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At least 163 records · Page 9Linked to original sources
PubMed1977

Facial palsy: unusual etiology.

This is a report of three patients who presented at the Mayo Clinic over a two-year period. All were initially diagnosed as having Bell's palsy but were later found to have a malignant neoplasm causing the paralysis. Two of the patients had breast carcinoma metastases involving the mastoid portion of the facial nerve. The third patient had an adenocarcinoma of the deep lobe of the parotid that involved the facial nerve distal to the stylomastoid foramen. The course of the facial paralysis in the two patients with the metastitic breast disease was almost identical. It consisted of episodes of pain in the mastoid area. generally in the late evening or during the night, often awakening the patient from sleep. This was then followed by peripheral facial-nerve paralysis, sometimes partial and at other times complete. These episodes lasted from 10 minutes to several hours and then resolved completely. They recurred over several months. The patients were completely asymptomatic and normal on examination in the intervals between episodes of paralysis until it became permanent. Metastatic lesions causing facial paralysis are extremely rare in the literature. In those cases that have been reported, the paralysis was progressive from the start and in the vast majority of cases was either painless or associated with other aural symptoms such as otorrhea, hearing loss, and periauricular swelling. There are two unusual features of these two cases: 1. the initial presentation of a breast metastasis as a facial paralysis; in the first case there were no other metastatic lesions present at diagnosis, whereas the second patient had other, asymptomatic, metastatic nodules; and 2. the multiple, brief, recurring episodes of facial paralysis, which have not previously been reported as a mode of presentation of metastitic disease. The third patient was diagnosed as having Bell's palsy. A facial nerve decompression was performed, and the nerve apparently looked normal. The paralysis failed to resolve. He was later found to have adenocarcinoma of the deep lobe of the parotid that involved the facial nerve distal to the stylomastoid foramen. A report of only one similar case could be found in the literature. The sequence of events in these three cases emphasizes the importance of submitting a patient suspected of having Bell's palsy to a thorough otoneurologic examination.

Adenocarcinoma↗
PubMed2005

Unilateral limb enlargement in a dog with a malignant peripheral nerve sheath tumor.

A 3- to 4-month-old female Golden Retriever dog presented with right hind limb enlargement. Physical examination of the limb and radiographic findings initially included soft tissue swelling with elongation, bowing, and cortical irregularity of the femur and tibia. During a period of approximately 7 months, pathology in the limb progressed to include tarsal laxity, muscle atrophy, avulsion of the gastrocnemius muscle, and luxation of the patella. During surgical intervention to shorten the limb and repair the patellar luxation, a large soft tissue cyst was identified along the caudal aspect of the femur and stifle. The limb was later amputated, and a final diagnosis of malignant peripheral nerve sheath (PNS) tumor of the sciatic nerve and surrounding soft tissues was made. The unilateral limb enlargement in this dog appears to have been because of the development and progression of a malignant PNS tumor. The presentation and associated pathologic changes in the limb are unusual for canine PNS tumor but have similarities with neurofibromatosis in the limbs of humans.

Amputation, Surgical↗
PubMed2006

Canine intraneural perineurioma.

A 6-year-old castrated German Shepherd Dog was presented with a 6-month history of progressive, nonpainful, left pelvic limb paresis. Magnetic resonance imaging revealed atrophy of left-sided epaxial and hypaxial muscles from L5-L7 and an enlarged L5 spinal nerve. Exploratory hemi-laminectomy revealed focally and cylindrically thickened L5 and L6 nerve roots. Histologic evaluation of a surgical biopsy specimen from the L6 dorsal nerve root, and the L5 nerve roots after later amputation revealed distended hypercellular fascicles. This distension was due to widely separated axons surrounded by concentric lamellations formed by neoplastic perineurial cells and their processes. These pseudo-onion bulbs were separated from each other by a basophilic myxoid stroma. The perineurioma cell processes were immunonegative for S-100 (alpha and beta chains) and collagen IV, but were immunoreactive for laminin. The central axons were also immunoreactive for NF-200 and S-100. The proliferative index of the perineurioma cells, as determined by MIB-1 immunoreactivity, was about 3%. Ultrastructurally, the widely separated, interdigitating perineurioma cell processes were connected by desmosomal-like junctional complexes to form continuous circles. Their processes were covered by a discontinuous basal lamina. Each centrally placed axon was normally, thinly, or completely unmyelinated and was surrounded by a normal Schwann cell. These morphologic and immunologic features distinguish this lesion from hypertrophic neuropathy and were consistent with intraneural perineurioma.

Animals↗
PubMed1997

Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum.

Lymphoma, mediastinal cysts, and neurogenic neoplasms are the most common primary middle and posterior mediastinal tumors. Lymphoma may involve the anterior, middle and/or posterior mediastinum, frequently as lymphadenopathy or as a discrete mass. Foregut cysts are common congenital mediastinal cysts and frequently arise in the middle mediastinum. Pericardial cysts are rare. Schwannoma and neurofibroma are benign peripheral nerve neoplasms, represent the most common mediastinal neurogenic tumors, and rarely degenerate into malignant tumors of nerve sheath origin. Sympathetic ganglia tumors include benign ganglioneuroma and malignant ganglioneuroblastoma and neuroblastoma. Lateral thoracic meningocele is a rare cause of a posterior mediastinal mass.

Combined Modality Therapy↗
PubMed2001

Presacral solitary giant neurofibroma without neurofibromatosis type 1 presenting as pelvic mass--case report.

A 35-year-old woman presented with a solitary neurofibroma in an unusual presacral location without neurofibromatosis manifesting as bilateral chronic sciatica for 2 years. She was initially considered as having a giant right ovarian mass, but was referred with a prediagnosis of solitary giant sacral nerve sheath tumor. The initial differential diagnosis was based on neuroimaging. A right-sided J incision with the extraperitoneal approach provided good exposure and handling of the tumor bed. Almost total excision without neurological deficit was possible. The histological diagnosis was neurofibroma. Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease are quite rare. Intrapelvic tumors are often diagnosed at a later stage. Neuroimaging is very helpful to delineate this unusual site and the extent of tumor development, and to determine the appropriate surgical intervention. A clear understanding of retroperitoneal anatomy is essential for safe removal of such tumors. Complete resection is preferred to prevent local recurrence and malignant transformation. Although root section is inevitable, neurological deficit is unlikely.

Adult↗
PubMed1993

MR imaging of the sacral plexus: abnormal findings.

Patients with symptoms of sacral plexopathy, radiculopathy, or sciatica may have disease originating in the neural elements, sacrum, bony pelvis, pelvic soft tissues, or gluteal region. As illustrated in our companion article, the sacral plexus and surrounding structures can be visualized in detail with MR imaging. This pictorial essay illustrates the MR findings in a number of abnormalities affecting the sacral plexus. Multiplanar MR examination is particularly helpful in defining neural levels of involvement by disease in this region.

Abscess↗
PubMed2004

Sonographic characteristics of peripheral nerve sheath tumors.

OBJECTIVE: We have found variability in the sonographic appearance of peripheral nerve sheath tumors. The purpose of this study was to characterize the sonographic appearances of pathologically proven peripheral nerve sheath tumors. CONCLUSION: Peripheral nerve sheath tumors are often hypoechoic with posterior acoustic enhancement and so may simulate a ganglion cyst. The presence of intrinsic blood flow on color Doppler sonography and peripheral nerve continuity suggests the diagnosis of peripheral nerve sheath tumor. Sonography cannot reliably distinguish neurofibromas from schwannomas.

Adolescent↗
PubMed1993

Orbital malignant peripheral nerve sheath tumors. Treatment with surgical resection and radiation therapy.

A series of three patients with primary orbital malignant peripheral nerve sheath tumors (MPNST) is presented. Two of our patients who were treated with surgery and postoperative radiotherapy are free of tumor recurrence. The third patient showed a good response to radiation therapy. While surgical excision remains the mainstay of therapy, our patients demonstrate the usefulness of adjuvant radiation therapy in this condition.

Adult↗
PubMed2001

Intraneural perineurioma of the common peroneal nerve. Case report and review of the literature.

Intraneural perineurioma, or localized hypertrophic mononeuropathy (LHM), is a focal lesion that produces a slowly progressive mononeuropathy in a peripheral nerve. The authors describe the clinical presentation, magnetic resonance (MR) neurography characteristics, and pathological characteristics of a perineurioma involving the peroneal nerve. Although there has been much debate surrounding the cause of this lesion, a literature review supports the argument that this is a neoplastic lesion, best referred to as intraneural perineurioma. Surgical management includes excision to prevent progression of palsy and placement of a nerve graft if clinically indicated. A 28-year-old woman presented with a 2-year history of progressive painless right peroneal nerve palsy. Magnetic resonance neurography revealed a right common peroneal nerve mass. At surgery, the mass was easily excised, leaving significant nerve fascicles intact. Intraoperative biopsy was not performed nor was a nerve graft placed. Pathological investigation demonstrated onion bulb-shaped whorls consistent with the appearance of intraneural perineurioma; immunochemical analysis confirmed the diagnosis. A review of the literature supports the argument that perineurioma, or LHM, is a neoplastic process, making "intraneural perineurioma" the most appropriate name. The authors also demonstrate the utility of MR neurography in the identification isolated nerve tumors and review the surgical management of this lesion.

Adult↗
PubMed2004

Primary malignant melanoma of the cervical spinal nerve root.

The authors report on a case of primary malignant melanoma of the 7th cervical spinal nerve root in a 45-year-old woman. Neuro-radiological features of this extra-dural mass were suggestive of a nerve sheath tumor. The lesion underwent total gross resection through the anterolateral approach. The patient's postoperative course was uneventful. Histopathological investigation confirmed malignant melanoma. There was no evidence of tumor recurrence or other melanotic lesions on regular follow-up examinations until the postoperative eighth month. When treating a common, benign-looking lesion of the cervical spinal nerve root, surgeons should be aware of the potential to encounter such a malignant tumor.

Cervical Vertebrae↗
PubMed2000

A new human malignant peripheral nerve sheath tumour-cell line, HS-sch-2, harbouring p53 point mutation.

Only a few human malignant peripheral nerve sheath tumour (MPNST)-cell lines have been reported, and their characteristics have not been fully established. In this study, we established a new human cell line, HS-Sch-2, from an MPNST of the ordinary type which arose in a 54-year-old woman without von Recklinghausen's disease. This cell line was characterized by chromosome analysis, immunohistochemistry, ultrastructural examination, and direct sequencing of the p53 gene. The HS-Sch-2 cells have grown for more than 48 months in vitro, and exhibited hypotriploid karyotypes with complex chromosome abnormalities lacking a specific pattern. Histological features of the heterotranplanted nude mouse tumours were essentially the same as those of the original MPNST, with positive reactions for S-100 protein and neuron-specific enolase but not for epithelial membrane antigen, fibronectin or CD34. Ultrastructural examination in vivo revealed intricate interdigitation of long cytoplasmic processes and basal lamina-like structures. In addition, direct sequencing of the p53 gene detected a point mutation from CGT to CAT at codon 273 in exon 8. This HS-Sch-2 cell line, which exhibits distinctive morphological characteristics of MPNST and a p53 point mutation, will be useful for biological and pathological investigations of MPNST.

Animals↗