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Phakomatoses (Neurocutaneous Syndromes)
Published in Swati Goyal, Neuroradiology, 2020
Neurocutaneous melanosis Hairy or deeply pigmented neviMelanosis of the leptomeninges (intensely enhancing)
Skin and subcutaneous tissue
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
A multidisciplinary management approach is advocated, with initial investigations examining for neurocutaneous melanosis as there may be leptomeningeal involvement Removal of GCPN should be considered for both aesthetic and oncological reasons
Melanocytic Tumors
Published in Dongyou Liu, Tumors and Cancers, 2017
Although both meningeal melanocytosis and meningeal melanomatosis involve the supra- and infratentorial leptomeninges and the superficial brain parenchyma (particularly in the cerebellum, brainstem, and temporal lobes), only meningeal melanomatosis spreads the malignant melanocytes from the leptomeninges to the Virchow–Robin spaces, and superficially within the brain substance. Nonetheless, both lesions generally occur in the setting of dermatologic syndromes (e.g., neurocutaneous melanosis syndrome and nevus of Ota). Similarly, while both meningeal melanocytoma and meningeal melanoma may affect any area of the meninges, they show a predilection for the spinal cord, posterior fossa and Meckel’s cave. Meningeal melanocytoma is a benign lesion, whereas primary malignant melanoma of the leptomeninges is malignant. However, neither of these lesions is associated with pigmented lesions elsewhere (e.g., benign congenital pigmented nevi or frank cutaneous malignant melanoma) [6].
Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles
Published in Fetal and Pediatric Pathology, 2020
A directly- or indirectly-related tethered spinal cord is common among patients with congenital spinal lipomatous malformations, and this can be the clinical setting that brings the patient to a surgeon. Associated diastematomyelia, hydromyelia, or a syrinx can be encountered. The patients may have dislocation of the hips, clubfeet, polydactyly, scoliosis, kyphosis, perineal anomaly, sacral agenesis, the Currarino syndrome (triad of sacral agenesis, presacral meningocele, and anorectal anomaly), upper gastrointestinal atresia, renal or cardiac anomalies, intraspinal arachnoidal cysts, epidermoid or dermoid cysts, a benign sacrococcygeal teratoma, or neurocutaneous melanosis [21, 24, 54, 62, 64, 73, 112–121].