Isolated brain metastasis of malignant choroidal melanoma 27 years after enucleation

Choroidal melanoma primarily metastasizes to the liver. Isolated extrahepatic metastases have rarely been reported and they generally resulted in death within 6 months. We describe a patient who developed an isolated brain metastasis 27 years after his left eye was enucleated for choroidal melanoma. The metastasis was successfully treated with surgery and radiotherapy. The patient is alive and disease free 3 years after treatment of the metastasis. Posterior location and other clinical and morphologic characteristics of primary choroidal melanoma could explain the unusually long latency of this solitary extrahepatic metastatic disease. Lifelong surveillance to detect early signs of metastasis is mandatory for any patient treated for choroidal melanoma.     

Clinical Course of a Presumed Metastatic Uveal Melanoma to the Contralateral Choroid

Abstract

We present the ultrasound and optic coherence tomography follow-up of a presumed choroidal metastasis from a contralateral melanoma. A 53-year-old male was diagnosed with uveal melanoma with extraescleral extension in his left eye. A year later, the fundus examination revealed a flat, gray-green, pigmented choroidal lesion in the right eye. The ultrasonography showed a mass, almost flat, and all these findings were compatible with a choroidal melanocytic lesion with risk factors for growth. One month later, melanocytic skin lesions appeared on the scalp, as well as small tumors. Three months later, an ultrasonography on B scan showed a growth of the tumor size. The patient developed a progressive deterioration and died. Three possibilities can explain the occurrence of a choroidal pigmented tumor in the contralateral eye: first, bilateral primary choroidal melanomas; second, both choroidal tumors are metastatic in origin from an unknown primary melanoma; and third, the contralateral tumor is a metastatic tumor from the primary choroidal melanoma.     

Calcification in choroidal melanoma after transpupillary thermotherapy

PURPOSE: To report the response of calcification in choroidal melanoma after transpupillary thermotherapy. METHODS: Interventional case report. A 66-year-old male with a posterior uveal melanoma measuring 10 x 8 x 4.4 mm underwent infrared diode laser transpupillary thermotherapy three times, each 12 weeks apart. For each treatment, a 3-mm spot size of 1-minute duration was used, and the power setting varied between 450 mW and 500 mW. RESULTS: Two months after the last transpupillary thermotherapy session, A- and B-mode echographic patterns strongly suggestive of intratumor calcification were observed. The tumor regressed to 2.8 mm in thickness, but calcification persisted. Local recurrence or distant metastasis was not detected during a follow-up of 14 months. CONCLUSION: Clinically detectable calcification is extremely rare in choroidal melanomas. Tumor regression with slowly progressive calcification may occur after transpupillary thermotherapy.     

Choroidal metastasis simulating uveal effusion syndrome: case report

We report a case of choroidal metastasis simulating uveal effusion syndrome. This patient with uveal metastasis from lung carcinoma presented with decreased vision in the left eye. Fundoscopic examination revealed pigmentary clumping, multiple choroidal nodules and serous retinal detachment. Fluorescein angioraphy revealed a leopard spots pattern similar to uveal effusion syndrome. Ultrasound revealed the presence of choroidal nodules with moderate internal reflectivity, choroidal thickening and serous retinal detachment. Optical coherence tomography showed retinal pigment epithelium tear and hyper-reflective nodules within the choroid and areas of serous retinal detachment. Image investigation demonstrated the presence of a solitary pulmonary nodule at the left apical lung. Choroidal metastasis can lead to a scenario that simulates uveal effusion syndrome. This possibility must be considered when having an atypical uveal effusion symptomatoloy and a systemic screening for systemic cancer should be performed.     

In vivo Confocal Microscopy in Differentiating Ipilimumab-Induced Anterior Uveitis from Metastatic Uveal Melanoma

This report aims to describe the facilitating role of in vivo confocal microscopy in differentiating inflammatory cells from a metastatic process in a patient with uveal melanoma and multiple systemic metastases who developed anterior uveitis while under ipilimumab treatment. A 43-year-old woman developed systemic metastases 11 months after treatment of amelanotic choroidal melanoma in her right eye with 30 Gy fractionated stereotactic radiotherapy. She first received temozolomide and then 4 cycles of ipilimumab 3 mg/kg/day. After the third cycle, severe anterior uveitis with coarse pigment clumps on the lens was seen in the left eye. Her left visual acuity declined from 20/20 to 20/80. Confocal microscopy revealed globular keratic precipitates with hyperreflective inclusions and endothelial blebs all suggestive of granulomatous uveitis. The uveitic reaction subsided after a 3-week course of topical corticosteroids, and her visual acuity was 20/20 again. Although uveal melanoma metastatic to the intraocular structures of the fellow eye is exceedingly rare and metastasis masquerading uveitis without any identifiable uveal lesion is even more unusual, it was still mandatory to rule out this distant possibility in our particular patient who already had widespread systemic metastases. Confocal microscopy was a useful complementary tool by identifying the inflammatory features of the keratic precipitates.Key Words: Ipilimumab, T-cell, Uveitis, Uveal melanoma, Confocal microscopy     

Hemorrhagic complication in an unsuspected macular choroidal melanoma

PURPOSE: To report a rare presentation of a hemorrhagic complication in a patient with unsuspected macular choroidal melanoma. DESIGN: Interventional case report. METHODS: A 32-year-old Chinese woman presented with disturbance of her left central vision. A hemorrhagic mass at the macula was noticed that was evacuated by submacular surgery. RESULTS: The mass expanded rapidly into a mushroom configuration after surgery. Fine-needle aspiration confirmed the diagnosis of malignant choroidal melanoma, and enucleation followed. Histology demonstrated moderately large epithelioid tumor cells with prominent nucleoli. The sclera was intact, and the optic nerve was not involved. The patient has been followed for 5 years without signs of local recurrence or metastasis. CONCLUSION: We should be alert for atypical presentations of choroidal melanoma. Unnecessary or inadvertent surgical intervention may induce tumor growth and subsequent local spread or metastasis.     

Unusual MRI findings in metastatic carcinoma to the choroid and optic nerve: a case report

A 51 year old man with biopsy proven pulmonary sarcoidosis and skin test positive for tuberculosis presented with features of an amelanotic flat choroidal mass suggestive of choroiditis. The mass enlarged despite corticosteroids and anti-tuberculous medications. A throrough systemic evaluation for possible primary tumor metastatic to the choroid was negative. Further clinical evaluation and magnetic resonance imaging suggested a diffuse primary choroidal malignant melanoma with optic nerve invasion. The eye was enucleated and the mass proved histopathologically to be a mucin secreting adenocarcinoma of unknown origin despite a repeat systemic work-up. The patient died three months after the onset of symptoms and three weeks after enucleation with diffuse metastases from an unknown primary cancer. Magnetic resonance imaging (MRI) is usually helpful in the differentiation of uveal melanoma from uveal metastasis. In this case, however, it suggested the diagnosis of a diffuse choroidal melanoma. The reason for the atypical MRI findings will be discussed.     

Photodynamic therapy with verteporfin of choroidal malignancy from breast cancer

PURPOSE: To evaluate the effectiveness of photodynamic therapy (PDT) with verteporfin for treating a submacular choroidal metastasis from breast cancer. DESIGN: Interventional case report. METHODS: Multispot PDT irradiation of tumor surface was performed in a 45-year-old woman who had a choroidal metastasis from breast cancer in the right eye. The response of uveal metastasis to photodynamic action was investigated with the use of fluorescein angiography and optical coherence tomography (OCT). RESULTS: Within four months after photosensitization, the exudative detachment of the macula was resolved, with improvement in the visual acuity following decrease in the tumor vascular permeability and absorption of subretinal fluid. CONCLUSIONS: PDT can effectively destroy a malignant tissue and induce an antitumor activity. PDT as an adjunct to chemotherapy is a possible treatment and might be appropriate for patients who require ocular treatment only.     

Pars plana vitrectomy in eyes containing a treated posterior uveal melanoma

PURPOSE: To determine the safety of pars plana vitrectomy in eyes containing a treated posterior uveal melanoma. DESIGN: Interventional case series. METHODS: Retrospective case series of patients with posterior uveal melanoma who underwent pars plana vitrectomy. Complications, vitreous cytology, local tumor control, and metastasis were assessed. RESULTS: Nine patients met study criteria. Tumors were treated with (125)I plaque radiotherapy (seven patients) or transpupillary thermotherapy (two patients). Vitrectomy was performed for vitreous hemorrhage (five patients), macular pucker (two patients), macular hole (one patient), and rhegmatogenous retinal detachment (one patient). Vitrectomy was performed at a mean of 24.7 months (range, 7-47 months) after melanoma treatment. Dispersion of tumor cells at vitrectomy was not observed in any patients. Melanoma cells were detected in the vitreous aspirate in one of seven cases examined cytologically. This patient had intratumoral and vitreous hemorrhage before plaque radiotherapy, underwent combined vitrectomy/cataract extraction, and developed intraocular tumor dissemination 56 months after vitrectomy. No other patients developed intraocular tumor dissemination. At mean follow-up of 24 months (range, 3-63 months) after vitrectomy, none of the nine patients developed systemic metastasis. CONCLUSIONS: Pars plana vitrectomy rarely may lead to intraocular tumor dissemination, although the risk of this complication is probably low if the tumor has been treated and has responded to therapy before vitrectomy. Vitrectomy should be approached with caution if a vitreous hemorrhage is present, especially if the hemorrhage occurred before tumor treatment, as this may seed tumor cells into the vitreous cavity.     

Intraocular melanoma spread to regional lymph nodes: report of two cases

PURPOSE: To report two cases of regional lymphatic spread of primary uveal melanoma. METHODS: The clinical records of two patients who underwent enucleation for uveal melanoma and later developed regional lymph node metastases were reviewed. One of the two eyes was initially treated with proton beam irradiation. Histologic sections of the enucleated eyes and excised lymph nodes were examined. RESULTS: The melanomas arose in the choroid and ciliary body of the two patients and spread to regional lymph nodes 2 years after enucleation. The choroidal melanoma recurred after irradiation, diffusely infiltrated the uveal tract, and extended into the conjunctiva via an emissary canal. The ciliary body melanoma spread through the trabecular meshwork to the conjunctiva. CONCLUSIONS: Choroidal and ciliary body melanoma may rarely exhibit regional lymph node metastasis. This mode of metastasis may occur after extraocular spread and invasion of conjunctival lymphatics.     

Transpupillary Thermotherapy in the Treatment of Choroidal Metastasis from Breast Carcinoma

BACKGROUND: Most patients who develop metastatic carcinoma to the choroid are managed by local radiation or chemotherapy. Since transpupillary thermotherapy (TTT) is currently gaining attention as an optional treatment for choroidal melanomas and hemangiomas, we sought to determine whether TTT is suitable for treatment of solitary choroidal metastasis at the posterior pole. METHOD: We report on a patient with decreased vision due to a serous macular detachment in a eye with a solitary choroidal metastasis from breast carcinoma, who was managed by TTT. RESULTS: After two months of follow up, total re-absorption of the serous macular detachment was achieved and the patient recovered full visual acuity in the treated eye. The choroidal mass became atrophic and hyperpigmentation of the retinal pigment epithelium and retinal folds in the macular region were observed. After six months of TTT, the ocular picture remained unchanged. CONCLUSION: TTT can be considered an acceptable therapeutic option for solitary choroidal metastasis associated with serous retinal detachment.     

Treatment considerations for primary uveal melanoma with choroidal metastasis to the fellow eye

We report two cases of primary uveal melanoma with metastatic involvement of the contralateral eye. Two female patients presented with primary choroidal melanoma. In the first case, primary enucleation of the affected eye was performed. Two years later, systemic tumor spread with contralateral choroidal melanoma was detected. A decision for observation of the ocular metastasis was made. In the second case, systemic tumor spread was already evident at time of initial diagnosis of the ocular melanoma. Six months later, a choroidal metastasis was detected in the fellow eye. Again, observation was recommended. In conclusion, systemic spread of primary choroidal melanoma may include a choroidal metastasis to the contralateral eye. Observation of the second affected eye may be prescribed.     

Benign Peripheral Nerve Tumor of the Choroid: A Clinicopathologic Correlation and Review of the Literature

A 30-year-old white male was found to have a small, nonpigmented choroidal tumor in the temporal portion of the macula in the left eye. A diagnosis of choroidal melanoma was made, based on the clinical appearance, fluorescein angiography, ultrasonography, and growth of the tumor. In spite of treatment with a cobalt plaque, the tumor continued to grow, and the eye was enucleated. Histopathologic study revealed a benign peripheral nerve tumor of the choroid, presumably a neurilemoma (Schwannoma). A correlation between clinical features, fluorescein angiography, ultrasonography, light microscopy, and electron microscopy is presented, and the literature on solitary peripheral nerve tumors of the uveal tract is reviewed.     

Treatment Considerations for Primary Uveal Melanoma with Choroidal Metastasis to the Fellow Eye

We report two cases of primary uveal melanoma with metastatic involvement of the contralateral eye. Two female patients presented with primary choroidal melanoma. In the first case, primary enucleation of the affected eye was performed. Two years later, systemic tumor spread with contralateral choroidal melanoma was detected. A decision for observation of the ocular metastasis was made. In the second case, systemic tumor spread was already evident at time of initial diagnosis of the ocular melanoma. Six months later, a choroidal metastasis was detected in the fellow eye. Again, observation was recommended. In conclusion, systemic spread of primary choroidal melanoma may include a choroidal metastasis to the contralateral eye. Observation of the second affected eye may be prescribed.     

Uveal melanoma presenting as cataract and staphyloma

Blind eyes can harbor a choroidal melanoma. We report a case of uveal melanoma presenting as staphyloma and complicated cataract in a 45-year-old female. The left eye was blind for six months. She underwent comprehensive ocular examination but fundus examination was precluded due to total cataract. The ultrasound of the eye showed a large mass filling the superior, nasal and inferonasal vitreous cavity with high surface reflectivity and low to moderate internal reflectivity. Magnetic resonance imaging (MRI) confirmed the diagnosis of choroidal melanoma. The patient underwent extended enucleation and histopathology was consistent with uveal melanoma.     

Choroidal melanoma metastatic to the contralateral choroid

PURPOSE: To report metastasis of choroidal melanoma to the contralateral choroid. METHODS: Interventional case report. In a 49-year-old Caucasian female, a primary choroidal melanoma, left eye, was diagnosed and treated with Ruthenium-106 plaque radiotherapy. The choroidal melanoma showed excellent regression with flattening of the mass and adjacent chorioretinal atrophy. RESULTS: Fifteen years after brachytherapy for choroidal melanoma, left eye, the patient developed a metastatic melanoma to the contralateral choroid as the first sign of metastasis. No history of oculo(dermal) melanocytosis or cutaneous melanoma existed. Systemic evaluation disclosed multiple metastases confined to the liver. CONCLUSION: Metastasis to the contralateral choroid can be the first sign of metastasis from choroidal melanoma.     

Long-term surgical outcome of posterior choroidal melanoma treated by endoresection

PURPOSE: To report the visual acuity, enucleation rates, and initial mortality after transvitreal endoresection of posterior uveal melanoma. METHODS: Twenty consecutive patients with medium-sized posterior choroidal melanomas were treated. Vitrectomy was performed, followed by posterior hyaloid dissection, retinotomy, melanoma removal with a vitrectomy probe, retinal reattachment with perfluorocarbon liquid, and silicone oil or gas exchange. RESULTS: Twenty patients (13 men [65%] and 7 women [35%]) were included in the study. The mean patient age +/- SD was 47.1 +/- 16 years (range, 22-70 years). Tumor thickness and tumor diameter ranged from 5.5 mm to 11 mm and 8.0 mm to 15.7 mm, respectively. Preoperative visual acuity ranged from hand motion to 20/40, and postoperative visual acuity ranged from no light perception to 20/30. Two eyes (10%) were enucleated during tumor resection due to severe bleeding. Enucleation was performed on 3 eyes (15%) during follow-up due to tumor recurrence at the edge of surgical coloboma in 1 patient, new focus of tumor in another sector distant from surgical coloboma in 1 patient, and painful blind eye in 1 patient. Fifteen eyes (75%) were saved. One patient (5%) died of metastasis to the liver. The mean follow-up period +/- SD was 89.55 +/- 38.4 months (range, 24-132 months. CONCLUSION: These data suggest that transvitreal endoresection of posterior uveal melanoma is an acceptable management option to conserve the globe and functional vision in selected patients. Distant metastasis is an infrequent event in this modality of treatment.     

Late choroidal metastasis secondary to papillary thyroid carcinoma.

PURPOSE: To report a case of late choroidal metastasis from papillary thyroid carcinoma. METHODS: Interventional case report. A 43-year-old woman who had been treated for papillary thyroid carcinoma 30 years earlier presented for evaluation of vision loss in the left eye. Eight weeks before this presentation, an ophthalmologist had diagnosed a metastatic uveal mass in the left eye of the patient. The clinical record was retrospectively reviewed. Ocular sonography was performed to confirm the diagnosis of choroidal metastasis. RESULTS: The choroidal mass had the typical characteristics of a metastatic lesion. The patient was treated with a combination of brachytherapy and chemotherapy. CONCLUSION: Papillary thyroid carcinoma can metastasize to the choroid many years after the initial diagnosis.     

Bilateral uveal melanoma: a case report

BACKGROUND: The occurrence of bilateral uveal melanoma is rare. This is the first reported case of bilateral melanoma of the choroidea in Austria. METHODS: The chart of a 62-year-old male patient with bilateral melanoma of the choroidea was reviewed. RESULTS: In October 1992, a patient was diagnosed with a choroidal melanoma in the left eye. After two treatments with a ruthenium applicator in 1992 and 1995, the left eye was enucleated in 1999 due to recurrent growth of the choroidal melanoma. In September 2005, the patient presented with a primary choroidal melanoma in the remaining right eye. Sonography of the liver as well as CCT and bone marrow scintigraphy showed no sign of metastatic disease. A ruthenium-106 brachytherapy was performed and the size and height of the melanoma showed decreases. In July 2006, the patient developed metastatic disease and died the following month due to metastasis of the liver. In the genetic analysis, monosomy 3 was detected in both melanoma, an amplification of c-MYC of chromosome 8 was detected in the melanoma of the right eye only. CONCLUSION: Bilateral uveal melanoma is a rare diagnosis. This case shows the importance of a thorough and recurrent examination of the second eye. The results of the genetic analysis are discussed.     

Orbital melanoma metastatic from contralateral choroid: management by complete surgical resection

We discuss a clinicopathologic correlation of orbital metastasis from a contralateral choroidal melanoma wherein the orbital tumor was removed completely by surgical resection. In October 1982, a 29-year-old woman was treated with cobalt-60 plaque radiotherapy for a choroidal melanoma in her right eye. The tumor responded well but recurred after 9 years, necessitating enucleation. In August 1999, 17 years after initial presentation, metastatic melanoma to liver, lung, and brain were found and the patient had a favorable response to chemotherapy and brain irradiation. Five months later, in November 2000, an enlarging orbital mass was documented to be compressing the left optic nerve. Complete surgical removal of the tumor was achieved by a superotemporal orbitotomy. Histopathologically, the tumor was a malignant melanoma with features similar to the choroidal tumor. The contralateral orbit can be the site of late metastasis from choroidal melanoma. In rare instances, an orbital metastasis can be completely removed surgically without subjecting the patient to orbital irradiation.