Orbital metastasis from cutaneous malignant melanoma

Orbital metastasis is a rare presenting sign of a cutaneous malignant melanoma. Fewer than 30 cases of orbital metastases have been published so far. A 69-year-old man was remitted to our clinic for removal of an orbital tumour verified with CT. Fine-needle aspiration biopsy revealed the tumour to be a metastasis from a cutaneous malignant melanoma diagnosed 4 years earlier. The patient died 3 months after detection of the orbital metastatic tumour. This case illustrates the value of simple fine-needle aspiration biopsy in the diagnosis of orbital tumours.     

Metastasis of choroidal melanoma to the contralateral choroid, orbit, and eyelid.

A 52-year-old woman was found to have a small juxtapapillary pigmented lesion in the choroid of the left eye. This lesion remained clinically stationary for one year, but subsequent growth prompted enucleation of the eye. The tumour was diagnosed histologically as a choroidal malignant melanoma of mixed cell type. Approximately 52 months later the patient developed proptosis of the contralateral (right) eye. Orbital ultrasonography showed a large mass in the right orbit, which was confirmed by needle biopsy to be a melanoma. In addition the patient was found to have metastatic melanoma to the choroid, right lower eyelid area, and liver. The development of simultaneous orbital, choroidal, and eyelid metastases from a contralateral choroidal melanoma is of ophthalmic interest and appears to be unique.     

Report of a case of malignant melanoma of the limbus (30-thy years of observation)

PURPOSE: To describe a case of a malignant melanoma of the limbus. MATERIAL AND METHODS: A 49-year-old female patient with a suspected malignant limbic melanoma in the left eye. The first signs appeared in 1975, manifested as reddening of the left eye on the temporal side with markedly dilated conjunctival vessels. In 1993, the lesion became pigmented and nine years later its progression was noted. In May 2003, lamellar keratoplasty of the left eye was performed under general anaesthesia. RESULTS: Histologic assessment--spindle cell malignant melanoma. The entire tumour was resected and the cosmetic result was very good. The results of accessory diagnostic studies were within normal limits. Over a 28-month follow-up period no local recurrence or metastases to other sites were observed. CONCLUSION: Patients with discoloration of the conjunctivae should be regularly evaluated by an ophthalmologist and an oncologist.     

Conjunctival melanoma at corneoscleral limbus on primary acquired melanosis. A case report

A 51-year-old Caucasian man consulted for a visual loss in the left eye due to corneal extension of a conjunctival melanoma. This conjunctival melanoma arose from primary acquired melanosis with atypia at the temporal corneoscleral limbus. The patient was treated using a combination of surgical excision with physical treatment by ocular proton therapy. Progression remained under control 11 months after treatment: no local tumour recurrence or metastasis was observed. Primary acquired melanosis with atypia must be regarded as a premalignant melanocytic lesion. Based on this case report, the authors focus on primary acquired melanosis and its risk of transformation to a conjunctival malignant melanoma.     

Cutaneous malignant melanoma metastatic to the eye, lids, and orbit

The incidence of malignant cutaneous melanoma is increasing faster than any other cancer. Thus, it will become an increasingly common source of metastatic disease to the eye, lids, and orbit. Herein, we have performed a systematic review of previously published cases including patient characteristics, clinical presentation, diagnostic techniques, current treatments, and outcomes. At the time of ocular diagnosis, nearly all reported patients had a known history of cutaneous melanoma and synchronous non-ocular metastases. Several aspects help in differentiating the tumors from primary uveal melanomas such as the presence of symptoms, rapidly growing multifocal tumors, vitreous seeding, and histopathological findings. Intraocular metastases (uvea, vitreous, retina, and anterior-segment) are more common and occur in younger patients than extraocular metastases (eyelids, orbit, and extraocular muscles). Palliative radiation therapy is often used for intraocular disease. Orbital metastases from cutaneous melanoma commonly involve the extraocular muscles resulting in diplopia and exophthalmos. The mainstays of extraocular treatment are surgical resection and radiation therapy. Unfortunately, there are few good options for systemic treatment of diffusely metastatic melanoma. Therefore, patients with ocular metastasis should be managed to prevent loss of vision or loss of the eye, and to maximize their quality of life.     

Simultaneous bilateral primary choroidal melanoma

The first published case from Norway of primary bilateral malignant melanoma of the choroid is presented. On the admission of a 73-year-old woman for a choroidal melanoma of the left eye, an asymptomatic tumour was detected in the periphery of her right eye. The left eye was enucleated. Histology confirmed the presence of a choroidal melanoma of the mixed type. After 3 1/2 years with regular follow-ups growth of the asymptomatic tumour was noticed. The fast growth was accompanied by a haemorrhage in the vitreous and a secondary retinal detachment. External radiation with a dose of 33.6 Gy resulted in a regression of the tumour, improvement of visual acuity to 0.5 and re-attachment of the retina, lasting the remaining 8 months of life. Multiple metastasis to the liver caused her death.     

Metastatic cutaneous melanoma to the vitreous cavity masquerading as intermediate uveitis

PURPOSE: To report a patient with metastatic cutaneous melanoma (MCM) presenting as intermediate uveitis. METHODS: We examined a 49-year-old man with malignant cutaneous melanoma and central nervous system (CNS) metastasis who was initially treated for bilateral intermediate uveitis. Biomicroscopic examination disclosed a normal anterior segment and vitreous organization with red blood cells (RBC) and some non-pigmented cells in both eyes. Funduscopy disclosed a retinal metastasis in the right eye, but vitreous organization precluded visualization of the retina in the left eye. RESULTS: The first diagnostic vitrectomy was negative for malignant cells in the left eye. However, progressive worsening of the condition persuaded us to repeat vitrectomy in the left eye and to do it in the right eye too. Vitreous samples were positive for malignant melanoma cells in both eyes. CONCLUSIONS: Metastatic cutaneous melanoma to the vitreous cavity is exceedingly rare and may masquerade as intermediate uveitis. It should be considered in the differential diagnosis of unusual uveitis and masquerade syndromes.     

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.     

Metastatic melanoma in the eye and orbit

OBJECTIVE: Presentation of a large series of patients with metastatic melanoma involving the eye and orbit. DESIGN: Retrospective clinical study. PARTICIPANTS: Thirteen cases of metastatic melanoma involving intraocular tissues, 6 cases of metastatic melanoma in the orbit, and 1 case of metastatic melanoma involving both the eye and the orbit, treated in Lausanne between 1986 and 2002, were identified from the computer files of the ocular oncology and orbitopalpebral surgery units of Jules Gonin Hospital. METHODS: The analysis is based on the demographic data concerning these patients, clinical data concerning the tumor, the treatment applied, the outcome, and the follow-up. MAIN OUTCOME MEASURES: Clinical presentation of intraocular and orbital metastasis, interval between primary tumor and ocular metastasis, survival of the patients, evaluation of various therapeutic protocols. RESULTS: Intraocular metastases (14 cases, 15 eyes) were situated in the choroid in 11 cases (isolated lesion, 6 cases; multiple lesions, 3 cases; diffuse involvement, 2 cases), in the iris and ciliary body in 2 cases, and in the retina and vitreous in 2 other cases. The primary tumor was a cutaneous melanoma in 8 cases, a melanoma of the contralateral eye in 3 cases, a mucosal melanoma in 1 case, and was unknown in 2 cases. The mean interval between the diagnosis of ocular metastases and the patient's death was 8.8 months (range, 1-48 months). The primary tumor in the 7 cases of orbital metastases was a cutaneous melanoma in 5 cases, a uveal melanoma in the contralateral eye in 1 case, and was unknown in 1 case. The mean interval between the diagnosis of orbital metastases and death was 19.7 months (range, 5-48 months). The patients were treated by various protocols. The best results, in terms of both local tumor control and preservation of visual function, were obtained with circumscribed proton beam radiotherapy or external beam irradiation, depending on the site and extent of the tumor. CONCLUSIONS: Metastatic melanomas to the eye and orbit are rare and generally occur in patients with disseminated metastases during the terminal stages of the disease, with a short life expectancy. Treatment is palliative and, among the various possible treatment options, circumscribed proton beam radiotherapy or global photon beam radiotherapy, at relatively high irradiation doses, seems to achieve the most favorable results.     

Metastatic cutaneous malignant melanoma of the vitreous and retina

A case of cutaneous malignant melanoma metastasising to the retina in a 71-year-old patient who presented with vitreous opacities is discussed. In view of the increasing incidence of cutaneous malignant melanoma in our community the possibility of ocular metastases merits consideration.     

Metastatic cutaneous malignant melanoma of the vitreous and retina

A case of cutaneous malignant melanoma metastasising to the retina in a 71-year-old patient who presented with vitreous opacities is discussed. In view of the increasing incidence of cutaneous malignant melanoma in our community the possibility of ocular metastases merits consideration.     

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.     

Treatment of non-resectable malignant iris tumours with custom designed plaque radiotherapy.

BACKGROUND--Plaque radiotherapy is the most common method of managing posterior uveal melanoma but its use for iris melanoma and iris metastases has not yet been evaluated. METHODS--Fourteen patients with non-resectable iris melanoma and four with iris metastasis were treated with plaque radiotherapy. The tumour response to treatment and the local side effects of the radioactive plaque were evaluated. RESULTS--In the iris melanoma group over a mean follow up of 26 (range 6-75) months, the tumour regressed in 13 of the 14 patients (93%) and recurred as diffuse seeding in one patient (7%). Despite large doses of radiation given transcorneally, the cornea developed epitheliopathy, abrasion, and oedema in only one case each. The major radiation side effects were localised iris vasculopathy without glaucoma in two cases, posterior synechiae in five cases, and cataract in six cases. In the iris metastasis group, tumour regression was observed in all four patients (100%) and radiation side effects were not evident over the relatively short mean follow up period of 8 (range 4-9) months. All of the 14 patients with irradiated iris melanoma have remained systemically healthy without metastasis while three of the four patients with irradiated iris metastases have died of metastases from the primary neoplasm. CONCLUSION--Custom designed plaque radiotherapy appears to be an effective alternative method of controlling non-resectable diffuse iris melanoma and solitary iris metastasis and has relatively few side effects.     

Metastatic tapioca iris melanoma.

A case of metastatic tapioca melanoma of the iris in a 12-year-old girl is reported. The patient had heterochromia, a red painful eye, and was treated for iritis with secondary glaucoma. In the course of 5 months iris lesions with the clinical appearance of tapioca pudding developed, and biopsy disclosed a melanoma. The eye was immediately enucleated, and pathological examination showed a melanoma with predominantly epithelioid-type cells which had infiltrated the angle, the posterior chamber, and the surgical wounds. Conjunctival extension was noted 10 months after enucleation, and regional lymph node metastases were found 4 months later. Previously reported cases are reviewed and compared with the present case.     

Optic nerve breast metastasis mimicking meningioma

Purpose: We report on an optic nerve breast metastasis masquerading initially as a central retinal vein occlusion and later as an optic nerve meningioma. Methods: A 60-year-old female presented with a left central retinal vein occlusion (CRVO). She represented 7 months later with left upper ptosis, proptosis and painful rubeotic glaucoma. Computed tomography (CT) and magnetic resonance imaging suggested an optic nerve meningioma. On referral to the regional orbital unit, a mild left external ophthalmoplegia was noted and, in view of previous right mastectomy and chemotherapy 3 years earlier, the left optic nerve was biopsied simultaneously with left enucleation of her painful eye. Results: Histopathology showed infiltration of the optic nerve and meningeal sheath spreading into the subretinal space and vitreous by malignant epithelial cells, consistent with breast origin. Further CT imaging and bone scans revealed no other metastases. Single field left orbit radiotherapy of 20 Gy was given in five fractions and Arimidex (Zeneca Pharmaceuticals, Cheshire, England) was commenced with the cessation of tamoxifen. The patient was also given an ocular prosthesis. Sadly, she lost vision in her other eye due to retrograde malignant invasion of her optic chiasm and died 6 weeks later. Conclusions: Orbital and choroidal metastases are relatively common but isolated optic nerve metastases are extremely rare. Progressive infiltration of the nerve is likely to enhance CRVO ischaemia and resultant rubeotic glaucoma. In the diagnosis of CRVO, proptosis or external ophthalmoplegia, the presence of pre-existing malignant disease should raise concerns, as delay in diagnosis may affect outcome, particularly if the metastases are sensitive to pharmacological therapy.     

Long-term progression in a case of transvitreal endoresection of a posterior choroidal malignant melanoma

BACKGROUND: In Japan, there are few reports of the prognosis of the endoresection of malignant choroidal melanoma. We previously reported this case of transvitreal endoresection of a posterior choroidal malignant melanoma. The tumor recurred and enucleation was carried out. Here we report the subsequent long-term clinical course. CASE: A choroidal tumor temporal to the macula was noted in the right eye of a 47-year-old male patient. The tumor size increased within the 1 year of observation and was suspected to be a malignant choroidal melanoma. Since the patient strongly wished to preserve his eyeball, he underwent infrared diode laser photocoagulation, resulting in further progress of the tumor. Three months later a transvitreal piecemeal endoresection was carried out. Four years after endoresection, the tumor recurred, and then the eyeball was enucleated. A pathological examination identified a spindle-cell type malignant melanoma located in the posterior part of the eyeball. No invasion was observed within the sclera or optic nerve. Neither local recurrence nor metastasis has been observed for 5 years after the enucleation. CONCLUSION: In this case, in spite of our utmost efforts, the eyeball could not be preserved. This case can provide useful information for considering endoresection as a management tool for choroidal melanoma.     

Bilateral breast metastases from choroidal melanoma

PURPOSE: To report an unusual case of solitary sequential bilateral breast metastases from choroidal melanoma. METHOD: Case report. RESULTS: A 48-year-old woman with a large choroidal melanoma in the left eye was treated with Iodine-125 brachytherapy and responded satisfactorily with decrease in tumor thickness. Thirty-seven months after treatment, she developed a solitary, circumscribed melanoma metastasis to the right breast, and 54 months after treatment, a similar metastasis was detected in her left breast. Both breast tumors were managed with lumpectomy. Systemic examination including magnetic resonance imaging of abdomen, chest, and head have been performed regularly and have been normal. At 61 months after treatment, the patient has no clinical evidence of metastatic disease elsewhere. CONCLUSIONS: Uveal melanoma rarely metastasizes to breast tissue. A breast nodule in a patient with a history of uveal melanoma is most likely a primary breast tumor but may rarely represent a metastasis from 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.     

Cutaneous melanoma metastasizing to the iris and choroid: a case report

BACKGROUND. Cutaneous melanoma is a primary tumour that rarely produces intraocular metastases. We report on a 47-year-old woman who was referred to our outpatient department because of anisocoria. METHODS. A cutaneous melanoma of the upper left arm had been excised 21 months ago with subsequent polychemotherapy for lymph node involvement. Visual acuity and intraocular tension were normal. The left iris showed a brownish, parapupillary, prominent nodule. In both eyes, multiple circumscribed whitish choroidal sub-pigment epithelial tumours were observed. RESULTS. General metastasis to lymph nodes, pleura, lungs, mucosa and skin was diagnosed. The patient died 2 months later. CONCLUSION. Metastases of cutaneous melanoma to the iris are extremely rare. The ophthalmologist may be helpful in monitoring treatment by quantitative documentation at follow-up.