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Central fibroma of the jaw, odontogenic or desmoplastic.

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.

Adult↗

Histologic and immunohistochemical evidence for considering ovarian myxoma as a variant of the thecoma-fibroma group of ovarian stromal tumors.

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.

Actins↗

Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: two distinct clinicopathologic entities.

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.

Adolescent↗

Three-level en bloc spondylectomy for desmoplastic fibroma of the thoracic spine: a case report.

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.

Adolescent↗

Comparative study of pagetoid dyskeratosis between acrochordons and soft fibromas.

BACKGROUND: Pagetoid dyskeratosis (PD) is considered a casual finding. We can find it in some conditions, including acrochordons and soft fibromas. OBJECTIVE: (1) to compare the presence of PD in soft fibromas and acrochordons and (2) to compare PD positive fibromas and PD negative fibromas. MATERIALS AND METHODS: We reviewed all acrochordons and soft fibromas diagnosed in the General Hospital of Lanzarote, Spain, between January 2001 and December 2002. We assessed the presence of PD, size, acanthosis, basal pigmentation, and the presence of pseudohorn cysts. RESULTS: Three hundred sixty one acrochordons and 164 soft fibromas were included in this study. There were striking differences in the presence of PD, size, acanthosis, and basal pigmentation between both entities. PD positive fibromas predominated in axillas. There were no other differences between PD positive fibromas and PD negative fibromas. CONCLUSION: Although soft fibromas and acrochordons are actually fibroepithelial polyps, including the presence of PD, there are striking differences between them. Thus, both conditions have to be considered as different entities. PD could be related to friction and moisture. PD has to be distinguished from other conditions such as Paget's disease, pagetoid melanoma, koilocytes, clear cell papulosis, among others.

Axilla↗

Effects of transforming growth factor-beta1 and tumour necrosis factor-alpha on cultured fibroblasts from skin fibroma as modulated by toremifene.

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.

Animals↗

[Cemento-ossifing fibroma: two cases].

INTRODUCTION: Cemento-ossifying fibroma is a rare benign tumor which can affect both jaw bones, particularly the mandible. CASE REPORTS: We report two cases of cemento-ossifying fibroma which illustrate the radiological and anatomic features of these tumors. Differential diagnosis between cementifying fibroma and ossifying fibroma was particularly difficult. The cementifying fibroma was treated by surgical resection followed by immediate reconstruction with an iliac bone graft. The ossifying fibroma was treated by enucleation-resection. DISCUSSION: The clinical, radiological and histological features of cementifying and ossifying fibroma allow distinction. Surgical treatment is achieved by enucleation resection for small-sized ossifying fibromas and mono-bloc resection with bone reconstruction for large-sized cementifying and ossifying fibromas.

Adult↗

Distinguishing features of focal cemento-osseous dysplasias and cemento-ossifying fibromas: I. A pathologic spectrum of 316 cases.

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.

Blood Vessels↗