Disease relevance of Angiofibroma

• Recently it was reported that MEN1 patients also exhibit several cutaneous tumors, including multiple angiofibromas, collagenomas, and lipomas [1].
• Weak to moderate CD9 reactivity was also observed on EC of juvenile nasopharyngeal angiofibromas, angiosarcomas, and Kaposi's sarcomas [2].
• Tuberous sclerosis (TSC) is an autosomal dominant inherited disorder with cutaneous lesions of ash leaf hypopigmented macules, shagreen patches, periungual fibromas, facial angiofibromas, forehead fibrous plaques, confetti hypopigmentation, and poliosis [3].
• Postirradiation malignant fibrous histiocytoma arising in juvenile nasopharyngeal angiofibroma and producing alpha-1-antitrypsin [4].
• The diagnosis of juvenile nasopharyngeal angiofibroma was confirmed by histopathology, and the diagnosis of combined hamartoma was established by typical ophthalmoscopic and fluorescein angiographic findings [5].

Psychiatry related information on Angiofibroma

• Tuberous sclerosis is a dominant hereditary disease characterized by the appearance of angiofibromas on the face, epileptic attacks, and mental retardation [6].

High impact information on Angiofibroma

• In addition, somatic mutation of the adenomatous polyposis coli gene, which causes adenomatous polyposis when mutated in the germline, could play a role in the pathogenesis of sporadic nasopharyngeal angiofibroma [7].
• Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas [8].
• Genetic evidence that juvenile nasopharyngeal angiofibroma is an integral FAP tumour [9].
• Angiofibromas and collagenomas were more frequent in MEN1 patients (64% vs. 8% and 62% vs. 5%; P < 0.00001) and were multiple in 77-81% of the MEN1 patients [10].
• Of 44 patients, nine (20%) had facial angiofibromas, tumours primarily occurring in association with tuberous sclerosis [11].

Chemical compound and disease context of Angiofibroma

• Expression of androgen receptors in nasopharyngeal angiofibroma: an immunohistochemical study of 24 cases [12].
• Twenty-four nasopharyngeal angiofibromas were obtained from archived tissue, and immunocytochemical studies were performed with antibodies to AR, PR, and ER [12].
• Carbon dioxide and pulsed dye laser treatment of angiofibromas in 29 patients with tuberous sclerosis [13].
• To better define the tumor, 12 patients with juvenile angiofibroma have been studied by axial and coronal high resolution computed tomography (CT) [14].
• Estrogen receptors in nasopharyngeal angiofibromas [15].

Biological context of Angiofibroma

• Somatic mutations of the MEN1 tumor suppressor gene detected in sporadic angiofibromas [16].
• This may indicate that angiofibromas of tuberous sclerosis are heterogenous with respect to the collagen types they contain, and that there may be disturbed cell growth or collagen synthesis, with individual variation from case to case [17].
• Cellular senescence of angiofibroma stroma cells from patients with tuberous sclerosis [18].
• Expression of growth factors, proto-oncogenes, and p53 in nasopharyngeal angiofibromas [19].
• Genetic heterogeneity of the MYC oncogene in advanced juvenile angiofibromas [20].

Anatomical context of Angiofibroma

• In this report, we cultured the angiofibroma stroma cells of three adult TSC patients and compared these cells with normal skin fibroblasts for their proliferative capacity, cell morphology and mitotic cycle using a stain for microtubules and the expression of the senescent associated beta-galactosidase (SA beta-Gal) [18].
• Cytoskeletal 55 kd protein unique to cultured cells from angiofibroma of tuberous sclerosis [21].
• Multinucleate cells exhibited intermediate filaments of the vimentin type, but were not stained by endothelial cell or macrophage markers, and were factor XIIIa-. This condition must be addressed in the differential diagnosis of other skin vascular proliferations, such as Kaposi's sarcoma and angiofibromas [22].
• Long-term exposure to fibroblast growth factor type 1 (FGF1) of fibroblast-like cells derived from neurofibromas of patients with neurofibromatosis type 1, from angiofibromas of patients with tuberous sclerosis, and from foreskin of unaffected donors resulted in the outgrowth of monosomy 6 in 7 out of 14 cell lines examined [23].
• Giant cell angiofibroma of the eyelids: an unusual presentation of tuberous sclerosis [24].

Gene context of Angiofibroma

• Expression of glial fibrillary acidic protein (GFAP) by cultured angiofibroma stroma cells from patients with tuberous sclerosis [25].
• It is unclear whether there is also a loss of hamartin, the product of TSC1 in TSC-associated and sporadic angiofibromas [26].
• Loss of expression of tuberin and hamartin in tuberous sclerosis complex-associated but not in sporadic angiofibromas [26].
• These results argue against the significant role of androgen receptor in the growth of nasal angiofibromas and corroborate previous observations of an unpredictable response of these neoplasms to antiandrogen therapy [27].
• OBJECTIVE: We investigated the expression of matrix metalloproteinase (MMP) and its tissue inhibitor (TIMP) in fibrous plaques, angiofibromas, and lesion-free skin specimens from patients with tuberous sclerosis complex [28].

Analytical, diagnostic and therapeutic context of Angiofibroma

• Cell cultures were derived from angiofibromas of three patients with tuberous sclerosis (TSC), from the unaffected skin of these patients, and from the skin of five healthy donors [29].
• The distribution of collagen types in angiofibroma and shagreen patch from patients with tuberous sclerosis was examined biochemically and immunohistochemically by indirect immunofluorescence microscopy using type-specific anti-collagen antibodies [30].
• METHODS: The expression of hamartin and tuberin was analyzed by immunohistochemistry in 59 TSC-associated and 12 sporadic angiofibromas using affinity-purified antibodies [26].
• Dermabrasion for the management of angiofibromas in tuberous sclerosis [31].
• A case of juvenile nasopharyngeal angiofibroma in a 14 year old boy is presented on account of the endocrinological investigation performed before, during, and after treatment with 15 mg/day diethylstilbestrol for more than one month [32].

References