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Results for “Nerve Sheath Neoplasms”

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At least 19 recordsLinked to original sources
PubMed1986

The intermediate filament complement of the spectrum of nerve sheath neoplasms.

The intermediate filament complement of the spectrum of nerve sheath neoplasms including 12 typical benign schwannomas, 1 ancient schwannoma, 2 cellular schwannomas, 6 neurofibromas and 4 malignant schwannomas was investigated by immunofluorescence microscopy, two dimensional electrophoresis, and immunoblot analysis. Studies were performed on freshly frozen tumor tissue samples; a broad spectrum of antibodies against all classes of intermediate filaments was utilized. Samples were also studied by electron microscopy, and immunohistochemically for S-100 protein and desmoplakins. By immunofluorescence microscopy, all nerve sheath neoplasms revealed intense positivity for vimentin throughout the cytoplasm while 2 benign schwannomas displayed co-expression of vimentin and glial filament proteins. Two-dimensional gel electrophoresis and immunoblot analysis confirmed the presence of vimentin and showed that it was the predominant protein in all tumors. Electrophoretic analysis of the 2 benign schwannomas that immunostained for glial filament proteins confirmed the presence of this protein which was shown to comigrate with a known human control sample. Neither immunofluorescence microscopy nor biochemical analyses revealed cytokeratin polypeptides, neurofilament proteins, desmin, or desmoplakin in any of the tumors. We conclude that while vimentin is the predominant intermediate filament expressed by the entire spectrum of nerve sheath neoplasms, at least occasional benign schwannomas are capable of co-expressing glial filament proteins. It remains to be determined whether the subgroup of nerve sheath neoplasms that co-expresses vimentin and glial filament proteins is otherwise distinguishable from their more frequent counterparts that express vimentin exclusively.

Adolescent↗
PubMed1987

Malignant nerve-sheath neoplasms in neurofibromatosis: distinction from benign tumors by using imaging techniques.

Malignant peripheral nerve-sheath neoplasms frequently complicate neurofibromatosis causing pain, enlarging masses, or neurologic deficits. However, similar findings sometimes also occur with benign nerve neoplasms. Our study was done retrospectively to determine if imaging techniques can differentiate malignant from benign nerve tumors in neurofibromatosis. Eight patients with symptomatic neoplasms (three benign, five malignant) were studied by CT in eight, MR in six, and 67Ga-citrate scintigraphy in seven. Uptake of 67Ga occurred in all five malignant lesions but not in two benign neoplasms studied. On CT or MR, all eight lesions, including three benign neoplasms, showed inhomogeneities. Of five lesions with irregular, infiltrative margins on CT or MR, four were malignant and one was benign. Of three lesions with smooth margins, one was malignant and two were benign. One malignant neoplasm caused irregular bone destruction. Accordingly, CT and MR could not generally distinguish malignant from benign lesions with certainty. However, both CT and MR provided structural delineation to help surgical planning for both types of lesion. 67Ga scintigraphy appears promising as a screening technique to identify lesions with malignant degeneration in patients with neurofibromatosis. Any area of abnormal radiogallium uptake suggests malignancy warranting further evaluation by CT or MR. Biopsy of any questionable lesion is essential.

Adolescent↗
PubMed1998

Perineurioma of the finger: case report of a rare peripheral nerve sheath neoplasm of pure perineurial cell lineage.

A 1.9-cm extraneural soft tissue tumor located on the palmar aspect of the index finger of a 50-year-old woman was excised. Depicting many histopathologic patterns and a full range of cellularity, the tumor was exclusively composed of epithelial membrane antigen/vimentin-positive and S-100 protein/Leu-7-negative neoplastic cells of perineurial lineage. Soft tissue (extraneural) perineurioma, a rare variant of benign peripheral nerve sheath tumor, should be included in the differential diagnosis of tumor and tumor-like conditions affecting soft parts of the hand.

CD57 Antigens↗
PubMed1991

Human peripheral nerve sheath neoplasm: expression of Schwann cell-related markers and their relation to malignant transformation.

We immunohistochemically examined the expression of Schwann cell-related markers, nerve growth factor (NGF) receptor, S-100 alpha- and beta-proteins, glial fibrillary acidic protein (GFAP), and galactocerebroside (gal C) in 5 malignant schwannomas, 21 benign peripheral nerve sheath tumors, and 4 apparently normal sural nerves. NGF receptor was expressed in the malignant schwannomas and benign peripheral nerve sheath tumors, but not in the endoneurium of apparently normal peripheral nerves. S-100 alpha-protein was observed in malignant schwannomas and in some neurofibroma cells. All cases were strongly positive for S-100 beta-protein but were negative for GFAP and gal C. Our results suggest that these Schwann cell-related markers may be useful in identifying peripheral nerve sheath neoplasma as well as their malignant transformation.

Adult↗
PubMed1978

Scanning electron microscopy of nerve sheath neoplasms.

Neurofibromas and schwannomas were clearly differentiable by scanning electron microscopy of thin microscopic sections. Neurofibromas exhibited ordered laminations of collagen with scattered fibroblast-like cells interspersed. Schwannomas contained proliferations of smooth tube-forming cells. These neoplastic characteristics were compared with cellular characteristics of normal nerve sheaths to determine the cell of origin in each neoplasm.

Animals↗
PubMed1994

Glandular peripheral nerve sheath tumours.

Glandular differentiation in peripheral nerve sheath neoplasms is a rare but well recognised phenomenon, believed to represent the least common of the various forms of heterologous differentiation which may occur in tumours of peripheral nerve. This group of tumours--the 'glandular peripheral nerve sheath neoplasms'--are the subject of this short review. The majority of glandular peripheral nerve sheath neoplasms arise in patients with von Recklinghausen's disease and follow a malignant course, but both benign and sporadic forms have now been described. Neither the presence nor the appearance of the glandular foci are believed to influence patient outcome. Predictions of likely biologic behaviour are best based upon an assessment of the nature of the accompanying spindle cell stroma. Glandular peripheral nerve sheath neoplasms must be distinguished from various tumours of soft tissue which may share similar histological features e.g. synovial sarcoma and the epitheloid nerve sheath tumours. Immunohistochemistry and ultrastructural examination may aid in this regard, and have also contributed to our understanding of the nature and origin of the glandular structures, though questions regarding their histogenic origins remain somewhat controversial.

Cell Differentiation↗
PubMed1998

Intraventricular perineurioma: case report.

OBJECTIVE AND IMPORTANCE: Perineurioma, a rare benign nerve sheath neoplasm occurring in either an intraneural or soft tissue form, has never been reported to arise in the central nervous system. CLINICAL PRESENTATION: We describe the clinical, radiological, and pathological features of a perineurioma arising in the choroid plexus of the third ventricle in a 65-year-old woman and causing obstructive hydrocephalus. INTERVENTION: The lesion, apparently unassociated with a nerve, was gross totally resected by frontal craniotomy using a left-sided transcallosal approach. Short-term follow-up showed no evidence of recurrence. CONCLUSION: Perineurioma of the variety found in soft tissue may occur in the central nervous system, wherein it shows the typical light microscopic, immunohistochemical (epithelial membrane antigen- and Collagen IV-positive, S-100 protein-negative), and ultrastructural (pinocytotic vesicles, discontinuous basement membrane) features.

Aged↗
PubMed1994

Benign glandular peripheral nerve sheath tumor. A case report.

The glandular peripheral nerve sheath tumor is a rare variant of nerve sheath neoplasms in which the focally occurring glands are lined by cells showing divergent differentiation. The vast majority of the reported nerve sheath tumors harboring these glands have been malignant. We herein present a case of benign glandular peripheral nerve sheath tumor in a 43-year-old woman who had no evidence of von Recklinghausen's disease. Histologically, the tumor is composed of spindle cell component and collections of glandular component. The glandular component occupied the central two-thirds of the lesion and was lined by a single layer of nonciliated cuboidal or columnar cells. No mitotic figures were recognized in the spindle cell area. This spindle cell area had neurofibroma-like features rather than schwannoma. Many of the spindle cells had positive reaction products for S-100 protein. The glandular lining epithelium were positive for cytokeratins (CAM 5.2, AE1/AE3, PKK1) and EMA. Some epithelial cells were immunoreactive for CEA, chromogranin, somatostatin and Leu-7. These immunohistochemical findings support the neuroendocrine differentiation of the epithelial element from the schwannian component.

Adult↗
PubMed1981

Ultrastructure of capillaries in acoustic neurilemmoma.

The microvasculature of normal acoustic nerve tissue and small intracanalicular and large extracanalicular neurilemmomas was analyzed by electron microscopy. The capillaries of normal acoustic nerve were nonfenestrated, but the microvasculature in nerve-sheath neoplasms was fenestrated. Hypertrophy and hyperplasia of endothelial cells were observed only in the small lesions. The proliferated and enlarge endothelial cells often partially occluded the vascular lumen and formed multichannel vascular lumina. Many fenestrae of capillaries were found in small neoplasms, but these were rarely identified in the large extracanalicular lesions. The gap junctions of endothelium in small nerve-sheath neoplasms were long, wavy, and convoluted, had no openings, and differed from those of large lesions in which the tight junctions were short, straight, and occasionally patent. Additionally, heavy vascularization and erythrocytes within basement membranes were observed only in large neurilemmomas. These ultrastructural features may constitute a basis for differences between large and small acoustic neurilemmomas noted in cerebrospinal fluid findings.

Capillaries↗
PubMed2002

Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as Bednár tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma.

We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednár tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.

Dermatofibrosarcoma↗
PubMed1985

Immunohistochemical recognition of human nerve sheath tumors by anti-Leu 7 (HNK-1) monoclonal antibody.

Using relatively high dilutions of anti-Leu 7 monoclonal antibody and a four-step peroxidase-antiperoxidase (PAP) reaction in paraffin-embedded tissues, we tested the affinity of this antibody to the cells of 47 human nerve sheath tumors and 22 other tumors in which the differential diagnosis with nerve sheath neoplasms is known to arise. Of all the nerve sheath tumors studied 68%, including 80% of the schwannomas, contained anti-Leu 7-positive cells. All 22 non-schwannian neoplasms were entirely negative. Specimens of eight experimental malignant rat schwannomas were also negative for anti-Leu 7 antibody. Our findings suggest that anti-Leu 7 monoclonal antibody is a promising marker that may facilitate the differential diagnosis between human Schwann cell and non-Schwann cell neoplasms.

Amputation Stumps↗
PubMed1990

Cellular peripheral neural tumors (neurofibromas) in children and adolescents: a clinicopathological and immunohistochemical study.

Nine examples of a cellular peripheral neural tumor (CPNT) were identified in a review of 139 peripheral nerve sheath neoplasms in children, which included 60 neurofibromas and 16 malignant peripheral nerve sheath tumors. The mean age at diagnosis of these nine patients was 7 years, with six presenting in the first decade of life and four were noted at birth. The male:female ratio was 0.5. Topographically, the tumors were located in the extremities, 4; head and neck, 3; and trunk, 2. One or another stigmata of von Recklinghausen's neurofibromatosis (VRN) was present in four patients. After initial resection, seven children remained well, but two developed a recurrence; the histology was identical to the original tumor in one case but overt malignant transformation had occurred in the second. This case was the only tumor-related death in this series. The CPNT was a circumscribed but nonencapsulated mass measuring 1.8-7.5 cm in greatest dimension in the subcutaneous and deep soft tissues and had a compact spindle cell pattern, occasional mitoses, and minor foci of typical neurofibroma. Immunohistochemical staining revealed vimentin expression in all seven cases, Leu-7 in six, myelin basic protein and S-100 protein in five, desmin in one, and actin in none. In contrast to neurofibroma and malignant peripheral nerve sheath tumors, CPNT tended to occur earlier, either congenitally or in the first decade, and slightly more commonly in females. The anatomical distribution and pattern of immunoreactivity were similar to neurofibroma. However, the cellularity and mitotic activity of these neoplasms were sufficiently disquieting as to raise concerns about the prognosis, and in one case, the tumor behaved in an unequivocally malignant fashion. When a peripheral neural tumor with the pathologic features described in this study is encountered, wide excision and careful clinical follow-up are recommended.

Actins↗