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Chondromyxoid fibroma of the nasal cavity with an interstitial insertion between chromosomes 6 and 19.

Chondromyxoid fibroma is an uncommon benign cartilaginous tumor that rarely presents in the sino-nasal region as a locally destructive, erosive lesion. Both clinically and histologically, it is a difficult diagnosis and can be confused with malignant processes such as myxoid chondrosarcoma. Histology of the tumor, especially with a small sample, can be challenging because of its heterogeneous nature showing an admixture of fibrous, myxoid, and chondroid areas. We are reporting unique cytogenetic findings in a case of chondromyxoid fibroma involving the floor of the nasal cavity with a clonal rearrangement between chromosomes 6 and 19. To our knowledge, karyotypes of 14 cases are reported in literature, with 11 cases reporting nonrandom, clonal abnormalities of chromosome 6. These results illustrate the distinctive nature of this tumor and may help identify genes involved in the pathogenesis of this tumor.

Chromosomes, Human, Pair 19↗

Juvenile ossifying fibroma of the maxilla.

Juvenile ossifying fibroma is a rare fibro-osseous neoplasm in young children. This lesion is locally aggressive and spreads quickly, and because it has a very high recurrence rate complete excision is essential. Reported here is a case of a massive juvenile ossifying fibroma of the maxilla in an 11-year-old male child. A titanium mesh was used to reconstruct the facial contour after a left total maxillectomy, achieving a satisfactory facial appearance.

Child↗

A case of hyperparathyroidism-jaw tumour syndrome found in the treatment of an ossifying fibroma in the maxillary bone.

Hyperparathyroidism-jaw tumour (HPT-JT) syndrome is characterized by parathyroid tumours as well as by ossifying fibromas of the mandible and maxilla, renal cysts, or Wilms' tumours. Recently, the gene responsible for HPT-JT syndrome has been identified as the HRPT2 tumour suppressor gene. In an 18-year-old male, a tumour in the maxilla was first diagnosed as an ossifying fibroma. During biochemical screening before surgery, the patient received a diagnosis of primary hyperparathyroidism. Neck computed tomography scanning showed a parathyroid tumour. Surgical excisions to remove the jaw tumour and parathyroid adenoma were performed. The postoperative course has been uneventful and a follow up at 2 years revealed no evidence of recurrence. The HRPT2 germline mutation of 39delC was detected in the proband, but not in his unaffected parents. These results suggested that the germline mutation occurred de novo.

Adenoma↗

Endoscope assisted removal of cementoossifying fibroma in the paranasal sinuses in a five-year-old girl.

We report a five-year-old girl with a sinonasal cementoossifying fibroma with CT findings that reveal the invasion of all paranasal sinuses and the remodeling of facial bones. It was possibly a congenital lesion and caused a slowly progressive nasal obstruction and anosmia. Tumor removal with transnasal endoscopic approach was applied and total removal of the mass was accomplished. It was found out to be the cementoossifying fibroma of the paranasal sinuses when the results of the pathological examinations were obtained.

Child, Preschool↗

Benign lymphangiomatous papules of the skin associated with ovarian fibroma.

Benign lymphangiomatous papules of the skin are considered reactive lymphatic proliferations either caused by disruption of the lymphatic flow or tissue damage produced by operation or radiation therapy. We report a 72-year-old woman with umbilical papules and vesicle-like lesions that led to the diagnosis of a large ovarian fibroma. Histologic study revealed dilated lymphatic spaces manifesting an anastomosing and branched pattern in the papillary and reticular dermis dissecting collagen bundles. The vessels were lined by plump endothelial cells with foci of intravascular papillary endothelial cell hyperplasia. After the ovarian fibroma was removed by laparotomy, umbilical lesions almost disappeared, leaving small flesh-colored papules. A periumbilical dermatosis may herald certain intra-abdominal diseases including those of neoplastic derivation. A heightened awareness of this association may lead to an early diagnosis with a potential for improved patient outcome. Benign lymphangiomatous papules have not been previously described in association with an untreated tumor, without previous operation or radiotherapy. This case advocates for disruption of the lymphatic drainage as the probable pathogenetic mechanism.

Aged↗

[Tendinous sheath fibroma causing "trigger wrist" and carpal tunnel syndrome].

There are few cases described of fibroma of tendon sheath located to the hand and the wrist. A case of fibroma of tendon sheath of lumbrical muscle of the index finger has been described. The tumor was responsible of a peculiar phenomenon of "triggering" of the wrist during the movements of flexion and extension of the index finger, and caused compression of the median nerve at the carpal tunnel. The intervention has brought to an early and long lasting relief of symptoms. Moreover, the histological aspect has been described and the relatively high possibility of recurrence of this lesion is underlined.

Adult↗

HRPT2 gene alterations in ossifying fibroma of the jaws.

Ossifying fibroma (OF) is a benign neoplasm related to bone characterized by a progressive enlargement of the affected jaw. Recently, the candidate tumor suppressor gene HRPT2 was identified and alterations in this gene were related with the Hyperparathyroidism-jaw tumor syndrome that is characterized by parathyroid adenoma or carcinoma, fibro-osseous lesions (mainly OF) of the jaws, and renal lesions. The purpose of the present study was to evaluate the HRPT2 gene in OF. Tumour and blood samples were obtained from 3 patients with OF and one with juvenile ossifying fibroma (JOF). The results demonstrated three novel mutations in two out of three genotyped OF's. Interestingly, one of these patients showed a germ-line mutation after blood analysis. RT-PCR amplification was performed to analyze HRPT2 mRNA expression and only wild-type HRPT2 transcript was found in all tumours. Investigation of the parafibromin protein by immunohistochemistry showed a similar pattern of immunolocalization with strong nuclear and cytoplasmic staining in all cases. In conclusion, the present study shows for the first time mutations of HRPT2 gene in OF and suggests that OF may arise due to haploinsufficiency of the HRPT2 gene.

Adolescent↗

Desmoplastic fibroma of the jaw: a case report and review of literature.

Desmoplastic fibroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart of soft tissue fibromatosis in both gnathic and extragnathic sites. It has a propensity for locally aggressive behavior and local recurrence. In the present report, we define the clinicopathological and radiographic features of a desmoplastic fibroma of the mandible in an 8-year-old white boy who initially presented with a 2-month history of a rapidly expanding, painless mass along the right inferior border of his mandible. A critical and comprehensive review of the English language literature is also provided.

Actins↗

Psammomatoid ossifying fibromas: immunohistochemical analysis and differential diagnosis with psammomatous meningiomas of craniofacial bones.

OBJECTIVE: To clarify the role of immunohistochemistry in the diagnosis of psammomatoid ossifying fibroma (PSOF), conventional cemento-ossifying fibroma (COF), and psammomatous meningioma (PM) of the craniofacial skeleton. STUDY DESIGN: The histology and immunohistochemistry of 4 PSOFs, 6 COFs, and 7 PMs was studied. Antibodies included EMA, cytokeratins, smooth muscle actin (SMA), desmin, vimentin, CD34, CD10, S-100 protein, and glial fibrillary acidic protein (GFAP). RESULTS: All PSOFs showed multiple round ossicles homogeneously distributed within a fibroblastic stroma. Psammomatous meningiomas had meningothelial features. All tumors, except 1 COF, were positive for EMA. All of them expressed vimentin, and none showed cytokeratins. Staining for SMA and S-100 protein was variable. CD10 was positive in all cases except 2 meningiomas. CD34 and GFAP stained only 1 case of meningioma each. CONCLUSIONS: The diagnosis of PSOF should rest on histologic features. An incorrect diagnosis of meningioma based on the expression of EMA should be avoided.

Actins↗

Peripheral granular cell odontogenic fibroma.

Peripheral odontogenic fibroma is a rare lesion that arises on the gingiva and can clinically mimic a variety of reactive lesions, benign neoplasms, and metastases. We describe a symptomatic lesion arising on the mandibular gingiva of a 58-year-old female with no history of trauma or dental disease in the area. An excisional biopsy showed the lesional stroma to contain numerous polyhedral granular cells with occasional interspersed islands of inactive odontogenic epithelium. We believe this to represent the fourth case of peripheral granular cell odontogenic fibroma to be reported in detail in the literature.

Female↗

[Myxoid pleomorphic fibroma of the skin].

Pleomorphic fibroma of the skin was described by Kamino et al 1 in 1989. It is a benign fibroblastic proliferation with cytologic atypia. This article describes a case of a myxoid variant of pleomorphic fibroma of the skin.

Fibroma↗

Left ventricular reconstruction after resection of a large fibroma.

Cardiac fibromas are rare tumors that are histologically benign but potentially lethal because of their location. The prognosis is related to complete resection. We report the case of a 15-year-old boy who, 1 year after partial excision of a large fibroma, underwent successful complete resection through a conventional surgical approach with left ventricular reconstruction.

Adolescent↗

Surgical issues in giant right ventricular fibroma.

Cardiac fibroma is a rare and benign tumor usually seen in infants and children. We report a 3-year-old asymptomatic child who presented with a giant right ventricular (RV) fibroma. He underwent complete surgical resection and is doing well at follow-up. Because of the size of the tumor and the potential need for resection of RV free wall, it is essential to have contingency plans to deal with postoperative RV failure.

Child, Preschool↗

Myocardial fibroma or fibrous hamartoma.

Early interest in myocardial tumors was directed primarily at their pathology. Today, however, interest must be in early diagnosis and planned operation utilizing cardiopulmonary bypass since such tumours, both benign and malignant, are potentially lethal due to their location and since most are resectable. The case of a patient with myocardial fibroma presented here offers an expanded pathological picture: the tumor contained fibrous tissue, cardiac muscle, vascular elements, adipose tissue, and smooth muscle. Smooth muscle has not been previously reported. A review of the literature reveals disagreement and confusion over proper nomenclature for the entity. Perhaps its varied histological presentation is better described as fibrous hamartoma than fibroma.

Adult↗

Psammomatoid (juvenile) ossifying fibroma of the orbit.

The clinical and pathologic features in 21 cases of a distinctive solitary fibro-osseous lesion affecting the orbit distinguished histologically by the dominance of numerous small round "psammoma-like" ossicles embedded in a cellular benign spindle cell stroma were studied. The patients were predominantly young, and 19 of the lesions were centered on the orbital plate of the frontal bone or ethmoid. A clinical course of slowly progressive symptoms over several years was typical but not uniform. The most common clinical diagnosis was fibrous dysplasia and mucocele. At least nine patients underwent two or more surgical operations; one patient had six recurrences over a 15-year period. There were no known deaths from the tumor, but its potentially massive size can result in considerable morbidity and cosmetic deformity. Although previously referred to as active juvenile ossifying fibroma, the term psammomatoid ossifying fibroma for this lesion is more descriptive and has historical precedence.

Adolescent↗

Ossifying fibroma arising in the right ethmoid sinus and nasal cavity.

Ossifying fibroma, a rare tumor entity, is a well-demarcated benign fibro-osseous tumor composed of bone, fibrous tissue and cementum. It is commonly found in the mandible, but also found in the maxilla and paranasal sinuses. Simple curettage is enough when the fibrous lesions are located in the mandible but a complete en bloc excision is required when these lesions are located in the maxilla and paransal sinuses to relieve symptoms and prevent recurrence. With the advent of sinonasal endoscopy in the mid 1980s, and subsequent advances in surgical techniques, endoscopic management of the fibrous-osseous lesions has become possible. In the current case study, we report a successful endoscopic removal of a huge ossifying fibroma located in the right ethmoid sinus and nasal cavity.

Adolescent↗

Fibroma of tendon sheath.

This report describes four patients with fibroma of tendon sheath. The tumors were slowly enlarging, firm nodules that were adherent to tendon sheaths of the volar aspect of the fingers or palm. One lesion was recurrent. Microscopically, they consisted of lobulated, hyalinized, collagenous masses that contained spindled and stellate fibroblasts and an array of slitlike spaces. There were small pseudopods and separate foci of tumor in adjacent connective tissue. These may explain the propensity of fibroma of tendon sheath to recur.

Adolescent↗