Primary orbital melanoma associated with oculodermal melanocytosis

A 29-year-old, otherwise healthy man presented with an approximately 5-months' history of left-sided exophthalmos. The patient had a bluish-black pigmentation on his left upper lid, and black pigmentation on the left conjunctiva and sclera, since birth. CT examination revealed a retrobulbar tumor in the left orbit, and the left medial rectus muscle was wider than the right one. The left orbital cavity and the superior orbital fissure were enlarged. All of the examinations were negative in respect of another primary tumor outside the orbit or of tumor dissemination. At surgery, a pigmented tumor was found and removed. The orbital tissues were darkly pigmented. Histological examination of the excised tumor showed it to be an encapsulated, circumscribed mass. The tumor was built up of fusiform and spindle-shaped cells containing black pigmentation, with very few mitoses and without necrosis. There has been no recurrence or metastasis in 3 years of follow-up. The consequences of the tumorous transformation of oculodermal melanocytosis as reflected in the published literature and the special clinical and histopathological characteristics of our case are discussed.     

Primary orbital melanoma associated with oculodermal melanocytosis

A 29-year-old, otherwise healthy man presented with an approximately 5-months' history of left-sided exophthalmos. The patient had a bluish-black pigmentation on his left upper lid, and black pigmentation on the left conjunctiva and sclera, since birth. CT examination revealed a retrobulbar tumor in the left orbit, and the left medial rectus muscle was wider than the right one. The left orbital cavity and the superior orbital fissure were enlarged. All of the examinations were negative in respect of another primary tumor outside the orbit or of tumor dissemination. At surgery, a pigmented tumor was found and removed. The orbital tissues were darkly pigmented. Histological examination of the excised tumor showed it to be an encapsulated, circumscribed mass. The tumor was built up of fusiform and spindle-shaped cells containing black pigmentation, with very few mitoses and without necrosis. There has been no recurrence or metastasis in 3 years of follow-up. The consequences of the tumorous transformation of oculodermal melanocytosis as reflected in the published literature and the special clinical and histopathological characteristics of our case are discussed.     

Ocular and oculodermal melanocytosis associated with uveal melanoma

Fifteen patients with ocular or oculodermal melanocytosis were found after reviewing 1210 cases of histologically proven uveal melanomas. The melanoma in each of these patients developed in the eye affected with ocular or oculodermal melanocytosis and not in the unaffected eye. In the one case of bilateral involvement with oculodermal melanocytosis, the patient developed the melanoma in the eye more affected with melanocytosis. In the only case of partial ocular melanocytosis, the melanoma developed in a sector of the eye affected with melanocytosis. A comparison of the prevalence of ocular or oculodermal melanocytosis in patients with uveal melanoma with the prevalence of ocular or oculodermal melanocytosis in the general population, implies that there is an increased incidence of uveal melanomas in patients with ocular or oculodermal melanocytosis.     

Glaucoma in oculodermal melanocytosis

Oculodermal melanocytosis (ODM) is a developmental disorder of the embryonic neural crest involving virtually all of the structures of the eye derived from this tissue. The authors examined 194 previously unreported Thai patients with ODM. Elevated intraocular pressures (IOPs) developed in the ipsilateral eye of 15 patients at initial examination and in 5 others during follow-up, for a total of 10.3% of the patients. Three patients had congenital or late congenital glaucoma, 14 had ocular hypertension or open-angle glaucoma, and 3 had acute angle-closure glaucoma. Five of the patients with open angles had acute pressure rises accompanied by anterior uveitis. Melanocytic hyperpigmentation of the anterior chamber angle was extensive in all patients except those with acute angle-closure glaucoma. There was no consistent correlation between the extent or density of angle pigmentation and the development of elevated IOP. A larger cup-to-disc ratio in the involved eye, presumably congenital, was present in an additional 19 patients. Glaucoma is common in eyes with ODM and may develop at any age. Patients with ODM and initially normal IOP should be examined at regular intervals. Patients with open angles and acutely elevated IOP should be examined carefully for signs of anterior uveitis and treated medically.     

Prevalence of ocular and oculodermal melanocytosis in Spanish population with uveal melanoma

Purpose

The aim of this study was to determine the prevalence of ocular and oculodermal melanocytosis (ODM) among patients with uveal melanoma (UM) in a Spanish population.

Methods

Retrospective review of the medical records of patients with ODM among patients with UM.

Results

Ten (11 eyes) of 400 patients (2.7%) with UM associated had ODM. The mean age at diagnosis of UM among patients with ODM was 62 years. One patient had bilateral tumours. UM was diagnosed during a routine-examination in two cases. All tumours were medium (7/11) or large (4/11) in size, with a mean maximum base of 13 mm and height of 7 mm. No patient had extraocular extension or metastatic disease at diagnosis. Enucleation was done in five cases and I-125-brachytherapy in six. The mean follow-up was 43 months. One patient died because of metastasis 2 years after enucleation; one patient is currently on treatment of systemic metastasis 11 years after.

Conclusions

ODM is more frequent in spanish population with UM than in American population. Despite the risk of UM in ODM, it is often diagnosed late when a conservative treatment is not indicated.     

Ocular and oculodermal melanocytosis

Thirty-three cases of ocular (27) or oculodermal (6) melanocytosis were reviewed to determine the ocular structures involved by the melanocytic hyperpigmentation. The hyperpigmentation was clinically documented to involve all quadrants of the eye in the majority of the subjects; however, nine persons had sectorial involvement. The choroid and episclera were involved diffusely or sectorially in all the subjects, and the anterior chamber angle and iris were involved in most cases. The conjunctiva, lens and optic disc were less frequently involved. Ten persons had a uveal malignant melanoma in the hyperpigmented eye, and in three of the cases the melanoma had arisen in the hyperpigmented sector. Melanocytic involvement of the trabecular meshwork was not clinically correlated with elevated intraocular pressure.     

Malignant melanoma of the optic nerve head in a case of oculodermal melanocytosis.

Malignant melanoma of the uveal tract, orbit, and brain have been reported to occur in patients with oculodermal melanocytosis. A 60-year-old Caucasian man with oculodermal melanocytosis developed a malignant melanoma of the optic nerve head in the left eye. This case is the first reported example of a malignant melanoma developing in the optic nerve associated with oculodermal melanocytosis. After presentation the patient refused surgery for 19 months and the progression of the tumour necessitated an exenteration of the orbit.     

Ocular melanocytosis, oculodermal melanocytosis and choroid melanomas

Among 837 cases of choroidal melanomas treated conservatively, 22 cases (2.69%) presented an ocular or oculodermal melanocytosis. This incidence is compared with the data of the literature. The role of melanocytosis on tumors' parameters and vital prognosis is studied.     

The fundus in oculodermal melanocytosis Is this a new observation?

An unusual case of bilateral oculodermal melanocytosis or the naevus of Ota is reviewed. In this condition, there is hyperpigmentation of ocular structures and the skin of the face. In this case, a pigmentary mottling of the fundus was observed using long wavelength light.     

Bilateral ocular melanocytosis with malignant melanoma of the choroid.

A woman with bilateral ocular melanocytosis developed a malignant melanoma of the choroid in one eye. The ocular melanotic hyperpigmentation, present since childhood, clinically involved the conjunctiva and episcleral and uveal tract of both eyes. To our knowledge this is only the second reported case of a uveal malignant melanoma in a patient with bilateral ocular melanocytosis. There is evidence that patients with ocular of oculodermal melanocytosis have an increased incidence of malignant melanoma of the uvea. It is possible that patients with bilateral ocular melanocytosis have a greater chance of developing of uveal malignant melanoma than do those with unilateral involvement.     

Choroidal melanoma growth patterns.

Thirty patients with choroidal melanoma in whom tumour thickness was found to increase on serial examination were retrospectively studied. Often when tumours started to increase in thickness, after a period of relative quiescence, the growth rate was relatively rapid. Increased tumour thickness was associated with increased tumour growth. During the phase of active tumour growth the height of the lesion tended to increase more than exponentially. Possible inadvertent biases in the selection of the patients studied prohibit wide-ranging conclusions from these data.