Cases reported "Angiofibroma"

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1/29. Giant cell angiofibroma of the orbit and eyelid.

    PURPOSE: To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN: Observational case series. MAIN OUTCOME MEASURES: Clinical and histopathologic features of giant cell angiofibroma. methods: light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS: A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION: Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.
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2/29. Angiofibroblastoma of the skin: a histological, immunohistochemical and ultrastructural report of two cases of an undescribed fibrous tumour.

    AIMS: The aim of this report is to present two cases of a distinct mesenchymal tumour of the skin that does not fit into one of the established entities. methods AND RESULTS: All cases of fibromyxoid tumours from the files of two dermatopathology centres, together with clinical data and histopathological records, were reviewed. Two cases of a lesion composed of stellate and spindle-shaped cells with the phenotype of fibroblasts embedded in a fibromyxoid to dense fibrous stroma were identified. Because of the large number of capillary-sized blood vessels and their peculiar distribution within the stroma, the name angiofibroblastoma of the skin is proposed for this peculiar neoplasm. In both cases, the tumour appeared as an indolent and slowly growing nodule on the extremities of adults. Immunohistochemical and, in one case, ultrastructural studies were performed. CONCLUSIONS: The lack of recurrences during a follow-up period of 9 and 3 years, respectively, after complete excision together with the bland histopathological appearance supports the benign character of the tumour.
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3/29. Angiectatic nasal polyps that clinically simulate a malignant process: report of 2 cases and review of the literature.

    BACKGROUND: Approximately 5% of inflammatory or allergic sinonasal polyps develop extensive vascular proliferation and ectasia with deposition of pseudoamyloid. These so-called angiectatic nasal polyps (ANPs) can grow rapidly and exhibit an aggressive clinical behavior that could simulate malignancy preoperatively. OBJECTIVE: To systematically address the differential histologic diagnosis of ANPs. methods: We evaluated by light microscopy, immunohistochemistry, and electron microscopy biopsy and resection specimens from 2 large ANPs (8 and 10 cm in diameter) that presented in 2 adult men with life-threatening epistaxis and facial deformity, respectively. RESULTS: The tumors were firm, lobulated, and covered by smooth, partially ulcerated mucosa. Histologically, clusters of dilated, thin-walled blood vessels embedded in pools of congo red-negative eosinophilic material, associated with patchy necrosis and atypical stromal spindle cells, were seen. Electron microscopy and immunohistochemistry (CD34, factor viii) confirmed the endothelial nature of the cells lining the spaces, whereas the atypical stromal cells were classified as myofibroblasts. CONCLUSIONS: These 2 cases represent extreme examples of ANPs that clinically simulate a malignant process. awareness of the histological features of ANPs should prevent confusion of such lesions with other vascular or spindle cell lesions of the nasopharynx that would require different treatment and carry a different prognosis.
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4/29. Cerebral angiofibroma: case report.

    OBJECTIVE AND IMPORTANCE: Intracranial fibromatous tumors are very rare lesions, with few reported cases. CLINICAL PRESENTATION: We report the case of a 34-year-old male patient who experienced seizures resulting from a cystic lesion in the left occipital region, which remained unchanged for 11 years. After the seizures increased in number, magnetic resonance imaging revealed a large cyst with a tumor nodule. INTERVENTION: A left occipital craniotomy was performed, and the tumor was removed. Pathological studies, including immunohistochemical and ultrastructural analyses, indicated that this neoplasm was composed of fibrous and angiomatous components, and a diagnosis of cerebral angiofibroma was established. CONCLUSION: Cerebral and meningeal fibromas are rare neoplasms that differ from solitary fibrous tumors and fibrous meningiomas. When a number of prominent blood vessels are present in a cerebral or meningeal fibroma, a diagnosis of angiofibroma can be considered. It is possible that some nodular brain tumors that were previously described as meningioangiomatosis could be reclassified as cerebral or meningeal angiofibromas.
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5/29. Giant cell angiofibroma of the inguinal region.

    Giant cell angiofibroma is a rare mesenchymal neoplasm most commonly arising in the soft tissues near the orbit. Recently, several cases of extraorbital giant cell angiofibroma have been reported. We report the light microscopic and immunohistochemical features of an additional case of extraorbital giant cell angiofibroma arising in the inguinal region that was clinically mistaken for an inguinal hernia. The patient was a 50-year-old woman who presented with a mobile, nonreducible, left inguinal mass. The tumor was 10.8 cm in greatest diameter, was well circumscribed, and appeared to be encapsulated. Histologically, the tumor was composed of a mixture of cytologically bland spindle-shaped cells and ovoid cells of varying cellularity with deposition in a variably collagenous and myxoid stroma. The tumor had prominent, various-sized blood vessels, often with perivascular hyalinization. In addition, scattered pseudovascular spaces filled with an amorphous eosinophilic material were present and lined by spindle-shaped and ovoid cells similar to those found throughout the neoplasm. Rare multinucleated floret-like giant cells were seen. Immunohistochemically, the tumor cells stained strongly and diffusely for both CD34 and bcl-2 while immunostains for S-100 protein, desmin, smooth muscle actin, and muscle-specific actin were negative. There is no evidence of local recurrence or metastasis 3 months following excision of the mass. This report emphasizes the recognition of this unusual tumor in extraorbital sites. We discuss the overlapping histologic and immunophenotypic features with giant cell fibroblastoma and solitary fibrous tumor and raise the possibility that these tumors could represent a histologic spectrum of CD34-positive dendritic interstitial cell neoplasms.
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6/29. Angiomyofibroblastoma of the male inguinal region.

    We present a case of benign angiomyxoid tumor arising in the inguinal region of a 27-year-old man. The tumor was a gelatinous mass completely encapsulated by a thin fibrous capsule with no hemorrhage or necrosis. Histologically, a proliferation of spindle cells as well as occasional pleomorphic cells was observed within the myxofibrous stroma, intermingled with abundant capillary-sized blood vessels. Immunohistochemical staining of the tumor demonstrated spindle, oval, and pleomorphic cells equally positive for vimentin, desmin, and CD34, but not for alpha-smooth muscle actin. Based on these histologic and immunophenotypic features, we conclude that this angiomyxoid tumor of the male inguinal region is indistinguishable from the female angiomyofibroblastoma of the pelvic and perineal regions.
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7/29. Cellular angiofibroma of the vulva with dna ploidy analysis.

    Cellular angiofibroma (CAF) is a recently described rare soft tissue neoplasm of the vulva (with only four reported cases) that typically occurs as a well-circumscribed solid rubbery vulvar mass in middle-aged women. The distinct histologic features of bland spindle cells admixed with numerous hyalinized medium to small blood vessels, and a vimentin-positive desmin-negative immunoprofile differentiates this neoplasm from other vulvar tumors such as angiomyofibroblastoma and aggressive angiomyxoma. In this report an additional case of CAF is presented with dna ploidy analysis and CD99 immunohistochemistry.
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8/29. Cellular angiofibroma of the vulva.

    BACKGROUND: The cellular angiofibroma is a benign mesenchymal neoplasm that clinically and histologically must be distinguished from biologically more aggressive lesions. It typically arises in women of late reproductive age and lends itself to cure by complete local excision. A report of an unusual case in a postmenopausal patient is presented. CASE: A 77-year-old woman presented with a painless vulvar mass that slowly enlarged over 3 years. Past history included a hysterectomy and bilateral salpingo-oophorectomy followed by estrogen replacement therapy. Surgical excision of the mass was performed and there is no evidence of recurrence 1 year postoperatively. Histopathologic examination revealed an admixture of hyalinized blood vessels and loose cellular stroma characteristic of a cellular angiofibroma. Immunohistochemical studies revealed stromal cell immunoreactivity for vimentin and CD34 and nonreactivity for desmin, actin, and S100 protein. The nuclei of the stromal cells demonstrated strong reactivity for estrogen and progesterone receptors. CONCLUSION: Mesenchymal lesions of the vulva and perineum include both benign and malignant neoplasms. The cellular angiofibroma is benign; however, other lesions including the aggressive angiomyxoma must be excluded when arriving at that diagnosis. The role of long-term estrogen therapy in the genesis of this tumor awaits further analysis.
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9/29. Giant cell angiofibroma of the nasolacrimal duct.

    PURPOSE: To describe clinical and histologic features of the first case, to our knowledge, of giant cell angiofibroma located in the nasolacrimal duct region in a 28-year-old woman. methods: Interventional case report. A left nasolacrimal duct tumor was excised en bloc by lateral rhinotomy. Histopathologic examination was performed with the use of light microscopy. Immunohistochemical staining included S-100 protein, muscle-specific actin, desmin, myoglobin, vimentin, and CD34. RESULTS: The lesion was characterized by haphazardly arranged oval to spindled cells, a myxoid and collagenous stroma, multinucleated giant cells, prominent blood vessels, and pseudovascular spaces. Tumor cells were strongly positive for vimentin and CD34 and were negative for other antigens. After excision, there has been no recurrence over 4 years of follow-up. CONCLUSIONS: Originally described as an orbital tumor, giant cell angiofibroma also may occur in the nasolacrimal duct and lacrimal sac region. This mesenchymal neoplasm should be included in the differential diagnosis of lacrimal drainage system tumors.
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10/29. Resection of nasopharyngeal angiofibroma with extranasal and extrapharyngeal involvement.

    The authors report their own experiences with the diagnosis and therapy of 7 cases of nasopharyngeal angiofibroma with extranasal and extrapharyngeal involvement, thereby attempt to formulate the surgical approach for resection of huge nasopharyngeal angiofibroma with extensive involvement. The results suggested that appropriate surgical approach was of paramount importance for eliminating the tumor and may effectively reduce traumatic injury during the operation. Elective embolization of the vessels feeding the tumor could reduce bleeding and therefore increase the safety of the operation.
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