachytherapy (radioactive dose to the tumour apex 150 Gy) in a series of 52 eyes with primary malignant choroidal melanoma (posterior to the equator, thickness

Background: To evaluate the effectiveness of transpupillary thermotherapy (TTT) as an adjunct to ruthenium brachytherapy for treatment of choroidal melanoma with reduced radioactive dose., Patients and Methods: In a prospective nonrandomised analysis 15 eyes with primary malignant melanoma of the choroid (posterior to the equator, thickness

Background: We have previously shown that histologically described microcirculation patterns (MCP) can be visualized with indocyanine green (ICG) angiography. We have designed a prospective study to evaluate the prognostic value of these angiographically imaged MCP in small choroidal melanocytic lesions. In this report we describe the design of the study, characterize the patient collective, and present the first results., Patients and Methods: In this prospective nonrandomized observational study, unilateral choroidal melanocytic lesions with 1.5-5.5 mm maximum apical height are observed until growth is determined according to defined criteria. Variables are demographic parameters, subjective symptoms, subretinal fluid, location and dimension of tumor, hemorrhage, color, orange pigment, and MCP determined by ICG angiography: normal, straight, parallel without crosslinking, parallel with crosslinking, arcs without branching, arcs with branching, loop, and network., Results: Seventy patients (22 males, 48 females; age: 33-88 years, median: 64 years) have been included up to now: 19 tumors showed growth so far (time to growth: 51-946 days, median: 127 days). The following parameters were statistically significantly correlated with time to tumor growth: flashes (p = 0.082), orange pigment (p = 0.012), subretinal fluid (p < 0.001), maximum basal tumor diameter (p = 0.001), maximum apical tumor height (p < 0.001), parallel with crosslinking (p < 0.001), arcs with branching (p = 0.006), loop (p < 0.001), and network (p < 0.001). Of these, complex MCP (parallel with crosslinking, arcs with branching, loop and/or network) showed the strongest correlation with time to tumor growth in a Cox regression model. Based on our data, the positive predictive value of imaging complex MCP (for growth within 12 months) is 78% and the negative predictive value is 98%., Conclusion: Our patient collective demonstrates comparable prognostic parameters for time to growth as described in the literature. In addition, the ICG angiographic detection of complex MCP is more strongly predictive of the time to growth than other clinically determinable factors. Thus, we recommend this examination for patients with small choroidal melanocytic lesions, if the patient is to be counseled regarding the likely biologic behavior of his tumor.

Background: Since introduction of brachytherapy using (106)ruthenium plaque therapy for treating malignant choroidal melanomas a number of comparative studies have shown that survival rates are equal between patients who underwent radiation or enucleation. When radiation treatment fails, the patient might be determined to choose removal of the tumor by pars plana vitrectomy., Patients and Methods: Between 1995 and 1998 a total number of 48 patients underwent (106)ruthenium plaque irradiation. In 9 patients tumor regression was not sufficient. Two of these nine patients decided explicitly against enucleation and choose to have the tumor removed from within the eye employing pars plana vitrectomy., Results: Intraoperatively, the previously radiated melanoma was easily removed with the vitrectomy cutter without significant bleeding. Postoperative recovery was without complications. Silicone oil removal was carried out in one patient after one year. Vision 9 months and 2.5 years after surgery was 0.2 and 0.02, respectively. The cosmetic results with parallel axis of the globes were very good and one patient gained binocular vision. No recurrent tumor growth was seen so far. Histologically, no mitotic figures were found., Conclusion: Malignant choroidal melanomas can be safely removed by pars plana vitrectomy with stable intraocular pressure. Previous radiation therapy alters the tumor, inducing fibrous tissue growth and necrosis of tumor cells. These tissue types are not prone to bleed when they are resected by the vitrectomy cutter. Long time studies have to elucidate the difference in survival rates between patients undergoing enucleation of the eye and patients who chose endoresection of the melanoma by pars plana vitrectomy.

A 59-year-old patient presented with metastatic uveal melanoma that developed in a nevus of Ota. The nevus of Ota or oculodermal melanocytosis contains an increased number of dermal melanocytes in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve. Malignant transformation arising in a nevus of Ota may occur in all anatomical sites influenced by the nevus. Most often the choroid is involved. Although the nevus of Ota is rare in Caucasians, associated melanoma is more common than in Asians or black people. The dermatologist should be aware of this problem and aim at an interdisciplinary management of these patients.

Background: Idiopathic sclerochoroidal calcification is a rare benign disorder of the choroid and sclera which has initially been described twelve years ago. Clinically, it is often mistaken for osteoma, choroidal metastasis or infiltration in lymphoma leading to exentsive further investigations., Case Report: A 68-year-old patient had been referred to our outpatient clinic because of unusual fundus changes on both eyes. Ophthalmoscopic examination revealed a yellowish placoid-like lesion in the superotemporal quandrant of the fundus of both eyes, the left lesion being more discrete. Flurescein angiography and echography led to the diagnosis of bilateral ISC., Conclusion: Although idiopathic sclerochoroidal calcification can easily be diagnosed by echographic and angiopraphic examination, it is frequently misdiagnosed for malignant tumors thus initiating excessive further investigation.

In early stages choroidal peripapillary choroidal melanoma may be confused with a choroidal nevus or melanocytoma., Case Report: A 41-year old female was diagnosed having a choroidal nevus in close proximity to the optic nerve head. Unfortunately the patient did not show up for annual review of this lesion. Ten years after the patient was examined for the second time. A massive increase in tumor size and prominence was noted including serous retinal detachment. Medical work up excluded any metastatic growth, and the globe was enucleated. Three years later, the patient is still healthy and there are no signs of metastatic spreading., Histological Work Up: Heavily pigmented peripapillary choroidal melanoma with fascicularly vasocentric proliferative structure, but no infiltration of the sclera or the lamina cribrosa of the optic nerve. The tumor cells were of midgrade size and some of the nucleoli were slightly enlarged. Mitosis and a syncytial structure were present. Immunohistochemically S-100 proteins, HMB 45 and NSE were found. The TNM classification was: ICD-O C 69.3; pT2, G1, S0, V0, pNx, pMx, microscopically RO., Conclusion: Pigmented juxtapapillary tumors resembling choroidal nevi require annual surveillance.

Background: About 3-10% of patients suffering from metastatic disease develop metastatic choroidal tumors. In this case report ocular symptoms were the first sign of a systemic malignancy., Case Report: A 65-year old man complained of an increasing painful redness of his left eye. He showed a white mass with 10 mm in thickness in the temporal chamber angle with "pseudohypopyon" and secondary glaucoma. Because of increasing pain the eye was removed. Histologic examination showed a small cell carcinoma of the ciliary body with high malignancy, which most probably has to be seen as a metastasis of a carcinoma of the lung, which was detected by internal examination concurrently. In addition, a metastasis in the adrenal gland was found. ACE polychemotherapy was performed, but 4 months later the patient died of metastatic disease with break down of the kidneys., Discussion: Based on literature data patients with systemic malignancy present themselves to a physician primarily because of ocular symptoms in 12-31%. In these cases differentiation of an uveal metastasis from a primary amelanotic melanoma can be difficult. The search for primary tumors has to be started in due time.

Background: The breast cancer metastasis is the most common intraocular tumor in females. Aim of this study was to determine incidence and risk factors for intraocular metastasis and to evaluate the benefit of an early treatment., Patients and Methods: 151 patients suffering from metastatic breast cancer were screened for the presence of intraocular metastasis. The medical history and the tumor status at the time of screening were evaluated and risk factors for intraocular metastasis were determined. In case of choroidal metastasis external beam radiotherapy was performed., Results: Intraocular metastasis was found in 7 out of 151 patients screened (4.6%). In one patient metastasis was located in the iris, in 6 patients in the choroid. Intraocular metastasis was only found in patients with more than one other organ system involved into disease (p = 0.002). In this subgroup of patients (n = 65) prevalence of intraocular metastasis was 10.8%. Other risk factors for intraocular metastasis were presence of lung metastasis or brain metastasis. In 5 out 6 patients with choroidal metastasis external beam radiotherapy was performed, resulting in durable regression of metastasis and stabilization or improvement of visual acuity., Conclusion: The prevalence of intraocular metastasis in metastatic breast cancer was determined to be 4.6%. It is most commonly located in the choroid and develops in the course of advanced metastatic disease. Since early external beam radiotherapy of choroidal metastasis prevents functional loss, patients at risk should be given an ophthalmological screening for intraocular metastasis.

Purpose: Choroidal melanomas usually present a characteristic appearance in MRI. Differing characteristics can cause problems in differential diagnosis between melanomas and other masses in the globe. The purpose of this study was to evaluate the appearance of choroidal melanomas with MRI in a large consecutive patient group., Methods: In a prospective study, 200 patients with choroidal melanomas were investigated with MRI using a 1.5 T scanner and a 5 cm surface coil. Both quantitative and qualitative evaluation of the resulting images was performed., Results: 78.5% of the melanomas presented with homogeneous signal intensities within the tumor due to a homogeneous pigmentation whereas 21.5% of the melanomas demonstrated a mixed pigmentation. Signal intensities of the homogeneous melanomas in the plain T1-WI were moderately or markedly hyperintense compared to the vitreous in 29.3% and moderately or markedly hypointense in the T2-WI in 37.1%. An accompanying retinal detachment was found in 65.5% and an extraocular growth in 7.0%., Conclusions: In 10% to 37% we observed the typical well known MR appearance, including homogeneous high signal in the T1-WI and low signals in the T2-WI. For further differentiation, morphological criteria (e.g. shape, size, and position) were used, which are also discussed.

Unlabelled: The purpose of this report is to evaluate the efficacy of transpupillary thermotherapy (TTT) to treat choroidal melanoma., Patients and Methods: Twelve patients with choroidal melanoma were treated. Four with small tumors close to the posterior pole received TTT alone; 8 were treated simultaneously with TTT and Ru-106 brachytherapy. To perform TTT a diode laser with a beam diameter of 3 mm was used. Exposure time ranged from 60 to 90 s until the retinal surface became light gray. Follow-up examinations were performed at 1-month intervals. If the tumor was partially regressed, additional TTT was performed to reach the endpoint of a chorioretinal scar., Results: All tumors exhibited a reduction of tumor height in a maximal follow-up period of 6 months. Four cases showed complete regression within 3 months. Side effects were minimal., Conclusions: Treatment with TTT may be useful as a complementary modality to brachytherapy. A longer follow-up time is necessary for final evaluation.

Unlabelled: The aim of our study was to develop noninvasive stereotactic radiotherapy for patients with choroidal metastases., Methods: The head of the patient was immobilized by an individual cast mask. The target volume and adjacent critical structures were three-dimensionally segmented based on ophthalmological findings and MRI. The beam angles were optimized by a beam's eye view technique with a micro-multileaf collimator. We use a linear accelerator with 6-MeV photons. A patient was treated in a phase I/II trial with a single dose of 22.5 Gy., Results: The accuracy of stereotactic positioning of the eye was 0.8 mm. The dose gradients were 15% mm. It was possible to spare the lens and the lacrimal gland in all cases. The treatment with four to six fields took 35-50 min and was tolerated without acute complications., Conclusions: We introduce a new method to treat choroid metastases by stereotactic radiotherapy. This method allows to concentrate the dose to the target and to spare adjacent critical structures. One of the advantages of this noninvasive treatment is the short treatment time. The clinical role of this method has to be evaluated based on long-term results of tumor control and visual outcome in these patients.