1/52. Choroidal melanoma with oculodermal melanocytosis in Hispanic patients. PURPOSE: To describe three Hispanic patients with oculodermal melanocytosis and uveal melanoma. METHOD: Case series. RESULTS: Three Hispanic patients with oculodermal melanocytosis and uveal melanoma underwent enucleation. The diagnosis of choroidal melanoma was confirmed by histopathologic examination. CONCLUSION: In the Hispanic population, uveal melanoma can occur in the presence of oculodermal melanocytosis. ( info) |
2/52. Partial unilateral lentiginosis with ocular involvement. Partial unilateral lentiginosis (PUL) is an unusual pigmentary disorder characterized by numerous lentigines grouped within an area of normal skin; the pigmented macules are often in a segmental distribution with a sharp demarcation at the midline. We report the first case of ocular involvement in a patient with this diagnosis. The patient, a 30-year-old Peruvian woman, had multiple brown macules on the left upper face in primarily a V1 and V2 distribution with a sharp demarcation at the midline of the forehead. The lesions first appeared near the hairline when she was 5 years of age, and then began to extend onto the face. She also had a discrete area of brown pigmentation on the left lateral bulbar conjunctiva. Because the patient had been previously diagnosed by several dermatologists as having either a speckled lentiginous nevus or a nevus of ota, we draw attention to the entity PUL and the possibility of ocular involvement. ( info) |
| PURPOSE: To report the development of a cavitary choroidal melanoma in a patient with nevus of ota and describe its magnetic resonance imaging (MRI) features. PATIENT: A 66-year-old man with right oculodermal melanocytosis and an ipsilateral choroidal cavitary melanoma was seen. diagnosis was suspected on the clinical, ultrasonographic and MRI findings. RESULTS: T1-weighted image showed a hyperintense solid mass containing hypointense cystic-like spaces delineated by hyperintense septa with respect to the vitreous. The reverse image was observed on T2-weighted images. A choroidal melanoma comprising epithelioid and spindle cells with multilocular cavities was documented histopathologically. CONCLUSIONS: There may be an association between cavitary melanoma and nevus of ota. Characteristic MRI findings could be helpful in the differential diagnosis of cavitary uveal melanoma. ( info) |
4/52. Neurocutaneous melanosis: a case of primary intracranial melanoma with metastasis. Neurocutaneous melanosis is a rare disorder characterized by the presence of large or multiple congenital melanocytic naevi and benign or malignant pigment cell tumours of the leptomeninges. Distant metastasis is unusual in primary leptomeningeal/intracranial melanomas. We present the case history of an adult male who had multiple primary intracranial melanomas associated with neurocutaneous melanosis (naevus of Ota) in the ophthalmic division of the left trigeminal nerve. Excision of the intracranial tumours was carried out in two stages, but the patient died 2 days after the second operation. autopsy showed multiple metastatic deposits in the liver. Symptoms and signs of raised intracranial pressure, the presence of Ota's naevus, and a dural-based mass or masses should alert the treating physician to suspect a primary leptomeningeal/intracranial melanoma. ( info) |
| The sturge-weber syndrome consists of a unilateral port-wine hemangioma of the skin along the trigeminal distribution and is accompanied by an ipsilateral leptomeningeal angioma. glaucoma is present in approximately half of the cases. The nevus of ota is a melanocytic pigmentary disorder, most commonly involving the area innervated by the trigeminal nerve. Elevated intraocular pressure, with or without glaucomatous damage, is observed in 10% of the cases. We report the first case of glaucoma associated with sturge-weber syndrome and nevus of ota in korea. ( info) |
6/52. Intracranial meningeal melanocytoma associated with ipsilateral nevus of ota. Case report. In this report, the authors review the case of a man with a neurocutaneous syndrome. He presented with an intracerebral melanocytoma associated with a blue nevus of the scalp; its location and its appearance during childhood supported the diagnosis of a nevus of ota. Meningeal melanocytomas are increasingly being diagnosed, but remain rare. Primary meningeal malignant melanoma is the first differential diagnosis to eliminate. Despite their common embryonic origin. the association of a melanocytoma with a nevus of ota is rare. A nevus of ota exhibits the same melanocytic proliferation and affects the trigeminal nerve territory. An ocular effect is not always observed. In contrast to an ocular lesion, a nevus of ota rarely transforms into a malignant melanoma. It is found only among caucasians. During 4 years of follow-up review after surgery, the patient remained asymptomatic. Other than antiepileptic therapy, he received no complementary treatment and cerebral imaging revealed no evidence of recurrence. ( info) |
7/52. Two cases of late onset Ota's naevus. Ota's naevus is among the dermal melanocytoses that show a distinct pattern involving skin innervated by the trigeminal nerve. Most cases present at birth or manifest clinically in early childhood. Cases of acquired lesions in adult onset have been reported rarely. We present two cases of late onset Ota's naevus which were confirmed by skin biopsies. Both patients underwent Q-switched alexandrite laser treatment with a dose of 8.0 J/cm2 given four or five times at 6 weekly intervals and showed some improvement. ( info) |
| nevus of ota is a benign pigmentary disorder that involves the skin innervated by the first and second branches of the trigeminal nerve. It is a dermal melanocytosis frequent in Oriental persons but uncommon in white persons. We report a case of nevus of ota in a white woman emphasizing the wide extension of the pigmentation and its association with ipsilateral sensorineural hypoacusia. ( info) |
9/52. Meningeal melanocytoma of the brain and oculodermal melanocytosis (nevus of ota): case report and literature review. BACKGROUND: Meningeal melanocytomas are rare benign pigmented tumors of meningeal melanocytes. nevus of ota is a melanocytic pigmentation of the sclera and the skin adjacent to the eye. We report a rare case of parietal convexity meningeal melanocytoma associated with ipsilateral mucocutaneous melanosis and review the literature. methods: A 17-year-old man presented with headache and blindness. There was left scalp and facial mucocutaneous melanosis. During surgery, speckled hyperpigmentation was seen in all layers of the scalp, bone, dura, and even ependyma. Pathologic examination of the tumor and scalp consisted of light microscopy and immunohistochemistry. In a literature review, the demographics (age, sex), clinical characteristics (site of lesion, duration of symptoms), therapeutic options (surgical excision, radiotherapy), and prognosis (recurrence, death, death related to the disease, and follow-up) were analyzed. For statistical analysis we used the independent sample t-test and chi(2) tests. RESULTS: Ninety-five cases (45 intracranial and 50 spinal) of meningeal melanocytoma have been reported. The median age of patients with intracranial lesions (40 years) was significantly lower than that of patients with spinal lesions (49 years) (p = 0.016). Of the 95 patients, 57.9% were female. In the nervous system, melanocytes are more common in the infratentorial and cervical areas, but more than half of the tumors have been located in the supratentorial region and the thoracic spine. In 46 months median follow-up, tumor recurrence rate and tumor related death rate were 26.3% and 10.5%, respectively. These were not statistically significant for different therapeutic modalities. There was a trend toward shorter survival for patients who underwent subtotal tumor resection without radiotherapy compared with other groups. CONCLUSION: The prognostic criteria, differential diagnosis, and the embryological aspects of meningeal melanocytoma are discussed with a review of the related literature. ( info) |
10/52. Bilateral type of nevus of ota presenting as agminated lentigines. We report a 30-year-old female patient with a history of hyperpigmented macules of the face since the age of 7 years. The clinical appearance, brown macules in multiple segmental or grouped patterns, was suggested to be agminated lentigines. However, histologic examination of the pigmented macule revealed scattered melanocytes within the dermis. We diagnosed this lesion as a bilateral type of nevus of ota presenting as agminated lentigines. The differential diagnosis from acquired bilateral nevus of ota-like macules was made. ( info) |