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Ovarian fibromas in prepubertal girls are rare. We describe two girls aged 8 and 11 years with extensively calcified ovarian fibromas. One patient had a single unilateral fibroma with metaplastic bone formation in the calcified area. The other patient had bilateral nodular fibromas suggesting the possibility of naevoid basal-cell carcinoma syndrome although broader manifestations are lacking at present.
It has been reported that ovarian fibromas display low signal intensity on both T1- and T2-weighted magnetic resonance images. We report an ovarian fibroma exhibiting low signal intensity on a T1-weighted image and high signal intensity on a T2-weighted image. Microscopically pronounced myxomatous changes were shown in the fibroma. The signal intensity of ovarian fibromas differs with the degree of myxomatous change.
This case report deals with the case history of a 45-year-old male patient who presented with an unusual case of a central odontogenic fibroma which destroyed and replaced the bone mass of the right maxilla. After clinical examination a biopsy was performed and histopathology was consistent with a central odontogenic fibroma with destructive growth. The treatment plan consisted of a surgical resection of the right maxilla. The histopathological evaluation showed a subtotal destruction of the maxillary bone mass by the odontogenic fibroma. Obvious problems concerning the differential diagnosis of odontogenic fibromas as well as current concepts regarding their exact designation and classification are discussed.
Ovarian fibroma rarely occurs in prepubertal girls. We report an ovarian fibroma in a 7-month-old female infant presenting as a right abdominal mass. The tumour was composed of oval-to-spindle cells forming interlacing fascicles and intermingling with collagen fibres. Immunohistochemically, the tumour cells were weakly positive for estrogen receptor and progesterone receptor but negative for inhibin. To the best of our knowledge, this is the youngest case of ovarian fibroma up-to-date in the English literature. Because Gorlin's syndrome was not present in the patient's family, other factors such as germline or somatic mutations may predispose the occurrence of this ovarian fibroma in infancy.
There have been only a few cases of desmoplastic fibroma of the spine in the literature and only one of them was purely located on the cervical spine. We report a new patient with the diagnosis of desmoplastic fibroma of the fourth cervical spine. The patient had the complaints of left arm and neck pain. After his radiological evaluation, a mass lesion was found on the left lamina of the fourth cervical spine. Surgical treatment was performed, and the histopathological examination revealed the diagnosis of desmoplastic fibroma. Patients with desmoplastic fibroma of the cervical spine may present with the arm and neck pain mimicking cervical disc disease. Higher index of suspicion by the clinicians must be practiced to make the appropriate diagnosis. Successful surgical outcome may be achieved in these patients.
We have identified 14 cases that over the last 40 years have been reported under a series of names, most commonly granular cell ameloblastic fibroma. An additional case in the mandibular premolar region of a 45-year-old woman is described. The tumor was conservatively removed and 4 years later shows no evidence of recurrence. On the basis of our examination of the clinical and histologic features of this lesion and a comparison with the previous cases, we agree with the recent suggestion that the tumor should be designated as a central odontogenic fibroma, granular cell variant. By means of S-100 protein immunostaining techniques, the granular cells in this lesion were compared with the granular cell population in a granular cell tumor (myoblastoma) and the mesenchymal component of an ameloblastic fibroma. The results reveal a lack of S-100 protein reactivity in the granular cells of the central odontogenic fibroma and suggest an origin of those cells different from the origin of cells in a granular cell tumor.
A 9-year-old boy who had had painless progressive proptosis of the right eye for 18 months, initially diagnosed as fibrous dysplasia, was found to have a circumscribed mass involving the frontal bone in the roof of the orbit. Biopsy of the mass led to the diagnosis of juvenile ossifying fibroma and the tumor was removed by a craniotomy approach. One year after surgery, the child appeared to be healthy. Ossifying fibroma can sometimes be differentiated from fibrous dysplasia both clinically and by computed tomography. Ossifying fibroma usually appears between the ages of 7 and 28 years and produces slowly progressive proptosis and displacement of the globe in a direction that depends on the original site of the tumor. Fibrous dysplasia usually develops during the first decade of life and produces facial asymmetry, proptosis, and displacement of the globe. An ossifying fibroma characteristically has well-defined margins, is round or ovoid, is usually monostotic, and produces expansion of the involved bone. Fibrous dysplasia is usually sclerotic and expands the bone throughout its length rather than in a localized fashion. Its borders are poorly defined and the lesion is often polyostotic.
BACKGROUND: Cardiac fibromas are rare tumors. Indications for and preferred type of operation are controversial, and little is known about early and late results of operation. METHODS: We retrospectively reviewed records of 18 patients who underwent resection of ventricular cardiac fibromas from 1964 to October 2002. Follow-up was obtained from current medical records and recent telephone and written correspondence. RESULTS: Seventeen patients had complete resection and 1 had subtotal resection of one or more fibromas. Tumors were located in the left ventricle (n = 12), septum (n = 4), or right ventricle (n = 2). A 2-month-old infant died intraoperatively. None of the surviving 17 patients had complete heart block. Follow-up lasted up to 33.7 years for a total of 172.1 patient-years. There were no late deaths. Fourteen patients were asymptomatic, 1 was in New York Heart Association functional class II, and 2 were in class III. There was no recurrence of tumor after complete resection and no change in size of residual tumor in the 1 patient who underwent subtotal resection. CONCLUSIONS: Although cardiac transplantation has been suggested by some as a preferable operation, most ventricular fibromas, even though extensive, can be completely resected with excellent early and late results. For patients with tumors extending into critical locations, subtotal excision can also give excellent late survival.
Collagenous fibroma (desmoplastic fibroblastoma) is a rare, benign tumor composed of spindle and stellate-shaped fibroblasts and myofibroblasts in a densely collagenous background. A t(2;11)(q31;q12) has been reported in one case of collagenous fibroma and a rearrangement of the 11q12 breakpoint in a second case. In the present study, we detected a t(2;11) identical to that previously described in a collagenous fibroma arising in the supraclavicular fossa of a 55-year-old man. This finding confirms the nonrandom association of t(2;11)(q31;q12) with collagenous fibroma.
The cemento-ossifying fibroma is probably the most frequent fibro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggressive fashion appear in the literature. At the present time, the relationship if these "aggressive" lesions to the "usual" fibromas is unclear. Two cases of "aggressive" cemento-ossifying fibroma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual fibromas do coincide. There are, however, diverging features warranting separation of different forms. These mild differences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made.
Desmoplastic fibroma of the long bones is very rare and has been mentioned in the literature, but there is no mention in the literature of desmoplastic fibroma occurring in relation to maxilla. The first case of desmoplastic fibroma of the maxilla is reported. Conservative surgery is recommended for desmoplastic fibroma of the maxilla to avoid facial deformity.
AIMS: To determine whether fibromas of tendon sheath represent a reactive fibrosing process or whether they are neoplastic, we investigated the chromosomes of the proliferating cells. A clonally occurring karyotypic abnormality would be an argument in favour of these fibromas being neoplastic. METHODS AND RESULTS: A biopsy specimen of a fibroma of tendon sheath was cultured after collagenase exposure leading to dissociation of the tissue. The cultured cells were harvested after 4 days and chromosome analysis done according to standard procedures. Ten out of 20 karyotyped cells showed an identical chromosome abnormality characterized by a t(2;11)(q31-32;q12). The other 10 cells showed a normal 46,XX karyotype. CONCLUSION: The clonally occurring chromosome abnormality found in this fibroma of tendon sheath suggests that this proliferation is neoplastic and not a reactive fibrosing process.
Recent cytogenetical studies have indicated that trisomy 12 is a feature of ovarian tumors in the thecoma-fibroma group. Ten cases of these ovarian tumors were studied in total, including two thecomas, two fibrothecomas, four fibromas, one cellular fibroma and one fibrosarcoma, to clarify the relationship between polysomy 12 and proliferative activity in these tumors. Each formalin-fixed, paraffin-embedded tumor tissue was examined by fluorescence in situ hybridization to determine copy numbers of chromosome 12 and by immunohistochemical staining of Ki-67 for evaluation of tumor cell proliferation. Gains of trisomy 12 were found in seven of the 10 cases, and the percentage of cells with tetrasomy 12, but not that of cells with trisomy 12, was significantly and positively correlated with percentage of Ki-67-positive cells, but significantly and inversely correlated with patient age. These findings suggest that tetrasomy 12 is an age-related aberration of chromosome 12 in ovarian tumors of the thecoma-fibroma group, and that such tumors exhibit more active proliferation in younger patients.
Collagenous fibroma (desmoplastic fibroblastoma) is a distinctive benign fibrous soft tissue tumor that typically occurs in the subcutaneous tissue or skeletal muscle in adults. We describe a case of collagenous fibroma in a 7-year-old boy who presented with a 1-cm solitary, firm nodule on the volar aspect of the metacarpophalangeal joint of the left little finger. Microscopically, the tumor was composed of spindle- and stellate-shaped cells embedded in a hypovascular, densely collagenous stroma. No mitotic figures, calcifications or necrosis were identified. Immunohistochemically, tumor cells were diffusely positive for vimentin, but negative for smooth muscle actin, muscle-specific actin, desmin, cytokeratin, S-100 protein or CD34. To our knowledge, this is the second reported case of collagenous fibroma in children. Our case report indicates that the clinicopathological features of collagenous fibroma in childhood are similar to those in adults.
A male child presented with suspicion of tricuspid stenosis. Echocardiography revealed the presence of a mass arising from the free wall of the right ventricle and causing right-ventricular inflow obstruction but not involving the tricuspid valve. The tumour was excised via right ventriculotomy using cardiopulmonary bypass, resulting in survival and a satisfactory clinical course. Histological examination showed the tumour to be a fibroma. Tricuspid stenosis is an unusual presentation of right ventricular fibroma. Careful examination is essential to early diagnosis of cardiac fibromas, particularly in children with unexplained cardiac problems. Echocardiography is still a good method for confirmation of the diagnosis and for follow-up. In view of the unknown natural history of cardiac fibroma, surgical excision should be undertaken. Early detection and prompt excision results in immediate and complete relief of symptoms and prolongs the life span.
Three patients with a combination of neurofibromatosis and multiple non-ossifying fibromas are presented. All patients possessed multiple osteolytic lesions with sclerotic margins, resembling non-ossifying fibromas. Two patients showed similar lesions in additional localizations. In two patients, non-ossifying fibroma was histologically verified. The simultaneous occurrence of neurofibromatosis and multiple non-ossifying fibromas can possibly be put down to a generalized mesodermal dysplasia. However, the definite explanation is unknown.
Two cases of ossifying fibroma of long bones are presented. This tumor is confused with monostotic fibrous dysplasia, but can be distinguished by its intracortical location, as demonstrated radiographically, and by its histologic pattern. Distinction from fibrous dysplasia is important since ossifying fibroma of long bone is a more aggressive lesion with different therapeutic implications. It appears that ossifying fibroma and adamantinoma of long bones are somehow related, and that lesions resembling fibrous dysplasia in association with adamantinomas of long bones are actually ossifying fibromas.
The case of recurrent bilateral ovarian fibromas occurring in a 22 year old Italian girl affected by Gorlin syndrome is reported. Ovarian fibromas occur in 75% of female patients with Gorlin syndrome and their recurrence has rarely been reported in the literature. Management is guided by the benign nature of the lesion and consists of surgical removal of the fibroma. Preservation of the normal ovarian tissue is recommended even though there is risk of recurrence of the fibroma.