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Amelogenesis imperfecta: multiple impactions associated with odontogenic fibromas (WHO) type.

Three types of amelogenesis imperfecta (AI) are recognised, namely hypoplastic, hypomature and hypocalcified varieties. We report on two cases of hypoplastic AI, the type which occurs most frequently. Both patients presented with multiple impacted permanent teeth. Odontogenic fibromas of the WHO type were found to be associated with the crowns of all the impacted teeth and are considered to have prevented normal eruption. Dentinal dysplasia found only in the furcation area of the multirooted impacted teeth was evident. The macroscopic, microscopic and radiological appearance of the affected teeth, pericoronal lesions and interradicular dentinal dysplasia are described, and the most likely origins of the odontogenic fibromas and calcifications observed, are discussed.

Adolescent↗

[Treatment of uterine fibroma using LH-RH analogs and gestrinone. Limits and indications].

Medical treatment of fibroma has changed radically in recent years with the introduction of analogs of LH-RH (luteinizing hormone releasing hormone). These agents, which are active only via parenteral administration, have proved remarkably effective, and are devoid of metabolic effects. They do engender some disagreeable adverse reactions, and unfortunately their efficacy is transitory and their cost high. Compared with these analogs, gestrinone, a progestogen as yet unused in treatment of luteal insufficiency, seems very interesting in the treatment of fibromas, due to its prolonged antigonadotropic effect and its antiprogesterone effect. Further studies are required to confirm the first results published by Coutinho.

Administration, Intranasal↗

[Ameloblastic fibroma--case report and discussion about relationship of mixed odontogenic tumors].

The authors have presented the case of ameloblastic fibroma of the upper jaw in 21 years old adult. Dimensions of the tumor, which were the result of long standing growth, and the occurring at the age when odontogenesis was practically completed, have supported the concept of distinct pathologic entity, but have not been agree with the theory of interrelationship of ameloblastic fibroma, ameloblastic fibro-odontoma and odontoma. In the context of those two studies the authors have discussed relationship of the mixed odontogenic tumors.

Adult↗

Chondromyxoid fibroma: report of six cases with immunohistochemical studies.

Chondromyxoid fibroma is a rare benign bone tumor of uncertain histogenesis that typically presents as an eccentric lesion in the metaphysis of a long bone of a young adult. We report S-100 positivity in six cases of myxochondroid fibroma, a finding consistent with the cartilaginous nature of the lesion and its possible relation to chondroblastoma.

Adolescent↗

Benign histiocytic fibroma of rib with CT correlation.

Benign fibrous histiocytoma is a benign tumor with fibroblastic and histiocytic differentiation, most commonly found in soft tissues and less frequently in adult bones. It is identical histologically to non-ossifying fibromas of childhood but differs in its clinical and radiological features. We here report a 26-year-old woman with benign histiocytic fibroma in a rare location, a rib, with computed tomographic correlation. Such correlation has not been reported previously.

Adult↗

Ameloblastic fibroma.

A case of an ameloblastic fibroma in the mandible of a 14-year-old boy with a six-month follow-up is reported. A brief comparison is made between the ameloblastic fibroma and the simple ameloblastoma.

Adolescent↗

[Bronchial hyperreactivity in non-asthmatic patients harboring a uterine fibroma].

One of the etiological factors of female late onset asthma lies in endocrine disorders and it often occurs during a period of hormonal instability (menopause or premenopause). Moreover various studies have shown increased allergic manifestations in women with gynecological problems (dysmenorrhea, premenstrual syndrome). Gynecological pathology is found in 30% of 67 women who suffer from late onset asthma. Prospective studies were carried out in 28 women with surgical uterine fibroma, to investigate bronchial hyperreactivity (BHR). The study included atopic research through questioning and allergy skin tests; spirographic respiratory function study, airway resistance, carbachol BHR with determination of the threshold dose (FEV1 20% decrease). Carbachol BHR was found in 8/28 persons (28%), but with no clinical manifestations. None of the patients smoke and only one suffers from mite-allergic rhinitis-conjunctivitis without asthma. BHR is often found in asymptomatic women with uterine fibroma. Is this BHR due to an autonomic dysregulation or to a hormonal malfunction? This hypothesis would require further studies to elucidate, since premenstrual asthma worsening is a well known phenomenon, which can be improved by progesterone. This may suggest a possible link between hormonal malfunction and BHR in women. A long term survey will facilitate detection of late onset asthma.

Adult↗

[Chondromyxoid fibroma of the skull base extending from the middle fossa to the posterior fossa--case report].

An extremely rare case of chondromyxoid fibroma of the skull base extending from middle fossa to posterior fossa is reported. The patient is a forty-seven year old woman who became paranoiac four months before admission and her gait was unsteady for one month. On April 27th, 1985, she fainted and was admitted to Kanto Rosai Hospital on April 30th. She was drowsy and disoriented. Neurologic examination revealed left pyramidal tract signs and impaired functions of right Vth (first and second branches), VIth and VIIIth cranial nerves. She was anemic and bleeding tendency due to thrombocytopenia was also recognized. Hematologist's diagnoses were myelodysplastic syndrome plus iron deficiency anemia. Neuroradiologic studies including CT scan, cerebral angiographies and MRI revealed a huge extra-axial skull base tumor extending from middle fossa to posterior fossa completely destroying the skull base. Stenosis of cavernous portion of internal carotid artery due to tumoral involvement was also noted. Surgical excision of the tumor was performed three weeks after the admission with the use of platelet transfusion. Transpetrosal transtentorial approach was performed removing the intracranial part of the tumor which was a semi-translucent jelly-like soft mass. The patient had a good post-operative course and improvement in the neurologic condition was recognized. Microscopically, the tumor showed marked lobular formation with small vessels and fibroblasts in the periphery of the lobule. The fibroblasts took stellate figures in the myxoid matrix at the center of the lobule. Chondroid pattern was also observed. Mitosis and nuclear atypism were not observed. Histological diagnosis was chondromyxoid fibroma.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral Angiography↗

[A case of chondromyxoid fibroma of the frontal bone].

A case of chondromyxoid fibroma of the skull is reported. A 20-year-old boy visited our clinic on December, 1982 because of a recurrent forehead tumor. He had a 4 X 4 X 1.5 cm tumor on the right side of forehead and a 3 X 3 X 0.5 cm tumor on the left. Neurological examination showed no abnormalities. Skull X-ray film showed a large round radiolucent area with clear sclerotic margin in the frontal bone and right orbit. Right carotid angiogram showed marked posterior displacement of the anterior cerebral artery, but no tumor stain. Plain CT scan showed a mass with iso to low density area in the frontal region. It was markedly and irregularly enhanced with contrast media. Surgery was performed entirely in the epidural space, and the tumor was completely removed. The post-operative clinical course was uneventful. Histologically, the three components of chondroid, myxomatous and fibrous tissues in this tumor led to the diagnosis of "chondromyxoid fibroma."

Adult↗

Chondromyxoid fibroma. A review of the literature and a report on our own experience.

The present paper comprises a review of the literature on chondromyxoid fibroma (CMF) and a report on nine cases originally classified as CMF, found in a review of all curetted bone lesions at our department from 1955 to 1978. At histologic revaluation only two cases fulfilled the criteria for true CMF, whereas six were classified as other benign bone lesions and one proved to be a chondrosarcoma. The chondrosarcoma case after recurrence was subjected to more mutilating surgery than would have been necessary if correct diagnosis had been established initially. The other six benign cases were all well and without signs of recurrence 5-11 years after curettage. In one of the two true CMF cases, i.e. in a young boy, there were for 2 years postoperatively radiological signs of a progressive recurrence, which was left without treatment, since the patient was well. Interestingly, radiograms 2 and 11 years postoperatively essentially showed the same picture, indicating spontaneous cessation of the process. Chondromyxoid fibroma is an extremely rare lesion. Histologic misinterpretation is not uncommon. In sporadic cases this may have serious implications. It may be questioned whether surgical treatment of CMF in young patients, known to have a high recurrence rate, is mandatory.

Adult↗

Surgical management of nasopharyngeal angio-fibroma.

Seventy cases of nasopharyngeal angio-fibroma are reported. Most of the patients were young adolescents between 13-24 years of age. 91% were males. The main symptoms were nose blockage and nasal bleeding, the latter was present in all the patients but the amount of blood varied from a few milliliters a time to a few hundreds of milliliters. Although angio-fibroma is benign, it invades the surrounding structures, especially the sphenoidal sinus, maxillary sinus and the ethmoidal sinuses, by expansion or by erosion. In 1/7th of the cases he palate is pushed downwards or bulges. Surgery may be the only methods to cure the disease. The main principle of surgery is complete removal of the tumour together with the periosteum to which it is attached. After thorough exposure, the extraction of the tumour should be as quick as possible, otherwise the bleeding will be profuse. As soon as the tumour has been completely removed, the hemorrhage stops spontaneously or only a minor bleeding remains. The majority of the blood supply is derived from the external carotid system, so ligation of the external carotid and injection of saline or 0.5% novocaine solution in the base of the tumour just before the extirpation of the tumour can reduce the loss of blood during the operation. However, usually a blood transfusion of 300-500 ml is necessary.

Adolescent↗

[Chondroma, chondroblastoma and chondromyxoid fibroma].

Chondromas are benign tumors characterized by the formation of mature cartilage. Typically, they involve the short bones of the hands and feet. The lesions may be either solitary or multiple as part of enchondromatosis affecting several bones. Cases of multiple enchondromatosis with predominantly unilateral distribution are generally referred to as "Ollier's disease" or when accompanied by multiple hemangiomas as "Maffucci's syndrome". In cases with multiple chondromas there is considerable risk of malignant transformation (approximately 50%). Treatment of single lesions (curettage and bone grafting) is usually uneventful, but any therapeutic approach to Ollier's disease or Maffucci's syndrome should be tailored to the individual distribution of lesions in the most severely deformed extremities. Chondroblastomas are uncommon benign tumors that are almost invariably situated in the epiphyses of long bones adjacent to the epiphyseal cartilage plate. Histologically, they are characterized by round or polygonal chondroblasts with focal accumulation of osteoclast-like giant cells. Intercellular substance is scanty, and calcification occurs typically at focal areas. Lung metastases are reported in rare single cases with high survival rates after local resection, indicating low-grade malignancy. Only a few recurrences have been reported following curettage and bone grafting. Avascular necrosis or chondrolysis with subsequent secondary osteoarthrosis may occur after surgical treatment of chondroblastoma of the upper femur epiphysis. These severe indirect sequelae should be considered both when planning surgery and during follow-up. Chondromyxoid fibromas are rare benign tumors that are mostly diagnosed in the second and third decade; they are mainly found in the proximal tibial metaphysis. Histologically, there are areas of chondroid, myxoid, and fibrotic tissues, of which the myxoid component is characteristic for chondromyxoid fibromas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Chondromyxoid fibroma of the occipital bone.

Chondromyxoid fibroma is an uncommon bone tumour, found predominantly in the metaphyses of the long bones of the lower limbs. The authors describe a patient in whom the tumour arose from the occipital bone. To the authors' knowledge, this is only the second report of chondromyxoid fibroma occurring in this location.

Adult↗

A case of a central odontogenic fibroma presenting a differential diagnostic problem.

Central odontogenic fibromas are rare benign tumors. We are presenting a case of a 67-year-old female with a central odontogenic fibroma which posed considerable difficulty in microscopic interpretation. The differential diagnosis ranged from metastatic carcinoma to a benign odontogenic neoplasm. The final diagnosis was made through persisting on multiple pathologic consultations, which allowed for conservative rather than aggressive surgery. Follow-up evaluation has confirmed the benign diagnosis.

Aged↗

[Apropos of a case of chondromyxoid fibroma of the talus. A review of the literature].

PURPOSE OF THE STUDY: Chondromyxoid fibroma (CMF) is a very rare tumor. CMF represents less than 1p. 100 of all benign osseous tumors. The upper part of the tibia is the most frequent localization. We report the second case of talus tumor published in the world literature. MATERIAL AND METHODS: A 20 years old man presented a lytic tumor of the talus. The histology diagnosed a Chondromyxoid fibroma. A complete excision was made. An autologous bone graft associated with blocks of coral were used for reconstruction. RESULTS: After six months sport activities were authorized. After 10 years follow-up, there is no recurrence, the coral has progressively disappeared, replaced by host bone. DISCUSSION: Only one case of talus tumor has been published in world literature. Feldman has collected 189 cases of CMF published before 1970. After 1970, 297 new cases published. Analysis of these 486 cases pointed out the frequency of differents localizations. CONCLUSION: This very rare tumor frequent between 10 and 30 years of age. The foot is the second localization after the tibia. A surgical conservative treatment with complete excision is recommended even in case of recurrence. Radiotherapy must be avoided in any case because of the risk of malignant degeneration.

Adult↗

Chondromyxoid fibroma of bone presenting as chronic back pain.

OBJECTIVE: To discuss a case of chondromyxoid fibroma presenting with low back pain. CLINICAL FEATURES: A 50-yr-old man had an 8-yr history of low back pain. This was diagnosed and treated as arising from the disc and caused by spinal stenosis. Magnetic resonance imaging findings supported the clinical findings. Years later, a plain radiograph of the pelvis revealed an incidental abnormality of the right ilium, and the patient was subsequently referred to a musculoskeletal tumor center for treatment. INTERVENTION AND OUTCOME: The lesion was surgically removed and the defect was reconstructed via bone allograft. CONCLUSION: Such tumors are a rare cause of back pain. Tumors of the pelvis can at times present as back pain. In cases of refractory back pain, an X-ray of the pelvis can be a useful screening investigation. Chondromyxoid fibromas are rare tumors best treated by excision if they are amenable or by curettage and bone grafting procedures.

Back Pain↗

TSC angiofibroma and ungual fibroma have different mutation signatures, with recurrent mutations in KMT2C.

PURPOSE: Tuberous sclerosis complex (TSC) is an autosomal dominant tumor suppressor syndrome characterized by tumors affecting multiple tissues, including skin, due to inactivating TSC1/TSC2 variants. Genome-wide profiling of somatic mutations in a unique collection of angiofibroma (FAF) and ungual fibroma (UF) TSC skin tumors was performed. METHODS: Genome sequencing was performed on 9 samples, comprising 4 FAF and 5 UF, along with 6 matched normal samples from 6 individuals with TSC. RESULTS: TSC-FAF and TSC-UF skin tumors have different mutation signatures, with a predominance of UV-related single-nucleotide variant (SNV; SBS7a and SBS7b) and dinucleotide variant (DNV; DBS1) signatures in FAF, and aging-related SNV (SBS1 and SBS5) signatures in UF. We also identified a novel DNV signature for TSC-UF, with frequent TG>CA and TT>GG substitutions. Furthermore, 3 inactivating somatic mutations in KMT2C were observed in 2 of 4 TSC-FAF and 5 mutations in other cancer genes. CONCLUSION: The distinct SNV mutation signatures seen in TSC-FAF and UF indicate that they develop through distinct pathogenic mechanisms, UV-induced mutagenesis in FAF, and aging-related mutagenesis in UF. The mechanism of the novel DNV signature in UFs merits further investigation. Our observation on the occurrence of KMT2C mutations suggests that KMT2C inactivation contributes to the pathogenesis of TSC-FAF.

Humans↗