Peripheral Nerve Tumors

Smooth muscle tumors Peripheral nerve sheath tumors Paraganglioma Solitary fibrous tumor Follicular dendritic cell tumor Langerhans cell histiocytosis Secondary tumors... 

NF1 neurofibromatosis type 1 benign and malignant peripheral nerve sheath tumors... 

Infections can damage nerves directly, or via exotoxins 621 Antibody- and cell-mediated mechanisms of neuropathy 621 Immune mechanisms contribute to paraneoplastic neuropathies 623 Toxins and hormone deficiency states are frequent causes of neuropathy 623 Many genetically determined neuropathies are now recognized 624 Neurofibromatosis is a frequent cause of peripheral nerve tumors 625... 

Neurofibromatosis is a frequent cause for peripheral nerve tumors. 

Marcus et al. reported that anti GM1 ganglioside antibody can be used as a T cell marker (11, 12, 13). In collaboration with Tada s group of the University of Tokyo we very recently found that antibody to asialoganglio-side GM1 specifically labels mouse natural killer cells and that the antibody selectively kills the cells in the presence of complement (14). Natural killer cells are known by their specific ability to attack tumor cells. We also have evidence that certain gangliosides are capable of eliciting autoimmune diseases experimentally which we called the ganglioside syndrome (JJ5). For example, some brain gangliosides produce autoimmune lesions in peripheral nerve in susceptible rabbits. This may correspond to the human... 

Tetraethyl- c8h20nci Y. Non-specific Neurofibromas peripheral nerve sheath tumor cell... 

Stonecypher MS, Byer SJ, Grizzle WE, Carroll SL. 2005. Activation of the neuregulin-1/ErbB signaling pathway promotes the proliferation of neoplastic Schwann cells in human malignant peripheral nerve sheath tumors. Oncogene 24 5589-5605. 

Wojtkowiak, J., et al. (2008). Induction of apoptosis in Neurofibromatosis Type 1 malignant peripheral nerve sheath tumor cell hnes by a combination of novel farnesyl transferase inhibitors and lovastatin. J Pharm Exp Ther 326 1-11. 

Of 22 patients given vindesine 2 mg/m on 2 consecutive days weekly, four developed a peripheral neuropathy with paresthesia, and five developed muscle weakness with loss of deep tendon reflexes (49). Neuropathies were pronounced at sites of pre-existing nerve damage. If a tumor had previously damaged peripheral nerves, or if a chordotomy had been performed, the paresthesia that developed at that site was often painful. Neurotoxicity... 

Emst H, Rittinghausen S, Wahnschaffe U, et al. 1987. Induction of malignant peripheral nerve sheath tumors in European hamsters with 1,1 -dimethylhydrazine (UDMH). Cancer Lett 35 303-311. 

Fetal fibroblasts myometrium vascular smooth muscle fetal myocardium vascular endothelial cells selected reticulum cells of lymphatic organs some CD30+ lymphoid cells (rarely) selected leiomyosarcomas, epithelioid hemangioendotheliomas, epithelioid angiosarcomas, primitive neuroectodermal tumors and Ewing s sarcomas, malignant peripheral nerve sheath tumors, and clear cell sarcomas... 

Anti-epithelial membrane antigen (M) Dako 1 400 NT Recognition of epithelioid sarcoma, synovial sarcoma, and selected peripheral nerve sheath tumors... 

Anti-CD99 (M) Dako 1 20 MWER Recognition of virtually all primitive neuroectodermal tumors and Ewing s sarcomas labels roughly 50% of synovial sarcomas and malignant peripheral nerve sheath tumors also present in lymphoblastic lymphomas/ leukemias presenting in soft tissue... 

KER, keratin, detected by a mixture of GAMS.2, MAK-6, and AEl /AE3 EMA, epithelial membrane antigen VIM, vimentin DES, desmin MSA, muscle-specific actin SMA, smooth muscle (alpha isoform) actin GALD, h-caldesmon S-IOOP, S-100 protein OCN, osteocalcin LM, laminin UL, Ulex europaeus I lectin binding FS, fibrosarcoma SGRMS, spindle cell rhabdomyosarcoma LMS, leiomyosarcoma MPNST, malignant peripheral nerve sheath tumor MSS, monophasic spindle cell synovial sarcoma SCAS, spindle cell angiosarcoma KS, Kaposi s sarcoma FOS, fibroblastic osteosarcoma. 

The Wilms tumor-1 (WTl) gene is located on the short arm of chromosome 11 (llpl3). It encodes a protein that is a critical determinant of urogenital development and is expressed in >80% of nephroblastomas. With regard to osseous and soft tissue tumors, WTl protein is not a specific marker and is potentially present in angiosarcoma, malignant peripheral nerve sheath tumor, synovial sarcoma, osteosarcoma, myxoid liposarcoma, and clear-cell sarcoma.Nevertheless, it is most often utilized (in a structured panel with other immunodeter-minants) as an adjunctive indicator of endothelial differentiation in diagnostic practice. ... 

Keratins are regularly found in synovial sarcoma, chordoma, parachordoma, epithelioid sarcoma, and adamantinoma. They are seen less commonly in leiomyosarcoma, malignant peripheral nerve sheath tumors, epithelioid angiosarcoma, and polyphenotypic tumors such as PNET. 

FIGURE 4.17 CD57 in malignant peripheral nerve sheath tumor (MPNST). Reactivity for S-100 protein, CD57, myelin basic protein, or collagen type IV is observed in greater than 85% of MPNSTs. 

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