1/306. Epidermal naevus syndrome and hypophosphataemic rickets: description of a patient with central nervous system anomalies and review of the literature.The epidermal naevus syndrome (ENS) is a rare dermatological condition consisting of congenital epidermal nevi associated with anomalies in the central nervous system, bones, eyes, hear or genito-urinary system. We report a new case of ENS associated with hypophosphataemic rickets. The girl was born with a mixed-type epidermal naevus and skeletal anomalies. Hypophosphataemic rickets was diagnosed at the age of 2.5 years. At 14 years of age. MRI of the head demonstrated right brain hypotrophy, a left temporal arachnoid cyst and asymmetric lateral ventricles. We reviewed the literature and found 13 reported cases of ENS associated with hypophosphataemic rickets. Conclusion We report a further patient with epidermal naevus syndrome and hypophosphataemic rickets, followed from birth to the age of 15 years, who had structural central nervous system anomalies with normal intellectual functioning. A comprehensive neurological work up is recommended in patients with epidermal naevus syndrome.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
2/306. A case of a malignant melanoma with late metastases 16 years after the initial surgery.We report a case of a pulmonary metastasis 16 years after the initial surgery for a malignant melanoma. The patient was a 58-year-old Japanese man. In 1976, he had a pigmented skin lesion with a diameter of 8 mm on his right third finger. He received an amputation of the finger and a dissection of the right axillary. Histological examinations of the tumor revealed a feature of a malignant melanoma with infiltration of the papillary layers of the dermis, 1.5 mm in thickness. The histological subtype was considered to be an acral lentiginous melanoma with a mixed spindle-epithelioid cell pattern. There was no regional lymph node metastasis. In December 1992, when he was 74-years-old, a round tumor in the left lower lung was discovered by chest radiography. In February 1993, he received a left lower lobectomy of the lung. Histological examination revealed a feature of a malignant melanoma with predominantly epithelioid cells and this was considered to be a metastasis from the initial skin lesion. Five months after the lobectomy, he died from a hemorrhage of a metastatic brain tumor. This case indicated the importance of periodic, life-long follow-up in treating malignant melanomas.- - - - - - - - - - ranking = 0.031350381328684keywords = brain (Clic here for more details about this article) |
3/306. Linear epidermal nevus and nevus sebaceus syndromes: a clinicopathologic study of 3 patients.BACKGROUND: Linear epidermal nevus syndrome and linear sebaceus nevus syndrome are rare neurocutaneous syndromes characterized by epidermal nevi, epilepsy, and mental retardation. Pathologic descriptions of the central nervous system findings in such patients are rare. DESIGN: We examined the clinicopathologic features of 2 patients with linear epidermal nevus syndrome and 1 with nevus sebaceus syndrome who underwent surgical resections for chronic epilepsy in a tertiary referral center with a high volume of epilepsy surgery. RESULTS: patients included 3 females, aged 11 months (patient 1), 8 years (patient 2), and 2 1/2 years (patient 3) at the time of surgery. The duration of seizures prior to surgery was 11 months, 6 years, and 28 months, respectively. Two patients had epidermal nevi involving the head region (patients 1 and 3), and 1 had a nevus sebaceus of Jadassohn (patient 2); patient 2 had a choristoma, and patient 3 had a dermoid cyst in the eye region. Patient 1 demonstrated hemimegalencephaly radiographically. Histologic examination of resected cortical tissue in patients 1 and 2 demonstrated severe diffuse cortical dysplasia characterized by a disorganized cortical architectural pattern, a haphazard orientation of cortical neurons, and increased molecular layer neurons. Gyral fusion was seen in patient 1. Pial glioneuronal hamartomas were observed in patient 1. Prominent cortical astrocytosis was seen in patients 1 and 2, and foci of microcalcification were evident in patient 1. Cortical dysplasia was milder in patient 3 and consisted of an increased number of molecular layer neurons. Neuronal heterotopia was observed in all 3 patients. CONCLUSION: The spectrum of neuronal migration abnormalities in the setting of these syndromes may be variable in terms of its histologic phenotypic manifestations.- - - - - - - - - - ranking = 0.16144160311189keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/306. Blue rubber bleb naevus: case report and literature review.Blue rubber bleb naevus syndrome is a rare cutaneous-digestive angiomatosis, described first by William Bean in 1958. Today, there are more than 200 cases published. The dangers of this syndrome include angiomata in the brain, kidneys or lungs (due to vasculature obliteration by in situ thrombosis). patients are scanned with technetium-labelled red blood cells in order to identify the affected organs. Multiple techniques are used to treat the lesions by gastrointestinal endoscopy.- - - - - - - - - - ranking = 0.031350381328684keywords = brain (Clic here for more details about this article) |
5/306. Diffuse neonatal hemangiomatosis with extensive involvement of the brain and cervical spinal cord.BACKGROUND: Diffuse neonatal hemangiomatosis (DNH) is a rare disorder first recognized at birth or during the neonatal period. DNH is characterized by numerous cutaneous and visceral hemangiomas involving three or more organ systems. MATERIALS AND methods: Although the skin and liver are most frequently affected, we present a case of DNH demonstrating an unusual predilection for the central nervous system (CNS). RESULTS AND CONCLUSION: We report the imaging findings in a patient with this disorder, paying particular attention to the features seen on cranial sonography and spinal MR imaging.- - - - - - - - - - ranking = 0.28684312842662keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
6/306. Fatal cutaneous squamous cell carcinoma with extension through the maxillary sinus and orbit into the brain.Cutaneous squamous cell carcinomas may cause death by metastasis or by local extension. We describe a deeply invasive cutaneous squamous cell carcinoma that caused death by direct extension into the brain.- - - - - - - - - - ranking = 0.15675190664342keywords = brain (Clic here for more details about this article) |
7/306. Leptomeningeal melanoma in childhood.BACKGROUND: Malignant melanoma (MM) is one of the least common types of childhood cancer, accounting for less than 1% of all pediatric malignancies. Neurocutaneous melanosis (NCM) is a rare phakomatosis consisting of congenital abnormal pigmentation of the skin and meninges. The meningeal lesions are particularly prone to malignant change. methods: The authors describe 5 patients with NCM and 1 with primary leptomeningeal melanoma (LMM) seen at 2 treatment centers in the north of england over a 13-year period (1984-1997). RESULTS: The clinical features, progress, radiological findings, and treatment of these patients are discussed. All six died within eight months of their diagnosis, illustrating the difficulties faced in treating patients with these conditions. The authors reviewed the published literature on NCM, concentrating on the various therapeutic strategies that have been tried. Very little consistency in approach was found. Malignant skin lesions in NCM may be less responsive than primary malignant melanoma, but the small number of patients with primary LMM or brain metastases of MM make comparisons with NCM difficult. The authors' own series illustrates well the piecemeal nature of therapy for patients with these rare conditions. CONCLUSIONS: The rate of incidence of MM melanoma in the U.K. is increasing, and it will represent an increasing proportion of the pediatric oncologist's workload. A consistent approach to the therapy of patients with metastatic MM and NCM is needed if we are to have any hope of offering more than palliative therapy to these children in the future.- - - - - - - - - - ranking = 0.031350381328684keywords = brain (Clic here for more details about this article) |
8/306. Cutaneous electrosurgery in a patient with a deep brain stimulator.BACKGROUND: Deep brain stimulators are implantable devices with electrical activity used to treat certain movement disorders such as essential tremor and Parkinson's disease. Similar to implantable cardiac devices, use of electrosurgery on patients with these devices may produce adverse effects. CASE REPORT: We describe the effects of electrosurgery on a patient with essential tremor and an implantable deep brain stimulator who required Mohs micrographic surgery to excise a basal cell carcinoma. The patient experienced immediate lancinating "electrical shock" using electrosurgery in the monopolar mode. The patient experienced no discomfort when a bipolar electrosurgical device was used or when his deep brain stimulator was "turned off." Appropriate positioning of the dispersive plate also reduced adverse effects. CONCLUSION: Dermatologic surgeons should be aware of patients with devices implanted in the CNS with electrical activity and proceed with caution when using electrosurgery. Different approaches can be utilized to help reduce adverse effects.- - - - - - - - - - ranking = 0.21945266930079keywords = brain (Clic here for more details about this article) |
9/306. Leptomeningeal melanomatosis with multiple cutaneous pigmented nevi: tumor cell proliferation and malignant transformation in an autopsy case.We experienced a rare case of leptomeningeal melanomatosis. The proliferative activity and nuclear accumulation of p53 in this tumor were examined, since the relationship between this tumor type and growth has not yet been elucidated. A 33-year-old Japanese man was shown to have leptomeningeal melanomatosis with multiple cutaneous pigmented nevi. The autopsy findings showed the presence not only of benign diffuse melanosis of the leptomeninges but also of leptomeningeal melanomatosis in the subarachnoid space and brain parenchyma. In the brain parenchyma, the direct invasion of tumor cells from the subarachnoid space and Virchow-Robin spaces filled with melanoma cells were observed. Multiple hemorrhagic areas invaded by melanoma cells were also present. Immunohistochemical staining with a monoclonal antibody to melanoma cells showed positivity in the tumor cells. Proliferation analysis using the MIB-1 antibody demonstrated that the labeling index of tumor cells invading brain parenchyma (2.54%) was higher than that in other lesions of the inner (0.89%) and outer layer (0.76%) of the subarachnoid space. Nuclear accumulation of p53 protein was rarely seen in the tumor cells. We reported a case of leptomeningeal melanomatosis. Higher proliferative activity was found in invading cells of the brain parenchyma. Malignant transformation of the tumor did not appear to be associated with p53 gene mutation.- - - - - - - - - - ranking = 0.12540152531474keywords = brain (Clic here for more details about this article) |
10/306. renal artery stenosis associated with epidermal nevus syndrome.Epidermal nevus syndrome is an unusual neurocutaneous disorder in which epidermal nevi are associated with abnormalities of the skeleton and central nervous system, including the eyes and somtimes the cardiovascular system. We treated a patient in whom the latter included renal artery stenosis. An 18-year-old man with epidermal nevi was diagnosed as having the syndrome based on the additional presence of scoliosis, an arachnoid cyst in the middle cranial fossa, and microphthalmos. hypertension was diagnosed when the patient was 15 years old. The plasma renin activity (9.7 ng/ml/h) was elevated. Right renal artery stenosis was demonstrated by angiography, and the abdominal aorta was narrowed distal to the ostium of the superior mesenteric artery. The plasma renin activity in the right renal vein (16 ng/ml/h) was higher than contralaterally (10 ng/ml/h). Several cardiovascular manifestations have been reported as a complication of epidermal nevus syndrome. hypertension in an individual with epidermal nevi and congenital anomalies should prompt a search for a vascular anomaly.- - - - - - - - - - ranking = 0.16144160311189keywords = central nervous system, nervous system (Clic here for more details about this article) |
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