Cases reported "Angiomatosis"

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1/8. Scrape cytology of meningioangiomatosis: a report of two cases with diagnostic cytologic features.

    BACKGROUND: Meningioangiomatosis is a rare, probably hamartomatous condition characterized by nonneoplastic intracortical proliferation of meningothelial cells, capillaries and fibroblasts. The lesion may mimic a tumor both clinically and radiologically. We present two cases of the entity, including its cytologic features. CASES: A 71-year-old man presented with an eight-month history of headache, change in vision, loss of depth perception and unsteady gait. Magnetic resonance imaging (MRI) revealed an infiltrative lesion of the left occipital lobe, diffuse in nature. Cytologic smears prepared at the time of intraoperative consultation showed numerous thin-walled capillaries together with bland spindle cells. Occasional large cells with prominent nucleoli were also present. Frozen section confirmed the presence of linear, small capillaries surrounded by fibroblasts and meningothelial cells, consistent with meningioangiomatosis. The second case was a 3-year-old girl who presented with a one-day history of seizure, vomiting, fever and perioral cyanosis. MRI revealed an enhancing lesion in a temporal lobe of the cerebrum. The patient underwent excision of the lesion. Intraoperative cytology showed numerous meningothelial whorls together with neurons and occasional capillaries. Both patients were well, one after three months and the other after six. CONCLUSION: Meningioangiomatosis is a lesion characterized by linear capillaries, meningothelial cells and neurons, some exhibiting nuclear atypia. The combination can lead to an erroneous diagnosis of more common conditions, such as meningioma (with intracortical extension) and anaplastic astrocytoma. Contrary to these more sinister diagnoses, meningioangiomatosis is a completely benign, presumably hamartomatous entity.
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2/8. Pseudoangiomatous stromal hyperplasia of the breast-cytological features of two cases and review of literature.

    Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a lesion characterized on histology by the presence of anastomosing slit like spaces embedded in a hyalinized fibrous stroma. Till date, the cytological features of PASH have been described in only 12 cases, of which two cases were diagnosed on aspiration cytology as suspicious for carcinoma and in one case, for phyllodes tumor. We describe the FNAC findings of two of our cases of PASH. The first case was diagnosed as a phyllodes tumor and the other case as a benign proliferative breast disease, possibly a fibroadenoma. A review of the published literature on cytology of PASH shows the morphological variations that can exist in the cytology smears of this lesion. FNAC findings of PASH are non-specific, and the role of FNAC in PASH is to confirm the benign nature of the lesion, rather than provide a definitive pre-surgical diagnosis.
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3/8. Histopathological study of five cases with sporadic meningioangiomatosis.

    We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle-cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti-CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki-67 proliferation index of all the cases was very low, less than 0.1%. neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.
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4/8. Angiomatous spindle cell lipoma: Report of three cases with immunohistochemical and ultrastructural study and reappraisal of former 'pseudoangiomatous' variant.

    Pseudoangiomatous spindle cell lipoma (PASCL) is a rare variant of spindle cell lipoma that is composed of spindle fibroblast-like cells and mature adipose cells, and that is further characterized by intratumoral branching spaces regarded originally to be non-vascular (hence the adjective 'pseudoangiomatous'). Reported herein are three cases of this tumor, with literature review of all six cases described previously. PASCL appears to have clinical features (age, sex, location, prognosis) similar to those of conventional spindle cell lipoma (SCL). Immunohistochemically, the spindle cells in all of the present cases were positive for CD34, as in conventional SCL. All lesions had at least mild positivity for sex steroid hormone receptors. The cells lining intratumoral dilated and branching spaces typical of this variant were strongly positive for lymphatic endothelial marker D2-40 in all cases, and for vascular markers CD31 and F-VIII in one case. Ultrastructural study confirmed the endothelial nature of these cells. The results indicate that pseudoangiomatous SCL is truly angiomatous and should be termed as such. It could represent a sex steroid hormone-dependent lesion.
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5/8. Skeletal angiomatosis limited to the hand: radiographic and scintigraphic correlation.

    Three-phase bone scintigraphy elegantly demonstrates the vascular nature of the expansile, lytic lesions of skeletal angiomatosis and the induced bony changes in the involved areas. Unusual features of the case include its confinement to the hand and rapid progression as shown by serial radiographs.
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6/8. Vertebral and epidural hemangioma with paraplegia in klippel-trenaunay-weber syndrome. Case report.

    A patient with a rare case of klippel-trenaunay-weber syndrome presented with paraplegia due to compression by a vertebral and epidural cavernous hemangioma. The metameric distribution of the large cutaneous vascular nevus provided the clinical clue to the nature of the spinal lesion. The association of the two lesions is explained in the basis of developmental anomaly.
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7/8. Retinal varicosity in Klippel-Trenaunay syndrome.

    A retinal varicosity in the Klippel-Trenaunay syndrome is observed and diagnosed in a 24-year-old female. fluorescein angiography was used to illustrate and specify the nature of the retinal vascular anomaly.
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8/8. Recurrent intraoperative choroidal effusion in sturge-weber syndrome.

    The formation of a massive choroidal effusion without hemorrhage during a trabeculectomy procedure was documented in a 17-year-old male with sturge-weber syndrome, most probably representing the youngest patient in the literature with this intraoperative complication. The occurrence of similar signs during a previous trabeculectomy procedure in the same eye, suggested the possible recurrent nature of this entity. Preparation of a posterior sclerotomy site before entering the eye during intraocular surgery in patients with Sturge-Weber Syndrome might help in the immediate management and in preventing complications due to the formation of massive choroidal effusion.
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