Cases reported "Angiomatosis"

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1/173. Localized soft tissue angiomatosis with subsequent development of angiolymphoid hyperplasia with eosinophilia.

    We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE.
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2/173. Meningioangiomatosis. A comprehensive analysis of clinical and laboratory features.

    Meningioangiomatosis (MA) is a rare, benign, focal lesion of the leptomeninges and underlying cerebral cortex characterized by leptomeningeal and meningovascular proliferation. It may occur sporadically or in association with neurofibromatosis type 2. Previous reports have emphasized histological and imaging features. Data on the management of these patients are sparse, and electrophysiological features of MA lesions have not been published. We assessed the clinical, electrophysiological, histopathological and imaging features as well as the surgical outcome in MA, and compared MA with and without neurofibromatosis. Seven patients with MA at our centre were investigated and their outcome was assessed. A review of the literature is included. MA exhibits a wide range of clinical, imaging, histopathological and electrophysiological features, making the diagnosis difficult. Sporadic MA cases are not associated with neurofibromatosis and the two disorders are genetically distinct. Medically refractory, localization-related epilepsy is the commonest presentation in sporadic cases, but atypical presentations also occur. Unlike sporadic cases, MA with neurofibromatosis is often found incidentally, does not produce seizures, occurs less frequently (ratio of 1:4), and is multifocal. MRI findings in MA correspond to the histological picture. However, the appearance on imaging is non-specific and may suggest cystic atrophy, angioma and tumours. Several abnormalities have been found in close proximity to MA lesions, i.e. meningioma, oligodendroglioma, arteriovenous malformation, encephalocoel and orbital erosion. In spite of histopathological diversity, MA lesions are either predominantly cellular or vascular. Immunohistochemical results are inconsistent among cases, add little to the diagnosis, and do not support a meningeal origin. Electrocorticographic recordings from the surface and within MA lesions revealed a spectrum of electrophysiological expressions. Intrinsic epileptogenicity of MA lesions was documented in some cases. Epileptogenicity was confined to the perilesional cortex in some patients and it was complex (extralesional, multifocal, generalized) in others. Only 43% of our patients became seizure-free postoperatively compared with 68% previously reported, and >70% of our patients and those in the literature continued to require antiepileptic drugs. This is in keeping with the diverse electrophysiology of MA and suggests a less optimistic postoperative outcome than previously recognized.
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ranking = 0.625
keywords = angiomatosis
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3/173. Multifocal meningioangiomatosis: a report of two cases.

    We report the CT and MR findings in two patients with multifocal meningioangiomatosis, neither of whom had a family history or stigmata of neurofibromatosis. All lesions were located in the cortical and subcortical areas and had round dense calcifications with eccentric cysts. The masses were associated with surrounding edema and gliosis.
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ranking = 0.625
keywords = angiomatosis
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4/173. angiomatosis of skin with local intravascular immunoglobulin deposits, associated with monoclonal gammopathy. A potential cutaneous marker for B-chronic lymphocytic leukemia. A report of unusual case with immunohistochemical and immunofluorescence correlation and review of the literature.

    Reactive cutaneous vascular proliferation or angiomatosis is associated with various conditions, but is rarely seen secondary to vascular occlusion. We report an unusual case of a 79-year-old female who presented with 8 month history of purpuric facial plaques, with painful crusted ulceration of the nose, later developing similar eruptions on hands, thighs and trunk. Biopsies showed marked angioproliferation, with intravascular (IV) hyaline deposits (PAS , fibrin /-; IgM , fibrinogen , and C3 ), associated with endothelial hyperplasia (factor viii , vimentin ). Immunofluorescence showed IV IgM, fibrinogen, and granular C3 deposits within vessel walls. Initially, extensive investigations only showed minimal monoclonal gammopathy of undetermined significance (MGUS) and repeatedly negative cryoglobulins. After a 3-year follow-up, the patient developed chronic lymphocytic leukemia (B-CLL). This case illustrates a difficult diagnostic challenge. Although this condition resembles other forms of reactive angiomatosis, it shows distinct features and should be considered in the differential diagnosis of unusual vascular proliferations of the skin. The cutaneous lesions are also considered a potential marker for an underlying systemic condition, which may require prolonged clinical follow-up. We believe this condition to be related to other rare cutaneous vascular proliferations associated with plasma cell and lymphoproliferative disorders. Furthermore, we suggest a common pathogenetic pathway resulting from the IV immunoglobulin deposits causing vascular injury, finally leading to the angiomatosis.
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ranking = 0.375
keywords = angiomatosis
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5/173. meningioma with meningioangiomatosis: a condition mimicking invasive meningiomas in children and young adults: report of two cases and review of the literature.

    Meningioangiomatosis is a malformative meningovascular proliferation that occurs sporadically and in patients with neurofibromatosis type 2. Its histologic features of perivascular proliferation of elongated fibroblast and meningothelial cells trapping islands of gliotic cortex may be erroneously interpreted as invasion when an overlying meningioma is present. We report two cases of meningioangiomatosis associated with meningioma and review the literature on the subject for a total of six cases. The age of patients ranged from 9 months to 33 years. All cases were single lesions, and none had clinical evidence of neurofibromatosis type 2. Meningiomas in children have been regarded as having more aggressive behavior than their adult counterparts, with more frequent invasion of the underlying brain. The lack of correlation between brain invasion and recurrence observed in series of meningiomas in young patients may suggest that some of these lesions are meningioangiomatosis associated with meningioma rather than invasive meningiomas.
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ranking = 0.875
keywords = angiomatosis
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6/173. Meningioangiomatosis: a case report and review of the literature.

    A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.
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ranking = 0.75
keywords = angiomatosis
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7/173. Intraparenchymal meningioma originating from underlying meningioangiomatosis. Case report and review of the literature.

    The authors present the case of a 20-year-old woman with right-sided temporal intraparenchymal meningioma originating from underlying meningioangiomatosis. The patient manifested temporal-type seizures as the initial symptom. She had no stigmata of neurofibromatosis Type 2. Neuroradiological studies revealed an intraaxial, contrast-enhancing lesion in the right temporal lobe. After gross-total removal of the tumor, histological examination revealed a transitional meningioma with surrounding neural parenchyma and features of meningioangiomatosis. Although there were no signs of anaplasia, necrosis, or increased mitosis, tumor islands were observed in the adjacent neuropil. The rare association of meningioangiomatosis and meningioma is discussed, along with radiological and surgical findings.
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ranking = 0.875
keywords = angiomatosis
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8/173. Bilateral optic neuropathy associated with diffuse cerebral angiomatosis in sturge-weber syndrome.

    Visual loss in patients with encephalotrigeminal angiomatosis or sturge-weber syndrome usually results from secondary glaucoma or from damage to the retrogeniculate pathways by the angiomatous lesions. Optic neuropathy has not been reported as a component of this syndrome. This report describes a patient who developed bilateral optic neuropathy with progressive visual loss associated with diffuse cerebral angiomatosis in the setting of sturge-weber syndrome.
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ranking = 0.75
keywords = angiomatosis
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9/173. Meningioangiomatosis with a predominant fibrocalcifying component.

    A case of meningioangiomatosis, resected from the parietal lobe in a 31-year-old female is presented. Macroscopically, the lesion was composed of five calcified nodules embedded within hardened elastic tissue. Histologically, cortical and subcortical calcified masses were found surrounded by a palisade of spindle and/or oval cells. In adjacent nervous tissue many pathological microvessels were observed and some were ensheathed by a perivascular proliferation of spindle cells. Moreover, gliosis with Rosenthal fibers and prominent connective tissue elements were observed. Immunohistochemical analysis based on monoclonal antibodies was performed. The spindle cells both within the palisades and the perivascular proliferations were vimentin and usually epithelial membrane antigenpositive. The possible pathogenesis of meningioangiomatosis is discussed and the role of angiogenesis within this lesion emphasized.
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ranking = 0.75
keywords = angiomatosis
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10/173. Eruptive pseudoangiomatosis arising in adulthood: 9 cases.

    Eruptive pseudoangiomatosis has only been reported in children up till now. The purpose of this study is to present the clinical characteristics of eruptive pseudoangiomatosis in adulthood and to discuss the potential role of infectious agents. A retrospective study concerning nine cases of eruptive pseudoangiomatosis observed over a ten year period was undertaken, concerning clinical, histological and epidemiological aspects of the disease. The patients, 8 women and one man had an acute eruption of numerous papules measuring 2-5 mm in diameter, disseminated on the face, limbs and trunk. The papules were erythematous and telangiectatic, surrounded by a white halo. The lesions blanched completely with pressure and refilled from the center on release. Histological examination was performed on 4 patients, showing dilated blood vessels with plump endothelial cells but no evidence of an increased number of vessels. Epidemiological data revealed the onset of the disease during hospitalisation for treatment of cancer or asthma in four patients and among the five remaining patients, two were hospitalized in a retirement home and one was a nurse in a psychiatric hospital. These data suggest that the eruption could be linked with a infectious disease despite the negativity of some serological investigations. We report for the first time cases of pseudoangiomatosis arising in adulthood.
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keywords = angiomatosis
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