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Gaurav Gupta and Allen Maniker

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas of ectomesenchymal origin. The World Health Organization coined the term MPNST to replace previous heterogeneous and often confusing terminology, such as “malignant schwannoma,” “malignant neurilemmoma,” “neurogenic sarcoma,” and “neurofibrosarcoma.” Malignant peripheral nerve sheath tumors arise from major or minor peripheral nerve branches or sheaths of peripheral nerve fibers, and are derived from Schwann cells or pluripotent cells of neural crest origin.

The Schwann cell is thought to be the major contributor to the formation of benign as well as malignant neoplasms of the nerve sheath. While this fact remains essentially true, the identity of cell of origin of the MPNST remains elusive, and has not yet been conclusively identified. It has been suggested that these tumors may have multiple cell line origins. In this review, the authors discuss the epidemiology, diagnosis, management, and treatment of MPNSTs.

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Shih-Shan Lang, Eric L. Zager, Thomas M. Coyne, Raj Nangunoori, J. Bruce Kneeland and Katherine L. Nathanson

. f: Neurofilament stain (NF-TA51) highlighting sparse encompassed axonal processes. g: INI-1 nuclear staining is preserved in the tumor cells, including the schwannoma tumorlets depicted here. Bar = 100 μm. Discussion Peripheral nerve sheath tumors include neurofibromas, schwannomas, and perineuriomas, all of which are generally considered discrete entities. 2 , 11 , 23 In recent years, there have been 66 reported cases of hybrid PNSTs, which include neurofibroma/perineurioma, schwannoma/perineurioma, and neurofibroma/schwannoma hybrid tumors. In the

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Samuel L. Barnett, Michael J. Wells, Bruce Mickey and Kimmo J. Hatanpaa

subtypes in malignant melanoma, and benign and malignant peripheral nerve sheath tumors . J Cutan Pathol 35 : 1014 – 1019 , 2008 12 Ohsie SJ , Sarantopoulos GP , Cochran AJ , Binder SW : Immunohistochemical characteristics of melanoma . J Cutan Pathol 35 : 433 – 444 , 2008 13 Posther KE , Selim MA , Mosca PJ , Stanley WE , Johnson JL , Tyler DS , : Histopathologic characteristics, recurrence patterns, and survival of 129 patients with desmoplastic melanoma . Ann Surg Oncol 13 : 728 – 739 , 2006 14 Quinn MJ , Crotty KA

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Howard Landy, Lynn Feun, Arnold Markoe, Sherri Patchen, Joy Bruce, Justin Marcus and Allan Levi

M alignant peripheral nerve sheath tumors are difficult to control despite aggressive treatment, which may include amputation of an extremity. The available literature provides only limited guidance regarding management of this disease. In this report we describe the treatment and follow-up review of a patient with NF1 and a recurrent median nerve MPNST. The patient underwent intraarterial and intravenous chemotherapy followed by additional surgery for gross-total tumor removal and postoperative radiotherapy; extended tumor-free survival was achieved. A review

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Darryl Lau, Dominic H. Moon, Paul Park, Shawn Hervey-Jumper, Paul E. McKeever and Daniel A. Orringer

T he term “malignant peripheral nerve sheath tumor” (MPNST) was coined by the World Health Organization to describe tumors originating from the connective tissue surrounding peripheral nerves, and it replaced previous nomenclature, including malignant neurilemmoma, malignant schwannoma, and neurofibrosarcoma. These tumors are rare, with an incidence of 0.001% in the general population. 15 , 17 Approximately 20%–50% occur in association with neurofibromatosis Type 1, 6 , 10 , 17 and up to 11% arise secondary to previous radiation therapy (RT). 10 , 23

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Jacob Schwarz and Allan J. Belzberg

was performed using a retropelvic approach. The tumor was dissected free of the peroneal and tibial nerves but the posterior femoral cutaneous nerve was sacrificed. Pathological examination confirmed a malignant peripheral nerve sheath tumor arising from a neurofibroma. Postoperative Course Postoperative examination demonstrated Grade −5/5 minimal weakness in the distribution of the sciatic nerve. The mass recurred within 2 months postsurgery. The patient underwent two rounds of doxorubicin and ifosfamide chemotherapy, followed by repeated gross-total surgical

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Yaxiong Li, Fengshi Fan, Jianguo Xu, Jie An and Weining Zhang

M alignant peripheral nerve sheath tumors (MPNSTs) are uncommon types of soft tissue sarcomas that usually arise from peripheral nerve sheaths and rarely involve the spinal roots. These tumors are believed to originate from Schwann cells or pluripotent cells of the neural crest. The estimated incidence of MPNSTs in patients without a history of neurofibromatosis is very low, approximately 0.001%. 7 Malignant peripheral nerve sheath tumors tend to recur locally at the original sites and metastasize to the lungs, liver, lymph nodes, and bones. 10 In this

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Eamon J. McLaughlin, Gregory G. Heuer, Robert G. Whitmore, John K. Birknes, Jean Belasco, Daniel Sterman, David W. Low and Phillip B. Storm

T he WHO uses the term MPNST to describe tumors that were previously known as malignant schwannoma, neurogenic sarcoma, and neurofibrosarcoma. These tumors are rare with an incidence of 0.001% in the general population 5 and represent only 5% of malignant soft-tissue tumors. Malignant peripheral nerve sheath tumors primarily affect adults in the 3rd–6th decades of life. 3 Approximately 50% of these tumors are associated with patients with neurofibromatosis Type 1. 8 Malignant peripheral nerve sheath tumors are highly aggressive tumors and carry a poor

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Daipayan Guha, Benjamin Davidson, Mustafa Nadi, Naif M. Alotaibi, Michael G. Fehlings, Fred Gentili, Taufik A. Valiante, Charles H. Tator, Michael Tymianski, Abhijit Guha and Gelareh Zadeh

P eripheral nerve tumors may be of neuroectodermal origin, derived from the neural sheath, or of nonneural sheath origin. Benign peripheral nerve sheath tumors (BPNSTs) include neurofibromas and schwannomas, which collectively constitute 10%–12% of benign soft-tissue neoplasms. 19 , 28 , 29 Malignant PNSTs (MPNSTs) represent 5%–10% of all soft-tissue sarcomas and occur in 0.001% of the general population. 12 , 30 Both BPNSTs and MPNSTs may occur sporadically, or in association with autosomal dominant neurofibromatoses (NFs). Sixty percent of patients with

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Gavin P. Dunn, Konstantinos Spiliopoulos, Scott R. Plotkin, Francis J. Hornicek, David C. Harmon, Thomas F. Delaney and Ziv Williams

M alignant peripheral nerve sheath tumors account for 3%–10% of all soft-tissue sarcomas, 3 , 20 with an incidence in the general population of 0.001%. 14 These tumors arise from major peripheral nerves. They can affect the trunk, extremities, head, and neck 1 , 18 , 25 and often involve major elements of the brachial plexus. 28 Approximately 50% of tumors affect patients with NF1 1 , 10 , 28 with a lifetime incidence of up to 10% in patients with specific NF1 gene microdeletions. 6 The overall 5-year survival rate of patients with MPNSTs ranges