Malignant peripheral nerve sheath tumor

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

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-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. 

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. 

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. 

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. 

Eolpe AL, Schmidt RA, Ghapman D, et al. Poorly differentiated synovial sarcoma immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors. Am J Surg Pathol. 1998 22 673-6S2. 

Smith TA, Machen SK, Fisher C, et al. Usefulness of cytokeratin subsets for distinguishing monophasic synovial sarcoma from malignant peripheral nerve sheath tumor. Am J Clin Pathol. 1999 112 641-648. 

King R, Busam K, Rosai J. Metastatic malignant melanoma resembling malignant peripheral nerve sheath tumor Report of 16 cases. Am J Surg Pathol. 1999 23 1499-1505. 

Laskin WB, Weiss SW, Bratthauer GL. Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol. 1991 15 1136-1145. 

Hoang MP, Sinkre P, Albores-Saavedra J. Expression of protein gene product 9.5 in epithelioid and conventional malignant peripheral nerve sheath tumors. Arch Pathol Lab Med. 2001 125 1321-1325. 

The ability of melanoma to simulate the appearance of various soft tissue sarcomas is also well documented. These include malignant peripheral nerve sheath tumors, gastrointestinal stromal tumors, epithelioid angiosarcoma, rhabdoid tumors, osteosarcomas, and primitive neuroectodermal tumors, to name a few. The detailed immunophenotypic properties of those lesions are provided elsewhere in this book. However, none of them manifests reactivity for gplOO-related melanocytic markers or tyrosinase, making those markers essential to the differential diagnostic process. 

Wick MR. Malignant peripheral nerve sheath tumors of the skin. Mayo Clin Proc. 1990 65 279-282. 

Olsen SH, Thomas DC, Lucas DR. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. Mod Pathol. 2006 19 659-668. 

Shimada S, Tsuzuki T, Kuroda M, et al. Nestin expression as a new marker in malignant peripheral nerve sheath tumors. Pathol Int. 2007 57 60-67. 

Noncarcinomatous lesions Plasma cell tumors, L H cells of Hodgkin lymphoma, few cells of non-Hodgkin lymphoma, anaplastic large cell lymphoma, malignant peripheral nerve sheath tumors, synovial sarcoma, leiomyosarcoma... 

Ordonez NG, Tornos C. Malignant peripheral nerve sheath tumor of the pleura with epithelial and rhabdomyoblastic differentiation report of a case clinically simulating mesothelioma. Am J Surg Pathol. 1997 21 1515-1521. 

Epithelioid synovial sarcoma is reactive for keratin in 100% of cases and Ber-EP4 in 90% of cases. Epithelioid malignant peripheral nerve sheath tumor is reactive for S-1 00 protein in 80% of cases. 

FIGURE 13.31 Malignant peripheral nerve sheath tumor of the dermis in a patient with neurofibromatosis (A). The tumor shows multifocal staining for S-100 protein (B). 

Leroy K, Dumas V, Martin-Garcia N, et al. Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1 a clinicopathologic and molecular study of 17 patients. Arch Dermatol. 2001 137 908-913. 

Gray MH, Rosenberg AE, Dickersin GR, et al. Glial fibrillary acidic protein and keratin expression by benign and malignant peripheral nerve sheath tumors. Hum Pathol. 1989 20 1089-1096. 

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