Cases reported "Angiomatosis"

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1/55. 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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2/55. 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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3/55. 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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4/55. angiomatosis of the hand demonstrated by contrast-enhanced magnetic resonance angiogram.

    Contrast-enhanced digital subtraction dynamic MR angiography has been applied to lower limb arteries, with accuracy comparable to that of conventional angiography. Application of this technique to hands has not been reported. A 2-year-old girl with a diffuse haemangioma of the right hand elegantly demonstrated by MR angiogram is presented. The feeding vessel and the relationship with surrounding palmar blood vessels were clearly identified. This provided essential preoperative information facilitating surgical resection, yet without the technical difficulties and morbidity associated with conventional angiography.
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ranking = 14.293637408556
keywords = blood vessel, vessel
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5/55. 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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6/55. 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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ranking = 14.293637408556
keywords = blood vessel, vessel
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7/55. Eruptive pseudoangiomatosis.

    We describe two children with acute onset and spontaneous resolution of angioma-like papules during a viral illness. The biopsy specimens from both patients showed a unique histologic appearance consisting of dilated dermal blood vessels with plump, hobnail-shaped endothelial cells. On the basis of the natural history and the histopathologic features we suggest the diagnosis of eruptive pseudoangiomatosis.
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ranking = 13.293637408556
keywords = blood vessel, vessel
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8/55. Meningioangiomatosis: report of three cases and review of the literature.

    Meningioangiomatosis is a rare condition, probably hamartomatous, characterized by proliferation of capillary-sized vessels, meningothelial cells, and fibroblasts within the cortex of the brain. Lesions may be single or diffuse and may be associated with neurofibromatosis type II. Clinically it presents with seizures but may be asymptomatic throughout life. We report 3 cases of meningioangiomatosis, 2 localized and 1 diffuse, all with different clinical manifestations. Differential diagnoses are discussed with a review of the literature. Since this condition is rare, close clinico-pathological correlation is essential. A correct diagnosis avoids further aggressive treatment.
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ranking = 1
keywords = vessel
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9/55. New micro vertical scissors for the surgical ablation of retinal angiomatous proliferation.

    PURPOSE: To develop new micro vertical scissors for the ablation of retinal angiomatous proliferation (RAP) to minimize possible retinal damage. DESIGN: Interventional case report. methods: New micro vertical scissors were developed and used for the ablation of RAP vessels. RESULTS: The length of the inner side of the tip is 180 microm, which is approximately one seventh that of regular vertical scissors. The scissors handled easily and the RAP vessels were ablated with less retinal damage compared with that associated with use of standard vertical scissors. Postoperatively, intraretinal hemorrhage was reduced and hyperfluorescence seen on indocyanine green angiography resolved. CONCLUSIONS: The new micro vertical scissors developed for the ablation of RAP vessels can be used safely and effectively during this surgical procedure.
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keywords = vessel
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10/55. Soft-tissue angiomatosis in adulthood: a case in the forearm showing a prominent myxoid adipose tissue component mimicking liposarcoma.

    Soft-tissue angiomatosis is a rare condition characterized by diffuse proliferation of benign vascular structures, accompanied by mature adipose tissue, which affect a large segment of the body in a contiguous fashion. Most patients present in childhood or adolescence. We report a case of large soft-tissue angiomatosis, located in the left forearm of a 65-year-old man, which showed extensive intramuscular and intermuscular infiltration, and embracement of the radius and the main vascular and neural bundles of the forearm. Due to the deep location, large size, ill-defined limits and non-homogeneous composition with areas suggestive of an adipose component in the radiological examination, the lesion raised the possibility clinically of a liposarcoma. The myxoid adipose tissue component of the lesion, a feature previously undescribed, and the predominance of small, capillary-sized vessels were considered concordant with the clinical diagnosis. This presentation is rare. The features of the lesion made complete surgical excision impossible and amputation of the extremity was considered the best treatment. The main differential diagnoses include infiltrating lipoma, myxolipoma, angiomyxolipoma, angiolipoma, intramuscular angioma, liposarcoma and low-grade myxofibrosarcoma. In conclusion, angiomatosis might simulate liposarcoma, particularly if the mass is large, heterogeneous, myxoid and appears later in life.
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keywords = vessel
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