1/60. A Laugier-Hunziker syndrome associated with esophageal melanocytosis.A 62-year-old Japanese woman came to our clinic because of melanotic macules on the lip, palatoglossal arch, lingual margin and palm. Endoscopic examination revealed a melanotic macule on the midesophageal mucosa but no polyposis in the gastrointestinal tract. Histologically, the specimens taken from the labial, esophageal and palmar lesions showed an acanthosis and basal hyperpigmentation in the epithelium. The patient had not taken any medication which could lead to pigmentation. As far as we know, this is the first case report of an esophageal melanocytic macule which occurred in a patient with Laugier-Hunziker syndrome. When confronted with an isolated pigment spot, we emphasize the necessity of systematic examinations for others. Because the pathologic relationship between Laugier-Hunziker syndrome and the esophageal melanocytic lesion is not proven, further studies should clarify this issue.- - - - - - - - - - ranking = 1keywords = melanocytic (Clic here for more details about this article) |
2/60. Primary invasive signet-ring cell melanoma.The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells.- - - - - - - - - - ranking = 1keywords = melanocytic (Clic here for more details about this article) |
3/60. Simultaneous occurrence of multiple melanoma in situ on sun-damaged skin (lentigo maligna), solar lentigo and labial melanosis: the value of dermoscopy in diagnosis.We report on a patient developing simultaneous occurrence of lentigo maligna lesions, solar lentigines and an extensive melanosis of the oral mucosa. Diagnostically, epiluminescence microscopy had a relevant role in the preoperative assessment and selection of suspicious pigmented lesions, as the lesions histologically labelled as lentigo maligna and solar lentigo were clinically indistinguishable. We review the clinical, dermoscopic and histopathologic differential diagnosis of solar lentigo, malignant lentigo and mucosal melanosis with other melanocytic and keratinocytic lesions and discuss the possible relationship between these entities.- - - - - - - - - - ranking = 0.5keywords = melanocytic (Clic here for more details about this article) |
4/60. Immunohistochemical detection of the c-met proto-oncogene product in the congenital melanocytic nevus of an infant with neurocutaneous melanosis.Transgenic mice overexpressing hepatocyte growth factor/scatter factor (HGF/SF) demonstrate extensive pigmented nevi in both skin and leptomeninges of the central nervous system resembling human neurocutaneous melanosis. We immunohistochemically detected HGF/SF receptor, Met, in a congenital nevus of an infant with neurocutaneous melanosis, indicating that deregulation of HGF/SF-Met signaling in the critical period of development may lead to this fatal syndrome.- - - - - - - - - - ranking = 2keywords = melanocytic (Clic here for more details about this article) |
5/60. 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.- - - - - - - - - - ranking = 0.5keywords = melanocytic (Clic here for more details about this article) |
6/60. Neurocutaneous melanosis with epilepsy: report of one case.Neurocutaneous melanosis is a rare congenital syndrome characterized by the association of large or multiple congenital melanocytic nevi and benign or malignant melanotic tumors in the central nervous system. patients with neurocutaneous melanosis usually have neurological symptoms early in life that progress rapidly due to the development of increased intracranial pressure or malignant melanoma. We report a 2-month-old female infant with multiple congenital melanocytic nevi and frequent seizure attacks. magnetic resonance imaging of the brain demonstrated several regions compatible with melanotic deposits. During follow-up for one year, she had normal development and was seizure-free under the treatment of phenobarbital and valproic acid. We suggest that infants with large or multiple congenital melanocytic nevi should receive regular clinical check-up and brain imaging to exclude the possibility of central nervous system lesions.- - - - - - - - - - ranking = 1.5keywords = melanocytic (Clic here for more details about this article) |
7/60. 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 = 0.5keywords = melanocytic (Clic here for more details about this article) |
8/60. dermoscopy in the diagnosis of tinea nigra plantaris.tinea nigra is a relatively uncommon dermatiaceous fungal infection, usually caused by Phaeoannellomyces werneckii, that may mimic a melanocytic lesion. We describe the value of epiluminescent dermoscopy of tinea nigra plantaris compared with other common diagnostic tools and procedures available (clinical appearance, potassium hydroxide [KOH], culture, culture mount preparation, and biopsy). A case of tinea nigra plantaris was evaluated clinically, microscopically with KOH, and dermatoscopically. Dermatoscopic findings were evaluated according to the Stolz system. dermoscopy, clinical presentation, and microscopy with KOH all confirmed the diagnosis, with dermoscopy being the fastest and simplest procedure. dermoscopy is a useful clinical adjuntive tool in differentiating tinea nigra from a melanocytic lesion.- - - - - - - - - - ranking = 1keywords = melanocytic (Clic here for more details about this article) |
9/60. Neurocutaneous melanosis in association with the Dandy-Walker complex, complicated by melanoma: report of a case and literature review.Neurocutaneous melanosis is a rare congenital neurocutaneous syndrome in which benign and malignant melanocytic tumors of the leptomeninges with large or numerous congenital melanocytic nevi develop. The Dandy-Walker malformation occurs as a broad posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilation of the fourth ventricle communicating with the posterior fossa. association of these entities is very unusual and only 10 previous reports were found in the literature. Our patient had multiple, medium-size to small melanocytic nevi present since birth. At 5 years of age the patient has intracranial pressure secondary to hydrocephalus. A diagnosis of Dandy-Walker malformation and suspected neurocutaneous melanosis was established after a skull computed tomography (CT) scan. Three months later the patient developed a right frontal tumor shown on the CT scan. The histologic finding was nevomelanocytic infiltration with strong pleomorphism. The tumor grew rapidly, producing neurogenic shock and death. The postmortem report indicated malignant melanoma.- - - - - - - - - - ranking = 2keywords = melanocytic (Clic here for more details about this article) |
10/60. 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 = 2.5keywords = melanocytic (Clic here for more details about this article) |
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