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Subungual pleomorphic fibroma.

BACKGROUND: Pleomorphic fibroma is a benign fibroblastic tumor characterized by pleomorphic, hyperchromatic cells or giant multinucleated cells embedded in a collagenous stroma. These cytologic features may lead to an incorrect diagnosis of malignancy. Most cases reported in the literature are located on trunk or extremities; the presentation as a subungual mass is rare. METHODS: We report an unusual case of a subungual pleomorphic fibroma in 66-year-old woman. Clinical information was obtained. Histologic examination and immunohistochemical studies were performed. RESULTS: A 66-year-old woman presented with a longstanding (40 years), subungual mass that deformed the nail of the left middle finger. Microscopic examination revealed a paucicellular tumor composed of hyperchromatic spindled, pleomorphic, floret-like giant cells embedded in haphazardly arranged collagen bundles in the dermis. No mitotic figures were seen. The tumor cells were vimentin-positive but did not stain with antibodies to S-100, cytokeratin, smooth muscle actin, factor XIIIa or CD34 negative. The diagnosis of a pleomorphic fibroma was made. Follow-up shows no evidence of tumor, 36 months after excision. CONCLUSION: Pleomorphic fibroma of the subungual region is an unusual cutaneous tumor with histologic features that may cause confusion with true sarcomas. This is only the second case reported of a subungual pleomorphic fibroma. Pleomorphic fibroma should be considered in the differential diagnosis of pleomorphic subungual tumors.

Aged↗

Two cases of supernumerary ovary: one with large fibroma with Meig's syndrome and the other with endometriosis and cystic change.

Reports of supernumerary ovaries are rare. We describe two such cases, one with fibroma and the other with endometriosis and cystic change. A large fibroma measuring 17.4 x 12.0 x 7.5 cm in size was found in the supernumerary ovary of the omentum in the first case of a 47-year-old married woman with Meig's syndrome. The second case was associated with endometriosis and cystic change, measuring 11 x 5 x 3 cm in size and located in the upper abdominal cavity. It was attached to the uterus of a 28-year-old pregnant woman who had neither fibroma nor Meig's syndrome. Histologically, corpus albicans and a few primordial germ cells were demonstrated, respectively. A fibroma showing a storiform pattern was found in the first case. The second case had endometriosis and a thin-walled cyst with bleeding and necrosis caused by torsion. Immunohistochemically, desmin, alpha-smooth muscle actin, c-kit, CA125, Na+/K+ATPase, overexpression of p53, myc and ras were all negative in the fibroma cells of the first case, and in the endometriosis and cyst wall of the second case. The fibroma cells were positive for vimentin and estrogen receptor, and the proliferating cell nuclear antigen was sporadically demonstrated in their nuclei. The mutation of the p53 gene at exons 5-8 was not detected by sequence analysis. Using RT-PCR, bax, bcl-2 and p16 were not detected either. Clinically, the two cases presented here did not show abnormal hormonal symptoms. They were diagnosed as abdominal tumors or masses. Based on these considerations, one might assume that supernumerary ovaries are probably more frequent than reported at present.

Adult↗

Depressed cell-mediated immunity in newborn rabbits bearing fibroma virus-induced tumors.

Tumors were induced in adult and newborn rabbits by inoculation of fibroma virus. Whereas tumors completely regressed in adult rabbits by 3 weeks after virus inoculation, newborn rabbits supported tumor growth for 3 to 4 weeks. In the latter case, some animals died at this time, others survived with a gradual regression of the tumors over an additional period of 5 to 6 weeks. Virus neutralization studies demonstrated antibodies to fibroma virus in the serum from both adult and newborn tumor-bearing rabbits. Newborn rabbits with progressively growing tumors failed to elicit a delayed cutaneous hypersensitivity reaction to fibroma antigens, whereas adult rabbits showed strong reactions as early as 7 days after tumor induction. Similarly, macrophage migration inhibition tests revealed that the lymphocytes from newborn rabbits with progressively growing tumors were only weakly reactive to fibroma antigens, as compared to lymphocytes from adult tumorbearing rabbits. In contrast, newborn rabbits that survived and regressed the tumors demonstrated strong cell-mediated immunity both by skin testing and migration inhibition. Virus growth studies in cell culture demonstrated that fibroma was unable to replicate in peritoneal macrophages from either newborn or adult rabbits. No differences were observed in growth of the virus in macrophages from tumor-bearing rabbits. The significance of these observations is discussed in respect of the possible role of cell-mediated immunity in fibroma tumor regression.

Animals↗

Studies on the polypeptides of poxvirus. II. Comparison of virus-induced polypeptides in cells infected with vaccinia, cowpox and Shope fibroma viruses.

Virus-induced polypeptides in cells infected with vaccinia, cowpox and Shope fibroma viruses were examined by SDS-polyacrylamide gel electrophoresis followed by autoradiography. At least 42 vaccinia virus-induced polypeptides were identified among the polypeptides of cells pulse-labeled with [35S]-methionine and/or of fractionated cells labeled with [14C]-leucine for 24 hr. They consisted of 15 polypeptides (early polypeptides) which were synthesized even in the presence of cytosine-1-beta-D-arabinofuranosyl-HCl, and 27 polypeptides (late polypeptides) which were synthesized only in the absence of cytosine-1-beta-D-arabinofuranosyl-HCl. By the same procedure at least 40 cowpox virus-induced polypeptides (14 early polypeptides and 26 late polypeptides) and at least 31 Shope fibroma virus-induced polypeptides (13 early polypeptides and 18 late polypeptides) were identified. Comparative studies of virus-induced polypeptides on the basis of migration in SDS-polyacrylamide gel electrophoresis revealed that 11 polypeptides were early polypeptides common to both vaccinia and cowpox viruses; 21 were late polypeptides common to both vaccinia and cowpox viruses; 4 were early polypeptides common to both vaccinia and Shope fibroma viruses; 7 were late polypeptides common to both vaccinia and Shope fibroma viruses; 5 were early polypeptides common to both cowpox and Shope fibroma viruses; 9 were late polypeptides common to both cowpox and Shope fibroma viruses; 4 were early polypeptides common to all three viruses; and 7 were late polypeptides common to all three viruses.

Cell Line↗

[Ossifying fibroma in maxilla. Report of case].

Since first reported by Montgomery in 1927, ossifying fibroma, a benign neoplasm of bone or bone tissue, has been the subject of numerous studies. In the present paper, a case of ossifying fibroma in a woman is presented. The woman was 49 years old. She visited our clinic with a complaint of swelling in the right maxillary region. Thorough examinations were conducted. From X-ray, CT, scintigraphy and biopsy findings, the case was diagnosed as ossifying fibroma. Under a general anesthetic, the tumor was extirpated. It measured 40 x 40 x 60mm. It's weight, including that of teeth, was 60.5 g. Histopathological examination revealed fibroblasts and hard, bone-like masses of varying shapes in the connective tissue. Nearly one year has elapsed since the surgical operation. The patient is doing well. Since 1957, a total of 107 cases of ossifying fibroma have been reported in Japan. The ages of the patients were mostly between 10 and 49. By sex, 42 were male and 65 female. By region, in 28 case, fibroma occurred in the maxillary part, 68 in the mandibular part, and 11 in the region extending from maxillary to mandibular area. More 70% of the cases were fibroma originating in the molar area.

Female↗

Ossifying fibroma of the maxillary sinus: a case report.

A case of ossifying fibroma of the maxillary sinus that occurred in a 45-year-old white female is reported. The lesion's radiographic, histologic and clinical behavior are examined. The clinical and radiographic features of ossifying fibroma distinguish it from monostatic fibrous dysplasia despite histologic similarities. The uncommon location of this ossifying fibroma in the maxillary sinus accounts for its large size, aggressive behavior and widespread osseous destruction. The prognosis is excellent after complete enucleation of the ossifying fibroma has been achieved. The benign fibro-osseous lesions of the jaws share similarities in radiographic and clinical appearance, histogenesis and histopathology, and consequently, pose difficulty in classification and treatment. Common histologic features of these lesions include an active proliferation of fibroblats, young and mature collagenous connective tissue, focal areas of mineralization which may resemble small cemeticles and/or irregular bone trabeculae, and multinucleated giant cells. Differential diagnosis of benign fibro-osseous lesions can therefore be made if clinical behavior, radiographic features, and hematologic changes are correlated with the histologic picture. Representatives of this group include true fibrous dysplasia, ossifying fibroma (both central and peripheral types), osteoid osteoma, osteoblastoma, cementifying fibroma, florid osseous dysplasia, proliferative periostitis of Garré, focal sclerosing osteomyelitis and osteitis deformans (Paget's disease).

Diagnosis, Differential↗

Sclerotic fibroma of tendon sheath.

Two cases of sclerotic fibromas directly related to the tendon sheath are presented. Fibroma in these 2 cases consisted of hypocellular, homogeneous collagen bundles that were closely related to the subjacent tendon sheath, and were described by the term 'sclerotic fibroma of tendon sheath'. It is possible that the sclerotic fibroma may arise from fibroma of tendon sheath and that common pathomechanisms exist between these two types of fibroma.

Adult↗

Peripheral odontogenic fibroma.

Peripheral odontogenic fibroma (WHO type) is an uncommon lesion of the gingiva; 18 well-documented cases have been published. It is considered to be the extraosseous counterpart of the central odontogenic fibroma. Because of the paucity of reported cases, the full histomorphologic spectrum of this lesion has not yet been established. This article presents nine cases of peripheral odontogenic fibroma that illustrate the variety of its histopathologic findings. The connective tissue ranged from loose (almost myxomatous) to markedly cellular to relatively acellular and well-collagenized. Islands and/or strands of odontogenic epithelium were present in all lesions. Matrix of mineralized material was present in three cases, and juxtaepithelial hyalinization was seen in one case. To avoid the introduction of additional diagnostic terms, we suggest that all these lesions be considered a spectrum of the peripheral odontogenic fibroma (WHO type). We also suggest that the term WHO type be used to distinguish peripheral odontogenic fibromas from the peripheral ossifying fibroma with which they have often been confused.

Adolescent↗

Peripheral odontogenic fibroma. Report of 5 cases.

The peripheral odontogenic fibroma (WHO type) is a relatively rare, benign, unencapsulated, exophytic gingival mass of fibrous connective tissue. Odontogenic epithelium is found within the gingival mass, but usually appears to play a minor role when compared to the fibrous component. According to the present concept, cases reported in the literature under the terms "odontogenic gingival epithelial harmartoma" "hamartoma of the dental lamina" and "peripheral ameloblastic fibrodentinoma" are actually examples of peripheral odontogenic fibroma. Review of the literature revealed only 30 acceptable cases that fit the present concept of peripheral odontogenic fibroma. Because of the paucity of reported cases, the histomorphological spectrum and the clinical features of this lesion have not yet been fully established. This article presents five new cases of peripheral odontogenic fibroma. The connective tissue ranged from markedly cellular to relatively acellular well collagenized. Islands and strands of epithelium were present in all five cases: in four they were scanty and in one abundant. A matrix of mineralized material was present in four cases. The peripheral odontogenic fibroma must be differentiated histologically from peripheral ossifying fibroma, which is a reactive lesion, and from the peripheral ameloblastoma and the calcifying epithelial odontogenic tumour.

Adult↗

The pericentromeric inversion, inv (6)(p25q13), is a novel diagnostic marker in chondromyxoid fibroma.

Chondromyxoid fibroma is a benign bone tumor that arises most commonly in the metaphysis of long bones in young adults. The cytogenetic features of this tumor are not well known. In this study, four chondromyxoid fibromas were karyotyped after short-term cell culture. All of the tumors contained clonal rearrangements of chromosome 6, and each of these rearrangements involved band 6q13. Two tumors contained a pericentromeric inversion, inv (6)(p25q13), which was described recently in chondromyxoid fibroma. 6q13 rearrangements are not associated with other types of bone and soft tissue tumors, and the inv (6)(p25q13) is reported only in chondromyxoid fibroma. Hence, this cytogenetic marker might be helpful in distinguishing chondromyxoid fibroma from chondrosarcoma and other histologic mimics. The consistent occurrence of 6q13 rearrangements suggests a specific oncogenic mechanism in chondromyxoid fibroma, most likely involving oncogene activation.

Adolescent↗

Odontogenic fibroma.

The odontogenic fibroma is a rare tumor that has generated controversy, perhaps disproportionately to its importance in the family of odontogenic tumors. The clinical and radiographic features are well documented but the histologic aspects have generated controversy. The behavior is benign, and published accounts indicate a low recurrence rate following treatment by curettage. The tumor recognized by the World Health Organization (WHO) is the legitimate odontogenic fibroma. Histologic variants include the granular cell type and the hybrid odontogenic fibroma giant cell-like tumor. Although the extraosseous "peripheral" odontogenic fibroma presents as a gingival enlargement clinically indistinguishable from other gingival lesions, its histomorphology is similar to the central tumor. A normal dental follicle around the crown of an unerupted tooth may histologically mimic the odontogenic fibroma and other odontogenic tumors.

Diagnosis, Differential↗

Recurrent digital fibroma, focal dermal hypoplasia, and limb malformations.

Recurrent digital fibroma of infancy generally is considered a sporadic tumor of childhood. We describe the case of a mother with recurrent digital fibroma at a young age who gave birth to a daughter with focal dermal hypoplasia, coloboma of the iris and eyelids, anal atresia, and extensive limb malformations. When the infant was 3 months old, fibromas started to appear at the fingertips. The cases of three additional patients are described, with a similar combination of multiple digital fibromas, pigmented marks on the temporal region, and limb malformations. One of these patients has consanguineous parents. The clinical findings overlap partially with Gorlin-Goltz syndrome, which has been renamed by some authors "microphthalmia with linear skin defects" (MLS). Since the skin signs are clearly different, however-more like those of Setleis syndrome ("forceps mark" temporal dysplasia)-the patients described here seem to have a new combination of congenital malformations. Deletion of distal Xp, known to occur in some MLS patients, was not detected using cosmids in fluorescence in situ hybridization. This pattern of digital fibroma with congenital malformations seems to represent a new syndrome.

Abnormalities, Multiple↗

Complex karyotypic abnormality in ovarian fibroma associated with Gorlin syndrome.

Nevoid basal cell carcinoma (NBCC) syndrome is an autosomal dominant disorder characterized by distinctive congenital malformations and a variety of benign and malignant neoplasms, including ovarian fibromas. We describe pathologic and cytogenetic findings in a large unilateral ovarian fibroma from a 12-year-old female with NBCC syndrome. The pathologic findings were characteristic for ovarian fibroma, but were unusual for the ovarian fibromas associated with NBCC syndrome because of the absence of calcification, the lack of bilaterality, and the presence of focal hypercellularity. The karyotype of tumor tissue showed complex numerical and structural abnormalities. Although there is frequent loss of heterozygosity of 9q22.3 and mutations in the PTCHgene in Gorlin syndrome, the ovarian fibroma in this case did not have cytogenetically detectable abnormalities of chromosome 9.

Basal Cell Nevus Syndrome↗

Bilateral ovarian fibromas in children.

The case of bilateral ovarian fibromas occurring in an 8-year-old black girl is reported. These lesions occur rarely in premenarchal females and may be a manifestation of Nevoid Basal Cell Syndrome. Calcifications are reported to occur rarely in ovarian fibromas but seem to occur frequently in fibromas in children. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma. Preservation of normal ovarian tissue is recommended with the acknowledged risk of recurrence of the fibroma.

Child↗

The central odontogenic fibroma: an attempt at clarification.

Three different but probably related central lesions of the jaws have been referred to previously as odontogenic fibromas. They are (1) hyperplastic dental follicles, (2) a fibrous neoplasm having a histologic appearance similar to that of a dental follicle, and (3) a more complicated lesion, illustrated in a WHO publication, consisting of fibrous connective tissue with varying amounts of odontogenic epithelium, dentin, and/or material resembling cementum. The terms simple odontogenic fibroma and odontogenic fibroma (WHO type) are proposed, respectively, for lesions 2 and 3 above. Some examples of lesion 3 may be confused histologically with the calcifying epithelial odontogenic tumor. More information is required on the biologic behavior of both 1 and 2. A central fibroma of the jaws should be considered a desmoplastic fibroma if it does not appear to have arisen from the odontogenic apparatus and is not a neurofibroma.

Dental Sac↗

Fibroma of tendon sheath. A clinicopathologic study of 32 cases.

We report 32 cases of fibroma of tendon sheath. Most cases presented as a painless mass in the distal portion of an extremity. Ganglion cyst was the most frequent clinical diagnosis. The median patient age was 30.5 years, and 60% of the patients were male. Only one lesion is known to have recurred. The lesions, which averaged 1.5 cm, were light tan, firm, and nodular. Histologic features common to all lesions were (a) a predominantly fibrous matrix containing (b) fibroblast-like spindle cells. Elongated, slitlike spaces were observed in many lesions, and nine cases had areas closely resembling nodular fasciitis. Myofibroblasts and fibroblasts were observed in the three cases studied by electron microscopy. The histologic findings were similar to those previously described for fibroma of tendon sheath. Although slitlike spaces are present in most instances, this finding is not specific for fibroma of tendon sheath, nor is it invariably present. Fasciitis-like changes have been noted in previous series. Our findings, as well as those from prior studies, indicate that fibromas of tendon sheath are heterogeneous. The diagnosis is made only after other fibrous, nodular lesions of the extremities are excluded. Fasciitis-like lesions heretofore classified as fibroma of tendon sheath are more appropriately classified as tenosynovial counterparts of nodular fasciitis.

Adolescent↗

Fibrosarcoma versus cellular fibroma of the ovary: a comparative study of their proliferative activity and chromosome aberrations using MIB-1 immunostaining, DNA flow cytometry, and fluorescence in situ hybridization.

We retrospectively analyzed the proliferative activity and the centromeric copy number of chromosomes 8, 12, and 17 in three cases of fibrosarcoma and eight cases of cellular fibroma of the ovary using MIB-1 immunostaining, DNA flow cytometry, and fluorescence in situ hybridization (FISH) on paraffin-embedded tissue specimens. In our study, both the MIB-1 labeling index (LI) and the proliferative index (% of cells in S + G2 + M phase) in fibrosarcomas were higher than those in cellular fibromas. The FISH analysis demonstrated the sole abnormality of a gain of trisomy 12 cells in all eight cases of cellular fibroma. Both a gain of trisomy 12 cells and a gain of tetrasomy 12 cells were observed in one case of fibrosarcoma. A gain of trisomy 8 cells was observed in all two fibrosarcomas in which signals were detected. By contrast, neither a gain of trisomy 8 cells nor a gain of tetrasomy 12 cells was observed in any of the eight cases of cellular fibroma. Chromosome 17 showed disomy in all eleven cases. On the basis of these findings, a gain of trisomy 8 cells is therefore considered to be an adequately effective marker to distinguish between cellular fibroma and fibrosarcoma of the ovary, and it may also be related to the proliferative activity of fibrosarcoma of the ovary.

Adolescent↗

Nuchal-type fibroma in two related patients with Gardner's syndrome.

Nuchal-type fibroma is a distinct subcutaneous and dermal fibrous tissue proliferation that has been previously definitely identified in one patient with Gardner's syndrome and has been possibly present in two others. Gardner's syndrome is an autosomal-dominant condition with variable expressivity that comprises epidermoid cysts, fibrous tumors, osteomas, intestinal polyposis, as well as other findings. We report two cases of nuchal-type fibroma presenting in a 13-year-old boy in the right upper back and in his 60-year-old grandfather in the upper chest at the posterior axillary line. Both individuals carried a diagnosis of Gardner's syndrome and neither of them had diabetes. Although the boy has as of now only presented with cutaneous manifestations of Gardner's syndrome, his grandfather has exhibited both cutaneous and intestinal evidence of this syndrome. In addition, the boy's mother and her sister have documented Gardner's syndrome. Light microscopic findings of nuchal-type fibroma from both patients include paucicellular, haphazardly arranged collagen bundles with entrapped adipose tissue. A marked diminution of elastic fibers was noted with Van-Gieson stains. The lesions were diffusely positive for CD34 and contained a few factor XIIIa-positive cells. Electron microscopic analysis revealed no differences between the collagen comprising the nuchal-type fibroma as compared with control dermal collagen obtained from skin away from the tumor. These cases strengthen the view that there is an association between nuchal-type fibroma and Gardner's syndrome.

Adolescent↗