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Fourth ventricular hemangioblastoma associated with pheochromocytoma and renal medullary fibroma.

Intraventricular hemangioblastomas are exceptionally rare. Of the cases described in the literature, very few were associated with von Hippel-Lindau disease. We present a highly unusual case of a fourth ventricular hemangioblastoma associated with a pheochromocytoma and a renal medullary fibroma. This may represent a forme fruste of the von Hippel-Lindau complex. A workup for papilledema resulted in the discovery of this rare finding.

Adrenal Gland Neoplasms↗

[Maxillary ossifying fibroma: apropos of 3 cases].

Three cases of ossifying fibroma of the maxilla are reported in two young females and in one child. The characteristic of this benign fibro-osseous tumour are described. The authors insist on the necessity of a confrontation between the clinical, the radiological and the histopathological aspects of this tumour to establish a diagnostic.

Adult↗

[Ileal intussusception, caused by fibroma, after cholecystectomy performed under microlaparotomy].

The development of minimally invasive surgery for the management of cholelithiasis has been based on the premise that abdominal exploration during cholecystectomy is unnecessary. In the current study, 575 patients undergoing micro- and modern mini-laparotomy cholecystectomy were evaluated to assess the incidence and significance of undetected intra-abdominal pathology. In one instance an intussusception occurred from an ileal fibroma. This patient required a reoperation on the 20th postoperative day, after the removal of the porcellaneous gallbladder. After the primary resection of the tumor and affected bowel area, the patient recovered completely. During 575 cholecystectomy, performed with the micro- and modern minilaparotomy method, only in one patient (0.17%) was significant pathology not detected.

Adult↗

[Osseous desmoid fibroma of the mandible. An unusual case in a 15-month-old infant].

The authors present a rare case, unique in the literature, of an osseous desmoid fibroma in which the histological diagnosis was made at the age of fifteen months. The signs were of a slight swelling with complete trismus of the mandible. After excision of the tumour, with a resultant defect in the superior ramus of the mandible, the trismus disappeared spontaneously after two weeks.

Diagnosis, Differential↗

[Bladder leiomyomas and fibromas. Apropos of 8 cases].

Eight cases of benign mesothelial tumors of the bladder are reviewed (7 leiomyomas and 1 fibroma). The authors describe the pathological and clinical features and the various possibilities of diagnosis of these uncommon tumors. The treatment is exclusively surgical and the prognosis is good in every case.

Adult↗

Allograft bone in the treatment of desmoplastic fibroma. A case report.

A 17-year-old girl had a desmoplastic fibroma of the distal femur. This rare tumor is managed by surgery alone and requires a marginal to wide resection because of its high risk of local recurrence. Had the tumor invaded into the epiphysis and marginal resection, it would have resulted in loss of articular femoral condyle. It was treated by en bloc proximal resection with distal intralesional curettage and anatomic specific allograft femoral replacement. There was no recurrence of the tumor three years after surgery, and function was excellent.

Adolescent↗

Desmoplastic fibroma. A rare oral tumor in children.

A report on confirmed cases of desmoplastic fibroma in a 30-month-old male and a nine-year-old male, both of which responded well to surgical therapy and neither of which has shown signs of recurrence. A 10-year clinical and radiographic follow-up is included.

Child↗

[Desmoid tumors (invasive fibromas) in the mouth and maxillofacial region (clinical aspects and pathology)].

The desmoid is the independent pathological entity of a non-metastasizing fibroma invading the local neighboring structures. It is derived from fascia, aponeuroses or tendines. In rare cases it is found in the oral and maxillofacial region. For a firm diagnosis, it is absolutely necessary to combine clinical data (location, size growth, behavior vis-a-vis adjacent structures) with pathologic-anatomic findings (invasion of the adjacent tissue, especially musculature). In this respect, examination of the marginal regions is of decisive importance. Because of the frequency of recurrences and the danger of sarcomatous degeneration, radical resection is the therapy indicated.

Adolescent↗

[Bladder fibroma. Report of a case].

Analysis of one case of benign vesical primitive connective tumour, with pathoanatomical description of vesical fibroma. The interest of this case rests on its rarity and uncertain origin, still subject to controversy. The paper presents the tumour's clinical history, pathological anatomy, therapeutical approach and prognosis.

Female↗

Cardiac fibroma and heart transplantation.

Successful orthotopic heart transplantation was performed in a 38-day-old child with a fetal echocardiographic diagnosis of a left ventricular mass and in a 40-year-old woman with cardiac murmur since childhood and an echocardiographic diagnosis of asymmetric septal hypertrophy. Pathologic examination of the removed hearts, consisting of gross, histologic, immunohistochemical, and ultrastructural studies, led to the final diagnosis of cardiac fibroma. Both patients were alive and in good condition at 35 and 28 months, respectively, after operation.

Adult↗

[Successful excision of a left ventricular fibroma in an adult patient].

A 48-year-old woman with negative T waves in leads I, a VL and V4-6 in the ECG was examined. The cross-sectional echography and the MRI revealed that she had a tumor in the free wall of the left ventricle. Under the cardiopulmonary bypass, the intramural fibroma (6 by 6 by 4 cm) of the left ventricle was resected and the defect was repaired by sutures of the outer myocardial layers to keep the left ventricular volume. Forty eight days after the initial operation, a reoperation was performed by interrupted sutures of the whole myocardial layers. She is doing well without any troubles 14 months after the reoperation.

Electrocardiography↗

[Right atrial fibroma simulating cyanotic cardiac vitium in a newborn (author's transl)].

The clinical picture in a cyanotic newborn suggests pulmonary atresia or tricuspid atresia. Angiocardiography shows a right atrial tumor, at necropsy a ping-pong-ball sized fibroma is found. Hypoxia and congestive heart failure occurred and the baby died on his eleventh day of life. Uncertain cardiac symptomatology should always raise the suspicion of a cardiac tumor. Cardiac tumors with predominant intramural growth can show pure cardiomegaly- and/or cardiac arrhythmias, congestive failure, while intracavitary growth may lead to various kinds of in- and outflow obstruction and/or embolisations.

Cyanosis↗

Intraventricular fibroma: MR and pathologic comparison.

This case report describes clinical, surgical, MR, and pathologic findings of an intraventricular fibroma, a rare, benign mesenchymal neoplasm. Relatively isointense with normal brain parenchyma on T1-weighted images, the tumor exhibited hyperintense signal on T2-weighted pulse sequences and enhanced intensely and homogeneously after intravenous administration of gadopentetate dimeglumine. Choroid plexus papilloma/carcinoma, meningioma, ependymoma, and subependymal giant cell astrocytoma should be included in the differential diagnosis.

Cerebral Ventricle Neoplasms↗

Desmoplastic fibroma of bone.

We present a rare case of benign intraosseous desmoplastic fibroma, occurring in the tibial bone of a 48-year-old woman. The plain film features consist of a lytic, expansile lesion, in the metaphysis and epiphysis of the bone. We present a case involving the long bone in which the correct diagnosis was suggested by CT and MRI. The appearance of the lesion on CT and MR is described.

Angiography↗

[Multiple perifollicular fibroma].

Perifollicular fibroma (PF) is a cutaneous hamartomatous proliferation of the pilar connective tissue sheath. It occurs predominantly on the face or neck in a solitary or multiple form. Histologically, it is characterized by a concentric arrangement of collagen fibers surrounding hair follicles. Other related proliferations include fibrofolliculoma and trichodiscoma, which are often complicated or have histological overlapping features, so they may represent the same spectrum of pilar mesodermal proliferation. Some individuals with multiple PFs have colonic polyposis, some of which develop into carcinomas, and there are suggestions of autosomal dominant inheritance. It appears that patients with multiple PFs and/or other connective tissue hamartomas may be at risk for colonic polyposis. Cutaneous signs may lead to an early discovery of digestive diseases.

Colonic Polyps↗

Collagenous fibroma (desmoplastic fibroblastoma): a report of seven cases.

We report the clinical features, light microscopic and ultrastructural appearance, and the immunohistochemical profile of seven benign fibrous tumors recently designated as desmoplastic fibroblastoma. The tumors occurred in four women and three men, who ranged in age from 41 to 76 years (mean, 53 yr). The tumors were located in the left thigh, right shoulder, right upper arm, left forearm, right neck, posterior neck, and foot and measured from 1.5 to 8 cm (mean, 4.6 cm) in greatest diameter. Four tumors were located in the subcutaneous tissue, two tumors were intramuscular, and one tumor involved the subcutaneous tissue and adjacent skeletal muscle. Six patients underwent a simple excision; one patient underwent a wide excision of the tumor. Grossly, the tumors were well circumscribed with a white to tan-white, bulging cut surface. Microscopically, they all displayed similar features and were composed of widely separated stellate- or spindle-shaped cells embedded in a hypovascular fibrous or fibromyxoid stroma. No mitotic figures, calcifications, or necrosis were identified. Five tumors were well demarcated, and two tumors had focally infiltrating margins. Ultrastructural examination performed on two cases showed that the tumor cells had features of fibroblasts and myofibroblasts. Immunohistochemical examinations performed on six cases showed that all of the tumors were diffusely positive for vimentin. One tumor showed diffuse staining for smooth muscle actin, and two tumors were focally positive for that antibody. One tumor showed diffuse staining for muscle actin, and another one was focally positive. Two tumors demonstrated diffuse faint staining for S100 protein. There was no staining for desmin, keratin, or CD34. Follow-up information was available in four cases and ranged from 1 to 60 months (average, 24 mo). No tumor recurred. Although the term desmoplastic fibroblastoma has been applied to this neoplasm, we think that the name collagenous fibroma is more appropriate for these tumors.

Adult↗