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Cases reported "Melanosis"

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1/20. iris mammillations as the only sign of ocular melanocytosis in a child with choroidal melanoma.

    An 8-year-old girl had visual loss in her left eye over 2 months. Ocular examination showed that visual acuity was counting fingers in the left eye. The left iris was moderately pigmented and thickened with numerous confluent, dome-shaped elevations on its surface, consistent with iris mammillations arising from ocular melanocytosis. There was total retinal detachment and an inferiorly located large amelanotic choroidal mass compressing the optic nerve. A specimen from a fine-needle aspiration biopsy showed spindle and epithelioid melanoma cells. The eye was enucleated. Pathologic examination showed that the bland melanocytes comprising the anterior border layer of iris formed focal aggregates, corresponding to the iris mammillations observed clinically. The uvea was diffusely thickened. Arising from the posterior choroid and obscuring the optic nerve head was a moderately pigmented spindle and epithelioid cell choroidal melanoma with diffuse lymphocytic infiltration and high mitotic activity. This case demonstrates that iris mammillations can be the initial manifestation of ocular melanocytosis in the absence of scleral pigmentation.
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ranking = 1
keywords = choroid
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2/20. Ocular melanocytosis and melanoma.

    The hypothesis that ocular melanocytosis is a precancerous condition that may lead to a choroidal melanoma should be seriously questioned for the following reasons: The incidence of malignant degeneration in a hyperpigmented eye is unknown and overreported. If the hypothesis were correct a bilateral melanoma would occasionally occur in patients with bilateral melanosis. No such case has been reported. In patients with unilateral melanosis the blue, unaffected eye may also develop a melanoma. A 67-year-old white woman with one dark and one blue eye provided the first such instance, although previous cases may not have been reported if their histologic picture was not unusual. The incidence of melanoma in our patients with unilateral melanosis was rare compared with the many melanomas developing in normal pigmented eyes (4/418). I found no statistically significant difference in the incidence of choroidal melanomas originating in the hyperpigmented or in the blue eye in patients with unilateral ocular melanocytosis.
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ranking = 0.28571428571429
keywords = choroid
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3/20. Two discrete choroidal melanomas in an eye with ocular melanocytosis.

    We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.
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ranking = 1.2857142857143
keywords = choroid
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4/20. Acquired anterior ocular melanocytosis following cataract extraction.

    The right eye of a 59-year-old man was the subject of extracapsular cataract extraction and posterior chamber lens implantation. He gradually developed superior sectoral scleral and diffuse iris hyperpigmentation within 7 years postoperatively. The iris was also uniformly thickened. He sustained 20/20 visual acuity in the pseudophakic eye. Despite marked pigmentation of the angle, his right intraocular pressure remained within normal limits. We use the term acquired anterior ocular melanocytosis because the pigmentary changes were confined to the anterior segment and the choroid was not involved. We conclude that surgical injury in the form of cataract extraction can induce episcleral/scleral hyperpigmentation and iris hyperchromia.
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ranking = 0.14285714285714
keywords = choroid
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5/20. Giant congenital melanocytic nevi in a patient with brain structural malformations and multiple lipomatosis.

    We present a 9-year-old boy diagnosed from birth with giant congenital melanocytic nevi. He had central structural brain malformations of hemimegalencephaly of the right frontotemporal lobe and left occipitoparietal lobe, choroid plexus hypertrophy, and a Dandy-Walker variant. In addition, he developed multiple lipomatoses. These lesions were cutaneous except for two at the cerebellopontine angles, which were present from birth. This patient represents a rarely documented example of two histopathologies resulting in serious complications. The diagnostic issues and histopathologic process are discussed.
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ranking = 0.14285714285714
keywords = choroid
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6/20. Primary choroidal melanoma in phakomatosis pigmentovascularis IIa.

    PURPOSE: To describe a previously unreported association between phakomatosis pigmentovascularis (PPV) IIa and primary choroidal melanoma. DESIGN: Case series. PARTICIPANTS: Three patients with PPV type IIa and choroidal melanoma. RESULTS: nevus flammeus was present unilaterally in each patient, involving the hemiface, the hemithorax, or both. Ocular melanocytosis also was present unilaterally in all patients involving the sclera, the iris, the choroid, the distribution of the trigeminal nerve, or a combination thereof. Renal cysts were noted in patient 1, and a nevus anemicus of the neck was noted in patient 3. A unilateral choroidal melanoma was detected in each of these 3 patients, in each patient involving the same eye in which melanocytosis had been seen. No metastases were found in any patient. CONCLUSIONS: Clinicians should be aware of the possibility that PPV, and in particular that occurring in patients with ocular melanocytosis, can be associated with choroidal melanoma.
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ranking = 1.2857142857143
keywords = choroid
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7/20. Primary acquired melanosis of the conjunctiva.

    BACKGROUND: Primary acquired melanosis (PAM) presents as a unilateral patchy area of conjunctival pigmentation mostly found in middle-aged or elderly white patients. Because PAM has the potential of becoming malignant, it is important to recognize PAM and to rule out other causes of pigmented lesions. The presence or absence of atypia is helpful in determining the potential for malignancy, because PAM without atypia is usually benign, whereas PAM with atypia may convert into a conjunctival melanoma. If atypia is present, the presence or absence of epithelioid cells and the pattern of intraepithelial growth are the main factors in determining the likelihood of neoplastic transformation. However, atypia can only be determined with histopathologic examination. Therefore, a biopsy is usually recommended. CASE REPORT: A 72-year-old white man presented for his annual examination with no visual complaints. His ocular history was remarkable for early cataracts and for a choroidal nevus. Slit lamp examination found a large dark area of elevated conjunctival pigmentation at the medial canthus that extended onto the superior bulbar conjunctiva and superior palpebral conjunctiva in the left eye. No conjunctival pigmentation had been noted previously. Results of a biopsy indicated primary acquired melanosis with atypia. The lesion was surgically excised, and the conjunctiva was reconstructed with an amniotic membrane graft. The patient was subsequently treated with topical 5-fluorouracil chemotherapy. There have been no signs of recurrence to date after his treatment. CONCLUSION: Because of the potential for malignancy, biopsies of all cases of primary acquired melanosis are indicated. If atypia is present, treatment options include local excision, cryotherapy, and topical chemotherapy.
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ranking = 0.14285714285714
keywords = choroid
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8/20. Two discrete uveal melanomas in a child with ocular melanocytosis.

    OBJECTIVE: To describe a case of two uveal melanomas in a child with mild ocular melanocytosis. methods: A 6-year-old girl was followed for 5 years with an ill-defined, slowly enlarging presumed choroidal nevus in the postequatorial fundus. Ocular oncology evaluation revealed mild sectorial scleral and uveal melanocytosis and an episcleral sentinel vessel superotemporally. Two discrete uveal melanomas were present. In the circumpapillary and macular region, tumor 1 was diffuse at 9.0 mm in base and 4.1 mm in thickness and with overlying subretinal fluid. In the ciliary body, tumor 2 was discovered by transillumination and was 6.0 mm in base and 2.2 mm in thickness. Enucleation was performed. RESULTS: Histopathologic analysis disclosed two discrete uveal melanomas in a bed of diffuse mild uveal melanocytosis. Tumor 1 was a mixed, predominantly epithelioid cell melanoma with active mitotic figures, and tumor 2 was a mixed, predominantly spindle cell melanoma. The choroid between the melanomas showed only benign, dendritic melanocytes consistent with melanocytosis. There was no extrascleral extension. CONCLUSIONS: Ocular melanocytosis can predispose to one or multiple uveal melanomas. Lifetime ophthalmic monitoring of affected patients is warranted.
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ranking = 0.28571428571429
keywords = choroid
(Clic here for more details about this article)

9/20. melanosis of the choroid plexua.

    A unique case of melanosis of the choroid plexus in a 74-year-old man who died of hypertensive cardiovascular disease is described. The cerebral lesion did not cause symptoms. The proposed pathogenesis is the conversion of choroidal epithelial lipofuscin into melanin. Our findings also may explain the origin of a previously reported primary malignant melanoma of the choroid plexus and of black cortical adenomas of the adrenal gland.
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ranking = 1
keywords = choroid
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10/20. Isolated unilateral hemichoroidal melanosis.

    Ocular melanosis is usually diffuse but may be sectoral. An unusual case of isolated unilateral hemispheric choroidal melanosis is presented in a patient with uveitis. Details of the embryologic basis for this condition remain speculative.
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ranking = 0.71428571428571
keywords = choroid
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