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Cemento-ossifying fibroma of the ethmoidal sinus manifesting as proptosis.

We report an aggressive cemento-ossifying fibroma of the ethmoidal sinus that initially manifested as proptosis and was misdiagnosed as meningioma after a frozen section was tested. Fibroosseous lesions containing cementum are considered to be of periodontal membrane origin and are most common in the mandible and maxilla. With the exception of the gigantiform cementoma, they are usually small, innocuous lesions following a benign course and are easily removed surgically. On rare occasions, the cemento-ossifying fibroma attains a large size, behaves aggressively, destroys bone, and requires radical surgery for complete excision. Such behavior appears likely when is is located in unusual sites such as the ethmoidal sinus. To our knowledge, our case is the third report of a cemento-ossifying fibroma arising in the ethmoidal sinus. Aggressive behavior has been a feature in all three instances.

Ethmoid Sinus↗

[Hysteroscopic treatment of uterine fibromas].

OBJECTIVES: To study retrospectively the long-term outcome after hysteroscopic treatment for intrauterine fibromas. METHODS: From October 1987 to January 1993, endouterine glycolol resection was performed in 239 patients with intrauterine fibromas. The operation had to be repeated in 39 patients due to incomplete resection or recurrent symptomatology. Patients presented with menometrorrhage, post-menopausal metrorrhage or infertility. RESULTS: Intra-operative complications were rare, mainly uterine perforations (7 cases) or metabolic syndromes due to reabsorption of glycine (5 cases). Mean follow-up was 2.5 years. The couple was sterile in 16 cases and 4 pregnancies were obtained (25%). Postmenopausal metrorrhage was present in 27 patients and the symptomatology disappeared in 24 (89%). In all, 196 women consulted for bleeding, good results were achieved in 81.1%. CONCLUSION: Endouterine resection is a sure, effective and long-lasting treatment for intrauterine fibromas.

Adult↗

[Role of endo-uterine resection using hysteroscopy in the treatment of sub-mucous hemorrhagic fibroma in the peri-menopausal period].

OBJECTIVES: To study retrospectively the long-term outcome after hysteroscopic treatment for intra-uterine fibromas. METHODS: From October 1987 to January 1993, endo-uterine glycocol resection was performed in 196 patients with intra-uterine fibromas. The operation had to be repeated in 39 patients due to incomplete resection or recurrent symptomatology. Patients presented with menometrorrhage. RESULTS: Intra-operative complications were rare, mainly uterine perforations (7 cases) or metabolic syndromes due to reabsorption of glycine (5 cases). Mean follow-up was 2.5 years. In all 196 women consulted for bleeding, good results were achieved in 81.1%. CONCLUSION: Endo-uterine resection is a sure, effective and long-lasting treatment for intra-uterine fibromas.

Adult↗

Malignant rabbit fibroma virus: observations on the culture and histopathologic characteristics of a new virus-induced rabbit tumor.

The clinical, histopathologic, and cultural characteristics of a newly isolated poxvirus, malignant rabbit fibroma virus (MV), were investigated. MV was isolated from tumors induced by an uncloned stock of Shope fibroma virus (SFV). MV, SFV, and rabbit myxoma virus were compared. Similarly to myxoma virus, MV grew to higher titer in vitro than did SFV and produced plaques rather than foci on rabbit kidney cell monolayers. Unlike the local, self-limited fibroblastic proliferations observed in SFV recipients, MV and myxoma caused a fulminant clinical syndrome characterized by malignant histology, metastases, and supervening fatal gram-negative infection with Pasteurella multocida. MV induced a large, protuberant local tumor and discrete metastases histologically resembling myxosarcomas. Draining lymph nodes contained metastases and showed diffuse cortical hyperplasia. Kupffer's cells were prominent in the liver, and macrophages were abundant in the splenic sinusoids. The lungs and trachea were spared, but the conjunctiva and nasal mucosa showed squamous metaplasia and atypia, with overlying Pasteurella infection and underlying tumor. Myxoma virus infection produced similar mucosal changes, but both of these as well as the epidermis overlying the myxomas showed cytoplasmic virus inclusions. Neither the skin nor the epithelial surfaces overlying MV-induced tumors nor the tumors themselves contained virus inclusions. Thus the tumor syndrome caused by MV differed from other known rabbit tumors. Endonuclease restriction digests showed that the MV genome resembled, but was distinct from, rabbit myxoma virus. Opportunistic infection associated with MV-induced disseminated tumor may be an experimental model for the infectious complications that often supervene in host-tumor relationships.

Animals↗

Calcified right ventricular fibroma causing outflow obstruction. Report of a case with successful excision.

A 5-year-old boy with clinical findings of pulmonic stenosis was found to have a large calcified mass in the right ventricular outflow region and a gradient of 120 mm Hg between the right ventricle and the pulmonary artery. At surgery, an ovalshaped tumor attached to the interventricular septum and obstructing the right ventricular outflow tract was removed. The child survived and is doing well. Histologically, the tumor had the characteristics of fibroma. A hemodynamic study three months after surgery showed almost complete abolishment of the gradient. To our knowledge this is the fifth reported case of calcified right ventricular fibroma with successful operation. In childhood intracardiac calcifications, together with obstruction, are highly suggestive of this lesion.

Angiocardiography↗

Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. VI. Fibrous tissue tumors (fibroma, fibromatosis, fibrosarcoma).

In a study of 256 nonepithelial neoplasms involving the nasal cavity, paranasal sinuses, and nasopharynx, 23 lesions were classified as fibrous tissue tumors, including four cases of "fibroma", six of fibromatosis, and thirteen of fibrosarcoma. The clinical findings associated with these lesions are described, their histologic features illustrated, results of therapy presented and clinicopathologic correlations made. The "fibromas" presented a small localized nodules. None recurred after local excision. Fibromatosis, a locally aggressive tumor, does not metastasize, but may cause considerable morbidity or even death due to local infiltration which may be difficult to control surgically. Fibrosarcoma may cause death either by local infiltration or by metastasis, but has a better prognosis than most other sarcomas of this region. We recommend that a large en block resection be performed initially for fibromatosis and fibrosarcoma growing in this area, after the diagnosis has been made by biopsy. In this series, including patients who had more than one operation, recurrent tumor was seen following 10 of 12 limited local excisions performed for fibromatosis and fibrosarcoma, but in only one of 13 patients after a large bloc resection. The problems involved in histologically differentiating fibrous tissue tumors from other lesions are discussed. A patient with the rare syndrome of multicentric fibromatosis with spontaneous regression of lesions is presented.

Adult↗

Fibroelastic hamartoma (fibroma) of the heart.

Approximately 70 cardiac fibromas (fibroelastic hamartomas) have been reported in the literature and at least 15 have been successfully excised. However there is no well-documented ultrastructural study of these lesions. A successfully excised cardiac fibroma (FEH) was studied by light and elctron microscopy. The tumor was composed mainly of fibroblasts admixed with bundles of collagen and elastic fibers. No muscle fibers were demonstrated in the central region of the tumor either by light or electron microscopy. Mast cells were occasionally present. The lack of encapsulation enabled fibroblasts with collagen and elastic fibers to extend between groups of myocardial fibers at the periphery of the tumor. Within these myocardial fibers ultrastructural changes were limited to the mitochondria and myofibrillar structure.

Child, Preschool↗

Long bone ossifying fibromas.

Ossifying fibromas involving the tibia were seen in two patients. In both patients, the radiographic appearance of the lesions suggested fibrous dysplasia, but histopathologic evaluation demonstrated findings similar to ossifying fibroma of the mandible and facial bones. Radiologic and pathologic recognition of this entity is necessary for proper treatment.

Adolescent↗

Juvenile ossifying fibroma: an ultrastructural study.

The ultrastructural features of a juvenile ossifying fibroma of the maxilla are described. The stromal portion of the tumor was composed of osteoblasts and to a lesser extent of fibroblasts. The bone spicules were rimmed by osteoblasts and osteoclasts. Calcification was seen to occur along the collagen fiber matrix, corresponding to calcification of osteoid, and also in the form of intracellular and extracellular crystallization. The latter form of calcification corresponded to so-called psammoma-like bodies, and was considered characteristic of this subtype of ossifying fibroma.

Bone Neoplasms↗

Juvenile fibromatosis resembling aponeurotic fibroma and congenital multiple fibromatosis. One case with pleuropulmonary involvement.

This article deals with a young school boy born in 1971 with a tumor in the palm of his right hand. We have considered this a juvenile aponeurotic fibroma. This tumor has recurred several times, leading to unavoidable amputation of the right wrist and hand. Seven years after the initial diagnosis, the axillary area became involved, then the pleura and lung. Such an unusual process leads to reconsidering the diagnosis and to regard this case as an intermediate form between Keasbey's juvenile aponeurotic fibroma and generalized fibromatosis.

Child↗

Desmoplastic fibroma of bone.

Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This study reviews 27 cases of desmoplastic fibroma, consisting of 9 from the Mayo Clinic files and 18 from our consultation files. There was a male predominance, and 74% of the patients were in the first 3 decades of life. The most frequent sites of involvement were the metaphysis of long bones and the mandible. Radiographically, the tumors were lucent, expansile lesions with well-defined margins. Histologically, they contained slender spindle cells and various amounts of collagen fibers. En bloc resection is the treatment of choice because a high incidence of recurrence was noticed after lesional curettage.

Adolescent↗

Aggressive psammomatoid ossifying fibromas of the sinonasal region: a clinicopathologic study of a distinct group of fibro-osseous lesions.

BACKGROUND: Psammomatoid ossifying fibromas represent a unique subset of fibro-osseous lesions of the sinonasal tract. They have distinctive histomorphologic features and a tendency toward locally aggressive behavior, including invasion and destruction of adjacent anatomic structures. METHODS: Seven cases of psammomatoid ossifying fibromas of the sinonasal tract were identified in the files of the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology. Medical records, including the clinical history, location of the lesions, radiographs, treatment, and follow-up were reviewed in each case. Follow-up information was available in all of the cases. RESULTS: Four of the patients were male and three were female. The patient's ages ranged from 5 to 54 years (median age, 33 years). Symptoms included facial swelling, nasal obstruction, pain, sinusitis, headache, and proptosis. Radiographic studies confirmed the presence of an osseous and/or soft tissue mass varying in appearance from well demarcated without invasion or erosion to invasive with bone erosion and intracranial extension. Sites of involvement included the nasal cavity and all paranasal sinuses, particularly the ethmoid and maxillary sinuses. Often, more than one sinus was involved and extension of disease included involvement of the orbit, nasopharynx, palate, and anterior cranial fossa. The histologic appearance was characterized by the presence of small mineralized (psammomatoid) bodies admixed with a cellular stroma with a variable amount of myxomatous material and scattered giant cells. Confusion with other osseous and soft tissue tumors may occur resulting in too limited or too aggressive management. En bloc surgical excision is the treatment of choice and may prove curative. Aggressive behavior with recurrence(s) or invasion into adjacent structures occurred. At the time of this writing, the patients are alive over follow-up periods ranging from 6 months to 7 years. CONCLUSIONS: Gnathic and midfacial fibro-osseous proliferations are a diverse group of lesions. A subset of these fibro-osseous lesions with predilection for the sinonasal tract were identified. These lesions are characterized by their distinctive histology, including psammomatoid ossicles and their locally aggressive growth. Complete surgical removal is the treatment of choice.

Adult↗

Nonrandom chromosome breakpoints at Xq26 and 2q33 characterize cemento-ossifying fibromas of the orbit.

BACKGROUND: Cytogenetic reports of histologically benign fibroosseous lesions are rare, with only nine previously reported cases. None of these previous studies revealed consistent numerical or structural chromosome aberrations, and to the authors' knowledge, no karyotypic abnormalities in cemento-ossifying fibromas of the orbit have been reported. METHODS: Short term in situ culture and Giesma-band chromosome methods were used to analyze three cementifying fibromas of the orbit: one from a 13-year-old African American male, one from a 14-year-old Hispanic male, and one from a 17-year-old white male. RESULTS: Cytogenetic findings in these three cases revealed the presence of identical chromosomal breakpoints occurring in all three tumors at bands Xq26 and 2q33. Two of the tumors showed an identical t(X;2)(q26;q33) reciprocal translocation as the sole abnormality. The third tumor revealed an interstitial insertion of bands 2q24.2q33 into Xq26 as the sole abnormality. CONCLUSIONS: The authors described new nonrandom breakpoints in fibroosseous lesions of the orbit, which can result from at least two different types of structural chromosomal aberrations. The identification of recurring breakpoints at Xq26 and 2q33 provides a new cytogenetic tumor marker for the identification of this tumor subtype. The sublocalization of breakpoints in this tumor should provide important information for the precise localization and characterization of genes involved in the histiogenesis of these lesions.

Adolescent↗

Cytogenetic abnormalities in an ossifying fibroma from a patient with bilateral retinoblastoma.

Cytogenetic analysis of a cemento-ossifying fibroma from a patient with nonfamilial bilateral multicentric retinoblastoma revealed three reciprocal translocations with the karyotype 46,XY,t(1;18)(q21;q21.3),t(3;10)(p13;q22),t(6;11)(p22;p15). Routine and high-resolution cytogenetic analysis of peripheral blood leukocytes showed an apparently normal, 46,XY chromosome pattern with no deletion of chromosome 13. Molecular analysis demonstrated no gross differences in the retinoblastoma gene or the TP53 gene between constitutional and tumor DNA. This is the first cytogenetic analysis of a cemento-ossifying fibroma and the first report of this tumor in a retinoblastoma patient. The data may be added to the small, but growing literature on cytogenetic aberrations in benign tumors and may lend insight into genes involved in cell proliferation and neoplastic transformation.

Adolescent↗

Transforming growth factor alpha, Shope fibroma growth factor, and vaccinia growth factor can replace myxoma growth factor in the induction of myxomatosis in rabbits.

The epidermal growth factor (EGF) homologues encoded by vaccinia virus, myxoma virus, and malignant rabbit fibroma virus have been shown to contribute to the pathogenicity of virus infection upon inoculation of susceptible hosts. However, since the primary structures of these growth factors and the disease profiles induced by different poxvirus genera vary substantially, the degree to which the various EGF homologues perform similar roles in viral pathogenesis remains unclear. In order to determine whether different EGF-like growth factors can perform qualitatively similar functions in the induction of myxomatosis in rabbits, we created recombinant myxoma virus variants in which the native growth factor, myxoma growth factor (MGF), was disrupted and replaced with either vaccinia virus growth factor, Shope fibroma growth factor, or rat transforming growth factor alpha. Unlike the control virus containing an inactivated MGF gene, which caused marked attenuation of the disease syndrome and substantially less proliferation of the epithelial cell layers in the conjunctiva and respiratory tract, the recombinant myxoma virus strains expressing heterologous growth factors produced infections which were both clinically and histopathologically indistinguishable from wild-type myxomatosis. We conclude that these poxviral and cellular EGF-like growth factors, which are diverse with respect to primary structure and origin, have similar biological functions in the context of myxoma virus pathogenesis and are mitogenic for the same target cells.

Animals↗

Case report 695: Cementifying fibroma of the proximal end of the tibia.

A case of a cementifying fibroma in the proximal end of the tibia in a 31-year-old woman is presented. The lesion was expanding but well-defined with amorphous central calcification. The unique, calcified, cementum-like particles in a fibrous stroma have been reported in only four previous cases in long bones. The histological and radiographic differences between a cementifying fibroma, bone cysts with cementum, and a true cementoma are discussed. The radiological differential diagnosis of fibrous dysplasia, giant cell tumor, and low grade central osteosarcoma was considered.

Adult↗

Epithelioid granulomata in a nonossifying fibroma. The possible drug-induced mechanism.

A 13-year-old boy affected by bursitis of the R knee and nonossifying fibroma of the distal metaphysis of the R femur, was treated in the weeks prior to surgical intervention with water-soluble aspirin tablets to relieve local pain. Unexpected epithelioid cell granulomata were observed inside the neoplastic fibrous stroma of the nonossifying fibroma. The coincidental appearance of a slight blood eosinophilia (7%) and perioral fixed drug eruption are clues that suggest that the histological bone findings may represent an expression of the hypersensitivity response elicited by aspirin. It cannot be ruled out that this unusual finding may be related to documented previous trauma, resulting in interstitial hemorrhages and subsequent reactive resorptive processes.

Adolescent↗

Intracerebral fibroma: light and electron microscopic study.

The light and electron microscopic findings of a surgically excised intracerebral mass is presented. The ultrastructural study led to definitely exclude both meningioma (that was the original diagnosis at surgery) and intracerebral schwannoma, while substantiating the uncommon final diagnosis of fibroma. The true intracerebral fibroma has exceptionally been described in the past. The seven so far reported cases are shortly reviewed.

Adolescent↗