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A first case of non-ossifying fibroma located in the mandibular condyle of a 20-year-old male is presented. Radiologic and histologic findings correspond in all details to those of non-ossifying fibromas of long tubular bones. The lesion's location within the condylar growth zone may strengthen the hypothesis that non-ossifying fibromas are disturbances in growth rather than true neoplasms.
The syndrome of perifollicular fibromas and colonic polyps was delineated 20 years ago by Hornstein and Knickenberg; it probably occurs more frequently than suggested by the literature. Multiple perifollicular fibromas were found in a mother and daughter. The mother also had colonic polyps. This dermo-intestinal syndrome varies in its clinical manifestations, but it is probably an autosomal dominant trait. We believe that the Horn-stein-Knickenberg syndrome and the Birt-Hogg-Dubé syndrome are identical. If perifollicular fibromas are observed and cannot be explained as postinflammatory sequelae of acne, the patient should be examined for colonic polyps as an appropriate from of cancer screening.
The periungual fibromas in tuberous sclerosis are also known as Koenen tumors. Joannes Henricus Maria Koenen was born on March 10, 1893, in Eindhoven and died in Bois-le-Duc May 29, 1956. From 1910 to 1918 he studied medicine at the University of Amsterdam. Then he worked as a physician at the "Coude-water" asylum in Rosmalen and from 1929 in the "Voorburg" asylum in Vught. In 1932 he published his publication concerning a family with tuberous sclerosis in which photographs of periungual fibromas are shown. His thesis in 1933 at the University in Leiden was titled "Imbecility in children. Its Importance in pedagogic and social regard, by reason of an investigation in some communities in Noord-Brabant". Later he was appointed director of a state mental institution. The periungual fibromas in tuberous sclerosis were first described by Richad Kothe in Munich in 1903.
A 12-year-old-girl presented with a fracture of an osteolytic lesion of the distal radius. A 7-year-old girl presented with a fracture of an osteolytic lesion of the femoral shaft. In both cases it was a non-ossifying fibroma with fracture misdiagnosed at pathology as aneurysmal bone cyst. Fractures through non-ossifying fibromas may alter the histological pattern of the initial lesion in two ways: firstly, by the presence of blood pigments due to the fracture, and secondly, by formation of new bone. Radiological-pathological correlation is essential to avoid histological errors after pathological fracture in a non-ossifying fibroma.
BACKGROUND: Fibro-osseous lesions of the jaws, including juvenile ossifying fibroma, pose diagnostic and therapeutic difficulties due to their clinical, radiological and histological variability. CASE REPORT: The case of an 8-year-old girl with a juvenile ossifying fibroma of the mandible is presented. Extirpation of the tumor and filling of the defect with cancellous bone was performed. After 9 months, a recurrence occurred which made a second extirpation of the tumor necessary. This was done without osteoplasty. After a 2-year follow-up the patient is free of tumor. DISCUSSION: Different classifications for fibro-osseous lesions of the jaws can be found in the literature. This makes a correct diagnosis and standardized treatment difficult. Based on the case presented here, the histological features of juvenile ossifying fibroma and the differentiation from osteosarcoma are discussed. At present, conservative surgical treatment seems to be adequate for these tumors.
Cardiac transplantation was performed in two infants with unresectable fibromas of the myocardium. In one patient, lip surgery was also required for unilateral cleft lip and palate. At autopsy, communicating hydrocephalus of mild to moderate degree was found in both cases. In the patient with facial clefts, there was also a large, ipsilateral cyst, or rhinocele, of the olfactory lobe. This unusual lesion, which seems to represent a previously unreported malformation, was apparently formed by segmental dilatation of a persistent olfactory ventricle. Cerebral or cranial anomalies are thought to be rare in cases of cardiac fibroma; however, macrocephaly was present in five patients. Furthermore, presenting abnormalities among previously reported cases included hydrocephalus in one case, and cleft lip and palate in another. These and other findings suggest that, at least in some cases, cardiac fibroma is a manifestation of a more extensive developmental disorder.
The biopsy service at Indiana University School of Dentistry, Department of Oral Pathology, accessioned 464 cases of giant cell fibroma from February, 1971, until May, 1980. From a study of these cases, it appears that the giant cell fibroma is a distinctive lesion with a characteristic age distribution, location, sex incidence, and histologic appearance. Clinically, the giant cell fibroma presents as an asymptomatic, papillary, pedunculated lesion, commonly regarded as a papilloma. It is fairly common in young persons and usually occurs on the gingiva. No sex predilection has been noted, and simple surgical excision appears to be the treatment of choice.
The DNA sequence has been determined for a 7-kb region within the terminal inverted repeats (TIR) of Shope fibroma virus (SFV), a poxvirus which induces benign fibromas in rabbits. This region of the SFV TIR, which flanks the junction of the TIR with the unique internal sequences of the viral genome, had previously been shown to be also present in the genome of malignant rabbit virus (MRV), a hybrid poxvirus derived from a recombination event between SFV and a related leporipoxvirus, myxoma. Unlike SFV, the recombinant MRV induces an invasive profile of tumors in infected rabbits, but the capacity to induce proliferant fibromas appears to have been derived from SFV. These SFV DNA sequences have been analyzed and their genetic organization shows a unique tandem arrangement of three large open reading frames (ORFs) which share considerable homology with each other. Very short spacer sequences are present between the majority of ORFs, all of which are transcribed toward the terminal hairpins of SFV. Unusual dyad symmetries flank two of the most closely related ORFs and evidence is presented that one SFV ORF (T9-L) which maps precisely at the TIR/unique sequence boundary was truncated during transposition to the left terminus from a progenitor copy (T9-R) at the right terminus. The origin of these putative viral genes is considered in light of the recent observation (C. Upton and G. McFadden, 1986, Mol. Cell. Biol. 6, 265-276) that a subset of this region of the SFV genome is closely related to, and may have been originally derived from, an endogenous covalently closed circular plasmid species detected in uninfected rabbit cells.
The role of the epidermal growth factor homologue in malignant rabbit fibroma virus (MRV) pathogenicity was investigated by constructing a viral growth factor deletion mutant (MRV-GF-). Since MRV is a recombinant virus with a myxoma virus background but possesses some terminal sequences derived from Shope fibroma virus, the growth factor gene in MRV is in fact identical to Shope fibroma growth factor (SFGF). Although no significant differences were detected in the in vitro characteristics of MRV and MRV-GF-, a pronounced attenuation was observed after inoculation of the test rabbits with MRV-GF-. Animals infected with wild-type MRV uniformly developed a fatal syndrome involving disseminated tumors accompanied by purulent conjunctivitis and rhinitis. In contrast, although MRV-GF- recipients developed similar initial signs of the MRV disease syndrome, 75% of these animals completely recovered from the viral and secondary bacterial infections and became immune to subsequent MRV challenge. Tumors in MRV-GF- recipients displayed earlier and more prominent inflammatory reactions than their wild-type MRV counterparts and contained fewer proliferating cells. Squamous metaplasia and hyperplasia of target epithelia were less pronounced in MRV-GF- than in MRV infection. We conclude that SFGF is a major virulence factor in MRV infection and is responsible for at least some of the cellular proliferation observed at tumor sites. In addition, the diminished ability of MRV-GF- to cause hyperplasia in nasal and conjunctival epithelia may decrease the extent of gram negative bacterial overgrowth as compared to the parental virus and hence contribute to the dramatic reduction in the lethality of MRV-GF- infection.
Cardiac fibromas are rare lesions which occur more frequently in infants and children than in the adult population. These tumors are nonmalignant proliferations of connective tissue most often found in the left ventricular myocardium or septal myocardium. No cytogenetic studies of cardiac fibromas have been reported. We report a case of an infant with a subepicardial tumor in whom the cytogenetic analysis showed a clonal reciprocal translocation, 46,XY,t(1;9)(q32;q22),inv(9)(p11q12)c. We review the literature regarding cardiac fibromas and briefly discuss the cytogenetics of benign fibrous neoplasias.
Desmoplastic fibroma is a rare, benign, fibroblastic tumour arising intra-osseously. A case in a 13 year-old male is described. A tumour, situated between the roots of the right mandibular canine and premolar, was excised in 1984. Morphologically, the characteristics of the tumour conformed well to those of a desmoplastic fibroma. The differential diagnosis of desmoplastic fibroma with reference to the histopathological and ultrastructural appearance of the tumour is discussed.
Six cases of the complex (World Health Organization) type of odontogenic fibroma are described. The relationships of these lesions to the simple type of odontogenic fibroma, desmoplastic fibroma, and to certain reactive lesions are discussed.
A case of desmoplastic fibroma of the right maxilla is reported. The lesion presented as a painless mass in the right posterior alveolar ridge of a 22-year-old white man. Histologically, the lesion was composed of interlacing fascicles of benign-appearing fibroblasts in a varying ground substance of collagenous and myxoid tissue. The lesion was not encapsulated histologically and was invading between bone trabeculae resulting in resorption of the bone. A review of the literature reveals that a total of 30 cases of desmoplastic fibroma of the jaws have been reported. All of the mandibular lesions except two were reported to have occurred in the left side, with the molar-ramus region favored. The case reported here is, to the best of our knowledge, the first reported case of desmoplastic fibroma occurring in the maxillary alveolar process.
The relationship of virus-induced immunological dysfunction and tumor dissemination was studied using two related tumor-causing leporipoxviruses: malignant fibroma virus (MV) and Shope fibroma virus (SFV). Recombinant viruses, produced by transferring MV's 10.7 kb BamHI C fragment to SFV, replicate in lymphocytes and suppress lymphocyte function in vitro. Those recombinants that replicate in lymphocytes and suppress lymphocyte function in vitro share about 3.5 kb from MV's C fragment. Some recombinants mimic MV in producing immune suppression and disseminated virus infection in vivo. Other recombinants, even some that are highly immunosuppressive in vitro (e.g. R71), only variably induce immune suppression in vivo, and do not cause disseminated disease. A segment of DNA from MV that transfers to Shope fibroma virus almost all of MV's virulence in vivo was identified.
We report the case of a 44-year-old man who presented with a chondromyxoid fibroma (CMF) of the sphenoid sinus, which filled the sinus and clinically and radiologically resembled a mucocele. Chondromyxoid fibromas are the least common cartilaginous neoplasms of bone, typically occurring in the metaphysis of long bones. They have occasionally been described in the facial bones, usually the mandible and maxilla, and rarely involve the paranasal sinuses. Chondromyxoid fibroma displays a relatively unique histological appearance, with characteristic lobulation, with spindle to stellate cells embedded in a myxoid or chondroid matrix. The periphery of the lobules tends to be more cellular than the center. Despite having been described nearly 60 years ago, the histogenesis of CMF remains controversial. Although CMFs are generally regarded as benign neoplasms, they may show an infiltrative pattern and may recur, particularly when they are in locations where complete surgical excision may be difficult or impossible.
Desmoplastic fibroma of bone is a rare tumor demonstrating the same histologic and biologic features of its soft tissue counterpart, aggressive fibromatosis. We report the second case of desmoplastic fibroma of bone with extensive chondroid metaplasia. The tumor arose in the left ischium of a 51-year-old male, with extension into adjacent musculature as a pseudoencapsulated mass. The infiltrating growth and quality of the fibrous component are characteristic of desmoplastic fibroma, and in addition, abrupt transitions into bland hyalin cartilage were frequent. Discriminating features of this lesion from other bone tumors capable of biphasic expression of fibrous and chondroid elements are discussed.
Odontogenic tumors composed of 2 distinct types of lesions are unusual. We report an odontogenic tumor that was composed of calcifying odontogenic cyst and ameloblastic fibroma that occurred in the right posterior maxilla of a 22-year-old Korean woman. The tumor had a cystic component with an ameloblastic epithelial lining and conglomerates of so-called ghost cells, and there were deposits of dentinoid material adjacent to the cyst. These are features characteristic of calcifying odontogenic cyst. Enamel organ-like epithelial islands were observed within a dental papilla-like stroma of the cyst wall. Additionally, a solid portion of the tumor had characteristic features of ameloblastic fibroma, i.e., a myxoid cellular stroma with numerous elongated islands of ameloblastic epithelium. Ghost cell masses were found in the area of ameloblastic fibroma as well. The distribution of the ghost cells suggests that this is a hybrid lesion rather than a collision tumor.
PURPOSE: To report a case of an isolated tarsal fibroma. METHODS: Case report. Excisional biopsy and histopathological evaluation were performed on a solid lesion originating from the tarsal conjunctival surface of an upper eyelid. RESULTS: Histopathological evaluation, including positive trichrome stains, was consistent with fibroma of the left upper tarsus. No recurrence has developed after a follow-up interval of a year. CONCLUSION: Tarsal fibroma is a rare condition that should be considered in the differential diagnosis of tarsal lesions.