|Year : 1996 | Volume
| Issue : 3 | Page : 165-166
Malignant melanoma of conjunctiva with xeroderma pigmentosa - a case report
Chaya Mehta, CN Gupta, Malavika Krishnaswamy
M S Ramaiah Medical College, Bangalore, India
M S Ramaiah Medical College, Bangalore
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mehta C, Gupta C N, Krishnaswamy M. Malignant melanoma of conjunctiva with xeroderma pigmentosa - a case report. Indian J Ophthalmol 1996;44:165-6
|How to cite this URL:|
Mehta C, Gupta C N, Krishnaswamy M. Malignant melanoma of conjunctiva with xeroderma pigmentosa - a case report. Indian J Ophthalmol [serial online] 1996 [cited 2020 Feb 21];44:165-6. Available from: http://www.ijo.in/text.asp?1996/44/3/165/24578
Xeroderma Pigmentosa is a rare autosomal recessive disease. It is seen in all races worldwide and has equal sex incidence. It is characterized by skin changes and neoplasia in exposed parts of the body due to UV radiation induced mutations. Malignancy in outer eye usually occur in second decade of life, the common tumours being basal cell and squamous cell carcinomas. Presented here is a rare and unusual case of xeroderma pigmentosa with malignant melanoma of conjunctiva in a 8 year old girl. Melanomas of conjunctiva, as such, are rare in children.
| Case report|| |
An eight year old girl diagnosed to have xeroderma pigmentosa was referred to us for ocular examination on 30 August 1993. The only offspring of a first degree consanguineous marriage, she had a history of freckles on exposed parts of the body including eyelids since the age of 3 months. She had no other contributory non-ocular finding. On examination, she was found to have severe photophobia. There was no signs of intraocular inflammation. Her vision, intraocular pressure (IOP) and fundus were normal. She was advised dark glasses, methyl cellulose eye drops and close follow-up.
One month later, she presented with a pigmented lesion on lower half of conjunctiva of the right eye extending from medial to lateral canthus and from the limbus inferiorly upto the fornix. The lesion was flat and soft, had a smooth surface and was immovable in places. Palpebral conjunctiva and eyelids were free of the lesion. Rest of the ocular examination was unremarkable. She was advised B-scan and excision biopsy which was refused by her parents. Two months later she returned with eccentric proptosis with eyeball pushed up [Figure - 1]. There was conjunctival chemosis. The lesion now extended to the upper nasal conjunctiva as well as 3 to 4 mm into the lower nasal quadrant of
the cornea. The pigmented lesion was raised 5 to 6 mm from the surface. It was nodular, soft and immovable iri places. Vision was reduced to 6/60 (induced astigmatism). The fundus and IOP were normal; ocular movements were restricted. The left eye and adnexa were normal. Except for freckles on exposed parts of the skin, detailed systemic examination was unremarkable. B scan showed growth in inferior quadrant of the orbit indenting the globe and extending posteriorly along the floor of the orbit [Figure - 2]. CT scan revealed the mass to be extending to the lateral rectus muscle and lacrimal glands laterally and lacrimal sac medially. Radiologically, a differential diagnosis of lymphoma or malignant melanoma was made. Blood, bone marrow and biochemical studies revealed no abnormalities.
There was no clinical or radiological evidence of metastasis.
She underwent orbital exenteration. Histopathological examination showed tumour cells with moderate pleomorphism, vesicular hyperchromatic nuclei, round to oval and spindle shaped cells with brown-black pigment in cytoplasm and few tumour giant cells [Figure - 3], pointing to a diagnosis of malignant melanoma. The tumour had infiltrated the extra-ocular muscles. Surgical margins and optic nerve were free of the tumour. On the second post-operative day patient developed disseminated intravascular coagulation and despite all measures expired.
| Discussion|| |
Xeroderma pigmentosa is characterized by photosensitivity, pigmentary changes, premature skin aging, photophobia, neoplasia and abnormal DNA repair to UV radiation induced damage due to defective endonuclease activity. Skin changes are noticed between 12 to 36 months in 75% of cases, but may rarely appear even earlier as in this case (3 months). Parts of the eye exposed to the sun are at greater risk and eyelids are involved in 80% of the cases. Photophobia is the usual presenting symptom. Commonly seen serious ocular complications include atrophy of the lower lid, conjunctival drying and shrinkage, decreased tear secretion, telangiectasia and ankyloblepharon. Exposure keratitis, vascularization, ulceration and nodular dystrophy also occur. Anterior uveitis follows corneal inflammation and actinic damage. Fundus lesions do not occur due to lack of exposure to UV light. Basal cell carcinoma and squamous cell carcinoma of lids and cornea (limbus) occur frequently. Melanomas of the skin are known to occur in xeroderma pigmentosa but are rare on the conjunctiva.
Conjunctival melanoma itself is a rare tumour, usually seen in middle age. It accounts for only 5% of melanomas in a large series of ocular melanomas. Melanomas in children are extremely uncommon. Duke-Elder mentions only 5 cases of malignant melanoma recorded in children between 1894-1937. Extensive literature review revealed reports of only 5 cases of conjunctival melanomas in children in the last 50 years. A case of malignant melanoma of conjunctiva in a child in a series of xeroderma pigmentosa patients was reported by Mortada in 1968 as mentioned by Duke Elder. To our knowledge this is the second such reported case.
Most cases of Xeroderma pigmentosa which begin in early childhood reach tumour stage and are fatal by 20 years of age due to multiple metastasis of squamous cell carcinoma and melanoma, infection or neurological complications. Exceptionally, growth may be so rapid and extensive that the entire conjunctival sac including both the fornices may be filled, cornea covered and globe hidden in neoplastic tissue. Our patient showed similar rapid growth. Prognosis in terms of metastasis is determined by tumour thickness. Using 0.8 mm thickness as a cut off point to predict the risk of metastasis, our patient though expected, did not show any clinical or radiological evidence of systemic metastasis. The treatment of malignant melanoma of the conjunctiva is surgical excision. Total eradication of the tumour at an early stage carries the best prognosis.
| References|| |
Osterlind A. Trends in incidence of ocular malignant melanoma in Denmark (1943-1982). Int J of Cancer 40:161-164, 1987.
Duke-Elder, System of Ophthalmology, Vol. VIII, Part-2, St. Louis, C.V. Mosby, 1965, pp 219-232.
Mcdonell J M, Carpenter J D, Jacobs P, Wan W L, et al. Conjunctival Melanotic lesions in children. Ophthalmology 96:986-993, 1989.
Duke Elder, System of Ophthalmology, Vol XIII, Part-1, St. Louis, C V Mosby, 1974, p 449.
Duke Elder, System of Ophthalmology, Vol. VIII, Part-2, St. Louis, C V Mosby, 1965, p 1222.
Felberg R, Maclean I W, Zimmerman L E. Malignant melanoma of conjunctiva. Human Pathology 16:136-143, 1985.
[Figure - 1], [Figure - 2], [Figure - 3]