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Half of the cases were reported in the extremities, with the lungs being the most common site of metastasis. We report a rare case of successful limb salvage surgery performed for a large exophytic malignant peripheral nerve sheath tumor of the right forearm with wide surgical margins followed by split-thickness skin graft and later a flexor carpi radialis tendon transfer\u00a0to extensor digitorum communis tendon.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>A 51-year-old Bedouin Arabic man presented to our institution with an incompletely excised, large, fungating, malignant peripheral nerve sheath tumor occupying most of his right forearm.\u00a0Staging\u00a0imaging\u00a0showed multiple lung nodules. He underwent wide local excision followed by skin graft and tendon transfer as a palliative measure to preserve the function of his dominant limb. The operation was performed without any complications, and the patient had an excellent postoperative result. Afterward, he was started on multiple lines of chemotherapy that failed because of disease progression, and the patient died 7 months after the operation.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>Clinicians must consider the possibility of soft tissue sarcoma even in a patient with a small, slow-growing, superficial mass. Furthermore, a wrong open biopsy or nononcological surgical procedure may lead to possible contamination and ultimately a more radical procedure than would have originally been necessary, where this can be prevented by an early referral to a highly specialized sarcoma center.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s13256-020-02427-4","type":"journal-article","created":{"date-parts":[[2020,7,6]],"date-time":"2020-07-06T23:06:49Z","timestamp":1594076809000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Fungating malignant peripheral nerve sheath tumor arising from a slow-growing mass in the forearm: a case report and review of the\u00a0literature"],"prefix":"10.1186","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8354-3323","authenticated-orcid":false,"given":"Samer","family":"Abdel Al","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamad K.","family":"Abou Chaar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wafa","family":"Asha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hani","family":"Al-Najjar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maysa","family":"Al-Hussaini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,7,7]]},"reference":[{"issue":"6","key":"2427_CR1","first-page":"2597","volume":"33","author":"VY Ng","year":"2013","unstructured":"Ng VY, Scharschmidt TJ, Mayerson JL, Fisher JL. 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A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"91"}}