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[Tracheal neurofibroma and Recklinghausen's disease].

Neurogenic tumours arising in the trachea are rare; the association with Von Recklinghausen's disease is exceptional. Only two cases of tracheal neurofibroma with neurofibromatosis have been reported. We report on another patient, a thirty year old man, who presented with acute shortness of breath; laser resection was performed via an endotracheal approach, then, a few days later, resection of the exotracheal tumor and tracheal segment was performed. Postoperatively, the patient did well for nine years without any respiratory complaint or neurofibromatosis relapse.

Adult↗

[A case of a primary neurofibroma of the left ventricle].

A neurogenic tumor of the heart is extremely rare, and previously only 8 cases of malignant schwannoma and 3 cases of neurilemmoma have been reported. The patient of this case was a seven year old boy and referred to our hospital by his school doctor for investigation of IRBBB on screening ECG. The chest-X-ray showed a bulging contour on left ventricular apex. Echocardiography and angiography showed a 4 x 4 cm solid mass in the anterior-lateral wall of the left ventricle near the apex. Thoracotomy was performed and during cardiopulmonary bypass the tumor was successfully removed without involvement of the endocardium. It was not encapsulated. The pathohistological findings of the specimen were compatible with neurofibroma, and S-100 protein was demonstrated immunohistochemically.

Child↗

[Pathologic dens fracture in irradiated malignant neurofibroma].

A pathological dens fracture in a 47 year old patient with malignant neurofibroma is described. These tumours are very rare but the prognosis is very poor because of rapid proliferation and early metastatic progression. In the described case, bone osteolysis remained unnoticed until manifestation of clinical symptoms. The pathological fracture occurred despite irradiation of the dens.

Axis, Cervical Vertebra↗

[Neurofibroma of the lacrimal sac].

A sole neurofibroma has been found in the lacrimal sac. It is the fourth case of this unusual localization presented in the world literature.

Adult↗

Unilateral congenital ptosis due to plexiform neurofibroma, causing refraction error and secondary amblyopia.

An 8-year-old boy with congenital ptosis of the right upper eyelid due to plexiform neurofibroma was operated on because of a rapidly worsening of his ptosis. Only partial removal of the tumor was possible. A hypermetropic astigmatism of the right eye was caused by the condition of the upper lid, with secondary amblyopia. This finding suggests that in cases of congenital ptosis the presence of a lid tumor must be suspected. Such a tumor causes ptosis and this can lead to refraction error and amblyopia.

Amblyopia↗

[Gastric neurofibroma. Report of 2 cases].

Two patients consulting for upper gastrointestinal bleeding, associated to impaired gastric emptying in one, were studied with X rays and endoscopy. A gastric neurofibroma was confirmed by histologic study in both patients. One patient was submitted to subtotal gastrectomy. An endoscopic resection of the tumor was performed in the other.

Aged↗

[Immunomorphologic study of the extracellular matrix in neurofibroma and benign schwannoma].

The distribution of type I, III, IV and V collagen and fibronectin in schwannomas and neurofibromas showed the similarity in the extracellular matrix structure of both tumours. Both the components of the interstitial connective tissue (collagen of I, III, V types and fibronectin) and component of basal membrane (the IV type collagen) are found in the fibrillar matrix of these two tumours. It is shown for the first time that matrix of rhythmical structures of schwannoma contains the collagen of type IV and fibronectin while the interstitial collagen of types I, III and V is absent. The application of the results obtained for the differential diagnosis of fibrous and neurogenic tumours is discussed.

Adolescent↗

Syndrome of inappropriate antidiuretic hormone secretion in a patient with intrasellar neurofibroma of the sixth nerve.

The syndrome of inappropriate antidiuretic hormone (SIADH) secretion has been described in a wide range of neurological and other disorders. We wish to add an extremely rare case of a solitary, large, invasive neurofibroma of the sixth cranial nerve extensively destroying the sella turcica in the skull base and causing inappropriate secretion of antidiuretic hormone in a 44-year-old black man in the absence of neurofibromatosis.

Abducens Nerve↗

Neurofibroma of vocal cord: case report.

A case of neurofibroma of the vocal cord is presented. Such an occurrence is quite rare, because of the unusual involvement site and because the lesion was not related to Von Recklinghausen disease. The pertinent literature is reviewed.

Adult↗

[Pelvic plexiform neurofibroma: typical aspects in CT].

Plexiform neurofibroma is a relatively uncommon but characteristic manifestation of neurofibromatosis. Two pelvic cases were found by CT in two young patients in whom it was the first fortuitous manifestation of the disease. The CT pattern, which seems to be in close relation with pathology, is described and illustrated. This pattern is so typical that there is no difficulty to establish the differential diagnosis. The question of sarcomatous transformation is more problematic by CT.

Adult↗

Giant neurofibroma of the pectoral region including the breast. Case report.

A case of a 56 years old female patient with a giant tumour of the left pectoral region developed in a 2 years time was reported. The tumour, free from skin and pectoral muscles, was after mammectomy 25 x 15 x 5 cm, 2.200 grs, multilobaed, albescent with hemorrhages zones and predominantly hard, the rest of the breast being extremely reduced in comparison with the volume of the tumour itself. The initial histological aspect was that of a benign spindle cell tumour and only after some neurohistological techniques the diagnosis established was neurofibroma. The literature data and the exceptional mammary localization of this tumour were reviewed.

Breast Neoplasms↗

Plexiform neurofibroma: report of an unusual presentation.

We describe a plexiform neurofibroma discovered incidentally in a patient who was involved in a motor vehicle accident. The lesion simulated a subcapsular liver hematoma or diaphragmatic rupture on sonography, computed tomography and angiography.

Adult↗

Unusual haemostasis for an unusual tumour. Catastrophic bleeding from a tactile neurofibroma affecting the thoracic vertebrae. Case report.

The symptomatology of a typical acute descending thoracic aorta dissection was imitated by profuse haemorrhage caused by a benign tumour composed almost exclusively of Wagner-Meissner like tactile corpuscles and fatty tissue. The tumour caused extensive destruction of the bodies of the fifth and sixth thoracic vertebrae at the level of the vertebro-costal articulation. Emergency cross clamping of the descending aorta and haemostasis of the bleeding from osteal defects by tamponade with bone polymethyl methacrylate appeared the only way to control the life threatening haemorrhage. It seems that an intrathoracic tactile neurofibroma with a similar case history has not been reported till now.

Aortic Dissection↗

[Malignant neurofibroma of the cervical portion of the vagus nerve].

The authors give an account of a malignant neurofibroma of the cervical portion of the vagus in Recklinghausen's disease in a 29 year-old man. The tumour was removed with part of the nerve. Relatively shortly after operation a local relapse of the tumour developed, with propagation into the posterior cranial fossa, which proved fatal. The authors discuss diagnostic difficulties, possibilities of treatment and the prognosis of malignant neurogenic tumours.

Adult↗