[Primary tumors of the bones. IV. Tumors of the osteogenic category: fibromas, giant cell tumors].
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Juvenile aponeurotic fibroma, isolated in 1953 by Louisa Keasbey is a rare tumour which is characterized by its recurrence and infiltration, but which has no tendency to metastasis. The case we report differs in its long-term outcome. The tumour diagnosed in a seven-year-old child has recurred locally for seven years, necessitated hand amputation, and finally developed a fibroblastic process extending to lung and pleura which caused the child's death. This new finding in Keasbey's disease led us to reconsider the nosological situation in juvenile fibromatoses and its reputation for benignity.
The clinical, cross-sectional echocardiographic, haemodynamic, and angiographic features of a case of cardiac fibroma in a newborn with unexplained pulmonary hypertension, and direct tumor involvement of the posterior leaflet of the mitral valve, are presented. The role of echocardiography in the pre-operative evaluation of cardiac tumors is discussed.
Loss of heterozygosity (LOH) studies in ovarian tumors, have highlighted the chromosomal regions at 9q22-31 and 9q32-34 as being potentially important in tumor development. We have investigated LOH at 9q22-31 in 85 patients with epithelial ovarian cancer, 15 with non-epithelial tumors and 16 with benign disease. Varying patterns of LOH were observed across the markers used between different tumors, the most common (71%) being interstitial discontinuous losses. LOH was frequent, and was detected at equally high levels in malignant (71%) and benign tumors (70%). LOH occurred in epithelial invasive tumors, borderline tumors, fibromas and dermoid tumors. In malignant epithelial tumors LOH at 9q22-31 was not significantly associated with patient clinical and pathological parameters; however, survival was 29 months at the 50th centile survival, in those women whose tumors displayed LOH compared with 60 months in women whose tumors retained heterozygosity. LOH at 9q22-31 was significantly associated with LOH at the p53 locus (p=0.02) and the ovarian suppressor locus at 3p21 (p=0.05). We conclude that the chromosome region at 9q22-31, flanked by the microsatellite markers D9S1796 and D9S53, is a frequent and early event in ovarian tumorigenesis. With the of extent of discontinuous LOH, high density deletion mapping of this region using LOH as a strategy to identify candidate genes may be problematic. However with the completion of the human genome sequencing project several candidate genes are identified.
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Recurring digital fibrous tumors of childhood are unusual inclusion-bearing fibrous tumors typically located on the fingers and toes of young children. We report here a histologically similar tumor located on the arm of a 44-year-old man.
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Technical and methodological procedures as well as results and follow-up studies of superselective angiographies and embolisations with different materials (gelfoam, lyophilised dura, silicone-elastomer) in 105 patients with vascular lesions (arteriovenous angiomas and fistulas, capillary haemangiomas), benign (glomus tumors, fibromas, meningeomas) and malignant tumors (sarcomas, metastases) of the cranio-facial region are described. Due to the clinical findings and follow-up studies up to three years there were different palliative and curative results which are exemplified. Therefore, a uniform performance of the embolisatory interventions is refused. The arterial approach, the different catheter systems as well as the actual technical procedure of the embolisations, with or without superselective pharmacoangiographies, has to be chosen according to the individual manifestation of the lesions and their anatomical and pathological conditions.
A calcified fibroma caused a so called cervical syndrome not responding to medical treatment. In the computerized tomography a compression of the cervical spinal cord could be demonstrated. From this the indication for the operation was given.
The desmoid is the independent pathological entity of a non-metastasizing fibroma invading the local neighboring structures. It is derived from fascia, aponeuroses or tendines. In rare cases it is found in the oral and maxillofacial region. For a firm diagnosis, it is absolutely necessary to combine clinical data (location, size growth, behavior vis-a-vis adjacent structures) with pathologic-anatomic findings (invasion of the adjacent tissue, especially musculature). In this respect, examination of the marginal regions is of decisive importance. Because of the frequency of recurrences and the danger of sarcomatous degeneration, radical resection is the therapy indicated.
Shope fibroma virus (SFV) causes a localized, self-limited, fibroblastic proliferation in adult rabbits. Extracts of Shope fibroma tumors were found to contain a second virus that induces a rapidly progressive disseminated tumor. Dissemination of this malignant fibroma is associated with activation of commensal mucosal infection with Pasteurella multocida, causing purulent conjunctivitis and rhinitis and resulting in death from nasal obstruction. We have isolated this new agent by two cycles of plaque purification. It is a poxvirus that is antigenically virtually identical to SFV as measured by a plaque reduction assay, but behaves differently both in vivo and in vitro. We have called this virus malignant rabbit fibroma virus (MV). Electrophoresis of restriction digests made with HIND III indicates that despite the antigenic similarity of SFV and MV, the locations of HIND III sites in the two viral genomes are quite different. These experiments have enabled us to determine that MV was present in small quantities in our initial uncloned stock of Patuxent strain SFV. Lymphocytes from rabbits bearing MV-induced tumors responded poorly to both B and T lymphocyte mitogens. This nonspecific immunologic dysfunction is evident at or before the time when metastases and Gram-negative infection develop, and it becomes more profound as the disease progresses. MV-induced tumors may provide a model for Gram-negative infection and decreased immunologic responsiveness associated with malignancies.
Chondromyxoid fibroma is an uncommon bone neoplasm, accounting in our series for less than 1% of all connective tissue tumors. The tumor is more common in males, and located mostly in the metaphyseal areas of the lower extremity. The tumor is benign and there have been no reports of metastases. The method of treatment that has been used since the initial identification of the tumor has been curettage, which has a 20-25% recurrence rate. In our 30 patients, the average length of followup was 11 years (range, 1-29 years. Most of the tumors were in the pelvis, proximal tibia, distal femur, and foot. Tumors that were treated with curettage alone did less well than those that were packed with allograft bone or polymethylmethacrylate. Tumors treated by excision did not recur. The most difficult problem with chondromyxoid fibroma is pathologic identity because it often is confused with more aggressive tumors that may metastasize.
Localized tumors of pleura should clearly be separated from diffuse pleural mesotheliomas due to clinical and pathological peculiarities. But there is some dispute on their proper interpretation and hence disagreement about their correct designation. Some call them "fibrous tumors (fibromas)" of pleura and appreciate by this term the fibroplastic potency of tumor cells and the lacking relation to overlying mesothelial surface cells, others prefer the name "fibrous mesothelioma" and allude to an at least abortive mesothelial differentiation within the tumor tissue. Examining 7 own tumor cases by means of light microscopic, topo- and immunohistochemical as well as electron microscopic methods the investigations were aimed at analysing the morphological structure of these tumors and subjecting it to a critical reappraisal with regard to a suited designation. Histologically, a marked variability of histological structure and texture was found, there were regions with similarities to fibromas, fibrous histiocytomas, hemangiopericytomas and nerve sheath tumors as well as blastema-like areas. Occasionally a vasoformative activity could be seen. In three tumors a few epithelioid cell complexes were visible, but neither keratin intermediate filaments nor UEA-I reactivity was to be demonstrated by immunohistochemistry. Antibodies against factor VIII associated antigen only marked endothelial cells of vascular structures. By the electron microscopic examination of 1 case abortive mesothelial differentiations were visualized (some lumen-like space formations, stub-like cellular projections of the cavity facing side, primitive intercellular junctions and fragmented basal lamina-like material). The findings inclusive the immunohistochemical results are discussed and jointly taken for sustaining the interpretation of these tumors as mesotheliomas and for militating against the classification as simple pleural fibromas or fibrous pleural tumors, respectively.
Purely benign mesenchymal spindle cell neoplasms of the breast are currently labeled under various terms in the literature (benign spindle cell tumor, fibroma, spindle cell lipoma, myofibroblastoma, solitary fibrous tumor, myogenic stromal tumor). The lack of strict diagnostic criteria to clearly indicate such mesenchymal neoplasms is the main reason which generated the risk of terming the same lesion under different names or, conversely, of collecting different types under the same term. Although such neoplasms exhibit morphological and immunophenotypical heterogeneity, they actually represent variations of the same tumor entity, likely arising from the uncommitted vimentin+/CD34+ fibroblasts of the mammary stroma, capable of multidirectional mesenchymal differentiation. To cover the entire spectrum of such lesions, the term "benign spindle cell tumors (BSCTs) of the mammary stroma" is advocated. BSCTs can be subtyped into four main groups by light microscopy (LM) and immunocytochemistry (ICC): fibroblastic, myofibroblastic, fibrohistiocytic, and mixed forms. A simple and practical approach to a nosologically correct diagnosis and a list of differential diagnoses are presented. The awareness of the diversity of morphological and immunophenotypical features of BSCTs of the mammary stroma, including uncommon variants, is helpful to avoid confusion with other monomorphic bland-looking benign and malignant spindle cell tumors and tumor-like lesions of the breast.
Odontogenic tumors composed of 2 distinct types of lesions are unusual. We report an odontogenic tumor that was composed of calcifying odontogenic cyst and ameloblastic fibroma that occurred in the right posterior maxilla of a 22-year-old Korean woman. The tumor had a cystic component with an ameloblastic epithelial lining and conglomerates of so-called ghost cells, and there were deposits of dentinoid material adjacent to the cyst. These are features characteristic of calcifying odontogenic cyst. Enamel organ-like epithelial islands were observed within a dental papilla-like stroma of the cyst wall. Additionally, a solid portion of the tumor had characteristic features of ameloblastic fibroma, i.e., a myxoid cellular stroma with numerous elongated islands of ameloblastic epithelium. Ghost cell masses were found in the area of ameloblastic fibroma as well. The distribution of the ghost cells suggests that this is a hybrid lesion rather than a collision tumor.