[Recurrence of nasopharyngeal fibroma. Study of 21 recurrences of 49 nasopharyngeal fibromas].
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Discerning desmoplastic fibroma from odontogenic fibroma may present considerable diagnostic difficulty. In the present article five cases of jaw fibroma serve to emphasize the various problems. Two cases represented desmoplastic fibroma. Both were treated by curettage, which was successful in one case but failed to provide a cure in the other. Factors responsible for this differing clinical course are discussed. A third case of desmoplastic fibroma proved to have been misdiagnosed, being a low-grade fibrosarcoma that subsequently showed dedifferentiation, and two cases were diagnosed as central odontogenic fibroma. After a discussion of the various histologic aspects of the reported cases, it is concluded tha desmoplastic fibroma is a well-delineated entity. Odontogenic fibroma, on the contrary, can exhibit a varying histomorphology, perhaps because of the existence of different entities that at present are taken together. To prevent the uncertainties concerning the odontogenic fibroma from making the definition of desmoplastic fibroma also indistinct, it is proposed that a central jaw fibroma that does not show the typical morphology of a desmoplastic fibroma should be considered an odontogenic fibroma.
Ovarian myxomas recently have been reported as new, distinct pathologic entities that show a myxoid, moderately cellular proliferation of spindle and stellate cells interspersed with areas of fibrosis, hemorrhage, and delicate vascular spaces. These histologic features are frequently seen in the thecoma-fibroma group of ovarian stromal tumors. For this reason, we propose that ovarian myxomas are part of the spectrum of differentiation in thecomas-fibromas of the ovary. To provide histologic and immunohistochemical evidence for this proposal, four ovarian myxomas were compared with 48 primary ovarian stromal tumors in the thecoma-fibroma group from 46 patients. The thecoma-fibroma group of stromal tumors included 23 thecomas, 23 fibromas, and two sclerosing stromal tumors. We found significant (> 25% of histologic appearance) myxoid change in six thecomas and one sclerosing stromal tumor. This myxoid change resembled the histologic appearance of an ovarian myxoma. Immunohistochemical studies on paraffin-embedded material showed vimentin immunostaining in all tumors. Smooth-muscle actin was present in all of the myxomas, in two of the two sclerosing stromal tumors, and in 20 (90%) of the 23 thecomas, but it was present in only 11 (48%) of the 23 fibromas. Desmin staining was not present in any of the four ovarian myxomas or in the two sclerosing stromal tumors, and only three (13%) of the 23 thecomas showed focal staining for desmin. Nine (39%) of the 23 fibromas expressed desmin. S100 protein was expressed in one fibroma and one thecoma, weakly. None of the ovarian myxomas or the thecoma-fibroma group of stromal tumors expressed cytokeratins as detected by three different monoclonal antibody cocktails, ie, cytokeratin AE1/AE3, cytokeratin CAM 5.2, or cytokeratin MAK-6. The ovarian thecoma-fibroma group of stromal tumors form a histologic spectrum of lesions in which clear-cut distinguishing points between various entities are difficult to define. The myxoid change, present in the thecoma-fibroma group of tumors, was indistinguishable histologically and immunohistochemically from ovarian myxoma. For this reason, we propose that ovarian myxomas may be at one end of the spectrum of differentiation in the thecoma-fibroma group of tumors, in which no remaining stromal tumor is detectable.
The term juvenile ossifying fibroma is used in the literature in naming 2 microscopically distinct fibro-osseous lesions of the craniofacial skeleton. One is characterized by small uniform spherical ossicles resembling psammoma bodies (psammomatoid juvenile ossifying fibroma). The other is distinguished by trabeculae of fibrillary osteoid and woven bone (trabecular juvenile ossifying fibroma). Three new cases of each type are reported, and the literature is extensively reviewed for published cases of these 2 entities.Psammomatoid juvenile ossifying fibroma is reported more commonly than trabecular juvenile ossifying fibroma. It affects patients from a wider age range (3 months to 72 years) and an older mean age range (16-33 years) as compared with 2 to 12 years and 8(1/2) to 12 years, respectively, for trabecular juvenile ossifying fibroma. In both types there is a slight male predominance and the lesions are unencapsulated and tend to infiltrate adjacent bone. A significant difference between the 2 tumors is their site of occurrence. Although psammomatoid juvenile ossifying fibroma occurs predominantly in the sinonasal and orbital bones, trabecular juvenile ossifying fibroma predominantly affects the jaws. Aggressive growth occurs in some-but not all-cases of both types. Such behavior may be related to younger patient age and the concurrent development of aneurysmal bone cysts, which is seen more frequently in psammomatoid juvenile ossifying fibroma. This study demonstrates that not only histologic but also demographic and clinical differences between psammomatoid juvenile ossifying fibroma and trabecular juvenile ossifying fibroma warrant their classification as 2 distinct clinicopathologic entities.
STUDY DESIGN: A case of desmoplastic fibroma of the thoracic spine treated by a three-level en bloc spondylectomy is reported. OBJECTIVES: To present a rare case of desmoplastic fibroma of the spine, and to emphasize the importance of at least a marginal resection of this tumor entity. SUMMARY OF BACKGROUND DATA: Desmoplastic fibroma is a rare tumor, with the literature reporting approximately 220 cases of the disorder in patients younger than 30 years of age. This disorder has a predilection for the mandible and the meta-diaphyses of long bones. A location of desmoplastic fibroma in the spine has been reported in only a few cases. Desmoplastic fibroma has a high tendency of local recurrence, especially after intralesional resection. METHODS: The reported case is that of a 14-year-old girl with a desmoplastic fibroma of the 9th, 10th, and 11th vertebrae. After confirmation of the diagnosis by CT-guided biopsy, a three-level en bloc spondylectomy with marginal resection of the desmoplastic fibroma was performed from the posterior approach. Stabilization was achieved with a multilevel pedicle screw instrumentation, and an autologous fibula was used for reconstruction. RESULTS: At this writing, 31 months after surgery, the girl has no evidence of recurrence and is pain free. CONCLUSIONS: Wide resection of tumors located in the spine actually is impossible to achieve because of the anatomic relations to the spinal cord, the major vessels, and the lung. As in the current case, a marginal resection is the maximal one to be achieved. Three of seven cases (43%) of desmoplastic fibroma in the spine treated by intralesional resection resulted in a local recurrence. These data clarify the importance of at least marginal resection of desmoplastic fibroma, if this is anatomically and technically possible. A local recurrence of desmoplastic fibroma in the spine can be impossible to treat surgically with a curative intention without a significant loss of function.
To determine how toremifene, an anti-oestrogen triphenylethylene derivate, reduces tumour mass, we investigated its modulation of TGF-beta1 and TNF-alpha in fibroma fibroblasts. Normal and fibroma fibroblasts, isolated from patients affected by Gardner's syndrome without or with fibroma manifestation, were cultured in vitro. Secretion of GAG, collagen and TGF-beta1 was increased in fibroma fibroblasts compared to healthy cells. The increase in TGF-beta1 secretion into the medium was associated with a parallel increase in TGF-beta1 gene expression and receptor number. Receptor cross-linking studies using radiolabelled TGF-beta1 revealed more receptors, particularly types I and II, in fibroma fibroblasts than in normal cells. Normal and fibroma fibroblasts did not synthesise TNF-alpha, but they had TNF-alpha membrane receptors, as shown by TNF-alpha assay. TNF-alpha secreted by human monocytes, which may be present in the peritumoral area, increased cell proliferation and GAG accumulation and was, in turn, enhanced by TGF-beta1 treatment. Both growth factors increased angiogenesis, as shown by the CAM assay. Toremifene reduced TGF-beta1 secretion by fibroma fibroblasts and TNF-alpha secretion by monocytes, thus downregulating cell proliferation, ECM macromolecule accumulation and angiogenic progression. We hypothesise that increased TGF-beta1 gene expression and TGF-beta1 secretion in fibroma fibroblasts as well as the subsequent rise in TNF-alpha production by monocytes may facilitate fibroma growth and that toremifene inhibits autocrine and paracrine growth factor production.
Focally expressed cemento-osseous dysplasia (periapical cemento-osseous dysplasia and focal cemento-osseous dysplasia) and cemento-ossifying fibroma (ossifying fibroma and cementifying fibroma) are two clinically recognized entities that are not easily differentiated histopathologically because of the lack of recognition of specific microscopic features. We have assessed 20 pathologic parameters for their ability to distinguish reliably between the two. Included in this study were 241 cases of focally expressed cemento-osseous dysplasia and 75 cases of cemento-osseous fibroma diagnosed from a combination of clinical, radiographic, and histopathologic information. Results revealed that 92.5% of focally expressed cemento-osseous dysplasia were composed of multiple small fragments of tissue whereas 88.0% of cemento-osseous fibromas showed a large intact specimen. Thick curvilinear trabeculae ("ginger root" pattern) or irregularly shaped cementum-like masses were typically seen in focally expressed cemento-osseous dysplasia, whereas thin isolated trabeculae with prominent osteoblastic rimming were more commonly observed in cemento-osseous fibroma. The stroma of focally expressed cemento-osseous dysplasia often displayed characteristic cavernous-like vascularity that was almost always associated with bony trabeculae. Free hemorrhage was frequently interspersed in the artifactual spaces throughout focally expressed cemento-osseous dysplasia. In contrast, the cases of cemento-osseous fibroma showed more cellularity in the stroma in which a storiform pattern was present in more than half the lesions studied. Giant cells, when present in cemento-osseous fibroma, were clustered in the center of the cellular stroma. The features described here allowed distinction histopathologically in 94% of cases studied. Three progressive stages of focally expressed cemento-osseous dysplasia and subtypes of cemento-osseous fibroma may be recognizable microscopically.
Cardiac fibroma and inflammatory myofibroblastic tumor (IMT) of the heart are rare lesions occurring in young patients and having pathologic similarities. We compared the morphologic and immunohistochemical features of seven cardiac fibromas, including one biopsied at birth and removed 4 years later, and two IMTs of the heart diagnosed at Marie Lannelongue Surgical Center (Le Plessis Robinson, France) between 1980 and 1999. Cardiac fibromas occurred in five females and two males and were surgically biopsied (n = 2) or removed (n = 6) between the ages of 8 days to 31 years (mean 7 +/- 12 years). Inflammatory myofibroblastic tumors were removed in two male patients, aged 13 weeks and 1 year, both alive and well 9 months and 5 years after surgery, respectively. Fibromas were ventricular lesions measuring 3 to 10 cm (mean, 5.7 +/- 2.2 cm). They contained entrapped myocytes and wavy elastic fibers. Three cases contained calcifications. Spindle cells were monomorphic. Their nucleus had a thin chromatin without nucleolus. Mitoses and extramedullary hematopoiesis were only observed in fibromas from patients younger than 5 months (n = 5) while prominent collagen fibrosis was present in fibromas from patients older than 4 years (n = 3). Inflammatory myofibroblastic tumors were endocardial lesions measuring 2 and 2.5 cm. They were covered by fibrin. Spindle cells were larger than in fibromas. Their nucleus had obvious nucleoli. They were associated with numerous inflammatory cells in a variable amount of myxoid background. Occasional mitoses and foci of necrosis were present. Spindle cells in both fibromas and IMTs strongly expressed smooth-muscle actin and were negative for desmin, CD34, S-100 protein, and p53. Our study shows that IMT must be considered in the differential diagnosis of cardiac fibroma especially in cases of inflammatory syndrome, location outside the ventricular myocardium, or multinodular lesions. Morphologic analysis permits the correct diagnosis, while immunochemistry shows a myofibroblastic differentiation in both lesions.
Leukocytes and macrophages, obtained from fibroma-immune rabbits and added to immune serum-fibroma virus mixtures, significantly increased the neutralization of fibroma virus as compared with immune serum alone. Immune cell suspensions from peritoneal exudates, regional lymph nodes, buffy coats, spleen, and liver were all effective in inhibiting fibroma virus. Approximately 2000 to 4000 immune cells/mm.(3) were necessary to cause an effect but no particular cell type could be implicated as responsible for the inhibition of fibroma virus. Normal cells did not consistently and significantly inhibit fibroma virus and cells from rabbits immunized with other viruses did not inhibit fibroma virus. Studies of the mechanism of action of the immune cells revealed: (a) that living cells were essential; (b) that normal cells, sensitized with immune serum, did not simulate the effects of immune cells; (c) that immune cells contained less preformed neutralizing antibody than an equivalent volume of immune serum, and (d) that inhibition of fibroma lesions was not the result of viral interference. It is suggested that the fibroma-neutralizing effect of immune cells is related to intracellularly placed antibody or to cellular transfer of an ability to form specific antibody in recipient animals.
Pleomorphic sclerotic fibroma is a benign neoplasm exhibiting features of sclerotic fibroma and pleomorphic fibroma. We report another such case. The tumor presented as a firm, 0.5-cm, flesh-colored papule on the forehead of a 72-year-old white man for an unknown duration. Histologic examination revealed a neoplasm in which the superficial portion showed features of a pleomorphic fibroma, the deeper portion showed features of a sclerotic fibroma, and a transitional area was present in between. We propose that pleomorphic fibroma, sclerotic fibroma, and pleomorphic sclerotic fibroma form a spectrum. Pleomorphic sclerotic fibroma may be used as a broad diagnostic term to encompass the spectrum.
Cells cultured from tumors induced in rabbits by inoculation of fibroma virus possessed virus-specific cell surface and cytoplasmic antigens. Tumor cell cultures were capable of a limited number of cell divisions before degenerating. Employing the (51)Cr-release test and the microcytotoxicity test, it was demonstrated that sera from rabbits with regressed fibroma tumors contained antibodies cytotoxic for cells infected in culture with fibroma virus. These sera were only weakly cytotoxic for cultured fibroma tumor cells. In addition, newborn rabbits bearing progressively growing tumors had serum antibodies cytotoxic for cells infected with fibroma virus in culture but not for fibroma tumor cells. Immunofluorescence studies also showed that the reaction of immune serum with the surface antigens of fibroma tumor cells was markedly weaker than with the surface antigens of cells infected with virus in culture. Furthermore, membrane cytotoxicity and immunofluorescence reaction were significantly reduced when cells were tested after prolonged incubation or cell division, or both, following infection with fibroma virus. The failure of tumors to regress in newborn rabbits in spite of the presence of cytotoxic antibodies is discussed in respect to a possible reduction or alteration of virus-specific surface antigens on proliferating tumor cells.
The present paper describes the relationship between ameloblastoma and ameloblastic fibroma deduced from a case diagnosed as "ameloblastoma combined with ameloblastic fibroma" arising in the mandible of a 5-year-old boy. Histologically, the tumor consisted of ameloblastoma in the central area and ameloblastic fibroma in the peripheral area; it clinically fits the characteristics of ameloblastic fibroma based on predominant age, manner of growth, and encapsulation. We reviewed the literature and discussed the relationship between ameloblastoma a ameloblastic fibroma in terms of tumorigenesis. It is assumed that ameloblastic fibroma can also be transformed into ameloblastoma, if the succeeding hard tissues are not formed, and the collagenous connective tissue substituting for the stromal mesenchymal tissue is formed by the inductive effect of the epithelial strands or other unknown factors. Several possibilities relative to the pathogenesis of ameloblastoma have been proposed by oral pathologists; however, to our knowledge, "ameloblastic fibroma can be transformed into ameloblastoma" has not hitherto been reported. The case we experienced here may be thought as an intermediate tumor pattern between ameloblastic fibroma and ameloblastoma.
Two different lesions of the gingiva that have been referred to previously as peripheral odontogenic fibromas are discussed. The first of these is the rare extraosseous counterpart of the central odontogenic fibroma (WHO type)1 and is therefore referred to in this article as the peripheral odontogenic fibroma (WHO type). It is probably treated adequately by simple excision, but a study of its biologic behavior is lacking. The second lesion is reactive, is common, and has a marked tendency to recur. It has been known by numerous synonyms, including calcifying fibrous epulis and peripheral ossifying fibroma, as well as peripheral odontogenic fibroma. The term peripheral ossifying fibroma should be retained for this lesion to avoid confusion with the peripheral odontogenic fibroma (WHO type).