|Year : 1991 | Volume
| Issue : 2 | Page : 70-73
Malignant melanoma of choroid
S Manohar, R Ramakrishnan, Tara Prasad Das, P Namperumalsamy
Aravind Eye Hospital & Post-Graduate Institute of Ophthalmology, Anna Nagar, Madurai - 625 020, India
Aravind Eye Hospital & Post-Graduate Institute of Ophthalmology, Anna Nagar, Madurai - 625 020
Source of Support: None, Conflict of Interest: None
Four cases of malignant melanoma of the choroid are reported due to rarity of the condition in India. One of the cases presented with Naevus of Ota. All the cases had typical clinical and investigative features. All cases were enucleated. Histopathologically three of them were of mixed type and one was of the epithelioid type. Two of the cases were seen in patients below 40 years of age.
Keywords: Malignant Melanoma, Choroid, Retinal Detachment, Ultrasou-nd, Flourescein Angiography
|How to cite this article:|
Manohar S, Ramakrishnan R, Das TP, Namperumalsamy P. Malignant melanoma of choroid. Indian J Ophthalmol 1991;39:70-3
| Introduction|| |
Malignant melanoma of the choroid is a rare disease in our country. Usually it presents in the 4th - 6th decades of life. The incidence of this tumour is 0.02 - 0.06% . In this communication, we report four cases of malignant melanoma of the choroid with typical clinical, investigative and histo pathological features. Even though this tumour is relatively rare and is less often reported, the tumour has to be borne in mind in eyes with secondary detachment. There is one report of malignant melanoma of the choroid which was misdiagnosed and treated as a case of primary open angle glaucoma in India  .As such, malignant melanoma of the choroid is not very often reported .
| MATERIALS & METHODS|| |
Over the past four years, we have clinically diagnosed six cases of malignant melanoma of choroid supplemented with the trans-illumination test, flourescein angiography and ultrasonography. There were two males and four females. The tumour in all the cases was advanced but there were no signs of metastasis. Two of these patients were below 40 years of age and two patients were referred as cases of retinal detachment. The involved eye was enucleated in four female patients who agreed for such treatment. The case report of these four patients are reported briefly.
| Case reports|| |
Case No. 1:
A sixty five year old female patient was referred to our hospital with history of gradual painless decrease of vision in the right eye of one and a half months duration which was diagnosed as retinal detachment. On examination, the anterior segment of both eyes were normal. Visual acuity in the right eye was hand movements and 6/6 in the left eye. Intra ocular pressures were within normal limits in both eyes. Fundus examination with the binocular indirect ophthalmoscope showed a pigmented solid mass in the nasal side of the optic disc and secondary retinal detachment over the tumour extending inferiorly upto and involving the macula in the right eye. There was no abnormality of the iris and ciliary body. Fluorescein angiography showed double circulation in the early arterio-venous phase, multiple punctate hyperfluorescent patches over the tumour mass, known as hot spots in the mid phase, and diffuse hyperfluorescence over the tumour mass in the late phase . Ultrasonically using a hand-held 10 Mhz focussed transducer, the tumour appeared as a well circumscribed solid mass with areas of acoustically quiet zone, and orbital shadowing [Figure - 1] and a typical tall spike with anglekappa in A-Scan associated with retinal detachment . The transillumination test was positive.
On general examination, there was no hepatomegaly or any distant metastasis. Liverfunction tests were normal. Based on these findings the diagnosis of malignant melanoma of the choroid was made. The eye was enucleated with the consent of the patient.
Pathologically, gross examination of the cut section of the eye ball showed a greyish-brown, mushroom shaped mass from the choroid, measuring about 6 x 4 x 4 mm situated on the nasal side. Microscopically, it was a cellular tumour composed of epitheloid and spindle cells, suggestive of mixed variety. There were moderate to-heavy pigments in the tumour cells [Figure - 2][Figure - 3].
Case No. 2:
A 35 year old female came to our hospital one year ago with history of painless decrease of vision in the right eye of one month duration. Her vision in the RE was 6/60 and 6/6 in the LE. Intraocular pressure was within normal limits in both eyes. On examination, there was naevus on the skin around the eyeball and on the right side of the cheek and there was associated conjunctival melanosis. The iris showed a hyperpigmented patch in the inferior quadrant, suggestive of naevus of Ota. The ciliary body was normal. Fundus examination of the right eye showed an elevated brownish mass inferior to the disc more towards the nasal side with secondary retinal detachment involving the inferotemporal quadrant. The left eye fundus was normal. There was no hepatomegaly or any distant metastasis. Ultrasonography [Figure - 4] and flourescein angiography showed features of malignant melanoma of the choroid.-She was advised to have p32 uptake study. But she didn't turn up for one year. After a year she came to the hospital with severe pain and complete loss of vision in the RE. Her vision at that time was only perception of light. She had severe corneal edema, hyphaema and a fixed pupil with vitreous haemorrhage. Intra ocular pressure was 60 mm of Hg. The eye was enucleated at this stage.
Pathologically gross examination of the eye ball showed an extensive brownish mass involving the whole of the eye extending from the choroid upto the lens, associated with vitreous haemorrhage. Histopathological examination revealed both spindle and polygonal cells suggestive of mixed type of malignant melanoma of the choroid.
Case No. 3:
A 33 year old female was referred to us as a case of retinal detachment in the right eye. On examination, her anterior segment in both eyes were normal. Vision in the right eye was HM and in the left eye was 6/6. Intra ocular pressure in both eyes were normal. Fundus examination of the right eye showed a brownish elevated choroidal mass just temporal to the disc with tumour retinal detachment inferiorly involving the macula. Flourescein angiography was typical of malignant melanoma of choroid with double circulation in early phase, hot spots in mid-phase and persistence of fluorescene in the very late phase [Figure - 5][Figure - 6][Figure - 7]. Contact ultrasonography shoved a solid homogenous choroidal mass with acoustic hollowing and orbital shadowing [Figure - 8]. There was no clinical signs of metastasis and liver function tests were normal. She was not willing for enucleation of the right eye immediately. Four and a half months later when she reported back, the tumour was more enlarged ophthalmoscopically and confirmed with ultrasonography [Figure - 9]. There was still no evidence of metastasis. At this time, the right eye was enucleated, which showed a large choroidal mass with overlying scleral pigmentation. Pathologically it was found to be a mixed malignant melanoma of the choroid.
Case No. 4:
A 54 years old female reported to our hospital with decrease of vision in the right eye of two months duration. On examination, the anterior segment in both eyes were normal. Vision in right eye was FCF and 6/6 in LE. Intra Ocular pressures in both eyes were normal. Fundus examination of the right eye showed an elevated brownish mass in the inferonasal quadrant with secondary detachment in the inferotemporal quadrant involving the macula. Ultrasonography and fluorescein angiography was typical of malignant melanoma of the choroid. There was no distant metastasis and liver function tests were normal. Enucleation was done. The cut section showed a large choroidal tumour with retinal detachment [Figure - 10]. Pathologically, the tumour was a cellular tumour with epitheloid cells.
| Discussion|| |
Malignant melanoma of the choroid is relatively rare in our country. Because of the relative rarity of the condition, this is not often suspected till the tumour is large enough to cause secondary retinal detachment. Even then it is not unlikely to be wrongly diagnosed as it happened in two of our cases who were referred as cases of primary retinal detachment. From time to time reports of malignant melanoma of the choroid  are reported in Indian literature. We reported one case in 1985 . Patients seen since then are reported in this communication.
Malignant melanoma of choroid is a disease of the 5th to 6th decades. In two of our patients, it was found in the third decade and both were females. The patients presenting .with macular edema and solid retinal detachment should be carefully examined for the presence of melanoma of the choroid, so that this can be treated early. The tumour usually arises from the outer layer of the choroid, breaks through the elastic layer of Bruch's membrane where it forms a collar-stud appearance as present in one of our case [Figure - 2] and later produces a solid retinal detachment. Failure of early diagnosis leads to extra ocular spread and serious complications and extensive surgical procedures. P 32 uptake study is one of the sophisticated study to diagnose suspicious cases of malignant melanoma with 80% accuracy though this is an invasive procedure. But this differentiates malignant melanoma of the choroid from haemangioma of the choroid and haemorrhagic retinal detachments. However, this could be done only in a few selected centres and is not always possible. Amongst the noninvasive procedures in addition to direct ophthalmoscopic visualization especially with binocular indirect ophthalmoscope, ultrasonography remains an important non-invasive diagnostic tool. The ultrasonic characters of malignant melanoma of the choroid are well known. Both B scan and A scan are useful, but we found the former easier to interpret. The B scan features of melanoma of the choroid are acoustic hollowing of the tumour, choroidal excavation and orbital shadowing (Ref - Coleman) due to homogenicity of the tumour. These are not seen in haemangioma of the choroid and haemorrhagic macular detachments. Even in cases where the tumour is seen ophthalmoscopically it is often useful to do ultrasonography to monitor the growth of the tumour in suspicious cases.
The treatment of the tumour at the stage, we saw our patients, is enucleation. But seen early, the tumour can be treated or operated upon.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10]
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