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Case Reports and Small Case Series
February 1999

Conjunctival Melanoma Associated With Extensive Congenital Conjunctival Nevus and Split Nevus of Eyelid

Arch Ophthalmol. 1999;117(2):268-269. doi:

Primary acquired melanosis and acquired conjunctival nevi are recognized to be precursors of conjunctival melanoma; however, it has not been known that congenital conjunctival nevi can be a precursor of conjunctival melanoma. We report a case of fatal conjunctival melanoma in a 72-year-old Japanese man. He was born with a large congenital black mole involving the right upper and lower eyelids and conjunctiva. He had a brown-black nodule in his inferior bulbar conjunctiva and the right parotid and submandibular lymph nodes showed marked swelling. Chemotherapy was performed to clinically diagnose metastatic conjunctival melanoma, but he died 5 months later due to systemic metastasis. Autopsy revealed conjunctival melanoma associated with extensive conjunctival nevi and split nevus of the eyelid.

Seventy-five percent of conjunctival melanomas arise in association with primary acquired melanosis.1 Histologic evidence of nevus or a history of a conjunctival lesion dating to childhood may be discovered in 20% to 30% of patients with conjunctival melanoma.1

Report of a Case.

A 72-year-old Japanese man had a large pigmented area around his right eye since birth, including the eyelid and conjunctiva. The black tone became more intense in the patient's first decade of life and had not changed since then. He first visited our institute on March 15, 1991, with his right vision obstructed by a black-brown nodule in the inferior bulbar conjunctiva.

The visual acuity and intraocular pressure showed no abnormalities. The right eyelid, conjunctiva, and caruncle were black, and the inferior aspect of the bulbar conjunctiva had a black elevated nodule. Small cystic lesions were present in other areas of the bulbar conjunctiva (Figure 1 and Figure 2). The ocular media, fundus, and optic nerve head showed no abnormalities. The right aspect of the parotid and submandibular lymph nodes showed marked swelling that was hard and did not reduce in size despite antibiotic therapy.

Figure 1.
A 72-year-old Japanese man with a brown-black nodule in the inferior aspect of the bulbar conjunctiva in the right eye. The eyelids and bulbar conjunctiva had wide areas of pigmentation.

A 72-year-old Japanese man with a brown-black nodule in the inferior aspect of the bulbar conjunctiva in the right eye. The eyelids and bulbar conjunctiva had wide areas of pigmentation.

Figure 2.
Small, cystic lesions were present in other areas of the bulbar conjunctiva.

Small, cystic lesions were present in other areas of the bulbar conjunctiva.

Metastatic malignant melanoma of the conjunctiva was clinically diagnosed. Several treatment options, including orbital exenteration, radiation therapy, and chemotherapy, were offered to the patient and the family. They desired chemotherapy only. The patient's condition deteriorated; he died 5 months later due to systemic metastasis of malignant melanoma.

Autopsy revealed a mass of conjunctival melanoma, composed of large epithelioid cells, in the bulbar conjunctiva inferior to the limbus (Figure 3, Figure 4, and Figure 5). Adjacent to the nodule, a narrow zone of pigmented cells, possibly melanophages, was present in the episclera (Figure 3, Figure 4, and Figure 6), that was extensively observed around the globe. Nests of cystic compound nevus (Figure 6) were extensively present in other areas of the bulbar conjunctiva. The palpebral conjunctiva and the intraocular components showed no abnormalities. Extensive intradermal nevus composed of small round cells (Figure 7 and Figure 8) were present on the skin of the eyelids. The nevus cells had features of congenital cutaneous nevus, with deep penetration in the skin and maturation at the base.

Figure 3.
A gross photographic view of the tumor located in the inferior bulbar conjunctiva.

A gross photographic view of the tumor located in the inferior bulbar conjunctiva.

Figure 4.
The tumor nodule is located in the conjunctiva (hematoxylin-eosin, original magnification ×100).

The tumor nodule is located in the conjunctiva (hematoxylin-eosin, original magnification ×100).

Figure 5.
The tumor is composed of moderately pigmented, markedly pleomorphic, large epithelioid cells (hematoxylin-eosin, original magnification ×400).

The tumor is composed of moderately pigmented, markedly pleomorphic, large epithelioid cells (hematoxylin-eosin, original magnification ×400).

Figure 6.
The bulbar conjunctiva has extensive compound nevi with cysts (hematoxylin-eosin, original magnification ×100).

The bulbar conjunctiva has extensive compound nevi with cysts (hematoxylin-eosin, original magnification ×100).

Figure 7.
The skin of the eyelid, with foci of intradermal nevus present in the dermis (hematoxylin-eosin, original magnification ×100).

The skin of the eyelid, with foci of intradermal nevus present in the dermis (hematoxylin-eosin, original magnification ×100).

Figure 8.
The nevus is composed of moderately pigmented, small round cells (hematoxylin-eosin, original magnification ×400).

The nevus is composed of moderately pigmented, small round cells (hematoxylin-eosin, original magnification ×400).

Comment.

Large congenital cutaneous nevi are associated with a risk of developing melanoma since childhood.1 Marghoob et al2 prospectively followed up 92 patients (median age, 3 years) with large congenital cutaneous nevi for an average of 5.4 years and found that the cumulative 5-year, life-table risk for the development of malignant melanoma was 4.5%. The calculated standardized morbidity ratio (adjusted relative risk) was 239-fold.2

It is known that congenital blue nevi of the eyelids and orbit can lead to eyelid and orbital melanoma. Tellado et al3 studied 21 orbital melanomas. They found that 90% of patients had an associated blue nevus and 47.5% of patients had some form of congenital melanosis.

Acquired conjunctival nevi are recognized to be precursors of conjunctival melanoma, but the risk of congenital conjunctival nevi for the development of melanoma is unclear.1

Gerner et al4 analyzed 343 surgically removed conjunctival nevi. Six cases that recurred during observation were originally classified as compound nevi. Among them, 1 recurred as malignant melanoma. They mentioned that in stationary and indolent cases in adults, excision as a routine procedure was unnecessary, since the risk of evolution of conjunctival nevi into melanoma was very low. In Japan, malignant melanoma of the conjunctiva is rare, with an estimated 6 to 7 cases per year.5

In our patient, the history indicates that large nests of nevus cells involving bulbar conjunctiva, caruncle, and eyelid skin had been present since birth. Generally speaking, large nevi are associated with a risk of developing melanoma.6 The many nevus cells of this patient probably increased the risk of developing melanoma, a condition that should be rare in Japanese patients.

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Article Information

This study was supported in part by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY (Dr Folberg).

Reprints: Karen Gehrs, MD, Department of Ophthalmology and Visual Sciences, University of Iowa Hospitals and Clinics, Iowa City, IA 52242 (e-mail: karen-gehrs@uiowa.edu).

Reprints: Yoshiaki Nawa, MD, Department of Ophthalmology, Nara Medical University, Kashihara, Nara 634-0813, Japan.

References
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Folberg  R Melanotic lesions of the conjunctiva. Spencer  THedOphthalmic Pathology An Atlas and Textbook. 4th ed. Philadelphia, Pa WB Saunders Co1996;125- 155
2.
Marghoob  AASchoenbach  SPKopf  AWOrlow  SJNossa  RBart  RS Large congenital melanocytic nevi and the risk for the development of malignant melanoma: a prospective study. Arch Dermatol. 1996;132170- 175Article
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Tellado  MSpacht  CSMcLean  IWGrossniklaus  HEZimmerman  LE Primary orbital melamomas. Ophthalmology. 1996;103929- 932Article
4.
Gerner  NNorregaard  JCJensen  OAPrause  JU Conjunctival naevi in Denmark, 1960-1980: a 21-year follow-up study. Acta Ophthalmol Scand. 1996;74334- 337Article
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Kaneko  A Incidence of malignant melanoma of the eye in Japan, 1977-1979. Nippon Ganka Gakkai Zasshi. 1982;86332- 355
6.
Grob  JJGouvernet  JAymar  D  et al.  Count of benign melanocytic nevi as a major indicator of risk for nonfamilial nodular and superficial spreading melanoma. Cancer. 1990;66387- 395Article
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