1/7. 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.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/7. Angiomyofibroblastoma of the vulva.A case of angiomyofibroblastoma of the vulva in a 53-year-old woman was examined by means of immunohistochemistry. Histological examination of the tumor revealed typical characteristics of vulval angiomyofibroblastoma. immunohistochemistry of the tumor cells revealed diffuse immunoreactivity for estrogen receptors, progesterone receptors, vimentin, and CD34. The stains for cytokeratin, epithelial membrane antigen, desmin, smooth muscle alpha-actin, muscle-specific actin, and S-100 were negative. These results were mostly consistent with those of previous reports and suggest that the tumors cells were derived from primitive mesenchymal cells which occur normally in this lesion and which show the potential for diverse lines of myoid differentiation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/7. Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature.BACKGROUND: Cellular angiofibroma (CA) of the vulva is a recently described condition, whose clinical and pathological features are poorly known. methods: We have encountered two cases of this very unusual tumor. Their clinical and pathological features were analyzed and compared to those reported in the literature. RESULTS: Both patients were middle-aged women. In each case, the lesion had the clinical appearance of a vulvar cyst, located in the lateral aspect of the clitoris and the right labium majus, respectively. Microscopically, the lesions were well circumscribed but not truly encapsulated. Both were composed of small spindle cells arranged in short fascicles and mixed up with relatively abundant small- or medium-sized rounded vessels. While mitotic activity was perceptible in both cases, no cellular atypia could be demonstrated. A striking feature seen in one case was the presence of pseudoangiomatous changes in the stroma, similar to those occasionally found in spindle cell lipoma. Phenotypically, the tumor cells consistently expressed vimentin, CD99, and both estrogen and progesterone receptors. A discrete CD34 or smooth muscle actin immunoreactivity was also found in one case. No expression of S-100 protein, Bcl-2 protein, CD117 (c-kit gene product), epithelial membrane antigen, desmin, or h-caldesmon could be demonstrated. CONCLUSION: This study further illustrates that CA of the vulva has distinct clinical and pathologic features that set it apart from the other soft tissue conditions involving this area. However, like many soft tissue neoplasms, this tumor also exhibits some variation in its histological or immunohistochemical features.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/7. Angiomyxofibromatous tumor of the falx cerebri.We report a distinctive angiomyxofibromatous lesion arising from the falx cerebri of a 48-year-old woman. The tumor was composed of bland-appearing, spindle, and stellate cells in a myxoid matrix with prominent vascularity. The tumor cells were immunopositive diffusely for vimentin and focally for S-100 protein, but were immunonegative for epithelial membrane antigen, CD34, MIC2, Bcl-2, glial fibrillary acidic protein, cytokeratin CAM 5.2, desmin, and smooth muscle actin. This lesion could not be categorized according to the current world health organization classification of tumors of the nervous system, thus underscoring a need to enhance our understanding of myxoid mesenchymal neoplasms and reassess their nosology.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/7. HMB-45 and tuberin in hamartomas associated with tuberous sclerosis.tuberous sclerosis (TSC) is an autosomal dominant condition characterized by various hamartomas. To assess whether these hamartomas have common features, we immunohistochemically analyzed an autopsy case of TSC with subependymal giant cell astrocytoma, hamartomatous lymphangioleiomyomatosis in the lungs and uterus, and angiomyolipomas in the liver, bilateral kidneys, parametrium, and a lymph node, using the specific antibodies for tuberin (the gene product of TSC2) and HMB-45 (a monoclonal antibody specific for human melanoma). Tuberin was ubiquitously expressed in the normal organs. It was negative or very weakly expressed in the hamartomas. In contrast, HMB-45 was commonly observed in the hamartomas except for the lesions in the brain. The subependymal giant cell astrocytoma was composed of cells immunoreactive for synaptophysin, neurofilament protein, glial fibrillary acidic protein, and S-100 protein but negative for HMB-45 and tuberin. The suppression of tuberin in the brain tumor and visceral hamartomas and an abnormal expression of HMB-45 in the visceral hamartomas were observed. We conclude that the hamartomas associated with TSC have common phenotypic and etiologic expressions and that the genes responsible for both tuberin and the antigen recognized by HMB-45 have important roles in the pathogenesis of these hamartomas.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/7. Extraorbital giant cell angiofibromas.We herein describe two unusual neoplasms showing histopathologic features consistent with those of giant cell angiofibroma, which was originally described as a neoplasm arising in the orbit in adults: one of them arose in the right submandibular region of a 48-year-old woman and the other in the right parascapular region of a 49-year-old woman. Macroscopically, although the latter was characterized by a lymphangioma-like cystic appearance, both tumors were well circumscribed and encapsulated. Microscopically, in both cases, pseudovascular spaces lined by a discontinuous row of multinucleated cells were seen against a background of spindle-shaped fibroblastic cell proliferation. In the second case, the tumor presented increased cellularity and plump and somewhat atypical nuclei of proliferating fibroblastic cells, compared with the tumor in the first case. Immunohistochemically, the mononuclear and multinucleated cells within these tumors were positive for vimentin and CD 34 but negative for any other antigens, including factor viii-related antigen, desmin, alpha smooth muscle actin, myoglobin, S-100 protein, LeuM1, lysozyme, alpha-1-antitrypsin, and cytokeratins (AE1/AE3 and CAM5.2). The features in these cases indicate that giant cell angiofibroma can arise in an extraorbital site in middle-aged patients and presents some histopathologic diversity.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
7/7. A cutaneous case of giant cell angiofibroma occurring with dermatofibrosarcoma protuberans and showing bimodal CD34 fibroblastic and FXIIIa histiocytic immunophenotype.Dei Tos and colleagues in 1995 reported a series of seven distinctive orbital tumors in adults which they named giant cell angiofibroma (GCA). The morphologic features are intermediate between giant cell fibroblastoma and solitary fibrous tumor with a richly vascularized, patternless spindle cell proliferation forming a collagenous or myxoid stroma with pseudovascular angiectoid spaces. The spindled tumor cells have large, rounded nuclei, sometimes with complex folded shape and pseudoinclusions. There also are multi- or mononuclear giant cells, and these tumor cells partly line so-called angiectoid spaces. Cells express human progenitor cell antigen CD34 and vimentin. One case in the buccinator fascia was also noted by the authors, but similar cutaneous lesions are thus far unknown. We report our experience with a polypoid tumor that ocurred on the thigh of a 49-year-old woman that conforms to the description of GCA. The tumor has variegated vessels admixed with patternless spindle and giant cell stroma with angiectoid spaces as well as areas of dermatofibrosarcoma protuberans (DFSP). Most tumor cells express vimentin and CD34, including giant and spindle cells lining angiectoid spaces. Focally up to 40% of the lesional cells express coagulation factor xiiia with histiocytoid to highly dendritic cytosomes. The DFSP component is composed of admixed CD34 and FXIIIa dendritic cells arranged in a storiform pattern. Tumor cells are negative for actin, desmin, S-100, and cytokeratin. The Ki67 proliferation index is 1% in GCA areas and 3% in DFSP areas; Ki 67 stains mainly fibroblasts. We conclude that this cutaneous GCA is a fibrohistiocytic tumor closely related to and representing a more organoid angioformative analog of GCF, with both being related histogenetically also to DFSP. These lesions represent part of a greater spectrum of fibrovascular tissue patterns, all probably derived from proliferations of interactive microvascular CD34 fibroblasts and FXIIIa histiocytes.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |