1/153. The clinical superiority of continuous exposure versus short-pulsed carbon dioxide laser exposures for the treatment of pearly penile papules. Treatment of pearly penile papules was performed both with a conventional continuous-wave (CW) and a newer generation high energy pulsed carbon dioxide laser. When compared to the short pulsed laser, the CW laser, using relatively low power densities, provided superior hemostasis and improved visualization of the operative field. Despite the increase in thermal injury, wound healing was not compromised. The results of this case report support the CO2 laser in CW mode as the infrared laser treatment of choice for exophytic lesions with increased vascularity. ( info) |
2/153. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study. We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type iv, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract. ( info) |
3/153. 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. ( info) |
4/153. 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. ( info) |
| 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. ( info) |
6/153. 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. ( info) |
7/153. Angiomyofibroblastoma of the female urethra. BACKGROUND: Angiomyofibroblastoma is a relatively recently described rare tumor of the superficial soft tissues. To date, 57 cases of angiomyofibroblastoma of the external genitalia in women have been reported. methods/RESULTS: We describe a case of a 24-year-old woman who presented with the urinary stream flowing out in the posterior direction and whose diagnosis was a urethral tumor, angiomyofibroblastoma. CONCLUSIONS: Angiomyofibroblastoma has a potential arising from the female urethra as well as other areas of the external genitalia. ( info) |
8/153. 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. ( info) |
9/153. 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. ( info) |
10/153. 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. ( info) |