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[Neurofibroma and Perineurial cell. Electron microscopic examinations of 9 neurofibromas (author's transl)].

In neurofibromas type I (plexiform) and type II (diffuse) the Schwann cell is the predominant cell in the neoplastic proliferation. Electron microscopic investigation of a neurofibroma with structures resembling tactile corpuscles (type III) revealed neoplastic proliferation of perineurial in stead of Schwann cells. The tactile-like (pseudo-Meissnerian) corpuscles (corpuscles neurofibromateux) are formed by neoplastic perineurial cells. These cells have a tendency to wrap themselves around longitudinal structures, such as collagen fibres or axons, and to come into direct contact with the latter. The close relationship between the axon and the neoplastic perineurial cell, analogous to that between axon and Schwann cell, points to a neuroectodermal origin origin of the perineurial cell. The electron microscopic appearances suggest that either the Schwann cell or the perineurial cell is the essential neoplastic component of neurofibromas, the fibroblastic proliferation being a secondary phenomenon.

Adolescent

An electron microscope study of "Pacinian neurofibroma".

An electron microscope study has been made of the "Pacinian neurofibroma". Unlike the usual neurofibroma the "Pacinian neurofibroma" is characterized by a proliferation of the so-called perineurial cells. Groups of surface vesicles, the absence of mesoaxons and a fragmented basallamina differentiate these perineurial cells from Schwann cells. The formation of the perineurial cells can be traced continuously from small and wide submicroscopic cellbands and clubshaped thickenings to ribbonlike cell complexes as well as to tactile-like structures. The most developed complexes and structures are visible with the light microscope. These formations do not correspond with real tactile corpuscles, rather they can be considered as neoplastic structures of the perineurium.

Axons

Incidence of neurofibroma in cattle in abattoirs in New South Wales.

The incidence of neurofibroma in slaughtered cattle in New South Wales during a four-year period is reported. Most of the cases were in adult Hereford females over 5 years old. In 24 of the herds to which neurofibroma cases were traced there was evidence that the disease had previously occurred in the herd. However, the data does not indicate close familial relationship among cases of neurofibroma.

Abattoirs

Plexiform neurofibroma infiltrating the facial nerve.

A plexiform neurofibroma was unusual in that it extensively infiltrated the main trunk and branches of the left facial nerve without compromising function, rather than forming a circumscribed, compressive mass. Histologic sections showed diffuse involvement of even minute branches of the facial nerve. Electron microscopy disclosed Schwann cell and fibroblastic proliferation in a collagenous matrix. Collagen fibrogenesis by fibroblasts, but not by Schwann cells, was noted. Many of the Schwann cells were free of any association with nerve axons, which suggests that some release of the Schwann cell from its usually well-defined functions is involved in neurofibroma formation.

Child

Surgical treatment of eyelid neurofibromas.

The classical surgical treatment of eyelid neurofibromas has been careful dissection of the tumor with preservation of much of the surrounding abnormal lid tissues. The reported outcome has been uniformly unfavorable. These tumors infiltrate extensively and are impossible to dissect out completely. However, the lateral location of lid neurofibromas allows "en bloc" resection of most of the tumor including the adjacent involved lid tissues. Levator function in these cases is potentially good and the lid will elevate well if the levator aponeurosis is joined to the tarsus laterally at the time of surgery. The four cases presented here indicate that this procedure is technically easier and may produce more acceptable results than other forms of treatment.

Adolescent

Neurofibroma and schwannoma of the oral cavity. A clinical and ultrastructural study.

The clinical parameters of fifty-five cases accessioned as neurofibroma and schwannoma of the oral cavity were reviewed. An electron microscope study was performed on several cases. The component cells of neurofibroma were spindle shaped, with bundles of collagen fibrils between cells. Junctional complexes were observed between adjacent cells. Component cells of the schwannoma showed numerous convoluted cytoplasmic processes. Reduplication of basal lamina was frequently observed and formed the material in the hyalinized area of Verocay bodies.

Adolescent

A combined neurofibroma-granular cell tumor of the middle cranial fossa.

A case of a combined neurofibroma-granular cell tumor in a 52 years old gunsmith is presented. The tumor developed after an intracranial trigeminal nerve operation 25 years previously. The solid tumor in the left middle cranial fossa had displaced and infiltrated the temporal lobe. It had expanded via the optic nerve into the left orbit, and further the apex partis petrosae was destroyed. The neurofibroma part shows histological aspects of malignancy, the granular cell tumor, considering its infiltrating and destructive growth, may be regarded as malignant as well. In intermingling portions of the tumor, transitional types of fiber-like and granular cells are prominent. In the peripheral zone of the tumor apparently reactively proliferated polynuclear astrocytes are seen with occasionally intracytoplasmatic lymphocytes (emperipolesis?). A short review of the literature and the theories concerning the histogenesis of the granular cell tumor is given. Whereas most authors in recent years suggest a Schwann cell origin, based on electron microscopic findings, this intermediate tumor type motivates us to postulate a mesodermal origin of the granular cell tumor. The question of viral influence is discussed briefly.

Aged

[Neurofibroma--neurofibromatosis--neurofibrosarcoma].

The author presents 14 patients with the gradations "neurofibroma--neurofibromatosis-neurofibrosarcoma". The great variability of forms of this disease ist emphasized. Apart from pure soft-tissue tumours, primary and secondary osseous changes were demonstrated in 4 patients. Intra-oral neurofibromas were seen in 5 cases. In 3 of these, the tongue was involved. Suggestions are given for the treatment of this clinical picture.

Adolescent

[Studies on neurinomas and neurofibromas in tissue culture (author's transl)].

10 acoustic neurinomas, 2 spinal cord neurinomas and 3 neurofibromas of von Recklinghausen's disease were elaborately studied by monolayer tissue culture method and Gelfoam organ culture method. Four morphological types were found in the acoustic and spinal cord neurinoma. (1) multinucleated cells (type I), dividing into bipolar spindle-shaped cells. (2) bipolar spindle-shaped cells (type II), forming of palisade fashion in the monolayer tissue culture method. (3) fibroblastic cells (type III), tending to predominate in the long-term cultures. (4) round cells (type IV), gathering to the Gelfoam mesh in the organ culture method. Neurofibroma of von Recklinghausen's disease was divided into cells of two types in the tissue culture. (1) spindle-shaped cells, rarely observed, and have been thinking that the cells originated from schwann cells. (2) round cells, having the macrophagic character, are predominant in the long-term culture.

Culture Techniques

Syndrome of multiple mucosal neurofibromas, pheochromocytoma and medullary thryoid carcinoma. Report of a case.

The present article reports a case of multiple endocrine neoplasia (MEN) syndrome, type IIb, including the following components: pheochromocytoma, medullary thyroid carcinoma, hyperplastic corneal nerves and multiple mucosal oral and ocular neurofibromas. The patients, a 35-year-old male also exhibited a series of other pertinent findings, i.e. thickened eyelids, marfanoid habitus and widened mandibular canal and mental foramina. In the discussion the clinical and histopathologic characteristics of the present case are compared with similar findings in other previously published cases. The multiple oral mucosal neurofibromas are described clinically and histopathologically and are stated as a pathognomonic early sign of the syndrome.

Adult

Malignant neurofibroma with glandular differentiation (glandular schwannoma).

A case of malignant shwannoma is reported with unusual elements in an 89-year-old female. A large mass was located in the subcutaneous tissue of the right lateral chest wall and measured 5 cm in the greatest diameter. Histologically the tumor was composed of neurofibroma and malignant schwannoma with glandular differentiation. Neurofibroma characterized by numerous hyaline neural nodules was located in the peripheral portion of the tumor, whereas malignant schwannoma occupied a large part of the central portion of the tumor. The glandular elements observed in some areas of malignant schwannoma consisted of cuboidal and columnar shaped cells and were arranged in tubular or tubulo-medullary fashion in which rosettes or pseudorosettes were found. Mucicarminophilic material was observed, both in the cytoplasm and in the lumen. Seven reported cases of peripheral nerve tumor with glandular differentiation are reviewed briefly.

Aged

Multiple halo neurofibromas.

The second case of multiple halo neurofibromatosis is reported. Halo neurofibromas are rare benign tumors that appear on the neck and trunk of adults. The clinical and histopathological differential diagnosis of the other neuroectodermally derived halo lesions, such as neural nevus, nevocellular nevus, spindle and epitheloid cell nevus, blue nevus, and malignant melanoma, also is considered. An autoimmune hypothesis is proposed to explain the depigmentation in the halo neurofibroma. The fact that the patient has café au lait spots that contain giant pigment granules probably indicates that he also has neurofibromatosis.

Biopsy

Adjacent malignant schwannoma and neurofibroma of intrathoracic vagus.

A 24-year-old man with neurofibromatosis and adjacent malignant schwannoma and neurofibroma of an intrathoracic vagus is reported. He survived for eight months, developing massive recurrence three months after surgical extirpation. Review of the 13 previously documented patients with neurofibroma of the intrathoracic vagus indicates that most patients have neurofibromatosis. The thickest portion of the intrathoracic vagi, the proximal portions and most often the left nerve, is the site of predilection. In about half, multiple neurofibromata of the nerve may be present. Malignant schwannoma of the mediastinum is discussed and it would appear that neurofibromatosis is not a predisposing factor.

Adult

Plexiform neurofibroma (Rankenneurofibrom) of the cauda equina.

The clinical and neuroradiological findings in a rare case of plexiform neurofibroma with bone scintigraphy, myelography and selective spinal angiography are described. The difficulties with the preoperative differential diagnosis from other intraspinal space occupying lesions are discussed and the importance of intraoperative biopsy and electrical stimulation is stressed.

Aged

Plexiform neurofibroma of the head and neck.

Ten patients with plexiform neurofibroma of the head and neck were observed at M.D. Anderson Hospital between 1956 and 1978. The clinical presentation and the long-term follow-up of the most interesting cases are presented. This is a chronic disease that causes cosmetic and functional deformity because of the size or the position of the tumor, or both. No patient exhibited malignant transformation. Because all of the disease cannot be removed, the surgical procedures should not be radical but should be designed to relieve symptoms or improve cosmesis.

Adult

Neurofibroma of the maxillary antrum.

A rare case of neurofibroma of the maxillary antrum is reported. The tumor was removed via a Caldwell-Luc approach. Its histopathologic characteristics are described.

Humans

Myxoid neurofibroma of the testis.

A case of an intratesticular myxoid neurofibroma is reported. These tumors, which arise from perineural and Schwann cells, commonly occur throughout the body but have not been reported previously to originate within the testis. The mucoid material within the tumor is translucent, which may confuse the diagnosis with that of a hydrocele.

Adult