1/27. Neurocutaneous melanosis associated with Dandy-Walker malformation. case report and review of the literature.Neurocutaneous melanosis is a rare dysmorphogenesis associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the leptomeninges anywhere in the central nervous system (CNS). It is interesting that almost 8-10% of patients had associated Dandy-Walker malformation in the literature, suggesting a common origin of the developmental abnormalities. In this article, we present a 2-year-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. We reviewed the literature and discuss the pathogenesis based on the preferred hypotheses so far.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/27. 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.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
3/27. Two cases of melanose neurocutanee with development of malignant melanoma: a microspectrophotometric and electron microscopic study.Two cases of neurocutaneous melanosis with development of malignant melanoma in the Japanese are presented. The first case was a 4-year-old boy in whom a retroperitoneal melanoma appeared with giant nevi, and cerebral and spinal melanosis. The second case was a 39-year-old man, in whom a primary leptomeningeal melanoma developed with leptomeningeal melanosis and smaller pigmented nevi. Microspectrophotometric and electron microscopic studies were made on the neoplastic and non-neoplastic melanotic tissues to elucidate the histogenesis of this rare disorder. Two different patterns of nuclear dna histograms, corresponding to melanosis and melanoma, were obtained by microspectrophotometry, Considerable variation in the ultrastructure of the melanocytes was seen by electron microscopy.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/27. Giant congenital melanocytic nevi in a patient with brain structural malformations and multiple lipomatosis.We present a 9-year-old boy diagnosed from birth with giant congenital melanocytic nevi. He had central structural brain malformations of hemimegalencephaly of the right frontotemporal lobe and left occipitoparietal lobe, choroid plexus hypertrophy, and a Dandy-Walker variant. In addition, he developed multiple lipomatoses. These lesions were cutaneous except for two at the cerebellopontine angles, which were present from birth. This patient represents a rarely documented example of two histopathologies resulting in serious complications. The diagnostic issues and histopathologic process are discussed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/27. Neurocutaneous melanosis in association with encephalocraniocutaneous lipomatosis.We describe a white female infant with neurocutaneous melanosis (NCM) and encephalocraniocutaneous lipomatosis (ECCL). Multiple, giant and small congenital melanocytic nevi (CMN) were observed on the head, neck and trunk and involved 70% of body surface area. Histologic examination of several CMN revealed atypical nodular proliferations of dermal nevomelanocytes. In a small (<1 cm) truncal CMN, single and dyscohesive intraepidermal nests of atypical nevomelanocytes simulating a superficial spreading melanoma, were observed. The placenta was grossly normal and histologically demonstrated multiple banal appearing nevomelanocytes within the stroma of its villi. At the 17-month follow-up no evidence of primary or metastatic melanoma was present. This previously undescribed association of NCM, ECCL and placental nevomelanocytes provides strong support for the hypothesized causal role of anomalous neural crest morphogenesis and migration in the development of all three disorders. The genetic mechanism underlying these complex birth defects has been hypothesized to result from the action of lethal autosomal dominant genes surviving by mosaicism.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
6/27. Neurocutaneous melanomatosis with a rapidly deteriorating course.Neurocutaneous melanosis is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We present serial MR imaging findings in the brain and spine of a child with congenital giant hairy nevi who developed progressive leptomeningeal melanomatosis and whose neurologic condition rapidly deteriorated.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/27. 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.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
8/27. Neurocutaneous melanosis.BACKGROUND: Neurocutaneous melanosis (NCM) is a rare phakomatosis characterized by a focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges. This syndrome is believed to result from an error in the morphogenesis of embryonal neuroectoderm. FEATURES: Two-thirds of patients with NCM have giant congenital melanocytic nevi, and the remaining third have numerous lesions but no giant lesions. Most patients present neurological manifestations early in life, which can be secondary to intracranial hemorrhages, to impairment of cerebrospinal fluid circulation, and to malignant transformation of the melanocytes. prognosis: The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/27. Atypical mole syndrome and congenital giant naevus in a patient with celiac disease.We describe a case of a 28-year-old woman affected by celiac disease (CD) associated with rare multiple disorders of the cutaneous pigmentary system: atypical mole syndrome and congenital giant naevus. Some other rare skin lesions have been reported in association with celiac disease such as cutaneous sarcoidosic granuloma, bullous pemphigoid, ichthyosis, alopecia areata, erythema elevatum diutinum, sclero-atrophic lichen and primary cutaneous amyloidosis. This is the 1(st) report concerning celiac disease and congenital disorders of the pigmentary system.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
10/27. Multiple giant congenital melanocytic nevi with central nervous system melanosis.A case of multiple giant congenital melanocytic naevi in whom central nervous system melanosis was detected at 6 weeks of age is described. The infant was asymptomatic, but presence of risk factors such as multiple naevi, giant naevi and naevi on scalp and posterior axial location prompted a magnetic resonance imaging study of the brain. To our knowledge, neurocutaneous melanosis at such a young age has not been reported in Indian literature.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
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