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Expression of Trk tyrosine kinase receptor is a biologic marker for cell proliferation and perineural invasion of human pancreatic ductal adenocarcinoma.

Pancreatic ductal adenocarcinoma (PDAC) is a widely known severe malignancy with a poor prognosis. Perineural invasion extending to the extra-pancreatic nerve plexus, a significant concern in the treatment is frequently present in this cancer. We analyzed immunohistochemical expression of neurotrophins (NGF, BDNF, NT-3) and the cognate receptors, Trk tyrosine kinase receptor family (TrkA, B, C) and p75NGFR in 28 surgically resected PDAC specimens. A comparative study between several clinicopathologic factors and Trk receptors revealed a significant correlation between increased expression of TrkA and cancer proliferation, as well as TrkC and cancer invasion, including venous and perineural invasion. The present findings revealed a novel mechanism in PDAC progression that is mediated via a NTs-Trk interaction.

Adenocarcinoma↗

[Intraneural perineurioma: an unusual nerve sheath tumor].

We report a case of intraneural perineurioma, developed in the left cubital nerve, in a five-year old girl. This intraneural tumor is rare and shows a typical histologic appearance: concentric whorls of perineurial cells EMA+, PS100-, around nerve fibers. This tumor must be distinguished from extraneural or soft tissue perineurioma, also composed of perineurial cells, with distinct clinical presentation and histological appearance.

Child, Preschool↗

Tumors of the soft tissues composed of large eosinophilic cells.

Soft tissue neoplasms composed of large eosinophilic cells include benign and malignant tumors with different degrees of biological aggressiveness. The main histotypes discussed in this review are the heterogeneous group of benign and malignant granular cell tumors with neural and non-neural differentiation, alveolar soft part sarcomas, rhabdomyomas, and rhabdomyosarcomas. The salient anatomic, clinical, morphological, and immunophenotypic features in differential diagnosis with metastatic melanomas, carcinomas, and paragangliomas are discussed separately for each histotype.

Adenoma, Oxyphilic↗

MR imaging of neoplasms of the lumbar spine.

MR imaging has evolved as the primary modality for imaging spinal neoplasms; however, neoplasms must be distinguished from unusual appearances of degenerative lesions. This article reviews MR imaging findings of a variety of neoplastic lesions of the lumbar spine. For example, extradural, metastatic tumors are the most common; nerve sheath tumors are the most common intradural extramedullary tumors; and ependymoma accounts for most of the tumors arising from the filum terminale.

Cauda Equina↗

Malignant peripheral nerve sheath tumors in children: a single-institution twenty-year experience.

A retrospective series of pediatric patients with localized malignant peripheral nerve sheath tumors (MPNST) treated during a 20-year period at one institution is reported. Between 1976 and 1996, 24 consecutive children were treated by a multimodality approach. Conservative surgery was the treatment of choice: primary radical surgery was performed in 10. Postoperative radiotherapy was administered in 12 and adjuvant chemotherapy in 19. Eight patients were alive without evidence of disease, six in first complete remission and two in second complete remission, after a median follow-up of 230 months. The 10-year event-free survival (EFS) and survival were 29% and 41%, respectively. Survival was 80% for the patients who underwent radical surgery, and 14% for the others; 71% for patients with tumors smaller than 5 cm, and 29% for those with tumors 5 cm or larger. Local recurrence was the major cause for treatment failure (13 of 17; 76%); the rate of local relapse was 33% v 75% in patients who either received or did not receive radiotherapy. Complete surgical excision remains the most effective treatment for MPNST and represents the main prognostic factor along with tumor size. Radiotherapy seems to play a role in achieving local control, whereas the role of chemotherapy is uncertain.

Adolescent↗

[Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component].

An exceptional case of malignant peripheral nerve sheath tumor with striated muscle differentiation and glandular component is reported, in a 52-year old man. This tumor measured 8 cm in diameter, and was localized in the chest wall, infiltrating the skeletal muscle. The mesenchymal portion of the tumor was composed mostly of spindle cells arranged in interlacing fascicles. Between these fascicles, there were large cells with abundant eosinophilic cytoplasm and clear elongated nucleus. Immunohistochemical study demonstrated cytokeratin, EMA and CEA expression in the glandular component and S100 protein expression in the major portion of the mesenchymal component. The large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, alpha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resection. Histological and immunohistochemical features were identical. We think that positivity of neoplastic striated muscle cells with alpha-SM actin reflects an early differentiation phase of these cells.

Carcinoembryonic Antigen↗

Leptomeningeal metastases from ethmoid sinus adenocarcinoma: clinico-radiological correlation.

A patient with sinonasal adenocarcinoma is presented with leptomeningeal metastases affecting multiple cranial nerves and spinal nerve roots. Head and neck cancer is known to be an extremely rare source for leptomeningeal metastatic spread. The cranial nerves, the spinal cord and roots and the cerebral hemispheres can be affected in case of leptomeningeal metastatic spread. Examination of the CSF is the hallmark of the diagnosis if leptomeningeal metastatic spread is suspected, but this case illustrates that the combination of specific clinical features on one hand and specific lesions on the Gd-enhanced T1-weighted MRI study on the other hand is reliable enough to make a presumed diagnosis if the CSF analysis remains negative. We suggest that in our patient direct leptomeningeal spread occurred through the cribriform plate to the CSF, followed by further spread in a gravity dependent way.

Adenocarcinoma↗

Pelvic ependymoma arising from the small bowel.

A 37-year-old woman underwent resection of an abdominal tumour which was adherent to the wall of the ileum. The diagnosis of an ependymoma was supported by evidence of typical perivascular pseudorosettes which stained positive for glial fibrillary acidic protein and contained abundant intermediate filaments within the elongated processes by electron microscopy. Flow cytometric study showed a diploid population of tumour cells. This is the first case of an ependymoma arising from the small bowel without any connection to the genital tract, the omentum or with the sacroccygeal area. As is the case with other unusual and ectopic localisations of ependymomas, prognosis of this tumour is difficult to evaluate.

Adult↗

Sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh.

Malignant peripheral nerve sheath tumour (MPNST) is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot.

Adult↗

[Carpal tunnel syndrome with an unusual cause: a malignant nerve sheath tumor of the median nerve].

We report on the follow-up of a patient who developed symptoms suggestive of carpal tunnel syndrome. Symptoms were however atypical with involvement of the nondominant hand and with selective, fascicular, electroneurographic changes. During the surgical decompression of the median nerve at the wrist a tumor was found, corresponding to an isolated malignant peripheral nerve sheath tumor (MPNST) of mild type. A course of local radiation therapy was completed, with no sign of recurrence, and a normalization of the serum level of neurone specific enolase.

Carpal Tunnel Syndrome↗

[Symptomatic neuroma of the sural nerve a rare complication of the harvesting of the nerve for grafting: case report].

The harvesting of the sural nerve for autologous grafting usually produces symptoms of low intensity and short duration. In rare occasions that procedure may lead to the formation of a symptomatic neuroma in the proximal stump. The symptoms of this complication are usually controlled by clinical treatment and the surgical procedure is left for the therapeutic failures. In this paper we present the case of a patient with a sural nerve neuroma submitted to surgical treatment by a variant of the centro-central anastomosis technique, developed for the treatment of amputation neuromas, that resulted in remission of the painful symptomatology. The different options of surgical treatment for this rare entity are discussed.

Anastomosis, Surgical↗

[Effect of histologic classification on surgical treatment of peripheral nerve tumors].

The influence of histological classification on the options of the peripheral nerve tumors surgery Peripheral nerve sheath tumors (PNST) constitute significant part of all tumors of peripheral nerves. In 1998-2000 years we have operated 7 such patients--3 females, 4 males in our department. The definite diagnosis was made by histology. Total removal was performed in all patients. The nerve continuity was preserved in 4 cases (3x neurofibroma, 1x schwannoma) and nerve had to be disconnected in 3 cases (1x neurobibroma, 1x plexiform neurofibroma as a part of von Recklinghausen disease (VRD), 1x malignant schwannoma). Good neurological outcome was in patients with preserved nerve continuity. Out of 3 patients with the need of nerve disconnection, graft and complete reinervation was performed in case of benign neurofibroma, but in the patient with von Recklinghausen disease and in the patient with malignant schwannoma the permanent leasion of nerve remained. We can conclude that histological classification directly predetermine the options of the peripheral nerve tumors surgery.

Adult↗