Nerve sheath tumors

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

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. 

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. 

Nerve sheath tumors may occasionally have GFAP- and EMA-positive cells. 

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. 

Determinants associated with non-neoplastic Schwann cells and benign peripheral nerve sheath tumors are frequently detected in MPNSTs. They show reactivity for... 

S-100 protein reactivity generally should be considered as melanomas. This is especially true if concomitant positivity is obtained for HMB-45, HMB-50, tyrosinase, or MART-1, because the latter four markers are only rarely associated with nerve sheath tumors. Despite the production of collagen type IV by nerve sheath cells, the immunohistochemical detection of this marker has limited value in the diagnosis of schwannian neoplasms because cells with smooth muscle, endothelial, and myo-fibroblastic differentiation may also synthesize it. 

Epithelioid Malignant Peripheral Nerve Sheath Tumors... 

Pleomorphic Malignant Peripheral Nerve Sheath Tumor... 

Malignant peripheral nerve sheath tumor (MPNST) may likewise assume a pleomorphic appearance. In most instances, that tumor variant shows patterns of reactivity for S-100 protein, CD56, CD57, and collagen type IV that are superimposable with those of better differentiated (spindle cell) MPNSTs." In the absence of focal S-100 protein expression and foci with conventional appearances, it is very difficult (if not impossible) to render this diagnosis with certainty. Unlike spindle cell variants of MPNST, pleomorphic subtypes have not been shown to be desmin-reactive. 

Round cell liposarcoma is a morphologic variant of high grade MLPS that may resemble extraskeletal chondrosarcoma, cellular peripheral nerve sheath tumors, or even metastatic poorly differentiated carcinomas. S-100 protein and vimentin are seen in all these tumor types. MPNSTs that are similar microscopically to round cell liposarcoma may express CD56 or myelin basic protein, unlike adipocytic lesions. 

Wick MR, Swanson PE, Scheithauer BW, et al. Malignant peripheral nerve sheath tumor An immunohistochemical study of 62 cases. Am Clin Pathol. 1987 87 425-433. 

Giangaspero E, Eratamico EC, Ceccarelli C, et al. Malignant peripheral nerve sheath tumors and spindle cell sarcomas An immunohistochemical analysis of multiple markers. Appl Pathol. 1989 7 134-144. 

Kawahara E, Oda Y, Ooi A, et al. Expression of glial fibrillary acidic protein (GFAP) in peripheral nerve sheath tumors A comparative study of immunoreactivity of GFAP, vimentin, SlOO protein, and neurofilament in 38 schwannomas and 18 neurofibromas. Am J Surg Pathol. 1988 12 115-120. 

Memoli VA, Brown EF, Gould VE. Glial fibrillary acidic protein (GFAP) immunoreactivity in peripheral nerve sheath tumors. Ultrastrucl Pathol. 1984 7 269-275. 

Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST) A clinicopathologic, immunohistochemical, and ultrastructural study of seven cases. Am J Surg Pathol. 1998 22 1368-1378. 

Abe S, Imamura T, Park P, et al. Small round cell type of malignant peripheral nerve sheath tumor. Mod Pathol. 1998 11 747-753. 

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