Cases reported "Melanosis"

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11/89. Neurocutaneous melanosis: a case of primary intracranial melanoma with metastasis.

    Neurocutaneous melanosis is a rare disorder characterized by the presence of large or multiple congenital melanocytic naevi and benign or malignant pigment cell tumours of the leptomeninges. Distant metastasis is unusual in primary leptomeningeal/intracranial melanomas. We present the case history of an adult male who had multiple primary intracranial melanomas associated with neurocutaneous melanosis (naevus of Ota) in the ophthalmic division of the left trigeminal nerve. Excision of the intracranial tumours was carried out in two stages, but the patient died 2 days after the second operation. autopsy showed multiple metastatic deposits in the liver. Symptoms and signs of raised intracranial pressure, the presence of Ota's naevus, and a dural-based mass or masses should alert the treating physician to suspect a primary leptomeningeal/intracranial melanoma.
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keywords = naevus, cell
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12/89. Diffuse melanosis arising from metastatic melanoma: pathogenetic function of elevated melanocyte peptide growth factors.

    The origin of diffuse melanosis resulting from metastatic melanoma is unknown. We examined the light microscopic and ultrastructural changes in the skin of an affected 35-year-old woman and determined the peripheral blood levels of melanocyte growth factors. A total of 7 biopsy specimens were examined by light and electron microscopy and immunohistology (S-100, HMB45, MART1, CD68, MAC387). serum/plasma levels of melanocyte growth factors of the patient were determined by enzyme-linked immunosorbent assays and compared with those of normal volunteers (n = 10) and amelanotic patients with metastatic melanoma (n = 10), matched to the UICC stage of the affected patient. Hyperpigmented but otherwise apparently normal skin of the patient displayed epidermal melanocyte hyperplasia, increased melanogenesis, and dermal pigment stored in histiocytes and other cells along with extracellular deposits. blood levels of alpha-melanocyte stimulating hormone, hepatocyte growth factor, and endothelin-1 were significantly elevated in the affected patient. Aberrant production of these factors may not only be responsible for activation of the pigment system in diffuse melanosis of metastatic melanoma, but also for increased proliferation, motility, and pigment incontinence of normal and malignant melanocytes.
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ranking = 0.0023167209991225
keywords = cell
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13/89. Melanotic macules of the tongue in a newborn.

    A newborn had 3 blue-black macules on the left side of the dorsal tongue. Clinical diagnosis was difficult. A biopsy specimen showed focal increase of melanin in the basal cells and subepithelial melanophages. The persistence of the lesions was unchanged at the age of 6 months, and the histologic features led to the diagnosis of oral physiologic melanosis.
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ranking = 0.0011583604995612
keywords = cell
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14/89. 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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ranking = 0.0011583604995612
keywords = cell
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15/89. Two discrete choroidal melanomas in an eye with ocular melanocytosis.

    We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.
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ranking = 0.0011583604995612
keywords = cell
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16/89. The histopathology of pigmentary dispersion syndrome with glaucoma.

    iris tissue and trabecular meshwork, obtained at time of trabeculectomy, was studied using the light and electron microscope in a 54-year-old woman with pigmentary dispersion syndrome with glaucoma. The specific defect was a loss of the outer epithelial cells of the iris with marked thinning of the remaining outer layers so that the two-cell architecture of the iris epithelium was maintained. In addition, the radial muscle layer was increased in both number and size of muscle fiber. It would appear that the pigmentary dispersion syndrome may represent a congenital or developmental abnormality of the iris epithelium, or both, and that the glaucoma which occasionally occurs in conjunction with this syndrome is of the usual open-angle type.
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ranking = 0.0023167209991225
keywords = cell
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17/89. Becker's naevus of the thigh with lipoatrophy: report of two cases.

    Although Becker's naevus is most common in males, cases with associated lipoatrophy are more frequently reported in women. Two women are described in whom Becker's naevus of the thigh was associated with localized lipoatrophy. Neither had any bony abnormality or functional deficit.
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ranking = 2.9965249185013
keywords = naevus
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18/89. A variant of junctional naevus of epithelioid and spindle cell type rich in melanophages.

    We describe a form of junctional melanocytic neoplasm with a massive production of melanin accumulated in the dermis. The pigment is stored in macrophages, which are by far the most numerous cellular component of the lesion. Another peculiar aspect is the occasional presence of a few melanocytes scattered in a pagetoid pattern above the dermo-epidermal junction in the spinous layer. The histological picture of this lesion is similar to a form of "tumoral melanosis" induced by a regressed malignant melanoma. The lesion had a worrisome clinical picture, its dark colour constituting a clinico-pathological diagnostic problem. The main clinical clues to the benign nature of this entity are the small lateral diameter, the uniform distribution of the pigment and the stability of the lesion over time; moreover, the patients are alive and well after a considerable length of time. Although a regressed dysplastic or malignant lesion cannot be totally excluded from a scientific point of view, we conclude that there is no sound morphological or clinical evidence that the lesion is other than biologically benign. The lesion is most likely another peculiar variant of epithelioid and spindle cell naevus.
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ranking = 2.5040542617485
keywords = naevus, cell
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19/89. 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.
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ranking = 0.0011583604995612
keywords = cell
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20/89. Peritoneal melanosis combined with serous cystadenoma of the ovary: a case report and literature review.

    Benign peritoneal melanosis is extremely rare and traditionally occurs in association with ovarian dermoid cysts, but rarely with peritoneal cyst, enteric duplication cyst or gastric triplication. The pathogenesis of peritoneal melanosis, in particular, the origin of the pigment-producing cells is unclear. We describe a case of peritoneal melanosis that was associated with ovarian serous cystadenoma in a young woman. Ovarian serous cystadenoma has not been previously described as a combined lesion of peritoneal melanosis. Based on the extremely rare incidence of this lesion and heterogeneous combined lesions, the possibility of an incidentally found, coexisting lesion couldn't be excluded. Here, we suggest that peritoneal mesothelial cells pinched off during the developmental period might be a source of pigment-producing cells.
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ranking = 0.0034750814986837
keywords = cell
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