1/6. Meningioangiomatosis occurring in a young male without neurofibromatosis: with special reference to its histogenesis and loss of heterozygosity in the NF2 gene region.A 16-year-old young male experienced persistent headache, and brain computed tomography and magnetic resonance imaging showed an abnormal mass with calcification in the right temporal lobe of the cerebrum. The tumor was located in the leptomeninges and cerebral cortex. In the leptomeninges, multiple calcified-fibrous nodules were noted. In this area spindle-shaped cells were arranged in a fascicular or storiform pattern. A few meningioma-like nodules were also present. With continuity of this leptomeningeal lesion, a diffuse infiltrative lesion composed of proliferating perivascular cells and hyalinized small vessels was also present in the cerebral cortex. The proliferating vessels were small and narrowed by proliferation of surrounding spindle-shaped cells. Immunohistochemically, the spindle-shaped cells had strong to moderate positivity for vimentin and CD34 and weak positivity for epithelial membrane antigen and S-100 protein. The maximum Ki67 labeling index was 0.3%. The spindle-shaped cells showed loss of heterozygosity on D17S929 and D17S282 microsatellite markers flanking the NF2 gene. These histopathologic and genetic findings are consistent with meningioangiomatosis, and meningioangiomatosis has been thought to be a neoplastic lesion of meningothelial cells. This is the first report of a genetic alteration in a case of meningioangiomatosis.- - - - - - - - - - ranking = 1keywords = spindle (Clic here for more details about this article) |
2/6. Plexiform neurofibroma of the uterine cervix: a case report and review of the literature.The female genital system is rarely affected in von Recklinghausen neurofibromatosis. The vulva is the most frequent genital location, but vaginal, cervical, uterine, and ovarian neurofibromas have rarely been reported. We describe a case of plexiform neurofibroma affecting the uterine cervix in a patient with chronic pelvic pain and menorrhagia who had multiple cutaneous neurofibromas and 1 large paraspinal neurofibroma. A small plexiform neurofibroma, which was not grossly visible, was confined to the uterine cervix and coexisted with a uterine leiomyoma and adenomyosis. There were no neurofibromas in the myometrium, fallopian tubes, or ovaries. Plexiform neurofibroma is a neoplasm that should be considered in the differential diagnosis of spindle cell neoplasms of the uterine cervix, especially in specimens from patients with neurofibromatosis.- - - - - - - - - - ranking = 1.1495351047913keywords = spindle cell, spindle (Clic here for more details about this article) |
3/6. Segmental neurofibromatosis of face.A 38-year-old man presented with asymptomatic skin lesions over the left side of the face of 5-years duration. He had multiple discrete soft-to-firm papules and nodules on the left side of the face along the distribution of the mandibular division of the trigeminal nerve. Histopathology examination of one of the nodules (face) showed a non-encapsulated tumor of the dermis with normal overlying epidermis. The tumor consisted of loosely spaced spindle cells and wavy collagenous strands in a clear matrix. These features were consistent with our clinical diagnosis of segmental neurofibromatosis. This case is reported for its rarity and typical manifestations.- - - - - - - - - - ranking = 1.1495351047913keywords = spindle cell, spindle (Clic here for more details about this article) |
4/6. Bilateral diffuse iris nodular nevi. Clinical and histopathologic characterization.BACKGROUND: Diffuse nodular nevus of the iris is an uncommon condition that presents with multiple verrucous excrescences distributed diffusely on the iris surface. methods: The authors describe 30 patients with bilateral diffuse iris nodular nevi and report associations with bilateral congenital cataract, neurofibromatosis, oculodermal melanocytosis, congenital ptosis, morning glory anomaly, Axenfeld anomaly, or Peters anomaly. RESULTS: iris nodules were uniform in size and distribution and were brown, as was the surrounding iris. light and electron microscopy of iridectomy specimens from one patient showed elevated plaques composed of aggregates of plump, lightly pigmented nevoid cells interwoven with mature, densely pigmented spindle-shaped uveal melanocytes. CONCLUSIONS: The authors report the largest clinical series and first ultrastructural description of bilateral diffuse iris nodular nevi, which represents a variant of neural crest development. No ocular complications could be attributed to the iris nodules, which should be differentiated from Lisch nodules and other pathologic iris lesions.- - - - - - - - - - ranking = 0.25keywords = spindle (Clic here for more details about this article) |
5/6. A 4-year-old boy with neurofibromatosis and severe renovascular hypertension due to renal arterial dysplasia.A 4-year-old boy had severe hypertension, cardiac failure, and signs of neurofibromatosis. Arteriography disclosed renal artery stenosis in both kidneys with signs of ischemia, particularly in the right kidney. Because of insufficient response to antihypertensive therapy, a right-sided nephrectomy was performed. Histological examination of this kidney showed segmental stenosis in all branches of the renal artery. The vascular lesions were characterized by an intimal proliferation of spindle cells in a mucoid matrix with destruction of the internal elastic membrane frequently accompanied by loss or attenuation of the media and fibrosis of the adventitia. Occasionally, a nodular arrangement of the spindle cells at the interface between intima and media was observed. Immunohistochemical studies demonstrate a smooth-muscle cell origin for these cells.- - - - - - - - - - ranking = 2.2990702095825keywords = spindle cell, spindle (Clic here for more details about this article) |
6/6. Protein-losing enteropathy caused by mesenteric vascular involvement of neurofibromatosis.A 44-year-old man with neurofibromatosis suffered from severe diarrhea and progressive hypoproteinemia. Enteric protein loss was confirmed by an alpha 1-antitrypsin clearance test. An x-ray study showed irregular mucosa in the distal segment of the ileum. Protein loss has subsided after ileocecal resection. laparotomy and radiological examinations failed to demonstrate neurofibroma or other tumors in the abdomen. The resected ileum revealed marked edema of the wall and a pseudomembranous enteritis-like appearance in the luminal surface. Microscopically, lymphatic vessels in the intestinal wall were dilated. Marked thickening of the intima with spindle cell proliferation was observed in the mesenteric arteries and veins. This finding is consistent with vascular changes in neurofibromatosis. These vascular changes in the mesentery due to neurofibromatosis may cause protein-losing enteropathy by altering the hemodynamic state and microvascular permeability in the intestine.- - - - - - - - - - ranking = 1.1495351047913keywords = spindle cell, spindle (Clic here for more details about this article) |