摘要
Melanocytic lesions of the CNS are rare tumours originating from melanocytes that are present in the leptomeninges. They consist of a spectrum of pigmented tumours ranging from melanocytoma to melanoma. A small group of these tumours have histopathological features between those of a benign melanocytoma and a malignant melanoma;these present as intermediate grade melanocytic neoplasms. Naevus of Ota is a blue hyperpigmented dermal lesion characterized by increased number of melanocytes in the distribution of ophthalmic and maxillary divisions of the trigeminal nerve. The association of an intracranial intermediate-grade melanocytic neoplasm with a nevus of Ota is extremely rare, with only 2 cases reported in the literature to date. As a result, their behavior and progression are still poorly understood. We present the first case of a familial naevus of Ota associated with intermediate-grade melanocytic neoplasm.
Melanocytic lesions of the CNS are rare tumours originating from melanocytes that are present in the leptomeninges. They consist of a spectrum of pigmented tumours ranging from melanocytoma to melanoma. A small group of these tumours have histopathological features between those of a benign melanocytoma and a malignant melanoma;these present as intermediate grade melanocytic neoplasms. Naevus of Ota is a blue hyperpigmented dermal lesion characterized by increased number of melanocytes in the distribution of ophthalmic and maxillary divisions of the trigeminal nerve. The association of an intracranial intermediate-grade melanocytic neoplasm with a nevus of Ota is extremely rare, with only 2 cases reported in the literature to date. As a result, their behavior and progression are still poorly understood. We present the first case of a familial naevus of Ota associated with intermediate-grade melanocytic neoplasm.
