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Chondromyxoid fibromas: a study of 10 cases.

Chondromyxoid fibroma (CMF) can be confused with a malignant tumor because of its potential for recurrence and occasional nuclear atypia. This study of 10 cases of CMF comprises 6 men and 4 women, with a mean age of 24.3 years. Tibia was the preferred site of involvement. The histology showed lobulated chondromyxoid matrix with peripheral fibrosis. Two cases had local recurrences following intralesional excision. The study emphasizes that despite nuclear atypia and recurrences, CMF is a benign disease.

Adolescent↗

TGF-beta1 drives partial myofibroblastic differentiation in chondromyxoid fibroma of bone.

Chondromyxoid fibroma (CMF) is a rare benign cartilaginous bone tumour with a lobular architecture containing stellate and myofibroblast-like spindle cells. The aim of this study was to investigate the presence, spatial distribution, and extent of myoid differentiation in CMF and to evaluate a possible causative role for TGF-beta1 signalling, which is known to promote smooth muscle actin (SMA) expression. Twenty cases were studied for immunoreactivity for muscle-specific actin (MSA), SMA, desmin, h-caldesmon, calponin, TGF-beta1, and plasminogen activator inhibitor type 1 (PAI-1). The extent of myofibroblastic differentiation was further investigated ultrastructurally, including immuno-electron microscopy using antibodies against MSA and SMA, focusing upon the different cell types in CMF. The expression of potential genes driving this process was quantified by Q-RT-PCR (TGF-beta1, fibronectin, its EDA splice variant, and PAI-1). Tumour cells, especially those with a spindled morphology, showed diffuse immunoreactivity for MSA, SMA, TGF-beta1, and PAI-1, while desmin, h-caldesmon, and calponin were absent. Ultrastructurally, neoplastic cells showed the presence of myofilaments and rare dense bodies, which were more prominent in spindle cells and less so in chondroblast-like cells. Immuno-electron microscopy confirmed the actin nature of these myofilaments. No fibronexus was identified. The functional activity of TGF-beta1 was demonstrated by the identification of PAI-1, a related downstream molecule both immunohistochemically as well as by Q-RT-PCR. There was a linear correlation between TGF-beta1 and PAI-1 expression. Fibronectin-EDA levels were low. We have therefore substantiated the presence of morphological, immunohistochemical, and immuno-electron microscopic partial myofibroblastic differentiation in CMF, driven by TGF-beta1 signalling.

Actins↗

Case report 713. Chondromyxoid fibroma of the third metatarsal.

In the case presented, the imaging features are those of an aggressive benign or less aggressive malignant lesion. The differential diagnosis radiographically included aneurysmal bone cyst with or without an accompanying lesion, giant cell tumor synovial sarcoma, and other mesenchymal sarcomas. Indeed, at times it is difficult to be certain whether the process originated in the bone or soft tissue. Pathological examination of the resected specimen showed the typical features of chondromyxoid fibroma. Grossly, the tumor was well demarcated and firm and composed of tan, translucent tissue that destroyed cortex but was confirmed by periosteum. Histologically, the tumor consisted of myxoid, chondroid, and fibrous elements. The tumor lobules were composed of predominately myxoid matrix containing stellate cells. Variable chondroid elements were present with immature appearing chondrocytes, containing eosinophilic cytoplasm and irregularly shaped nuclei.

Adult↗

Chondromyxoid fibroma of the sternum. Case report.

We report the second case of chondromyxoid fibroma (CMF) of the sternum, documented in the literature. In this case, only histology of the biopsy was useful in diagnosing CMF before definitive surgery. A wide subtotal resection of the sternum and reconstruction with a Gore-Tex soft tissue patch was performed.

Adult↗

Sacral chondromyxoid fibroma.

A 15-year-old girl with a limp and weakness and wasting of the left leg, was found to have a large chondromyxoid fibroma of the sacrum. The lesion presented difficulties in diagnosis because of its unusual site, neurological presentation, large size and extensive extension into the soft tissues of the pelvis and buttock.

Adolescent↗

Chondromyxoid fibroma of bone.

Chondromyxoid fibroma is a benign, although potentially aggressive tumor, with a cartilage-like matrix, accounting for approximately 1% of all bone tumors. It usually affects the metaphyseal region of long bones of patients in their first or second decade of life. An additional peak of incidence has been observed between 50 and 70 years of age. Three cases are presented here: 10-, 13-, and 52-year-old patients, with lesions in the proximal tibia, the proximal humerus, and the proximal femur, respectively. The literature is reviewed in terms of clinical behavior, diagnostic procedures, prognostic factors, treatment, and outcome. Preferred treatment is complete local excision with tumor-free margins. Intralesional curettage with or without local adjuvants shows a local recurrence rate of approximately 25%. Radiation therapy may be useful in nonresectable cases but bears the well documented risk of radiation-induced malignancies.

Adolescent↗

Congenital chondromyxoid fibroma of the ethmoid: case report.

This report describes a congenital case of chondromyxoid fibroma (CMF) arising from the ethmoid bone. We believe it to be the second case of congenital CMF that has been documented, and the third case of CMF arising in the ethmoid. We describe the radiographic features of this rare entity and indicate the necessity for careful correlation between radiographic and histological findings to distinguish CMF from chondrosarcoma.

Chondroblastoma↗

Chondromyxoid fibroma of paranasal sinuses: report of two cases presenting with nasal obstruction.

Chondromyxoid fibroma (CMF) is a rare, benign cartilaginous tumor that often occurs in the metaphyses of long bones. Tumors of the craniofacial bones are extremely rare and most often involve the mandible and the maxilla. This report presents the clinicopathological and radiological features of two unusual cases of CMF arising in the paranasal sinuses that presented with nasal obstruction. The tumors arose in the sphenoid and ethmoid sinuses and were treated by curettage and resection, respectively. One of the two patients was 20 days old, suggesting a possible congenital origin.

Aged↗

Chondromyxoid fibroma of the acromium with soft tissue extension.

Chondromyxoid fibroma is an unusual, benign tumor of cartilaginous origin and represents less than 1% of all primary bone tumors. It usually involves the long bones around the knee joint or the flat bones of the pelvis or ribs. Soft tissue extension is also thought to be rare in these lesions. They are usually eccentrically located in the metaphyses of the long bones and centrally in the flat bones. The radiographic appearances are characteristically those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. Histologically, they display a lobulated pattern with spindle-shaped cells lying within a myxoid matrix with areas of hyaline cartilage. The differential diagnosis includes giant cell tumor, chondroblastoma or enchondroma as well as chondrosarcoma. The rarity of these lesions may render the diagnosis difficult to make, especially when the lesion involves an unusual site such as the acromium.

Acromion↗

Chondromyxoid fibroma of the sacrum.

A 30-year-old man with a 7-month history of mild sacral pain and intermittant left sciatica was found to have an expansile lesion in the sacrum on a plain radiograph. Biopsy confirmed a chondromyxoid fibroma which was removed surgically. A 1-year follow-up showed no recurrence. The case is the fifth to be reported. Plain film and MRI appearances, histology and treatment are described. The previously reported cases are reviewed and the current literature is discussed.

Adult↗

Shope fibroma virus growth factor exhibits epidermal growth factor activities in newborn mice.

A synthetic 55-residue peptide consisting of the carboxyl portion of the predicted genomic DNA sequence of Shope fibroma virus growth factor (SFGF residue 26-80) was found to exhibit epidermal growth factor-transforming growth factor activities in newborn mice. The synthetic SFGF accelerated precocious incisor eruption and eyelid opening in newborn mice and also retarded the overall growth rates of hair, body weight and body length when administered in dosages of 4 to 6 micrograms per gram of body weight. The results of whole animal studies indicate that SFGF belongs to the EGF-TGF alpha family and exerts similar biologic effects in newborn animals.

Animals↗

The peripheral odontogenic fibroma.

An extensive review of the English-language literature has produced only five undoubted cases of peripheral odontogenic fibroma. This article reports ten new cases. The age and sex of the patients and the distribution by site and histology of the lesions are compared with those of other cases of bifrous tumors and neoplasms of odontogenic origin. It is postulated that this lesion may be more closely related to the fibrous euplis than was previously realized. Both lesions show hyperplasia of the basal layer of the covering ipithelium into double strands resembling odontogenic epithelium. It is suggested that this hyperplasia may be related to induction of the covering epithelium by primitive ectomesenchymal remnants within the gingivae. Proliveration of odontogenic rests within the peripheral odontogenic frbroma may be related to the same stimulus.

Adolescent↗

Cystic, melanotic ameloblastic fibroma with granulomatous inflammation.

A large mandibular tumor which had the cellular features of ameloblastic fibroma but was cystic and complicated by granulomatous inflammation is described. The epithelial component contained melanin. The pathogenesis of the cystic change is discussed, and the lesion is compared to the proposed papilliferous variant of this odontogenic neoplasm.

Adolescent↗

Recurrent central odontogenic fibroma.

This article presents a case of a central odontogenic fibroma which recurred 9 years after it was enucleated. Very few cases of this tumor have been recognized and reported. The clinical and histopathologic criteria and the differential diagnosis of the lesion are stressed.

Adult↗

Central odontogenic fibroma of the WHO type.

Two examples of central odontogenic fibroma, WHO type, are reported. The radiographic and microscopic features are discussed and illustrated. Both were treated by curettage, and neither has recurred after 10 and 9 years, respectively. We postulate an ectomesenchymal-epithelial interaction in the histogenesis of this unusual tumor.

Adult↗