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[Malignant neoplasms of the peripheral nerve sheaths with cellular immunophenotype of perineural cells (perineural sarcoma?)].

2 cases of soft tissue malignant tumors formally related to the group of spindle cell sarcoma are described. The majority of cells expressed an epithelial membranous antigen and vimentin but were S-100 protein unreactive, i.e. had a phenotype characteristic of perineural cells. Monoclonal antibodies to 5 types of filaments protein of intermediate type, neurone specific enolase and collagen type I, II, III, IV, V were also used as well as electron microscopic investigation. Problems of differential diagnosis from schwannomas, fibrosarcomas, monophasic spindle cell "synovial sarcoma" and validity of integrating these tumors into an independent variety of malignant peripheral nerve sheath tumors are discussed.

Adult↗

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↗

MR imaging of benign peripheral nerve sheath tumors.

In a retrospective, nonblind review of MR imaging of 15 benign peripheral nerve neoplasms in 13 patients, the signal pattern of the tumors (including contrast-enhanced images) and stage were assessed. One lesion was subcutaneous, 9 intramuscular, 2 intermuscular and 3 extracompartmental. One lesion was located to the trunk, 5 to the upper extremity and 9 to the lower. The signal on T1-weighted spin-echo images was homogeneous isointense compared to adjacent muscle in 11 lesions and in 2 slightly hyper- and in 2 slightly hypointense. T2-weighted spin-echo images, acquired in all but one examination, showed a hyperintense signal, homogeneous in 8 and centrally inhomogeneous in 6 lesions. Postcontrast T1-weighted images of 11 lesions showed a strong signal, with an inhomogeneous enhancement in the center of the lesion similar to that obtained in T2-weighted images. In 2 cases there were signal characteristics indicating bleeding in the tumor. In one lesion both the nonenhanced and contrast-enhanced T1-weighted images showed a hypointense signal in the tumor center suggestive of intramuscular myxoma. All lesions were well delineated without reactive edema. In all cases, anatomic tumor location was correctly assessed. Although the findings were not pathognomonic for neurinoma, MR imaging provided valuable information confirming the clinical and cytologic assessments.

Adult↗

CD44-independent hepatocyte growth factor/c-Met autocrine loop promotes malignant peripheral nerve sheath tumor cell invasion in vitro.

Malignant peripheral nerve sheath tumors (MPNSTs) are invasive peripheral nerve neoplasms that express both the receptor tyrosine kinase c-Met and its ligand hepatocyte growth factor (HGF). The combined expression of these proteins has been implicated in tumor cell growth and metastasis. However, HGF/c-Met autocrine activity requires the presence of a serine protease, the HGF activator (HGFA), and, in some cells, the CD44 transmembrane glycoprotein. Here, we found that HGFA, HGF, c-Met, and CD44 are coexpressed in MPNSTs but their localization did not correlate with increased cell proliferation. The ST8814 MPNST cell line also expresses all of these proteins, can convert pro-HGF to active HGF, and exhibits constitutive c-Met phosphorylation. Blocking c-Met activity or expression inhibits the invasive behavior of these cells but not their proliferation. Interestingly, although a CD44 splice variant contributes to MPNST cell invasion and interacts with c-Met and HGF in ST8814 cells, it is not required for c-Met activation. These data indicate that an HGF/c-Met autocrine loop can promote MPNST invasion through a CD44-independent mechanism and suggest that c-Met, HGFA, and HGF are potential molecular targets to inhibit MPNST metastasis.

Autocrine Communication↗

Lesser known aquarium fish tumor models.

The repeated use of particular species for experimental oncology in fish increases their future value by accumulating background information for these models and justifies the establishment of genetic stock centers. However, the wide diversity that exists within the class Osteichthyes and Chondrichthyes suggests that the ideal surrogate models for studying some types of neoplasms might be found among lesser known species. To help assess cultured fish as surrogates for some other types of human neoplasia, we examined cases in the archives of the Registry of Tumors in Lower Animals and reviewed reports in the literature. Spontaneous and induced neoplasms originating from a spectrum of cell types were seen in more than 215 fish species commonly raised in aquaria or cultured for study among 69 families. Prominent families include the Poeciliidae (livebearers), Cyprinidae (carps and minnows), Cichlidae (cichlids), Cyprinodontidae (killifish), Characidae (tetras), Adrianichthyidae (medakas), Aplocheilidae (rivulins), and Salmonidae (salmon and trout). The following are examples of potential fish tumor models that have received less consideration than some others: papilloma and carcinoma of the urinary bladder in oscar (Astronotus ocellatus); osteogenic neoplasms, peripheral nerve sheath tumors, and ependymoblastoma in coho salmon fingerlings (Oncorhynchus kisutch); and nephroblastoma resembling Wilms' tumor in Japanese eels (Anguilla japonica).

Journal Article↗

Ciliary body schwannoma.

PURPOSE: Intraocular schwannomas are very rare, benign, peripheral nerve neoplasms. The authors report a case of ciliary body schwannoma. METHOD: A 39-year-old Korean woman presented with slowly decreasing visual acuity and proptosis of the right eye for 4 years. At the time of her visit, the eyeball protruded and deviated laterally. She could not sense light with her right eye. We enucleated the eyeball. The enucleated eyeball with tumor was examined histopathologically, immunohistochemically, and under electron microscope. RESULTS: Microscopic examination revealed spindle cells and characteristic Antoni type A and B areas with Verocay bodies. Immunohistochemical study showed tumor cell expressions of S-100 and vimentin, but other immunohistochemical studies were negative. Electron microscopic examination demonstrated Luse body. CONCLUSIONS: We diagnosed the tumor as a schwannoma arising from the ciliary body on the basis of the above mentioned results. Schwannomas are very rare intraocular neoplasms, but they are benign so we should differentiate from other intraocular neoplasms.

Adult↗

Surgical outcomes of 654 ulnar nerve lesions.

OBJECT: In this article the authors present a retrospective analysis of 654 surgical outcomes in patients with ulnar nerve entrapments, injuries, and tumors during a 30-year period. METHODS: Data were gathered between 1968 and 1998 at Louisiana State University Health Sciences Center. Mechanisms of injuries or lesions included 460 entrapments at the elbow level (70%), 76 lacerations (12%), 52 stretches/contusions (8%), 34 fractures/dislocations (5%), 12 gunshot wounds (2%), two injection-induced injuries (0.3%), and 13 nerve sheath tumors (2%). In cases of entrapment, direct operative recordings uniformly demonstrated a slowing of conduction at the elbow, even in cases in which preoperative noninvasive studies had been nondiagnostic. Intraoperative electrical "inching" studies also demonstrated significant conduction abnormalities that lie just proximal to and through the olecranon notch rather than distal, beneath the flexor carpi ulnaris muscle. There were only eight exceptions to this. Lesions not in continuity due to the injury required primary or secondary end-to-end sutures or graft repair. Aided by intraoperative nerve action potential recording, lesions in continuity received either external or internal neurolysis and split repair or resection followed by end-to-end suture or graft repair. Functional recoveries of Grade 3 or better were seen in 81 (92%) of 88 patients who underwent neurolysis, 42 (72%) of 58 patients who received suture repair, and 24 (67%) of 36 patients who received graft repair. Nevertheless, fewer Grade 4 or 5 recoveries were reached than those seen in patients with radial or median nerve injuries. Nerve sheath tumors were resected with preservation of preoperative function in five of seven patients. CONCLUSIONS: Although difficult to obtain, useful functional recovery can be achieved with proper surgical management of ulnar nerve entrapments and injuries.

Action Potentials↗