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[Hypoglycemia associated with pleural fibromas. Study of insulin-like growth factors (IGF) and pathogenic considerations].

PURPOSE: Pathogeny of hypoglycemia associated with non-islet-cell tumors is unclear. We discuss the mechanisms of this syndrome. CURRENT KNOWLEDGE AND KEY POINTS: We report three cases of spontaneous hypoglycemia revealing pleural fibroma. Endocrine tests before surgery showed low serum growth hormone and insulin-like growth factor I (IGF-I), reduced serum IGF-II levels in two patient. Insulin-like growth factor binding protein-3 (IGFBP-3) was low and electrophoretic profile of IGF-II was the 'big IGF-II' type. We discuss the mechanisms of hypoglycemia associated with non-islet-cell tumor. Impaired formation of the ternary complexes and its consequences seem the main pathogenic factor. FUTURE PROSPECTS AND PROJECTS: Analysis of IGF and IGFBP and in situ measurements of IGF mRNA could help in understanding this syndrome and allow therapeutic considerations in the management of hypoglycemia by corticosteroids and growth hormone.

Adrenal Cortex Hormones↗

Recurring digital fibroma of childhood.

A case of recurring digital fibroma of childhood is presented. The natural history of this condition over several years is documented. The literature is reviewed, with particular reference to the management of the condition. Surgeons should be aware of the clinical features and the principles of management of this condition, as patients may be referred because of associated finger or toe deformities.

Disease Progression↗

Juvenile aponeurotic fibroma.

A large aponeurotic fibroma situated on the dorsum of the hand of a fourteen-year-old Nigerian male is reported. Out of forty-three juvenile aponeurotic fibromata of the hand documented in the literature, only two cases occurred on the dorsum.

Adolescent↗

Desmoplastic fibroma of the jaw bones.

A review of the literature has disclosed 15 cases of desmoplastic fibroma in the jaws including the present case. Fourteen out of 15 cases have affected the mandible with a female: male ratio of one to two. Ten of the reported cases were found in the first and second decades of life. Although histologically benign, the tumor usually shows locally aggressive behavior and may recur when treated conservatively. The difficulties in clinical and histological diagnosis are stressed. The paucity of reported cases prevents substantiation of guidelines for therapy.

Adult↗

Chondromyxoid fibroma.

A brief review of the literature concerning chondromyxoid fibroma is presented. 7 previous cases and the present case of the tumor in the jaws are described. Parallels to the extra oral lesions are drawn and in the light of previously published data, distinctive diagnostic features are discussed. The importance of close cooperation between the surgeon, the radiologist and the pathologist is stressed to avoid a misdiagnosis and radical treatment of the malignant counterpart of a rare benign tumor.

Adult↗

Intratendinous fibroma of flexor tendon.

Fibroma of the tendon sheath is a benign lesion usually found in contact with tendon sheath or on the epitenon layer. This case report describes the intratendinous location not previously described. Impairment of flexor tendon excursion is relieved by longitudinal tenotomy and tumor excision.

Child↗

Juvenile aponeurotic fibroma.

Juvenile aponeurotic fibroma is an uncommon benign tumor which occurs mostly in the hands and wrists of young children. It has a tendency to recur, especially in younger individuals, and this, combined with a rather cellular microscopic appearance, has led to mistaken diagnoses of malignancy. No instances of metastasis have been reported. Conservative therapy, consisting of excisional biopsy without sacrifice of vital structures, is indicated.

Adolescent↗

Juvenile aponeurotic fibroma: a case report.

Juvenile aponeurotic fibroma is a rare lesion of the palmar surface of the hands and, occasionally, of the feet of children. It is benign tumor which is self-limited in growth, but which has a strong propensity to recur. Grossly and histologically it exhibits a tendency to invade all surrounding structures, but does not metastasize. Rapidity of growth seems to slow down with increasing age. It should be recognized and treated conservatively by repeated surgical removal.

Child, Preschool↗

Fibroma of tendon sheath.

Fibroma of tendon sheath is a benign soft tissue tumor that has a predilection for the hand. It is histologically distinct from giant cell tumor of tendon sheath, a lesion with which it is commonly confused. A case is reported in a 35-year-old man whose tumor, which extended the whole length of the index finger, had been present for 6 years. This particular tumor is probably more common than has been previously recognized.

Adult↗

Juvenile aponeurotic fibroma of the hand.

Juvenile aponeurotic fibroma is a rare benign tumor found primarily in the extremities of children and young adults. Clinically, it presents as a painless, mobile solitary mass in the hand. It may represent the cartilage analog of fibromatosis. Since it has a distinct tendency toward local recurrence, wide excision without sacrifice of function is the indicated treatment. Two cases are presented illustrating the diagnostic features and treatment of this disease process.

Adult↗

Recurring digital fibroma of childhood.

A recurrent digital fibroma of childhood is reported. This case illustrates difficulties in the management of these recurrent tumors. Despite tumor-free margins on the excised tumors, recurrence occurred at other sites. Recurrences or new primary lesions are reported in 75% of the cases. Because of the rare tendency of these lesions to regress and the high recurrence rate, an individualized approach based on lesion location and behavior is recommended.

Amputation, Surgical↗

Intratendinous aponeurotic fibroma.

Juvenile aponeurotic fibroma is a distinctive fibroblastic tumor usually found in young children. The tumor has a predilection for the palms and soles, although it can occur elsewhere, either superficially in subcutaneous tissues or in deeper musculofascial and paraskeletal tissues. In this case the tumor occurred in an adult within the substance of the flexor pollicis longus tendon, a location not previously reported in the literature. Because of the unique location of the tumor, it was excised without sacrificing the tendon. Since recurrence of these lesions is common, continued observation was necessary. Magnetic resonance imaging in this patient 26 months after the operation showed restoration of the normal tendon contour.

Adult↗

[Ovarian fibroma associated with Demons-Meigs syndrome and elevated CA 125].

We report a rare case of ovarian fibroma in a young woman. When ovarian masse is associated with Meigs' syndrome and elevated CA 125 serum level a malignant process may be suspected. But a negative cytological examination of ascitic effusion and absence of peritoneal implant recommend performing limited surgical procedures.

Adult↗

Primary right ventricular tumor (fibroma) simulating cyanotic heart disease in a newborn.

The clinical, echocardiographic, cineangiocardiographic and autopsy findings in a newborn infant with right ventricular fibroma are reported. The tumor caused severe right ventricular outflow obstruction simulating cyanotic heart disease. Echocardiography allowed a definitive diagnosis of the tumor mass at 10 hours of age. The clinical implications of the case are discussed.

Cineangiography↗

[Juvenile aponeurotic fibroma (Keasbey's tumor) with metastatic progression. Apropos of a case].

The juvenile aponeurotic fibroma, isolated in 1953 by Louisa Keasbey is a rare tumour which is characterized by its recurrence and infiltration, but which has no tendency to metastasis. The case we report differs in its long-term outcome. The tumour diagnosed in a seven-year-old child has recurred locally for seven years, necessitated hand amputation, and finally developed a fibroblastic process extending to lung and pleura which caused the child's death. After this new finding in Keasbey's disease, we are led to reconsider the nosological situation in juvenile fibromatoses and its character of benign disease.

Adolescent↗

[Pleural fibroma revealed by hypoglycemia].

Pleural fibroma or solitary fibrous tumor of the pleura (SFTP) is a rare tumor generally discovered fortuitously or in patients with non-specific respiratory symptoms, more rarely with hypoglycemia. We report the case of a 51-year-old man with a history of smoking (30 pack-years) and no other disease history whose chest x-ray revealed an enormous opacity occupying the lower two-thirds of the left hemithorax. This radiological anomaly had led to episodes of lipothymia three months earlier together with mental confusion in the morning as well as an episode of hypoglycemia (0.48 g/l) which rapidly resolved after administration of hypertonic dextrose. Ultrasonography and chest scan were performed later and confirmed the tissular nature of the opacity which was well limited with a left base. Minimally abundant homolateral pleural effusion was also noted. The diagnosis of SFTP revealed by hypoglycyemia was the most probably diagnosis. Emergency thoracotomy enabled removal of a hard tumor weighing 2115 g. Histology confirmed the diagnosis of benign SFTP. Hypoglycemia in the context of SFTP would be related to tumor secretion of insulin-like proteins. Complete emergency resection is required to avoid the risk of life-threatening hypoglycemia.

Fibroma↗

Aortic valvular fibroma as a source of systemic emboli in POEMS syndrome.

In a young woman with POEMS syndrome and two systemic embolic events, transesophageal echocardiography revealed a small mobile aortic valve mass. At surgery, an aortic valvular papillary fibroma, with areas of attached thrombus, was removed. This case underlines the importance of transesophageal echocardiography in identifying cardiac sources of emboli.

Adult↗

Cardiac fibroma mimicking apical hypertrophic cardiomyopathy: a case report and differential diagnosis.

A 55-year-old man was seen with palpitations, dyspnea, syncope, and a large apical left ventricular mass. A diagnosis of apical hypertrophic cardiomyopathy had been made by cardiac catheterization 10 years previously. Transthoracic echocardiography performed during the current evaluation, however, was suggestive of a primary cardiac tumor. At operation, a large fibroma was excised. Knowledge of the echocardiographic appearance of apical left ventricular abnormalities can be helpful in determining the most appropriate choice of therapy.

Cardiac Catheterization↗