1/3. 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.- - - - - - - - - - ranking = 1keywords = hyperpigmentation (Clic here for more details about this article) |
2/3. 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.- - - - - - - - - - ranking = 1keywords = hyperpigmentation (Clic here for more details about this article) |
3/3. A new neurocutaneous syndrome possibly related to Ota's nevus.The case of a 42-year-old Sudanese man is reported who had developed hyperpigmentation of the left face and rapidly progressive right hemiparesis followed by myoclonus at the age of 30. Blue-grey hyperpigmentation was noted in the territory of the left ophthalmic, maxillary and mandibular trigeminal nerve branches, with alopecia of the right face and axilla. CT of the brain revealed left-sided paraventricular calcifications and MRI showed atrophy of the left cerebral hemisphere and mesencephalon, a calcified haemangioma in the left basal ganglia, an old infarct in the right central pons, and cerebellar atrophy. This case resembles Ota's syndrome; however, atypical distribution of the facial nevus, the epidermal location of hyperpigmentation, and the combination of vascular and degenerative cerebral malformations indicate that this is a new hitherto unreported neurocutaneous vascular syndrome.- - - - - - - - - - ranking = 3keywords = hyperpigmentation (Clic here for more details about this article) |