|Year : 1993 | Volume
| Issue : 1 | Page : 26-29
Malignant melanoma of the conjunctiva
AK Grover1, A Rastogi1, K Uma Chaturvedi2, LD Sota1, AK Gupta1
1 Guru Nanak Eye Centre, New Delhi, India
2 Maulana Azad Medical College , New Delhi, India
A K Grover
12 Mata Sundri Lane, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Grover A K, Rastogi A, Chaturvedi K U, Sota L D, Gupta A K. Malignant melanoma of the conjunctiva. Indian J Ophthalmol 1993;41:26-9
Malignant Melanoma of the conjunctiva is uncommon. In pigmented races it is rare. We have come across only two case reports from India in the last three decades , Malignant Melanoma of the conjunctiva poses a challenge to the ophthalmologist because of difficulty in the clinical differentiation of early malignancies from primary acquired melonosis (PAM), absence of definitive guidelines of management and the difficulties in making an unequivocal histological diagnosis of malignancy, as the microscopic diagnosis lags behind the clinical diagnosis .
We are presenting two cases, one a typical case of malignant melonoma, while the second highlights the difficulties mentioned. It also brings out the recent trend towards conservative management.
A 70 year old female presented with history of a large pigmented swelling in the left eye which had grown rapidly during the last three months. There was no history of anorexia or weight loss. The general physical and systemic examinations were non contributory.
Examination of the left eye revealed a large pigmented mass 1.5 x 2cm in diameter occupying the lateral 2/3 of the left lower lid. It was projecting inwards from the palpebral conjunctiva and causing eversion of the lower lid [Figure - 1]. The skin was adherent over the swelling. Both the bulbar and palpebral conjunctiva showed widespread melanoma. The superior limbus was deeply pigmented. Visual acuity with aphakic glasses was 6/18. There was a restriction of abduction of the left eye.
The right eye had a visual acuity of 6/60 due to an immature senile cataract. There was no evidence of pigmentation in this eye. The case was diagnosed as malignant melanoma of the conjuctiva and a total exenteration was carried out. The defect was covered with a partial thickness skin graft taken from the thigh. Histopathology revealed a frankly malignant melanoma of the conjunctiva with features of pleomorphism [Figure - 2]. The upper palpebral conjunctiva showed increased junctional activity. Focal deposits were present in the anterior corneal layers. The rest of the eyeball including the optic nerve and the orbital fat were found to be free of malignancy.
A 45 year old female presented with a history of a rapidly growing pigmented growth on the inner aspect of the right eye for the last one year. She had a small pinhead size asymptomatic naevus at the same site since childhood. The patient did not suffer from any loss of weight or appetite. Examination of the right eye revealed a mulberry like pigmented growth 1 x 1.5cm in size arising from the caruncle and plica semilunaris and extending to the bulbar conjunctiva [Figure - 3]. The height of the tumour was about 0.5cm. The tumour was firm, non tender, and did not bleed on touch. It was associated with extensive and patchy conjunctival melanosis involving the palpebral and bulbar conjunctiva and fornices. Both upper and lower puncta were spared. The eye was otherwise normal with a corrected visual acuity of 6/6. The left eye showed no abnormality. Systemic examination and investigation showed no evidence of metastasis. Urine was negative for melanin. Wide excision of the medial aspect of both upper and lower lids including the mass with a 5mm clear zone beyond the area of conjunctival pigmentation was carried out under general anaesthesia [Figure - 4]. The lacrimal sac, canaliculi and puncta had to be sacrificed. Repair of the defect was carried out by grafting of buccal mucus membrane to replace the bulbar conjunctiva, while a tarsoconjunctival flap from the upper lid was used to repair the large defect in the lower lid and the medial canthus. The medial ends of the upper and lower lids were anchored to the medial periosteum. The skin could be apposed by direct suturing. Ptosis and ocular motility restriction seen in the early post operative period recovered over a period of two months, leaving-only a moderate restriction of motility in abduction.
Histopathology revealed circumscribed nests of pigmented naevus cells in the subepithelium [Figure - 5]. There was moderate mitotic activity and nuclear pleomorphism. Some of the cells showed large vesicular nuclei with prominent nucleoli [Figure - 6]. Possibility ofa compound naevus with pseudosarcomatous change or a dysplastic naevus was suggested by the histopathologist, with the condition that in view of the recent increase in size, early malignant changes could not be excluded. The margins of the excised mass were free from evidence of any malignancy. Over a follow up period of 9 months the patient showed no evidence of recurrence or metastasis after which she was lost to follow up. The patient came 26 months after surgery with swelling of both lids in the operated eye for 4 months. She was found to have a recurrence with two large pigmented nodules arising from the fomices and bulging through the lids [Figure - 7]. Clinically there was no evidence of orbital involvement or systemic metastasis.
Ultrasonography of the abdomen revealed evidence of metastasis in the liver. The eye was exenterated. Histopathology revealed a frankly malignant melanoma involving retro-ocular tissue and peri-ocular tissue. The conjunctiva lining the lids and the layers of the eyeball were free. The posterior most part of the orbital contents showed infiltration by occasional cluster of melanoma cells.
| Discussion|| |
Classical cases of conjunctival melanoma present no difficulties with regard to clinical diagnosis. The histological features are unequivocal and the alternatives in management are relatively clear cut. Case I typifies such a classical case. The clinical diagnosis could be made easily by the history of a rapidly growing large pigmented mass in an elderly patient. Associated presence of widespread bulbar and palpebral melanosis suppported the diagnosis. The histological diagnosis was unequivocal. The need to carry out an exenteration was also not in doubt considering the size and extent of the malignancy.
The difficulty arises when the clinical and histological diagnosis suggest a malignancy but are inconclusive. The differentiation from an acquired melanosis without malignant change may be difficult. Case-II exemplifies the difficulties that may be encountered. The clinical features which suggested the diagnosis of malignant melanoma within an area of primary acquired melanosis (PAM) at initial presentation in this patient were:
a) History of rapid increase in size with episodes of bleeding from the mass in a middle aged patient.
b) Involvement of caruncle, plica and palpebral conjunctiva.
c) Presence of a pedunculated, elevated mass nearly 5mm in thickness.
The histopathology in case II did not give an unambiguous diagnosis of malignancy when it was initially excised
However, a number of features were suggestive of malignancy. The presence of increased junctional activity, moderate mitotic activity, nuclear pleomorphism and the presence of large nuclei with prominent nucleoli in some cells suggested the possibility of malignancy . The subsequent clinical course and histopathology after extentration following recurrerence confirmed malignant nature of the tumour.
The risk factors in conjunctival melanomas that have been reported to be associated with death are:
a) Involvement of palpebral and bulbar conjunctiva or caruncle.
b) Paucity of small polyhedral cells.
c) Moderate atypia.
d) A relative lack of inflammatory response.
All of these features were seen in case II and suggest a relatively bad prognosis. This was confirmed when the patient came back with a recurrence after 26 months inspite of a wide excision.
Primary acquired melanosis (PAM) is a lesion of dark pigmentation due to melanin. It is not present since birth and is not due to any local factors or systemic disease . It is generally accepted that PAM occurs during middle age and should not be diagnosed in children, just as a diagnosis of junctional naevus should be avoided in adults . Previously PAM was considered to be precancerous in all cases. Now it is definitely known that many cases remain benign. 75% of the malignant melanoma of conjunctiva arise from the primary acquired melanosis , When a pigmented lesion shows a recent rapid increase in size, when there are multiple lesions with no continuity between them, when the thickness of the lesion is more than 2mm, then it is suggestive of malignancy.
The pathologist looks for evidence of melanocytic hyperplasia in all cases of PAM. The presence of cytological atypia determine whether the lesion is benign or precancerous . When the lesion is frankly malignant the histopathology reveals anaplastic changes in the junctional zone of the epithelium. The cells tend to separate, become large and pleomorphic with prominent nuclei and nucleoli. Abnormally increased melanin may be seen in the cytoplasm. In many cases the histopathology alone may still not be diagnostic but when correlated with the clinical picture and in the presence oftumour cells i n the substantia propria these lesions should be considered malignant ,. Histological diagnosis may lag behind the clinical diagnosis. This was so in our patient where the rapid increase in size, the site of the lesion, and bleeding suggested malignancy, though the histology was inconclusive.
There has been a gradual shift in the management of cases of malignant melanoma of the conjunctiva.
The choice of treatmet is surgical excision where feasible, followed by close observation. We followed this line of treatment in case II. Unfortunately the patient did not come for regular follow up and reported very late after recurrence. While excising, particular emphasis needs to be placed on the margins of the tumour and a wide excision should be carried out, cryotherapy or laser therapy is indicated if the tumour is identified at the excised margin. Cryotherapy- ,, and laser ablation[ 14] kill melanocytes while preserving the substantia propria. Incompletely treated lesions may metastasize locally or distantly via the lymphatics. Radiotherapy has also been successfully tried in localized cases of melanomas. The patients must be followed up to look for recurrence of pigmentation, ipsilateral pre-auricular, submandibular, or cervical lymphadenopathy. Orbital exenteration may be required only in patients with massive orbital invasion by the tumor or in patients with widespread cancerous melanosis which are too extensive for excision  as was the case in case 1, or with recurrence as seen in case II. However, it is not known to enhance survival [13,.Once distant metastasis has occurred there is no effective treatment beyond palliation.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]