1/36. Nuchal fibroma associated with scleredema, diabetes mellitus and organic solvent exposure.A case of scleredema diabeticorum of Buschke associated with nuchal fibroma and organic solvent exposure is reported. The patient presented with a neck mass causing discomfort and restriction of movement. Histological examination showed this to be a nuchal fibroma. Additionally, there was widespread induration of the skin of his trunk which was asymptomatic. A biopsy showed features of scleredema. This is the first reported association of these two conditions, both of which show increased and thickened collagen bundles without significant fibroblast proliferation. They differ by the occurrence of mucin in scleredema, although this is not always demonstrable, particularly in late lesions. The possibility that nuchal fibroma is an end stage, localized form of scleredema is canvassed. The patient's medical history included insulin-dependent diabetes mellitus with complications of retinal vessel thrombosis and peripheral neuropathy. The patient also had significant past exposure to a wide variety of chemicals, including organic solvents.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/36. 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 = vessel (Clic here for more details about this article) |
3/36. fibroma of a tendon-sheath presenting as toe deformity.We describe a rare manifestation fibroma in a tendon-sheath in an 83-year-old man. The patient complained initially of a slowly progressive spreading, apart of the right second and third toes. A mass was found in the plantar aspect of the foot. It was completely excised at surgery. Histological examination revealed sparse spindle or stellate cells with slit-like vessels in the dense collagenous matrix. There had been no recurrence at follow-up 17 months after surgery.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/36. Localized birt-hogg-dube syndrome with prominent perivascular fibromas.The autosomal dominant birt-hogg-dube syndrome is a cutaneo-intestinal condition that manifests on the skin in the form of multiple, skin-colored small papules that, histologically, prove to be mantleomas (fibrofolliculomas and trichodiscomas). These cutaneous lesions usually appear in the region of the head, neck, and upper part of the trunk. To date, only a single report in the literature describes the localized occurrence of this syndrome. We now describe a localized form of the birt-hogg-dube syndrome in a man with multiple mantleomas that were confined to the left half of the face, and which, in part, were arranged in the form of plaques. Another striking finding in this patient was a conspicuous vascular component in the lesions, characterized by a pronounced, well-demarcated fibrosis in the region of cutaneous blood vessel proliferations. Because perivascular fibromas have already been observed in other patients with Birt-Hogg-Dube-syndrome, the perivascular fibroma, with fibrofolliculoma and trichodiscoma, must be included within this syndrome's spectrum of skin changes.- - - - - - - - - - ranking = 12.103502064177keywords = blood vessel, vessel (Clic here for more details about this article) |
5/36. Giant-cell fibroblastoma: a case report emphasising the presence of hyperplastic subplasmalemmal linear densities in continuity with granular matrices in the extracellular space.The histological, immunohistochemical and ultrastructural features of a case of giant-cell fibroblastoma from the soft tissues of the chest wall in a 48-year-old female are described with special reference to the cell surface and matrix. Subplasmalemmal linear densities (SLDs) characterised cell surfaces, and exhibited excessive development of the dense external component: foci of identical dense material were present in the matrix. The nature of these dense foci, both the external component of the SLD and those free in the extracellular space, was investigated by light microscope immunostaining for fibronectin, laminin and collagen IV. All three proteins stained vessels. There was weaker but positive staining for tumour cell surfaces and matrix, consistent with the widely dispersed nature of the dense foci. Given their fine structural appearance, these dense foci can be referred to as granular matrices. Given also that the matrix protein immunostaining pattern is consistent with the distribution of these granular matrices as observed by electron microscopy, they may be provisionally interpreted as a kind of basement-membrane-related granular matrix. The presence of these proteins emphasises the point that, while giant-cell fibroblastoma fibroblasts lack a lamina, they nevertheless bear basement-membrane-related proteins organised, however, in a non-laminate fashion. The observations reinforce the need to qualify immunostaining results by ultrastructural investigation in order to understand the organisation of immuno-detected proteins and are discussed in terms of their diagnostic and possible biological significance.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/36. 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 = 12.103502064177keywords = blood vessel, vessel (Clic here for more details about this article) |
7/36. Antiangiogenic treatment of mesenteric desmoid tumors with toremifene and interferon alfa-2b: report of two cases.PURPOSE: Desmoid tumors are uncommon, benign, fibrous lesions occurring sporadically and in association with familial adenomatous polyposis. Typical clinical features include a locally aggressive behavior, an unpredictable course, and a high propensity for recurrence after surgical resection. There are no standard medical or surgical approaches, and no markers for monitoring medical therapy of desmoid tumors. methods: We report two cases of mesenteric desmoid tumors treated with interferon alfa-2b and toremifene, a novel regimen devised to block angiogenesis. Pre- and posttreatment desmoid tumor tissues were obtained in one patient during a repeat resection for recurrent stenosing Crohn's disease and examined for mean vessel count and cellular proliferation levels by immunostaining for the endothelial surface antigen CD31 and the proliferation associated nuclear antigen, Ki-67, respectively. We assessed plasma D-dimers, a potential marker of angiogenic activity, and followed this throughout the course of antiangiogenic therapy in our two patients. RESULTS: Examination of posttreatment tissue revealed a significant decrease in microvessel density (P<0.02) and Ki-67-positive nuclei (P<0.0001) compared with pretreatment tissue. Both patients demonstrated a prompt and sustained drop in previously elevated plasma D-dimer levels, which correlated clinically with lesion regression and sustained remission. CONCLUSIONS: Treatment with toremifene and interferon alfa-2b was successful and well tolerated in our two patients. Our data suggest a combined antiangiogenic and antiproliferative mechanism of action. Furthermore, normalization of previously elevated plasma D-dimers may emerge as a strategy to monitor treatment efficacy in mesenteric desmoid tumors.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/36. Vanek's tumor (inflammatory fibroid polyp). Report of 18 cases and comparison with three cases of original Vanek's series.Eighteen cases of Vanek's tumors are presented. The patients included nine men and nine women between the ages of 45 and 93 years (mean, 66.2 years). Nine cases were clinically diagnosed as polyps of the gastric antrum, five cases as polyps of the stomach (not otherwise specified), one polyp was located in the ileum and the three remaining polyps in the small intestine (not otherwise specified). The thirteen polyps with available size information measured from 0.4 to 5 cm in the greatest diameter (mean, 2.2 cm). Immunohistochemically, the affections were positive for vimentin (18/18) and CD34 (15/18). All the cases negative for CD34 also lacked concentric onion skin-like formations of the spindle cells around glands and vessels. The different immunophenotype and absence of concentric formations could be explained by the existence of two different lesions commonly designated as Vanek's tumor (inflammatory fibroid polyp) or by the hypothesis of various evolutional stages. In the differential diagnosis, it is important to distinguish namely eosinophilic gastroenteritis, gastrointestinal stromal tumor, inflammatory pseudotumor, hemangioendothelioma, and hemangiopericytoma. In contrast to gastrointestinal stromal tumors, genetically no substitution, deletion, or insertion occurred in c-kit exon 11 in all analyzed samples. Likewise, no deletion or insertion in part of c-kit exon 9 was observed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/36. Pleomorphic hyalinizing angiectatic tumor of soft parts: ultrastructural analysis of a case with original features.Pleomorphic hyalinizing angiectatic tumor, a rare neoplasm of uncertain lineage resembling malignant fibrous histiocytoma and schwannoma, was first described in 1996 by M. E. F. Smith et al. (Am Surg Pathol. 20:21-29). To date, less than 100 cases have been reported in the international literature. It occurs in subcutaneous and intramuscular soft tissues of extremities or trunk in adults without sex predilection. All lesions are composed of sheets and fascicles of spindled and pleomorphic cells associated with clusters of thick-walled ectatic vessels surrounded by a perivascular hyaline material and inflammatory cells such as mast cells. About one-half of these neoplasms express CD34. No patient has developed metastases but occasional local recurrences are possible. This tumor of uncertain lineage is suggested to be an aggressive locally growing low-grade sarcoma. Only 3 cases were previously studied by electron microscopy and appeared to consist of primitive fibroblastic cells. The authors report histological and ultrastructural characteristics of a new case of PHAT excised from the right buttock of a 66-year-old man with the presence of ganglion-like cells, a feature that has not been previously reported, and unusual central ischemic necrosis. The features of this case are suggestive of a fibroblastic origin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/36. Spontaneously ruptured uterine angioleiomyoma.Angioleiomyoma is an uncommon type of leiomyoma of the uterus that originates from smooth muscle cells and contains thick-walled vessels. A 45-year-old woman with the complaint of lower abdominal pain was admitted to the hospital. In the operation a ruptured, bleeding uterine tumor was seen. She underwent total hysterectomy and bilateral salpingo-oophorectomy. On pathologic examination of the specimen, the tumor was diagnosed as angioleiomyoma. Here, an unusual clinical presentation of uterine angioleiomyoma was reported.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
| | Next -> |