Cases reported "Melanosis"

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1/4. Cerebral mass due to neurocutaneous melanosis: eight years later.

    Neurocutaneous melanosis (NCM) is associated most commonly with giant congenital melanocytic nevi (CMN), in particular those on the scalp or in a posterior axial location that are accompanied by satellite congenital nevi. It also can occur in patients with multiple medium-sized CMN. In general, the prognosis of those with symptomatic NCM is poor, even in the absence of malignancy, while the prognosis of those with asymptomatic NCM detected via screening varies and is more difficult to predict. Herein we report an asymptomatic patient with a giant CMN and multiple satellite nevi who had a screening magnetic resonance imaging (MRI) study at age 5 months that showed a rounded area of increased signal in the right temporal lobe on T1-weighted images, suggestive of parenchymal melanosis. This melanotic mass was resected at age 10 months, and histologic examination of the surgical specimen showed prominent perivascular collections of benign, pigment-containing melanocytes within cerebral tissue. The patient remains healthy 8 years later. His excellent long-term outcome and other reports of NCM with localized central nervous system (CNS) involvement apparent on MRI may have implications for management, including early imaging of patients with high-risk CMN and potential surgical intervention for NCM.
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2/4. Neurocutaneous melanosis with transposition of the great arteries and renal agenesis.

    Neurocutaneous melanosis (NCM) is rare and is characterized by the proliferation of melanocytes in the central nervous system. A 6-day-old infant boy was referred to our department with giant congenital melanocytic nevi and convulsions. On physical examination the patient had a giant black-brown pigmented nevus covering his face, neck, scalp, shoulders, back, chest, and abdomen. Numerous satellite lesions were noted on the face, neck, and upper extremities. In the right bulbar conjunctiva, a brown plaque was present. magnetic resonance imaging (MRI) showed hyperintense areas in the brain on short repetition time/short echo time sequences, compatible with intraparenchymal melanin deposits. No leptomeningeal abnormality was seen. Further investigation also revealed agenesis of the right kidney and transposition of the great arteries. Transposition of the great arteries, which has never been reported in NCM, may be an incidental finding. We present a case of NCM associated with agenesis of the right kidney and transposition of the great arteries.
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3/4. Neurocutaneous melanosis, neurofibromatosis and spinal meningioma: an unusual association.

    The case of 38-year-old woman bearer of a congenital giant naevus "en pelerine" with numerous neurofibromas and other satellite naevi was reported: the patient was afflicted by spastic tetraparesis, more pronounced on the right side. MRIscan of the spine revealed the presence of a cervical spinal tumor shown histologically to be a psammomatous meningioma. The skin picture was consistent with neurocutaneous melanosis; the rarity of its association with neurofibromatosis and spinal meningioma is discussed in the light of embryologic arguments.
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4/4. Neurocutaneous melanosis associated with inferior vermian hypoplasia: MR findings.

    OBJECTIVE: We describe the MR findings of two cases of neurocutaneous melanosis (NCM) associated with inferior vermian hypoplasia (IVH). MATERIALS AND methods: Two cases of NCM associated with IVH are presented. Both patients had congenital giant hairy nevi and multiple satellite lesions. RESULTS: magnetic resonance imaging revealed IVH as well as hyperintensities in the pons and the cerebellar leptomeninges on T1-weighted images. The hyperintensities may be ascribed to the T1-shortening effect of melanin. A right parietal tumor in one patient and unilateral ventriculomegaly in another patient were also depicted. CONCLUSIONS: Magnetic resonance demonstration of the T1-shortening leptomeningeal infiltrations adds weight to the diagnosis of NCM. The implication of the concurrence of NCM and IVH is discussed.
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