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PubMed1983

Immunohistology of malignant rabbit fibroma virus--a comparative study with rabbit myxoma virus.

Malignant rabbit fibroma virus (MV) causes a syndrome that consists of disseminated malignant tumors and immunosuppression complicated by severe Pasteurella multocida infection and death. Tissues from rabbits given MV and rabbit myxoma virus were examined by direct immunofluorescence with the use of antibody against virus antigens. Primary and metastatic tumors caused by MV and rabbit myxoma virus were composed of soft tissue cells containing virus antigens. Skin appendages and epidermis overlying the respective tumors showed scant MV but abundant myxoma virus antigen. Both viruses were present systemically in the reticuloendothelial system. Epithelial cells from the liver, kidney, and lung of myxoma virus-infected rabbits contained virus, whereas in MV tumor-bearing rabbits, these cells were uninvolved. However, nasal mucosal and conjunctival epithelia, the locations of Pasteurella infection, showed squamous metaplasia and contained large amounts of MV and myxoma antigens. By analogy to other respiratory tract pathogens, these epithelial changes were probably etiologically significant for development of pasteurellosis in rabbits bearing virus-induced tumors. Thus by immunopathologic as well as clinical examination, MV produces a syndrome distinct from that seen with rabbit myxoma virus. MV induced severe immunosuppression despite T-lymphocyte hyperplasia in the lymphoid tissues observed. The combination of a systemic virus infection, epithelial alterations that impaired clearance mechanisms, and immunologic dysfunction is likely to contribute to the inability of rabbits given MV to survive their gram-negative infection.

Animals↗
PubMed1986

Psammomatoid ossifying fibroma.

Fibro-osseous lesions represent a variety of bone proliferations each characterized by different morphologic patterns of osteoid production. Psammomatoid ossifying fibroma (POF) is characterized histologically by numerous small round ossicles resembling psammoma bodies and is a locally invasive lesion of facial and cranial bones. Two cases of POF arising in the ethmoid sinus and involving the orbit are presented to emphasize the importance of complete surgical removal of involved bones. Histologically, portions of POF may demonstrate other patterns of osteoid production, which resemble fibrous dysplasia and Paget's disease of bone. The variation in radiodensity in POF on computed tomography is a function of the density of psammomatoid ossicles and of the coexistence of other "minor" forms of bone proliferation.

Child↗
PubMed1988

Epibulbar fibroma of the conjunctival substantia propria.

A 39-year-old black woman had a 12-year history of slowly progressive left temporal juxtalimbal conjunctival swelling. The lesion was freely movable over the surface of the globe and was believed to be situated within the substantia propria. Histopathologic evaluation of the excised tissue disclosed that it was a collagenous mass of lamellar architecture, with a hypocellular dispersion of mesenchymal cells and a scattering of capillaries in the absence of inflammation. Electron microscopic examination revealed that the lamellar arrangement had regular lobular subdivisions composed of collagen fibers approximately 50 nm in diameter. Compressed fibroblasts occupied the peripheries of the lobules; the former exhibited abundant rough-surfaced endoplasmic reticulum and failed to elaborate basement membrane material. Delicate processes of the fibroblasts extended into the centers of the collagenous aggregates. To our knowledge, this is the first convincingly documented case of an acquired fibroma of the substantia propria of the epibulbar conjunctiva. The differential diagnosis in this case included related fibroblastic and simulating nonfibroblastic lesions.

Adult↗
PubMed1996

Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.

BACKGROUND: Desmoplastic fibroma (DF) is an extremely rare bone tumor. The recommendations for therapy are often based on limited personal experience, and the rate of local recurrence in the published cases is very high. Therefore, an analysis of treatment results of published cases was performed. Furthermore, DNA analysis of the tumors from two patients was also performed. METHODS: The clinical, radiologic, and histologic data of two patients with DF of the long bones are presented. DNA flow cytometry was performed on both DFs, three cases of abdominal fibromatosis, and three cases of extraabdominal fibromatosis. One hundred eighty-nine patients analyzed in the literature and our own 2 patients were evaluated with regard to epidemiologic, clinical, and histologic data, with particular emphasis on treatment results. RESULTS: DNA analysis of the locally infiltrating tumors revealed indices of proliferation between 21.5% and 24%, noticeably elevated values in comparison with extraosseous desmoid tumors (8.04%). Magnetic resonance imaging (MRI) was most valuable for imaging the intraosseous and extraosseous extent of DF. The evaluation of 191 patients (189 from the literature, 2 of the authors) showed the numbers of males and females to be equivalent, with a mean age of 23 years. DF has been reported in almost all bones, with a tendency to occur in the mandible and the long bones. Approximately 12% of patients presented with a pathologic fracture (20 of 161 patients). Infiltrative growth in the soft tissue was documented in 48% of patients. Three patients developed metastases after local recurrence. Analyzing the treatment results, the authors found a recurrence rate of 55-72% after nonresection procedures, and 17% after resection. No recurrences are reported after resection with wide surgical margins. The recurrence rate of tumors of the extremities was 55%, and 25% of these patients eventually required an amputation. CONCLUSIONS: Considering the "semimalignant" character of this entity and the poor treatment results in patients with recurrent tumors, marginal or wide resection for primary treatment is recommended. The superior imaging quality of MRI greatly facilitates preoperative planning.

Adolescent↗
PubMed1999

Nuchal-type fibroma: a clinicopathologic study of 52 cases.

BACKGROUND: Nuchal-type fibroma (NTF) usually arises in the posterior aspect of the neck. Previously published reports describe only 11 cases and provide limited clinicopathologic information. METHODS: Fifty-two examples of NTF from 50 patients were analyzed from the files of the Soft Tissue Registries of the Armed Forces Institute of Pathology in Washington, DC, and the Faculty Hospital in Pilsen, Czech Republic. RESULTS: The age of the patients ranged from 3 to 74 years (mean, 40 years). There were 41 males and 9 females. Thirty-six NTFs were located in the posterior neck region and 16 were from extranuchal sites. Two patients had had lesions excised from both a nuchal and an extranuchal location. The mean greatest tumor dimension was 3.2 cm. Microscopically, all examples had a superficial (subcutaneous and sometimes dermal) component and consisted of paucicellular, thick bundles of lobulated collagen fibers with inconspicuous fibroblasts. Entrapped adipose tissue and traumatic neuromalike nerve proliferations were typically present. Skeletal muscle infiltration was also seen in a minority of cases. Eleven of 25 patients (44%) for whom clinical information was available reportedly had diabetes. Gardner's syndrome was documented in one patient and was possibly present in two additional individuals. During follow-up, five patients had local recurrences, but none of the recurrences were destructive and all were ultimately controlled by local reexcision. CONCLUSIONS: NTF is a rare, tumorlike accumulation of collagen that often affects the posterior neck region but can also occur in a number of other sites. The process has a strong association with diabetes and also appears to be linked to Gardner's syndrome. Local recurrence probably reflects the persistence of local or systemic factors related to its pathogenesis.

Adolescent↗
PubMed1983

Osteofibrous dysplasia (ossifying fibroma of long bones). A study of 12 cases.

Osteofibrous dysplasia (ossifying fibroma of long bones) is one of the fibro-osseous lesions that affects the tibia and fibula in the first decade of life. A study of 12 patients with this lesion showed the high rate of recurrence after surgical intervention. Most commonly, the lesions are eccentrically located in the diaphysis of the tibia. Pseudarthrosis may develop in cases where the lesion is situated in the distal portion. Roentgenologic and histopathologic features are basically similar to those of monostotic fibrous dysplasia of long bones. However, bony or osteoid trabeculae covered by osteoblasts enable distinction between osteofibrous dysplasia and monostotic fibrous dysplasia, as determined histologically. The current evidence indicates that surgery should not be attempted in patients under 10 years of age.

Bone Neoplasms↗
PubMed1992

The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors.

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Adolescent↗
PubMed1993

Osteosarcoma. Low-grade intraosseous-type osteosarcoma, histologically resembling parosteal osteosarcoma, fibrous dysplasia, and desmoplastic fibroma.

BACKGROUND: Low-grade intraosseous osteosarcoma is a rare variety of osteosarcoma and it is difficult to recognize. METHODS: A series of 10 patients with low-grade intraosseous osteosarcoma is reported. These patients were identified from the Istituto Rizzoli files, which includes approximately 1000 cases of osteosarcoma. Clinical data and radiographic and histologic features were studied. RESULTS: The radiographic appearance confirmed malignancy in five patients and suggested it in two. A benign lesion was diagnosed in three patients. Three lesions resembled parosteal osteosarcoma, two appeared similar to fibrous dysplasia, and two had features of desmoplastic fibroma. A mixed histologic pattern was found in three other tumors. Recurrence after intralesional excision in all patients indicated the aggressive nature of this lesion. The development of metastases in two patients and progression in the grade of malignancy in one of these highlighted the malignant nature of the tumor. CONCLUSIONS: The correct diagnosis would permit adequate treatment with wide surgical margins.

Adolescent↗
PubMed1986

Fibroma of tendon sheath.

This report describes a 60-year-old woman with a fibroma of tendon sheath of left hand. Ultrastructural study of the tumor reveals the nature of the tumor cells as fibroblasts, thus distinguishing it from the more common giant cell tumor of tendon sheath believed to be derived from synovial cells.

Carpal Tunnel Syndrome↗
PubMed1984

Maturation of Wilms' tumor pulmonary metastases to benign fibromas after therapy.

A 3-year-old girl with Wilms' tumor developed pulmonary metastases 5 months after diagnosis. All but one of the visible lesions disappeared after thoracic irradiation and multiple-agent chemotherapy. Subsequent thoracotomy revealed 17 small nodules in addition to the visible lesion, and all of them proved to be fibromas. It would appear that the Wilms' tumor metastases to the lung were made to mature to benign neoplastic processes after the treatments given.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Parovarian fibroma with heterotopic bone formation of probable wolffian origin.

Parovarian neoplasms of wolffian duct origin are very unusual. This article presents the first report of a case of parovarian fibroma with heterotopic bone formation of probable wolffian duct origin. The tumor cells present among mature collagen bundles were fibroblast-like cells, while those at the sites of cellular proliferation were either undifferentiated mesenchymal cells or had differentiated into osteoblasts. In the bone matrix, the tumor cells had differentiated into osteoblasts and connective tissue had ossified.

Adult↗
PubMed1996

Adenocarcinoma of the breast metastatic to benign ovarian fibroma.

An elderly woman presenting with a perforated benign gastric peptic ulcer was found to have widespread dissemination of breast carcinoma. Several small discrete metastases were present within a large benign ovarian fibroma. The rare phenomenon of tumor-to-tumor metastasis is discussed in the context of ovarian pathology.

Adenocarcinoma↗
PubMed1999

Shope fibroma virus DNA topoisomerase catalyses holliday junction resolution and hairpin formation in vitro.

The telomeres of poxviral chromosomes comprise covalently closed hairpin structures bearing mismatched bases. These hairpins are formed as concatemeric replication intermediates and are processed into mature, unit-length genomes. The structural transitions and enzymes involved in telomere resolution are poorly understood. Here we show that the type I topoisomerase of Shope fibroma virus (SFV) can promote a recombination reaction which converts cloned SFV replication intermediates into hairpin-ended molecules resembling mature poxviral telomeres. Recombinant SFV topoisomerase linearised a palindromic plasmid bearing 1.5 kb of DNA encoding the SFV concatemer junction, at a site near the centre of inverted-repeat symmetry. Most of these linear reaction products bore hairpin tips as judged by denaturing gel electrophoresis. The resolution reaction required palindromic SFV DNA sequences and was inhibited by compounds which block branch migration (MgCl2) or poxviral topoisomerases. The resolution reaction was also slow, needed substantial quantities of topoisomerase, and required that the palindrome be extruded in a cruciform configuration. DNA cleavage experiments identified a pair of suitably oriented topoisomerase recognition sites, 90 bases from the centre of the cloned SFV terminal inverted repeat, which may mark the resolution site. These data suggest a resolution scheme in which branch migration of a Holliday junction through a site occupied by covalently bound topoisomerase molecules, could lead to telomere resolution.

DNA Topoisomerases, Type I↗
PubMed1994

Characterization of the Shope fibroma virus DNA ligase gene.

The Shope fibroma virus (SFV) DNA ligase gene has been cloned and sequenced, and the biochemical requirements of the gene product have been determined in vitro. The SFV ligase gene maps to the BamHI L1/L2 boundary and spans 1.7 kb. The gene is predicted to encode a 559-amino-acid protein of M(r) = 63,139 which shares 45% amino acid identity with Orthopoxvirus ligases. The C-terminal two-thirds of the protein appears to encode the catalytic domain and shares distant homology with many ligases. The N-terminal homology is shared between only Orthopoxviruses and Leporipoxviruses and suggests that DNA ligases may be composite structures consisting of two independently evolved protein domains. Although the the gene encodes features characteristic of both early and late poxviral genes, Northern analysis showed that SFV ligase is expressed as a late gene product. In order to prove the identity of the protein it was expressed as a glutathione S-transferase fusion in Escherichia coli, affinity purified, and shown to be a Mg2+.ATP-dependent ligase in vitro. The recombinant protein can also form a covalent ligase.AMP complex characteristic of ATP-dependent DNA ligases. The SFV ligase gene can be disrupted and is thus not essential for viral growth in culture. This was shown by recombining a PCR product, encoding a P7.5 promoter and E. coli guanine phosphoribosyltransferase gene (gpt) into the open reading frame, and selecting for gpt+ viruses. This work provides insights into the evolution of Orthopoxviruses and Leporipoxviruses and strains suitable for a detailed analysis of the role DNA ligases play in poxviral recombination.

Amino Acid Sequence↗
PubMed1998

Shope fibroma virus RING finger protein N1R binds DNA and inhibits apoptosis.

Shope fibroma virus (SFV) N1R gene encodes a RING finger protein that localizes to virus factories within the cytoplasm of infected cells. Altered proteins, with deletions and site-specific mutations, were transiently expressed in vaccinia virus-infected cells to discern regions of the protein that are required for localization. We have determined that at least part of the RING finger region is necessary for localization but that the RING motif alone is not sufficient. A chimeric protein, however, in which the RING finger region of the herpes simplex virus-1 ICP0 protein replaces the SFV N1R RING motif does localize to virus factories. A region of five highly conserved amino acids at the amino terminus of SFV N1R is also critical for localization. We report that the SFV N1R protein binds double- and single-stranded DNA, suggesting a mechanism for localization, and that overexpression of this protein in vaccinia virus-infected cells reduces apoptosis-associated fragmentation of nuclear DNA.

Amino Acid Sequence↗
PubMed1999

The complete genome sequence of shope (rabbit) fibroma virus.

We have determined the complete DNA sequence of the Leporipoxvirus Shope fibroma virus (SFV). The SFV genome spans 159.8 kb and encodes 165 putative genes of which 13 are duplicated in the 12.4-kb terminal inverted repeats. Although most SFV genes have homologs encoded by other Chordopoxvirinae, the SFV genome lacks a key gene required for the production of extracellular enveloped virus. SFV also encodes only the smaller ribonucleotide reductase subunit and has a limited nucleotide biosynthetic capacity. SFV preserves the Chordopoxvirinae gene order from S012L near the left end of the chromosome through to S142R (homologs of vaccinia F2L and B1R, respectively). The unique right end of SFV appears to be genetically unstable because when the sequence is compared with that of myxoma virus, five myxoma homologs have been deleted (C. Cameron, S. Hota-Mitchell, L. Chen, J. Barrett, J.-X. Cao, C. Macaulay, D. Willer, D. Evans, and G. McFadden, 1999, Virology 264, 298-318). Most other differences between these two Leporipoxviruses are located in the telomeres. Leporipoxviruses encode several genes not found in other poxviruses including four small hydrophobic proteins of unknown function (S023R, S119L, S125R, and S132L), an alpha 2, 3-sialyltransferase (S143R), a protein belonging to the Ig-like protein superfamily (S141R), and a protein resembling the DNA-binding domain of proteins belonging to the HIN-200 protein family S013L). SFV also encodes a type II DNA photolyase (S127L). Melanoplus sanguinipes entomopoxvirus encodes a similar protein, but SFV is the first mammalian virus potentially capable of photoreactivating ultraviolet DNA damage.

Amino Acid Sequence↗
PubMed1993

Case report 787: Desmoplastic fibroma.

An unusual case of desmoplastic fibroma of the pelvic bones is presented, showing spontaneous arrest of growth. The tumor showed a marginal sclerosis, a rather rare finding in these lesions and a phenomenon probably related to the tumor's very slow growth in the presented patient.

Aged↗