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At least 55 records · Page 3Linked to original sources
PubMed1998

Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic, immunohistochemical, and ultrastructural study of seven cases.

Most malignant peripheral nerve sheath tumors (MPNST) are schwannian in nature. The pathologic features of MPNST with perineurial cell differentiation remain to be characterized. To determine the clinicopathologic, immunohistochemical, and ultrastructural characteristics of perineurial MPNST, 121 MPNST from the Mayo Clinic Tissue Registry were examined. Of these 23 spindle cell tumors with long processes disposed in whorls or storiform patterns, features typical of perineurioma, were studied. On the basis of immunohistochemistry (epithelial membrane antigen+/S-100-), 5 perineurial MPNST were identified among 23 tumors selected. These and two previously characterized perineurial MPNST are the subject of this study. None of seven tumors was associated with NF-1. Patients included five males and two females ranging in age from 11 to 83 years (mean, 45.7 years). The tumors measured 1.5 to 30 cm (mean, 9.1 cm) and arose in the extremities (two), trunk (two), face (one), mediastinum (one), and retroperitoneum (one). Only one tumor was nerve associated (phrenic nerve). All tumors were surgically removed. No encapsulation or neurofibroma components were noted. Necrosis was seen in three lesions. Four tumors were classified as high-grade malignant and three as low grade. Mitotic indices varied from 1 to 85/10 high-power fields (median, 16). Immunoreactivities included epithelial membrane antigen (100%), vimentin (100%), Leu-7 (57%), and CD34 (14%). Stains for S-100 protein, muscle markers, and cytokeratin were nonreactive. Ultrastructurally, perineurial-like cells were noted in three tumors and cells intermediate between perineurial and Schwann cells in one. Four tumors recurred and two metastasized; no deaths of disease were noted at follow-ups of 28 to 98 months (mean, 66.9). In conclusion, 4% of MPNST show perineurial cell differentiation. An NF-1 association has yet to be described. Nerve involvement is infrequent. Their immunophenotype (epithelial membrane antigen+/S-100-) frequently indicates ultrastructural perineurial differentiation. The prognosis of perineural MPNST appears to be more favorable than that of conventional MPNST.

Adult↗
PubMed1997

Perineurial cell tumor (perineurioma) with granular cells.

A form of benign cutaneous tumor with perineurioma findings and with the presence of associated granular cells is described. The two cases studied consisted of whorls made up of a high number of circumferentially arranged flattened cells, with perineurial characteristics, including bipolar cell processes, pinocytotic vesicles, a basal lamina, a positive immunoreactivity for EMA, and absence of immunostaining for S-100 protein. The granular cells, enclosed within the whorls, contained densely packed vesicles, particles with an apparently solid core, as well as membrane-limited vacuoles with disintegrating cellular organelles and electron-dense amorphous material. While failing to demonstrate any immunoreactivity for EMA, the granular cells showed positivity for S-100 protein, which supports their Schwann-cell origin. Due to its morphological and immunohistochemical characteristics, this peculiar form of tumor can be considered as a perineurioma with perineurial cell whorls and granular cell changes occurring in associated Schwann cells at the center of the whorls.

Adult↗
PubMed2003

Outcomes of surgery in 1019 brachial plexus lesions treated at Louisiana State University Health Sciences Center.

OBJECT: Outcomes of 1019 brachial plexus lesions in patients who underwent surgery at Louisiana State University Health Sciences Center during a 30-year period are reviewed in this paper to provide management guidelines. METHODS: Causes of brachial plexus lesions included 509 stretches/contusions (50%), 161 plexus tumors (16%), 160 thoracic outlet syndromes (TOSs, 16%), 118 gunshot wounds (12%), and 71 lacerations (7%). Many features of clinical presentation, including prior treatment, patient's neurological status, results of electrophysiological studies, intraoperative findings, and postoperative level of function, were studied. The minimum follow-up period was 18 months and the mean follow-up period was 42 months. Repairs were best for injuries located at the C-5, C-6, and C-7 levels, the upper and middle trunk, the lateral cord to the musculocutaneous nerve, and the median and posterior cords to the axillary and radial nerves. Conversely, results were poor for injuries at the C-8 and T-1 levels, and for lower trunk and medial cord lesions, with the exception of injuries of the medial cord to the median nerve. Outcomes were most favorable when patients were carefully evaluated and selected for surgery, although variables such as lesion type, location, and severity, as well as time since injury also affected outcome. This was true also of TOSs and tumors arising from the plexus, especially if they had not been surgically treated previously. CONCLUSIONS: Surgical exploration and repair of brachial plexus lesions is technically feasible and favorable outcomes can be achieved if patients are thoroughly evaluated and appropriately selected.

Action Potentials↗
PubMed1990

Ophthalmic manifestations of perineural spread of facial skin malignancy.

Facial skin malignancy, when complicated by perineural spread (PNS), can lead to devastating orbital, intracranial and paranasal sinus involvement, the management of which requires a multidisciplinary approach and which itself may be devastating. Adequate surgical excision with frozen section control, and specific inspection of histological specimens for perineural invasion, are essential to avoid this problem. This report presents four patients who manifested such involvement, and emphasises the importance of recognition of such ominous symptoms as facial pain, paraesthesiae and weakness. Thorough neuro-ophthalmic examination is mandatory to detect signs of nerve involvement which may indicate perineural spread both at the initial assessment of primary tumour and at suspected recurrence. In this series, the most common sensory and motor nerves affected were, respectively, single, small branches of the ophthalmic and facial nerves.

Aged↗
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↗
PubMedDate not supplied

Malignant peripheral nerve sheath tumor of the pancreas with perineurial cell differentiation.

The authors report a malignant peripheral nerve sheath tumor (MPNST) that developed in the body of the pancreas of a 76-year-old woman. The well-demarcated tumor, 4.5 x 3.5 cm in size, was composed of spindle-shaped cells arranged in a storiform pattern or sweeping fascicles. Ultrastructurally, the tumor cells had elongated cytoplasmic processes with numerous pinocytotic vesicles, and were covered with discontinuous basal lamina-like substances. Immunohistochemically, tumor cells were diffusely positive for epithelial membrane antigen, vimentin, and CD34, but negative for S-100 protein and Leu 7. It was therefore concluded that the tumor was a MPNST with perineurial cell differentiation. Primary MPNST of the pancreas is extremely rare and the immunohistochemical and ultrastructural studies are necessary for its diagnosis.

Aged↗