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A case of a neoplasm called non-osteogenic fibroma or non-ossifying fibroma is reported. The neoplasm was situated in the cervical part of the spine and was removed surgically. A peculiarity of this case is rare occurrence of this neoplasm and oligosymptomatic stationary course of the disease.
Intra-osseous fibromas of the jaw are classified by origin. Intra-osseous odontogenic fibromas have odontogenic epithelia, while desmoplastic fibromas do not. However, it is often difficult to determine the odontogenic origin for central fibromas. Three subjects with a diagnosis of intra-osseous fibroma were examined. Case 1 was a 35-year-old man found to have a panoramic radiograph from the right premolar to the mandibular ramus in the mandible that exhibited multilocular radiolucency. Within the radiolucency, small-radioopaque bodies were observed. Case 2 was a 13-year-old female, in whom a panoramic radiograph from the left premolar to the molar in the mandible showed multilocular radiolucency. Case 3 was a 51-year-old female who exhibited a heart-shaped radiolucency in the panoramic radiograph of the left first molar area in the mandible. We also reviewed the literature for previously reported cases of intra-osseous odontogenic and desmoplastic fibroma. In 64 cases of intra-osseous odontogenic fibroma and 68 cases of desmoplastic fibroma we extracted data on age, sex, location, and radiographic findings. Based on the analysis of the reported literature cases, re-evaluation of the patients in our study revealed that case 1 could be classified as desmoplastic fibroma, while cases 2 and 3 were intra-osseous odontogenic fibromas.
Sclerotic fibroma was first described in association with Cowden's disease by Weary et al. in 1972. In 1989, Rapini and Golitz detailed 11 cases of solitary sclerotic fibroma (SFS) in the absence of Cowden's disease, suggesting the term SFS of the skin. Classic histological features include hypocellular, hyalinized bands of collagen sharply demarcated from the surrounding skin. Numerous authors have described sclerotic fibroma-like changes in other entities including melanocytic nevi, dermatofibromas, lipomas, tendon sheath fibromas, giant cell collagenomas, neurofibromas, angiofibromas, erythema elevatum diutinum, and folliculitis. Dissension has arisen, with some dermatopathologists asserting that sclerotic fibroma is just an evolutionary end-point of a previous lesion. Others contend that SFS is a distinct lesion and cite recurrent cases and/or proliferation marker studies to corroborate this view. We detail the histopathological findings of lesions consistent with the classic description of SFS and compare these to sclerotic changes observed in an intradermal nevus, blue nevus, erythema elevatum diutinum, neurofollicular hamartoma, angiofibroma, neurofibroma, accessory nipple, and dermatofibromas. Sclerotic fibroma-like change may be seen in a variety of lesions and may represent a common reaction pattern in the skin.
We observed clonal chromosome abnormalities in two fibrous soft tissue tumors diagnosed as collagenous fibroma (desmoplastic fibroblastoma). The involvement of the same band of the long arm of chromosome 11, 11q12, was observed in both tumors. The presence of hitherto unreported similar chromosomal abnormalities in this tumor supports the neoplastic nature of this lesion. In addition, a possible relationship with fibroma of tendon sheath, which also shows rearrangement of 11q12, is suggested. 11q12 might be a common genetic denominator of benign fibroblastic lesions, such as collagenous fibroma and fibroma of tendon sheath.
The case of a 30-year-old woman with multiple loose bodies in the shoulder joint is presented. The patient complained of discomfort of her right shoulder. Conventional radiographs failed to reveal any abnormality of the shoulder. On MRI numerous loose bodies were detected that resembled synovial chondromatosis. Histologically, the loose bodies were composed of a benign fibroblastic lesion with diagnostic features of fibroma of tendon sheath, each surfaced by synoviocytes. The clinicopathologic features of intra-articular fibroma of tendon sheath are reviewed. We propose using the term "synovial fibroma" for tumors with histopathologic features of fibroma of tendon sheath arising in the synovium of joints.
Malignant rabbit fibroma virus (MRV) has been shown to be a lethal tumorigenic poxvirus of rabbits derived from a recombination event between Shope fibroma virus (SFV), which induces benign fibromas in rabbits, and myxoma virus, the agent of myxomatosis. We have cloned and sequenced all of the MRV recombination junctions, which are located near the left and right terminal inverted repeat (TIR) regions, and present a composite map of the MRV genome with respect to the relevant gene products. The two junctions closet to the MRV termini, at identical positions at the left and right ends, are at nucleotide 5272 and result in an in-frame fusion protein (ORF T-5) in which the N-terminal 232 aa are derived from an SFV sequence linked to a C-terminus derived from myxoma. At the left MRV TIR the recombination junction distal from the terminus maps to nucleotide 9946 but leaves the adjacent gene virtually unchanged from its SFV homolog. At the right terminus, the relevant junction sequences from MRV and myxoma could not be cloned in wild-type Escherichia coli but were maintained stably in a recA recBC sbcB host. The SFV/myxoma junction at this location maps 5' to a growth factor gene (SFGF) which is related to those encoding epidermal growth factor and transforming growth factor-alpha. As a result, the myxoma growth factor gene has been deleted in MRV and replaced in toto by the SFV gene. The recombination junction upstream from the SFGF gene creates an in-frame fusion in ORF T11-R in which the N-terminal amino acids are derived from myxoma and the remainder from SFV. In summary, MRV has received the following ORFs from SFV: at the left terminus T5 (fusion), T6, T7, and T8; at the right terminus, T5 (fusion), T6, T7, T8, T9-R, SFGF, and T11-R (fusion).
BACKGROUND: Solitary sclerotic fibroma (SF) presents as a well circumscribed dermal nodule, composed of sparse spindle cells with alternating wavy collagen fibers arranged in a storiform pattern. The histogenesis and nature of this histologically distinct lesion are uncertain. Whether this peculiar tumor represents a true hamartoma or a degenerating end of various fibrous lesions such as pleomorphic fibroma (PF), dermatofibroma, or angiofibroma is still controversial. High proliferating index of spindle cells in SF argues against the possibility of being a degenerating end product of another lesion. METHODS: We studied morphological features and immunoprofile of eight SFs, in comparison with four PFs, one collagenized dermatofibroma, two angiofibromas, and two periungual fibromas. Immunostains for CD34, CD31, O13 (CD99), Factor XIIIa, S-100, CD68 (KP-1), and MIB-1 were carried out using a labeled streptavidin-biotin method with DAKO-automated immunostainer. Paraffin blocks of two SFs were reprocessed for electron microscopic studies. Clinical data of all patients with SF were also reviewed. RESULTS: Spindle cells and pleomorphic cells in SF and PF showed diffuse immunoreactivity for CD34 and O13 but were negative for CD31, S-100, and CD68. Spindle cells in one dermatofibroma and one angiofibroma were positive for Factor XIIIa. Proliferating index (MIB-1) was very low in all cases of SF, contradicting some previous reports. CONCLUSIONS: SF is a fibrotic lesion with cells positive for CD34 and O13. It shares a common immunoprofile with PF but is distinct from dermatofibroma and other common spindle cell lesions of skin. O13 expression in SF has not been previously described.
We report a new case of pleomorphic fibroma of the skin arising on the face. The lesion was polypoid and fibrous, located on the dermis, and formed by coarse collagen bundles with sparse cells. It was also characterized by the presence of cellular atypia and pleomorphism without mitosis. Immunohistochemistry showed staining for vimentin and actin and negativity for S-100, CD34, CD68, alpha-1-antichymotrypsin and alpha-1-antitrypsin antigens, supporting a myofibroblastic origin. A few isolated cells also showed granular cytoplasmic detection of factor XIIIa. Although the disorder has been previously considered to be a tumour of preferential localization on the trunk or extremities, this does not always hold true as four of the 14 reported cases arose on the head. Pleomorphic fibroma is a benign condition and is we believe a variant of sclerotic fibroma.
A proliferative activity study analysing morphometric and quantitative aspects of nucleolar organizer regions (NORs) and proliferating cell nuclear antigen (PCNA) expression was conducted in 10 cases of peripheral ossifying fibroma (POF) and 10 cases of ossifying fibroma (OF). For NOR identification, the silver staining technique (AgNOR technique) was used. PCNA expression was determined by immunohistochemical staining using the PC10 antibody. The AgNOR analysis for the two lesions showed a profile characteristic of benign lesions. OF showed higher AgNOR number and PCNA expression than POF. Our results suggest increased proliferative activity in OF compared with POF.
Malignant rabbit fibroma virus (MV) is a recombinant poxvirus derived from Shope fibroma virus (SFV) and rabbit myxoma virus (D. S. Strayer, E. Skaletsky, G. F. Cabirac, P. A. Sharp, L. B. Corbeil, S. Sell, and J. L. Leibowitz, 1983a, J. Immunol. 130, 399-404; W. Block, C. Upton, and G. McFadden, 1984, Virology 140, 113-124). We report here the transcriptional mapping of early RNAs transcribed from the SFV sequences within MV and from the corresponding regions in SFV. Hybridization analysis and S1 nuclease mapping of RNA using viral DNA probes were used to define 5' and 3' ends of the various transcripts. The RNAs described here are transcribed in one direction in a densely arranged head to tail fashion similar to that described for some vaccinia virus early transcriptional units. At late times of infection the early SFV RNAs are not detected whereas the early MV RNAs are present in minor amounts. The early SFV and MV transcripts range in size from 3170 to 425 nucleotides (nt) long. All of the longer transcripts are produced as a result of read through transcription. Three MV transcripts contain fused SFV and rabbit myxoma virus sequences due to transcription through the recombination junction region in the MV genome. Two other MV transcripts are transcribed from a unique initiation site near another recombination junction region resulting in RNAs that are composed of SFV sequences having unique 5' ends.
The 10.7-kb BamHI "C" restriction fragment of malignant rabbit fibroma virus (MV) contains genes that are important for its immunosuppressive activity. When this fragment is transferred to a related avirulent leporipoxvirus, Shope fibroma virus (SFV), recombinant viruses show clinical features characteristic of MV: they replicate in lymphocytes and alter immune function in vitro, induce disseminated tumors in recipient rabbits, and are immunosuppressive in vivo. The 10.7-kb BamHI "C" restriction fragment of MV was sequenced in its entirety. Its DNA sequence and the 14 ORF's derived from analyzing this sequence are discussed. Analysis of known open reading frames to which the ORF's from MV's Bam "C" fragment show homology permits us to identify some MV ORF's showing high degrees of similarity to known and postulated proteins produced by vaccinia virus. Functions for some of these vaccinia proteins are known, while functions for others are hypothetical or unknown. Further analysis of genetic determinants of MV's virulence has indicated that two overlapping restriction subfragments of the BamHI "C" fragment can transfer MV's virulent behavior to SFV. The 0.7-kb region in which these two subfragments overlap includes the C-terminus of MV orf C-7 and the N terminus of MV orf C-8. These correspond to the C- and N-termini, respectively, of SFV orf's D-9 and D-10 and to vaccinia orf's D-6 (early transcription factor) and D-7 (subunit of RNA polymerase). We sequenced the region of SFV's BamHI "D" fragment in this area and illustrate here the comparative sequences of this portion of SFV's genome and orf's. On the basis of comparisons between MV, SFV, and vaccinia in this area we discuss the potential significance of these observations.
This study presents previously unreported data on a series of 400 peripheral ossifying fibromas (POFs) and 13 peripheral odontogenic fibromas (PODFs). The differences between the two lesions are discussed, and comparisons are made with other reports in the literature. It is concluded that the lesions represent separate pathologic entities.
The present study examined histological difference between ossifying fibromas (OF, n=5) and peripheral cemento-ossifying fibromas (PCOF, n=7). Bone morphogenetic proteins (BMP)-2 and -4, osteopontin (OPN), osteocalcin (OCN) and proliferating cell nuclear antigen (PCNA) were used for the immunohistochemical examinations. Oxytalan fibers present at the periodontal tissue were stained to determine the tumor cell origin. Many OFs showed high immunohistochemical reactions for BMP-2, -4 and OPN compared to those of PCOFs. PCNA index (IP) of OFs was significantly higher than that of PCOFs. All the PCOFs showed a high expression of oxytalan fibers. Only two OFs exhibited a small number of oxytalan fibers. These results suggest that PCOF has only little ability to form hard tissue and seems to be a reactive lesion. The expression of oxytalan fibers reveals that OF does not only originate from periodontal tissue.
A father and his son were seen because of multiple wartlike lesions on their fingers and palms. On histologic examination, these lesions showed changes compatible with vascular fibromas, myxoid fibromas, and, in a few instances, verruca vulgaris. We are not aware of reports of similar cases in the dermatologic literature.
Psammomatoid ossifying fibroma (POF) is a diagnostic designation for a sinonasal or orbital fibroosseous lesion characterized by numerous small round mineralized spherules lying in a fibroblastic stroma. We report 3 cases. One occurred in the frontal sinus of an 18-years-old male; 1 in the frontal sinus of a 20-year-old male and 1 in the maxillary sinus of a 36-year-old male. Rather limited excision resulted in a disease-free follow-up time from 2 to 6 years. The histomorphology of POF was compared with other fibro-osseous lesions of the craniofacial skeleton including the jaws. It appeared that POF is similar to cemento-ossifying fibroma occurring in the jaws and the necessity for different names for lesions only differing in their site of occurrence is considered to be at least debatable.
We describe a unique benign mesenchymal tumor in paraspinal location in a 13-year-old patient with Gardner's syndrome. The Gardner's syndrome in this patient consisted of multiple (more than 100) polyps throughout the entire colon with most in the cecum and rectum, three osteomas in the frontal area of the skull and one in the third right rib, and multiple superficial skin tumors. One of these cutaneous tumors was excised and histologically diagnosed as an epidermal cyst. Both father and uncle of this patient suffered from Gardner's syndrome as well. Microscopically the mesenchymal tumor was histologically different from nuchal type fibroma and fibromatosis. It consisted of a diffusely-growing fibrous mass composed of dense collagenous fibers and relatively numerous, bland-looking, spindle-shaped cells. The collagen fibers had haphazard spacing with no lobular arrangement. The collagen fibers were of a very coarse quality. No entrapment of adipose tissue, skeletal muscle or peripheral nerves was seen in the lesion. Immunohistochemically the tumor was vimentin positive and smooth muscle actin, muscle-specific actin, S-100 protein, cytokeratin and desmin negative.
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