Bilateral choroidal metastasis from carcinoma ex pleomorphic adenoma of the parotid gland

The histological, clinical and angiographic findings are reported of a 34-year-old man with bilateral visual loss who had left parotidectomy with subsequent radiotherapy due to carcinoma ex pleomorphic adenoma of the parotid gland 1 year before. Funduscopy disclosed choroidal masses with surrounding serous retinal detachment in both posterior poles. At the time of ocular diagnosis, lung, pleura and pharynx metastases had recently been revealed. Because of the extent of disease and its poor prognosis, no treatment was offered. Although parotid gland carcinoma usually spreads via lymphatics, choroidal involvement may rarely occur due to haematogenous dissemination.     

后部眼球壁灰褐色隆起物声像学诊断

目的：研究后部眼球壁灰褐色隆起物－脉络膜血肿及脉络膜黑色素瘤的声像学表现,以便对其进行声像学鉴别诊断。方法：对FFA确诊为脉络膜血肿和手术病理证实为脉络黑色素瘤的17例患者B超检查所见,进行声像学分析。结果：9例脉络膜血肿患者,B超检查表现为：6例后极部球壁局限性增厚,随增益降低出现层间透声裂隙,增益越低透声裂隙越明显,裂隙中散在弱回声光点;3例病变范围大、隆起高,与浅层视网膜脱离相似,但其光带较厚,缺乏后运动,且膜下见细小弱回声光点。8例脉络膜黑色素瘤中5例表现为球壁半球形实性隆起物,3例为蘑菇形。肿物在声像图上均表现为前缘光滑锐利,内回声前方多而强,向后渐少而弱,见脉络膜凹陷及后方声影,3例蘑菇形肿物均见头部周围及附近视网膜浅层脱离。结论：脉络膜血肿与脉络膜黑色素瘤的声像学表现有明显差异。     

Primary transpupillary thermotherapy (TTT) for malignant choroidal melanoma

PURPOSE: To evaluate the efficacy of transpupillary thermotherapy (TTT) as the only method of treatment for small choroidal melanoma. PATIENTS AND METHODS: In a prospective non-randomized analysis, 20 patients with primary choroidal melanoma (posterior to the equator with base < or = 2 and thickness < or = 4.5 mm) were treated with TTT as the only method of treatment (diode laser at 810 nm, beam diameter 3 mm, power setting 0.3-0.9 W, exposure time 20-37 min). During follow-up of at least 6 months, clinical aspects, ultrasonographic tumour thickness, fluorescein and indocyanine green angiographic patterns, visual acuity and ocular side-effects were recorded. RESULTS: In 17 eyes the tumour regressed significantly within 3 months after one treatment session (to a flat chorioretinal scar in 15 eyes). Despite a clinically flattened chorioretinal scar, fluorescein and indocyanine green angiography revealed that choriocapillary vessels in the heat-treated areas of 15 eyes remained perfused. Three amelanotic melanomas showed almost no response to TTT after repeated treatment at higher power settings. Visual acuity remained unchanged or improved in 12 eyes. Ocular side-effects included posterior synechia of the iris (1), macular oedema (2) and temporary retrobulbar pain (2). No patient showed tumour recurrence or metastases during follow-up. CONCLUSIONS: Preliminary results obtained by this study demonstrate good efficacy and visual outcome following TTT as the only method of treatment for small choroidal melanoma. However, indocyanine green angiographic findings suggest that tissue damage in the choroidal layer might be less effective, which perhaps may lead to a higher rate of tumour regrowth. Long-term follow-up is required to obtain data on late ocular side-effects, tumour recurrence and metastasis.     

Ultrasound detection of posterior scleral bowing in young patients with choroidal melanoma

BACKGROUND: In evaluating young patients with choroidal melanomas, which are uncommon in people less than 30 years old, we noted that some had the ultrasound appearance of posterior scleral bowing. The purpose of this study was to determine the incidence of posterior scleral bowing in young patients with choroidal melanoma. METHODS: We reviewed the ultrasonograms of 24 patients less than 30 years of age (mean age 25 years [standard deviation (SD) 4.7 years]) who presented to an ocular oncology service in Toronto between May 1984 and May 1997. In all cases the diagnosis was choroidal melanoma. Histologic specimens were available in six cases. RESULTS: Scleral indentation posterior to the normal curvature of the globe was identified in 14 patients (58%). These patients had a mean tumour height of 4.4 mm (SD 2.0 mm) and mean tumour diameter of 9.5 mm (SD 2.9 mm). In the 10 patients with no scleral bowing the mean tumour height was 5.7 mm (SD 3.1 mm) and mean tumour diameter 11.5 mm (SD 3.8 mm). Pathological study (results available in nine cases in the scleral bowing group and four cases in the group without bowing) showed that all the tumours in the former group and three of those in the latter group were of the more indolent cell types, either mixed with predominantly spindle cells, or spindle cell. The six patients with histologic specimens were all in the scleral bowing group. The histologic features supported the ultrasound finding of posterior scleral bowing. No transscleral invasion occurred over the tumour region. INTERPRETATION: Posterior bowing of the sclera at the tumour site was observed in over half of young patients with choroidal melanoma. This phenomenon can be detected by ultrasonography and has a different appearance from that of choroidal excavation.     

Choroidal melanoma: A review of the experience of the Sydney Eye Hospital Professorial Unit 1979–1995

Purpose: This study examines 90 patients presenting with choroidal or ciliochoroidal melanoma to the Professorial Unit at the Sydney Eye Hospital. The indications for treatment, and the outcome for the eye and vision are presented together with an account of mortality and the incidence of metastases. Methods: A retrospective analysis of 90 choroidal melanoma patients managed by one surgeon over a 16-year period was undertaken. Initial findings, investigations performed, incidence of metastatic disease, treatment received and complication rates and mortality, where applicable, were recorded. Results: The group was followed for an average of 64 months (range, 5–172 months). Primary treatment was with either Iodine¹²⁵ (¹²⁵1) brachytherapy, local excision or enucleation. Radiation retinopathy was prominent in ¹²⁵1 cases resulting in poor visual acuity when the tumour resided in the posterior pole. Local excision even of large tumours was effective particularly if peripheral. Overall metastatic disease was seen in 11% with 5-year survival rates for the metastatic group being 10%. Prognosis after diagnosis of metastases was poor. Conclusions: Specific therapy for choroidal melanoma must relate to the size and location of the tumour at the time of diagnosis. Visual outcome relates directly to the proximity of the tumour to the optic nerve and fovea. Metastatic disease latency can be prolonged; therefore caution about prognosis is required long after therapy is given. The 5-year survival is encouraging with all forms of therapy. However, as the natural history of ocular melanoma is variable and not fully delineated it is important to monitor the effects of conservative therapy. Further long-term survival data is required to distinguish whether one form of treatment is advantageous over the others, although case-control studies are difficult for ethical and practical reasons. In this regard the Collaborative Ocular Melanoma Study (COMS) will provide further evidence for the safety and efficacy of conservative therapy with brachytherapy compared to enucleation.     

Malignant changes in a giant orbital keratoacanthoma developing over 25 years

PURPOSE: To report a patient with a history over 25 years of a slowly growing, large, invasive crateriform tumour filling the anterior part of the orbit. METHODS: A 61-year-old male presented with a large tumour of the left orbit. Exenteration was performed with subsequent histological analysis of the excised mass. RESULTS: The main tumour showed the characteristic features of a keratoacanthoma. However, the posterior aspect of the tumour disclosed the morphology of a squamous cell carcinoma. Six months later, the patient presented with metastases to lymph nodes, lung and mediastinal tissue. A leukemoid reaction was diagnosed by fine needle biopsy. CONCLUSION: The giant variety of keratoacanthoma may fail to regress and can transform into a squamous cell carcinoma. In our patient, the development of a chronic lymphoid leukemia raises the possibility that it may be the underlying cause for the transformation of the posterior part of the keratoacanthoma into a frank squamous cell carcinoma.     

Accuracy of choroidal melanoma diagnosis by general ophthalmologists: a prospective study

PURPOSE: To estimate the proportion of 'false positives' in patients referred with a diagnosis of suspected choroidal melanoma by general ophthalmologists to an ocular oncology centre. METHODS: A prospective study of patients referred by general ophthalmologists to an ocular oncology centre was undertaken over a 14-week period. The diagnosis was made clinically in patients receiving radiotherapy or phototherapy and was confirmed by histopathology in patients requiring fine needle aspiration biopsy (FNAB) or enucleation. RESULTS: A total of 132 new patients were seen in 10 consecutive ocular oncology clinics between 29 March 2004 and 5 July 2004. The mean age was 62 years (range 28-88 years) and 60 (55%) were female. Among the 83 suspected malignant posterior segment lesions, the suspected diagnosis included choroidal melanoma (73), choroidal metastasis (6), 'choroidal tumour' (3), and 'Solid retinal detachment' (1). Only 50 of the 73 suspected melanomas were confirmed (68.5%; 95% CI, 57-78%), the oncologist's diagnosis in the remaining 23 being choroidal naevus (10), choroidal metastasis (1), circumscribed choroidal haemangioma (2) and others (10). Only one of six patients with suspected metastases had this condition, the remainder having melanoma (1), lymphoma (1), circumscribed choroidal haemangioma (1), and others (2). The 'choroidal tumours' and 'solid detachments' were found to be chorio-retinal disciform scar (1), varix of vortex vein (1), eccentric CNV (1), and subretinal haemorrhage (1) CONCLUSION: Approximately 30% of patients referred to an ocular oncology service with the diagnosis of choroidal melanoma have an incorrect diagnosis.     

眼内肿瘤的彩色超声多普勒诊断分析

目的研究眼内肿瘤的彩色超声多普勒诊断特征，为鉴别诊断提供依据。方法应用彩色超声多普勒诊断仪对９２例（１０５只眼）眼内肿瘤进行检查，观察各种肿瘤的血流特征，并测定病变内血管的血流参数。用ＳＡＳ软件对测定结果进行统计学分析。结果视网膜母细胞瘤、脉络膜黑色素瘤、脉络膜血管瘤、视盘血管瘤、脉络膜转移癌，Ｃｏａｔｓ病及原始玻璃体增生症（ｐｅｒｓｉｓｔｅｎｔｈｙｐｅｒｐｌａｓｔｉｃｐｒｉ－ｍａｒｙｖｉｔｒｅｏｕｓ，ＰＨＰＶ）的病变上均可发现血流信号；而脉络膜骨瘤和视乳头黑色素细胞瘤的病变内无血流信号。脉络膜黑色素瘤的视网膜中央动脉和脉络膜血管瘤的眼动脉、视网膜中央动脉及睫状后短动脉的血流参数均较正常值下降（Ｐ＜０．０５）；而脉络膜黑色素瘤与脉络膜血管瘤比较，睫状后短动脉的血流速度，前者明显高于后者（Ｐ＜０．０５）。结论彩色超声多普勒对眼内肿瘤的诊断及鉴别诊断有重要价值。     

Choroidal mass as the presenting sign of small cell lung carcinoma

CASE REPORT: To report the case of a 58-year-old man with blurred vision and metamorphopsia who had an amelanotic choroidal mass in the right eye as the presenting sign of a small cell lung carcinoma. Systemic screening failed to reveal a tumor elsewhere, and the lesion was initially treated as a primary ocular tumor. Discovery of the primary site was made 10 months after the ocular diagnosis, and the patient was then treated with systemic chemotherapy and local radiation therapy. COMMENTS: The ophthalmologist has a crucial role not only in the management of ocular metastases but also in the diagnosis of the primary nonocular malignancies that present as a choroidal mass. The possibility of ocular metastases in patients with choroidal masses should always be considered whether or not there is a diagnosis of cancer elsewhere.     

Pseudomelanomas of the choroid

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.     

Ocular phototherapy

Phototherapy can be translated to mean ‘light or radiant energy-induced treatment.'' Lasers have become the exclusive source of light or radiant energy for all applications of phototherapy. Depending on the wavelength, intensity, and duration of exposure, tissues can either absorb the energy (photocoagulation, thermotherapy, and photodynamic therapy (PDT)) or undergo ionization (photodisruption). For phototherapy to be effective, the energy has to be absorbed by tissues or more specifically by naturally occurring pigment (xanthophyll, haemoglobin, and melanin) within them. In tissues or tumours that lack natural pigment, dyes (verteporphin, Visudyne) with narrow absorption spectrum can be injected intravenously that act as focal absorbent of laser energy after they have preferentially localized within the tumour. Ocular phototherapy has broad applications in treatment of ocular tumours. Laser photocoagulation, thermotherapy, and PDT can be delivered with low rates of complications and with ease in the outpatient setting. Review of the current literature suggests excellent results when these treatments are applied for benign tumours, particularly for vascular tumours such as circumscribed choroidal haemangioma. For primary malignant tumours, such as choroidal melanoma, thermotherapy, and PDT do not offer local tumour control rates that are equivalent or higher than those achieved with plaque or proton radiation therapy. However, for secondary malignant tumours (choroidal metastases), thermotherapy and PDT can be applied as a palliative treatment. Greater experience is necessary to fully comprehend risks, comparative benefits, and complication of ocular phototherapy of ocular tumours.     

Breast Carcinoma Metastatic to the Choroid: Analysis of G7 Patients

Two hundred fifty patients with breast carcinoma had ocular examinations between 1973 and 1980. One hundred fifty-two patients were referred because of ocular symptoms or signs, and 98 were asymptomatic. Of the symptomatic patients, 58 had choroidal metastases; nine of the asymptomatic patients with stage IV metastatic breast cancer had choroidal involvement (9.2%). For the 67 patients with metastases, the median age at ocular diagnosis was 51 years. The median interval from primary to choroidal metastases was three years. Survival after ocular diagnosis ranged from one week to four years, with a median of nine months. Among the 67 patients, choroidal tumor deposits occurred bilaterally in 27 patients (40.3%), in 18 right eyes (26.9%), and in 22 left eyes (32.8%). Irradiation led to stabilization or improvement in visual acuity in virtually all cases. Patients in whom acuity was not threatened were observed on medical therapy. Four of these patients eventually required irradiation for increasing disease. Choroidal metastases tended to follow pulmonary dissemination, and to occur with or before central nervous system disease. Choroidal metastases are a common, treatable event in breast carcinoma and may represent the smallest detectable clinical lesion.     

Unusual course of hypernephroma metastasis

11 years after removal of a kidney because of hypernephroma a choroidal metastasis of this tumor grew. Clinically an inflammatory process had been suspected, thus leading to late correct diagnosis. The clinical and histological findings are presented. In the overall statistic of ocular metastasis seen in the university-eye-hospital in Hamburg, hypernephromas hold the 3rd place in the rank-list of the most common primary tumors. Thus, in case of a suspected ocular metastasis, in differential diagnosis a hypernephroma should be excluded.     

Choroidal osteoma: evidence of progression and decalcification over 20 years

Choroidal osteoma is a rare, benign, ossifying tumour of the choroid of unknown aetiology. In contrast to other types of intraocular ossification, choroidal osteoma is found typically in young healthy females in the second or third decades of life with no history of systemic or ocular disease. Choroidal osteoma is a deep, pale yellow lesion with distinct geographic borders at the juxtapapillary or macular region, with branching ‘spider’ vessels on the surface of the tumour. These features should help differentiate choroidal osteoma from other types of intraocular tumour and the diagnosis can be confirmed with ultrasonography and computerised tomography. Here we report an initially unilateral case of choroidal osteoma, which decalcified over 20 years but during the same period the fellow eye also developed a choroidal osteoma to become a bilateral case. Despite the benign nature of the tumour, vision may be compromised by gradual atrophy of the overlying retina, serous retinal detachment, accumulation of sub‐retinal fluid and sub‐retinal haemorrhage associated with choroidal neovascularisation. Frequent examinations are recommended for patients with choroidal osteoma, for early detection of a subretinal neovascular membrane and potential treatment with laser photocoagulation.     

Combined positron emission tomography/computed tomography for evaluation of presumed choroidal metastases

BACKGROUND: Choroidal metastases are the most common intraocular malignancy and are the first sign of systemic malignancy in approximately one-third of patients. Of patients with no previous diagnosis of cancer, oncological evaluation fails to find the primary lesion in approximately 50% of cases. Newer imaging modalities such as combined positron emission tomography/computed tomography (PET/CT) may improve the yield of the systemic work-up. METHODS: Consecutive patients presenting with presumed choroidal metastases were evaluated with whole body combined PET/CT scanning. RESULTS: Four patients presenting to a tertiary referral hospital with choroidal metastases as the first sign of systemic malignancy were evaluated. In all four cases, PET/CT demonstrated the ocular lesion, and the primary malignancy which was confirmed by tissue biopsy. False-negative results were seen in two cases of cerebral metastases. PET/CT demonstrated lesions not visible on CT or magnetic resonance imaging in two cases. CONCLUSIONS: Combined PET/CT is a useful addition to the work-up of patients with choroidal metastases. It provides the opportunity to detect lesions not visible with other imaging modalities and the ability to image patients with contraindications to magnetic resonance imaging. It is essential to correlate PET images with clinical information and the results of other imaging modalities and tissue biopsy remains the gold standard in the diagnosis of malignancy. False positives and negatives can occur with PET/CT, and further research is needed before this promising technology becomes a routine part of the evaluation of patients with choroidal metastases.     

Extramedullary plasmacytoma of the orbit

PURPOSE: To report a case of extramedullary plasmacytoma of the orbit. METHODS: A 71-year-old patient presented with diplopia eyelid fullness and limitation of ocular motility in the left eye. Visual acuity was counting fingers, intraocular pressure 34 mmHg and fundus eye examination showed choroidal folds in the involved eye. RESULTS: CT scan showed a mass filling the superior and external left orbit without bone destruction. A biopsy was performed revealing that the tumour was composed of plasmacytoid cells positive with immunohistochemical stains for Kappa light chains and epithelial membrane antigen. Systemic work up was negative. The diagnosis of extramedullary orbital plasmacytoma was made. The patient was treated with external beam radiotherapy (40 Gy) and has remained disease free for four years (49 months). CONCLUSION: Extramedullary plasmacytomas of the orbit are extremely rare tumours. Accurate and early diagnosis is essential for the therapeutic approach. Extensive medical work up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving the orbit is needed when the diagnosis of orbital extramedullary plasmacytoma is suspected because treatment and prognosis are very different.     

Ocular complications in systemic lupus erythematosus--choroidal and retinal pigment epithelial changes

PURPOSE: Cotton wool patch and retinal hemorrhage are common ocular findings in systemic lupus erythematosus(SLE). Immunosuppressive treatment with corticosteroids may modify choroidal and retinal findings. We examined ocular complications, mainly choroidal and retinal pigment epithelium (RPE) changes in patients with SLE. MATERIALS AND METHODS: Fifty SLE cases[3 males, 47 females, 41 +/- 15 (mean +/- standard deviation) year-old] were examined and treated in the departments of Internal Medicine and Ophthalmology of Osaka University Medical Hospital. RESULTS: Complications included cataract in 16 cases, glaucoma or ocular hypertension in 9 cases, and choroidal and retinal changes in 15 cases. Occlusive retinal vasculitis, retinal pigment epithelial dysfunction, such as pigment epithelial detachment, choroidal neovascularization and multiple posterior pigment epitheliopathy, in 6 cases, and branch retinal vein occlusion in 2 cases were detected as fundus complications. Average time period from the first detection of SLE was 13 years in patients with RPE changes. CONCLUSION: Long term corticosteroid therapy is probably responsible for the RPE complications.     

Incidence and clinical characteristics of symptomatic choroidal metastasis from lung cancer

Purpose: To determine the clinical characteristics of symptomatic choroidal metastasis (CM) resulting from metastatic lung cancer. Methods: Twenty‐two consecutive patients with symptomatic CM resulting from lung cancer were retrospectively reviewed for ocular findings, medical history and systemic disease. All patients underwent a complete screening for further organ metastasis by computed tomography (CT) and bone scintigraphy. Annual frequency of CM was determined and compared with the incidence predicted from ocular screening studies. Results: In eight of 22 (36%; 95% confidence interval [CI] 17–59) patients, lung cancer had been diagnosed before occurrence of CM, with a median interval of 13 months. In 14 patients lung cancer was detected after diagnosis of CM, with a median interval of 1 month. Choroidal metastasis was unilateral, solitary and located close to or at the posterior pole in the majority of patients. Further organ metastasis with a median number of three affected organ systems was present in 19 (86%; 95% CI 65–97) patients. Median survival after diagnosis of symptomatic CM was 13 months, by contrast with 2 months in lung cancer patients with CM identified in an ocular screening study. The mean number of patients in Berlin diagnosed with symptomatic CM was 1.4 per year, which was two orders of magnitude less than predicted from screening studies. Conclusions: Symptomatic choroidal lung cancer metastasis in the majority of patients presents as a solitary tumour before diagnosis of lung cancer in patients with multiple organ systems affected by metastatic disease. Contrary to predictions from ocular screening studies, it is a rare clinical entity.     

Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma

CASE REPORT: A 30-year-old man was referred to our ocular oncology service with a diagnosis of amelanotic choroidal melanoma of the left eye. The following tests were performed: ophthalmoscopy, fluorescein angiography, indocyanine green angiography, ultrasonography, magnetic resonance imaging and biopsy. DISCUSSION: The diagnosis of giant nodular posterior scleritis, as suggested by ultrasonography, was confirmed by biopsy. A comprehensive medical evaluation was performed, but no etiology was found. The histology revealed a granuloma compatible with ocular sarcoidosis. A rapid response was obtained by systemic steroid administration (1 mg/kg). Sarcoidosis continues to be a challenge in diagnosis. It is important to distinguish nodular posterior scleritis from choroidal melanoma.