{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T00:15:14Z","timestamp":1768436114566,"version":"3.49.0"},"reference-count":45,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2000,2]]},"abstract":"<jats:p content-type=\"fine-print\">\u2713 Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. Patients with NF5 present with caf\u00e9-au-lait macules, freckles, and\/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain, neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.<\/jats:p>","DOI":"10.3171\/jns.2000.92.2.0342","type":"journal-article","created":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T17:33:09Z","timestamp":1242235989000},"page":"342-346","source":"Crossref","is-referenced-by-count":33,"title":["Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis"],"prefix":"10.3171","volume":"92","author":[{"given":"Jacob","family":"Schwarz","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Allan J.","family":"Belzberg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Pediatr Radiol","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1007\/s002470050082","article-title":"MR imaging differentiation of benign and malignant peripheral nerve sheath tumors: use of the target sign.","volume":"27","author":"Bhargava","year":"1997"},{"key":"b2","series-title":"Peripheral Neuropathy","first-page":"1623","article-title":"Peripheral nerve tumors","volume":"2","author":"Birch","year":"1993"},{"key":"b3","series-title":"Nat Genet","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1038\/ng0296-144","article-title":"Loss of NF1 results in activation of the Ras signaling pathway and leads to aberrant growth in haematopoietic cells.","volume":"12","author":"Bollag","year":"1996"},{"key":"b4","series-title":"Neurofibromatosis","first-page":"244","article-title":"Neurofibromatosis type 1 in a child of a parent with segmental neurofibromatosis (NF-5).","volume":"2","author":"Boltshauser","year":"1989"},{"key":"b5","series-title":"Ann Surg","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1097\/00000658-199109000-00015","article-title":"The role of multimodality therapy in soft-tissue sarcoma.","volume":"214","author":"Brennan","year":"1991"},{"key":"b6","series-title":"Arch Surg","doi-asserted-by":"publisher","first-page":"1289","DOI":"10.1001\/archsurg.1987.01400230075014","article-title":"Local recurrence in adult soft-tissue sarcoma. A randomized trial of brachytherapy.","volume":"122","author":"Brennan","year":"1987"},{"key":"b7","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"1457","DOI":"10.1056\/NEJM199011223232105","article-title":"Altered expression of the retinoblastoma gene product in human sarcomas.","volume":"323","author":"Cance","year":"1990"},{"key":"b8","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"1644","DOI":"10.1002\/1097-0142(19940315)73:6<1644::AID-CNCR2820730616>3.0.CO;2-V","article-title":"Preoperative and postoperative adjuvant combination chemotherapy for adults with high grade soft tissue sarcoma.","volume":"73","author":"Casper","year":"1994"},{"key":"b9","series-title":"Dermatology","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1159\/000247405","article-title":"Bilateral segmental neurofibromatosis.","volume":"185","author":"Cecchi","year":"1992"},{"key":"b10","series-title":"Am J Hum Genet","first-page":"484","article-title":"Somatic mosaicism in a patient with neurofibromatosis type 1.","volume":"58","author":"Colman","year":"1996"},{"key":"b11","series-title":"Cancer Res","first-page":"794","article-title":"Molecular abnormalities of mdm2 and p53 genes in adult soft tissue sarcomas.","volume":"54","author":"Cordon-Cardo","year":"1994"},{"key":"b12","series-title":"A Clinical, Pathological, and Genetic Study of Multiple Neurofibromatosis.","author":"Crowe","year":"1956"},{"key":"b13","series-title":"Ann Surg Oncol","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1007\/BF02307086","article-title":"Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience.","volume":"2","author":"de Cou","year":"1995"},{"key":"b14","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"2006","DOI":"10.1002\/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6","article-title":"Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.","volume":"57","author":"Ducatman","year":"1986"},{"key":"b15","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"867","DOI":"10.3171\/jns.1996.84.5.0867","article-title":"Acute presentation of a neurogenic sarcoma in a patient with neurofibromatosis type 1: a pathological and molecular explanation. Case report.","volume":"84","author":"Feldkamp","year":"1996"},{"key":"b16","series-title":"Arch Surg","doi-asserted-by":"publisher","first-page":"1285","DOI":"10.1001\/archsurg.1992.01420110027007","article-title":"Management of small soft-tissue sarcoma of the extremity in adults.","volume":"127","author":"Geer","year":"1992"},{"key":"b17","series-title":"JAMA","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1001\/jama.1997.03550010065042","article-title":"The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2.","volume":"278","author":"Gutmann","year":"1997"},{"key":"b18","series-title":"J Am Acad Dermatol","doi-asserted-by":"publisher","first-page":"864","DOI":"10.1016\/S0190-9622(97)80013-8","article-title":"Segmental neurofibromatosis: case reports and review.","volume":"37","author":"Hager","year":"1997"},{"key":"b19","series-title":"Am J Clin Pathol","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1093\/ajcp\/106.3.282","article-title":"p53 expression in neurofibroma and malignant peripheral nerve sheath tumor. An immunohistochemical study of sporadic and NF1- associated tumors.","volume":"106","author":"Halling","year":"1996"},{"key":"b20","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"1253","DOI":"10.1002\/1097-0142(19900915)66:6<1253::AID-CNCR2820660627>3.0.CO;2-R","article-title":"Malignant peripheral nerve sheath tumors of the buttock and lower extremity. A study of 43 cases.","volume":"66","author":"Hruban","year":"1990"},{"key":"b21","series-title":"Brain","doi-asserted-by":"publisher","first-page":"1355","DOI":"10.1093\/brain\/111.6.1355","article-title":"Von Recklinghausen neurofibromatosis. A clinical and population study in southeast Wales.","volume":"111","author":"Huson","year":"1988"},{"key":"b22","series-title":"Virchows Archiv A","first-page":"19","article-title":"Immunohistochemical and molecular analysis of p53, MDM2, proliferating cell nuclear antigen and Ki67 in benign and malignant peripheral nerve sheath tumours.","volume":"427","author":"Kindblom","year":"1995"},{"key":"b23","series-title":"Proc Natl Acad Sci USA","doi-asserted-by":"publisher","first-page":"820","DOI":"10.1073\/pnas.68.4.820","article-title":"Mutation and cancer: statistical study of retinoblastoma.","volume":"68","author":"Knudson","year":"1971"},{"key":"b24","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"1403","DOI":"10.1056\/NEJM199411243312102","article-title":"Neurofibromatosis type 1 due to germ-line mosaicism in a clinically normal father.","volume":"331","author":"L\u00e1zaro","year":"1994"},{"key":"b25","series-title":"Genes Chromosom Cancer","doi-asserted-by":"publisher","first-page":"250","DOI":"10.1002\/gcc.2870100405","article-title":"TP53 mutations are frequent in malignant NF1 tumors.","volume":"10","author":"Legius","year":"1994"},{"key":"b26","series-title":"Eur J Surg Oncol","doi-asserted-by":"publisher","first-page":"474","DOI":"10.1016\/S0748-7983(95)96713-3","article-title":"Soft tissue sarcoma therapy: prospects for future studies.","volume":"21","author":"Lejeune","year":"1995"},{"key":"b27","series-title":"N Engl J Med","first-page":"684","article-title":"Neurofibromatosis 2. (Bilateral acoustic neurofibromatosis.)","volume":"318","author":"Martuza","year":"1988"},{"key":"b28","series-title":"Curr Opin Genet Dev","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/S0959-437X(95)90053-5","article-title":"Ras signaling and NF1.","volume":"5","author":"McCormick","year":"1995"},{"key":"b29","series-title":"Proc Natl Acad Sci USA","doi-asserted-by":"publisher","first-page":"5435","DOI":"10.1073\/pnas.87.14.5435","article-title":"Chromosome 17p deletions and p53 gene mutations associated with the formation of malignant neurofibrosarcomas in von Recklinghausen neurofibromatosis.","volume":"87","author":"Menon","year":"1990"},{"key":"b30","series-title":"Br J Dermatol","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1111\/j.1365-2133.1994.tb06893.x","article-title":"What is segmental neurofibromatosis?","volume":"130","author":"Moss","year":"1994"},{"key":"b31","series-title":"Ann Intern Med","doi-asserted-by":"publisher","first-page":"39","DOI":"10.7326\/0003-4819-113-1-39","article-title":"NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update.","volume":"113","author":"Mulvihill","year":"1990"},{"key":"b32","series-title":"Brain Pathol","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1111\/j.1750-3639.1997.tb01067.x","article-title":"Neurofibromatosis 1 and 2.","volume":"7","author":"Pollack","year":"1997"},{"key":"b33","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"946","DOI":"10.1227\/00006123-198706000-00021","article-title":"Segmental neurofibromatosis.","volume":"20","author":"Rawlings","year":"1987"},{"key":"b34","series-title":"Curr Probl Cancer","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/S0147-0272(82)80016-0","article-title":"Neurofibromatosis: clinical heterogeneity.","volume":"7","author":"Riccardi","year":"1982"},{"key":"b35","series-title":"J Am Acad Dermatol","doi-asserted-by":"publisher","first-page":"518","DOI":"10.1016\/S0190-9622(84)80104-8","article-title":"Neurofibromatosis heterogeneity.","volume":"10","author":"Riccardi","year":"1984"},{"key":"b36","series-title":"Arch Dermatol","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1001\/archderm.1987.01660310085020","article-title":"Segmental neurofibromatosis.","volume":"123","author":"Roth","year":"1987"},{"key":"b37","series-title":"Neurology","first-page":"76","article-title":"Familial transmission of segmental neurofibromatosis.","volume":"33","author":"Rubenstein","year":"1983"},{"key":"b38","series-title":"Neurosurgery","first-page":"1122","article-title":"Segmental neurofibromatosis of the sciatic nerve: case report.","volume":"31","author":"Sieb","year":"1992"},{"key":"b39","series-title":"J Am Acad Dermatol","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1016\/S0190-9622(84)80092-4","article-title":"Bilateral dermatomal neurofibromatosis.","volume":"10","author":"Takiguchi","year":"1984"},{"key":"b40","series-title":"J Am Acad Dermatol","doi-asserted-by":"publisher","first-page":"866","DOI":"10.1016\/0190-9622(90)70306-3","article-title":"Segmental neurofibromatosis.","volume":"23","author":"Trattner","year":"1990"},{"key":"b41","series-title":"Ann Surg Oncol","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1007\/BF02303627","article-title":"Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience.","volume":"2","author":"Vauthey","year":"1995"},{"key":"b42","series-title":"Science","first-page":"181","article-title":"Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients.","volume":"249","author":"Wallace","year":"1998"},{"key":"b43","series-title":"Ann Surg","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1097\/00000658-199203000-00012","article-title":"Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity.","volume":"215","author":"Williard","year":"1992"},{"key":"b44","series-title":"Hum Genet","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1007\/s004390050341","article-title":"Somatic mosaicism for deletion of the entire NF1 gene identified by FISH.","volume":"99","author":"Wu","year":"1997"},{"key":"b45","series-title":"Cell","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1016\/0092-8674(90)90024-9","article-title":"The neurofibromatosis type 1 gene encodes a protein related to GAP.","volume":"62","author":"Xu","year":"1990"}],"container-title":["Journal of Neurosurgery"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/92\/2\/article-p342.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/92\/2\/article-p342.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg\/92\/2\/article-p342.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,27]],"date-time":"2023-03-27T05:07:36Z","timestamp":1679893656000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg\/92\/2\/article-p342.xml"}},"subtitle":["Case report"],"short-title":[],"issued":{"date-parts":[[2000,2]]},"references-count":45,"journal-issue":{"issue":"2"},"URL":"https:\/\/doi.org\/10.3171\/jns.2000.92.2.0342","relation":{},"ISSN":["0022-3085"],"issn-type":[{"value":"0022-3085","type":"print"}],"subject":[],"published":{"date-parts":[[2000,2]]}}}