1/38. Expression of CD34 in sclerotic ("plywood") fibromas.CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor xiiia or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/38. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/38. Solitary fibrous tumor of the thoracic spine. Case report and review of the literature.Solitary fibrous tumor (SFT) is a neoplasm first described as a tumor of mesenchymal origin involving soft tissues. The authors provide a review of the literature with detailed pathological analysis and radiological description of SFTs involving the central nervous system. The authors report a rare case of a SFT of the thoracic spine in an adult man presenting with myelopathy. magnetic resonance imaging revealed a well-circumscribed, intradural, extramedullary mass at the T2-3 level. Histological examination demonstrated a proliferation of predominantly spindle-shaped cells with a collagen-matrix background. Immunohistochemical staining was positive for vimentin and CD34 and negative for S-100 and epithelial membrane antigen with an MIB-1 labeling index of 2.6%. review of the literature revealed 10 cases in which this tumor, frequently found in the pleura, was involved in the central nervous system. Although rare and their clinical significance as yet unknown, SFTs may be considered in the differential diagnosis of intradural spinal cord lesions. The natural history is also unknown, but these tumors appear to be biologically benign.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/38. Aggressive CD34-positive fibrous scalp lesion of childhood: extrapulmonary solitary fibrous tumor.Although solitary fibrous tumor (SFT) was originally described as a pleural tumor, an increasing number of extrapleural sites of SFTs have been documented. This has been attributed not only to the heightened awareness of the spectrum of histopathological features that characterizes SFTs but also to the recognition of the role of CD34 immunostaining in soft tissue tumors in general, and in SFTs in particular. Despite the large number of documented extrapleural SFTs in adults, cranial SFTs are rare, having been documented in the meninges, scalp, and infratemporal fossa. Extrapleural SFTs are, to date, an unrecognized entity in children. We document an aggressive fibrous scalp lesion in a 30-month-old female child that demonstrated features common to benign cranial fasciitis and SFT. However, based on bright, diffuse CD34 antigen immunopositivity, a diagnosis of SFT was made. The need to include the CD34 antigen stain in a panel of immunohistochemical markers used to assess spindle cell lesions of childhood is emphasized.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
5/38. Meningeal solitary fibrous tumor as an unusual cause of expohthalmos: case report and review of the literature.OBJECTIVE AND IMPORTANCE: We report an extremely rare case of a patient with meningeal solitary fibrous tumor with orbital involvement presenting as unilateral exophthalmos. This rare tumor should be considered in the differential diagnosis for aggressive dural-based lesions. CLINICAL PRESENTATION: A 54-year-old man presented with a protruded eyeball on the right side and left hemiparesis. Computed tomography and magnetic resonance imaging demonstrated a huge mass, markedly enhanced in and around the right anterior clinoid process, which extended to the orbit and middle cranial fossa. cerebral angiography revealed a richly vascular tumor fed by branches of both the right external and internal carotid arteries. A presumptive diagnosis of meningioma or hemangiopericytoma was considered. INTERVENTION: The tumor was exposed through an orbitozygomatic approach. At surgery, the tumor was grossly firm to hard and had destroyed the dura, orbital roof, anterior clinoid process, temporal bone, and muscle. Histological analysis revealed that the tumor was composed of spindle-cell proliferation in a collagen-rich background, but it exhibited regional variations. The vascular network demonstrated irregular vascular lumina with a "hemangiopericytoma-like" pattern. Histological features included high cellularity and a high degree of pleomorphism. Immunohistochemical analysis of the tumor demonstrated diffuse positive staining for CD34 and vimentin. The tumor displayed no positive staining for cytokeratin, epithelial membrane antigen, glial fibrillary antigenic protein, S-100 protein, and factor XIII. CONCLUSION: Meningeal solitary fibrous tumor is considered a unique pathological entity. Wider use of immunohistochemical screening should enable analysis of the real incidence of these tumors; larger series and longer follow-up duration will allow conclusions to be drawn regarding treatment and prognosis. Differential diagnosis is discussed and the literature is reviewed.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
6/38. Two cases of supernumerary ovary: one with large fibroma with Meig's syndrome and the other with endometriosis and cystic change.Reports of supernumerary ovaries are rare. We describe two such cases, one with fibroma and the other with endometriosis and cystic change. A large fibroma measuring 17.4 x 12.0 x 7.5 cm in size was found in the supernumerary ovary of the omentum in the first case of a 47-year-old married woman with Meig's syndrome. The second case was associated with endometriosis and cystic change, measuring 11 x 5 x 3 cm in size and located in the upper abdominal cavity. It was attached to the uterus of a 28-year-old pregnant woman who had neither fibroma nor Meig's syndrome. Histologically, corpus albicans and a few primordial germ cells were demonstrated, respectively. A fibroma showing a storiform pattern was found in the first case. The second case had endometriosis and a thin-walled cyst with bleeding and necrosis caused by torsion. Immunohistochemically, desmin, alpha-smooth muscle actin, c-kit, CA125, Na /K ATPase, overexpression of p53, myc and ras were all negative in the fibroma cells of the first case, and in the endometriosis and cyst wall of the second case. The fibroma cells were positive for vimentin and estrogen receptor, and the proliferating cell nuclear antigen was sporadically demonstrated in their nuclei. The mutation of the p53 gene at exons 5-8 was not detected by sequence analysis. Using RT-PCR, bax, bcl-2 and p16 were not detected either. Clinically, the two cases presented here did not show abnormal hormonal symptoms. They were diagnosed as abdominal tumors or masses. Based on these considerations, one might assume that supernumerary ovaries are probably more frequent than reported at present.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/38. Non-functioning ovarian fibroma with extensive calcification: case report.A case of a non-functioning ovarian fibroma with extensive calcification in a young Ugandan female is described. Ovarian fibromas occasionally may be bilateral and associated with benign ascites and pleural effusion called Meigs' syndrome or related to a rare hereditary condition known as Gorlin's syndrome. Rarely if functioning, the tumour may produce hormones to cause diabetes mellitus or hypoglycaemia or secrete carbohydrate antigen 125 to clinically simulate ovarian carcinoma.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/38. Solitary fibrous tumours of the lower gingiva: a case report.Solitary fibrous tumours (SFTs) are rare lesions in the oral cavity. Typically they arise in the pleura. We report a lesion occurring in the lower anterior gingiva. In histochemical examination, the spindle-shaped, neoplastic cells stained strongly for CD34 antigen and vimentin, but did not stain for desmin, smooth-muscle actin, muscle actin and S-100 protein. The expression of CD34 antigen and vimentin were useful for the differential diagnosis.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/38. Superficial acral fibromyxoma: report of two cases.Superficial acral fibromyxoma (SAFM) is a rare soft tissue tumor that has recently been delineated as a separate entity. We report 2 cases of SAFM and discuss its pathological features and differential diagnosis. Both patients had lesions on the toe. In 1 patient, the tumor was found after nail extraction, which had been performed for the treatment of onychomycosis, whereas in the other patient the tumor itself was the reason for seeking dermatological assistance. Biopsies from both cases demonstrated similar features. There was a moderately circumscribed, non-encapsulated tumor extending through the whole dermis. The neoplasm was composed of spindle and stellate cells with slight nuclear atypia arranged in a loose storiform, partly fascicular growth pattern. In 1 case, strands of cells with rather wavy nuclei were seen at the periphery of the tumor. Mitotic figures were scarce. The neoplastic cells were embedded in a myxoid stroma with increased numbers of small blood vessels and scattered mast cells. Immunohistochemically, the tumor cells showed weak focal positivity for CD34 and stained negatively for S-100 protein and alpha-smooth muscle actin. In 1 case epithelial membrane antigen (EMA) was negative, whereas in the second case focal expression of EMA by neoplastic cells was seen. alcian blue staining revealed abundant mucinous material within the stroma. In conclusion, SAFM represents a distinct entity in the spectrum of cutaneous myxoid tumors. The differential diagnosis of SAFM includes various myxoid neoplasms and tumors with a predilection for distal parts of the extremities.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/38. Solitary fibrous tumor of the hypoglossal nerve.Summary: We report a case of solitary fibrous tumor (SFT) causing isolated hypoglossal nerve palsy. The neuroimaging appearance of the tumor was indistinguishable from that of schwannoma or meningioma. Immunohistochemical tests demonstrated strong reactivity for CD34 but an absence of staining for S100 and epithelial membrane antigen; this profile is indicative of an SFT. SFTs are mesenchymal tumors that can affect the dura-covered segments of cranial nerves. They may be considered in the differential diagnosis of an isolated cranial nerve palsy.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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