1/109. Genital angiofibromas in tuberous sclerosis: two cases.Cutaneous lesions are an important feature of tuberous sclerosis (TS). Facial angiofibromas usually begin to appear at the end of the first decade of life and are considered to occur in about 88% of adults with TS. They are only rarely reported on sites other than the face or front. In this paper, we report two patients with the complete syndrome of TS that had, in addition to classic facial lesions, multiple papules on the genital area. Histopathology confirmed the diagnosis of angiofibroma. One patient's lesions were misdiagnosed as genital warts and were so being treated before the correct diagnosis was made. Although we don't know the incidence of genital angiofibromas in TS patients, we believe that they might be underreported, because dermatologists generally don't look for them when they see these patients.- - - - - - - - - - ranking = 1keywords = angiofibroma (Clic here for more details about this article) |
2/109. Does the tuberous sclerosis complex include intracranial aneurysms? A case report with a review of the literature.BACKGROUND: tuberous sclerosis is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of symptoms and clinical features. OBJECTIVE: diagnosis can be very difficult in cases with incomplete manifestations (formes fruste) lacking the classic signs of the disease. MATERIALS AND methods: We report a case fulfilling the diagnostic criteria for tuberous sclerosis (shagreen patches, hypomelanotic macules, renal cysts and angiomyolipomas, and "migration tracts" in the cerebral white matter) in association with a giant intracranial aneurysm, but lacking mental retardation, epilepsy and facial angiofibroma. RESULTS: Fourteen other cases of tuberous sclerosis and intracranial aneurysms, all but one without any clear sign of polycystic kidney disease, were found in the literature. CONCLUSION: We suggest that vascular dysplasias in general and aneurysms (mainly intracranial) in particular can be added to the other non-primary diagnostic features for the clinical diagnosis of tuberous sclerosis.- - - - - - - - - - ranking = 0.14285714285714keywords = angiofibroma (Clic here for more details about this article) |
3/109. Giant cell angiofibroma of the eyelids: an unusual presentation of tuberous sclerosis.The authors describe a patient with a history of mental retardation and seizures who presented with unusually large edematous, pedunculated eyelid lesions of unknown etiology. The histopathology revealed features consistent with giant cell angiofibroma, a variant of the angiofibromas commonly seen in tuberous sclerosis.- - - - - - - - - - ranking = 0.85714285714286keywords = angiofibroma (Clic here for more details about this article) |
4/109. 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.- - - - - - - - - - ranking = 1.1428571428571keywords = angiofibroma (Clic here for more details about this article) |
5/109. Endoscopic treatment of juvenile nasopharyngeal angiofibroma.Traditional treatment of juvenile nasopharyngeal angiofibromas (JNAs) has included open surgical approaches for the majority of tumors. At the University of washington Medical Center (UWMC), endoscopic techniques have been used for the removal of some small JNAs. This report describes the institutional experience in treating these tumors. The medical records of 15 patients at UWMC treated over a 15-year period for JNA were reviewed. Three patients were treated only by an endoscopic approach, and one patient had a combined endoscopic and open procedure. All three of the patients treated only by the endoscopic approach were disease free with a minimum of 24 months follow up. The one patient treated with a combined endoscopic and open approach had recurrence of disease. Endoscopic removal after embolization effectively treated three patients with early stage JNAs. Indications for this procedure are discussed.- - - - - - - - - - ranking = 0.71428571428571keywords = angiofibroma (Clic here for more details about this article) |
6/109. Extraorbital giant cell angiofibroma.A number of intriguing fibrovascular mesenchymal proliferations with benign or low grade malignant potential have recently been described. Giant cell angiofibroma was introduced as an entity by Dei Tos et al. in 1995 and initially considered to be a lesion of the orbit. We describe an extraorbital example, indicating that giant cell angiofibroma is not confined to the orbit. Immunologically, giant cell angiofibroma is positive for CD 34, bcl-2 and vimentin, and negative for epithelial and muscle markers, and S-100. The tumor shares several morphological and immunological properties with giant cell fibroblastoma and solitary fibrous tumor, yet it features a histology sufficiently characteristic to allow its categorization as a separate entity. The recommended treatment is complete but conservative excision. Metastases have not been reported.- - - - - - - - - - ranking = 1keywords = angiofibroma (Clic here for more details about this article) |
7/109. Nasopharyngeal angiofibroma in a patient with haemophilia A: a bleeding tumour in a bleeding-prone patient.Nasopharyngeal angiofibroma is a highly vascular tumour which occurs almost exclusively in adolescent males. Although it is histologically benign, it may cause serious clinical problems because of its tendency to bleed profusely during surgery. This paper presents the first case of nasopharyngeal angiofibroma in a patient with haemophilia A, another well-known disease of bleeding tendency. In this case the tumoural mass was surgically removed with effective factor viii replacement without any bleeding complication.- - - - - - - - - - ranking = 0.85714285714286keywords = angiofibroma (Clic here for more details about this article) |
8/109. Application of artificial dermis prior to full-thickness skin grafting for resurfacing the nose.Two patients with nasal skin defects resulting from excision of rhinophyma and multiple angiofibromas were treated with artificial dermis followed by full-thickness skin grafts taken from the postauricular region. The secondary skin grafts took completely in both patients, and the postoperative results were excellent. Although a two-stage operation is required, application of artificial dermis prior to full-thickness skin grafting is a reliable method for resurfacing the nose.- - - - - - - - - - ranking = 0.14285714285714keywords = angiofibroma (Clic here for more details about this article) |
9/109. adult-onset angiofibroma and multiple endocrine neoplasia type I.multiple endocrine neoplasia type 1 (MEN 1) predisposes affected persons to neoplasms of the parathyroid glands, the endocrine pancreas, the anterior pituitary, and the duodenum. We report the first case of adult-onset multiple angiofibromas of the central face associated with MEN 1. Seven siblings also developed adult-onset angiofibromas, none with evidence of tuberous sclerosis. Basic fibroblast growth factor (BFGF) is elevated in many patients with MEN 1 and may play a pathogenetic role.- - - - - - - - - - ranking = 0.85714285714286keywords = angiofibroma (Clic here for more details about this article) |
10/109. Giant cell angiofibroma of the oral cavity: report of a new location for a rare tumor.Giant cell angiofibroma is a rare, soft tissue tumor that was first described in the orbit. Since then, several case reports have described this tumor in a number of extra-orbital sites, suggesting a wider anatomic distribution than is generally recognized. The tumor typically acts in a benign fashion with only rare local recurrences but no tendency to metastatic disease. Here, we report the first case of a giant cell angiofibroma in the oral cavity. The tumor presented as a soft tissue nodule on the buccal mucosa of a 60-year-old man. The histologic differential diagnosis included a number of other uncommon soft tissue neoplasms, including giant cell fibroblastoma, solitary fibrous tumor, and pleomorphic lipoma. The histologic and immunohistochemical features of this tumor and differentiation from other histologically similar soft tissue neoplasms are briefly discussed.- - - - - - - - - - ranking = 0.85714285714286keywords = angiofibroma (Clic here for more details about this article) |
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