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To better understand this condition and to evaluate its association with nerve-territory overgrowth, the authors attempted to compile the world\u2019s literature on published LN cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>PubMed and Google Scholar databases were searched to identify published articles on LN cases, using a variety of terms. Publications in all languages were assessed. All publications with cases determined likely to be LN were read. Cases that provided clear clinicopathological and\/or radiological evidence of LN were labeled as \u201cdefinite\u201d and cases that demonstrated features of LN (e.g., nerve-territory overgrowth) but lacked definite proof of nerve involvement were labeled as \u201cprobable.\u201d<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>Initial screening revealed a total of 2465 papers. After exclusions, 281 publications reported cases with a definite diagnosis of LN and 120 articles reported cases with a probable diagnosis of LN. The authors identified 618 definite and 407 probable cases of LN. Sex distribution was balanced (51% female). Early diagnosis was common, with two-thirds of patients having symptoms in the 1st decade of life. The most commonly affected nerve was the median nerve (n = 391). Nerve-territory overgrowth was common (62% definite LN; 78% combined cases); overgrowth was exclusive to the territory of the affected nerve in all cases but 5.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p>The authors present a comprehensive review and analysis of the literature of LN cases. One of the main findings was the nerve-territory overgrowth was associated with LN, especially when present earlier in life. The authors believe that all cases of LN associated with overgrowth can be explained on anatomical grounds, even in the few reported cases in which this is not immediately obvious.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2018.12.jns183050","type":"journal-article","created":{"date-parts":[[2019,4,9]],"date-time":"2019-04-09T19:59:58Z","timestamp":1554839998000},"page":"1286-1294","source":"Crossref","is-referenced-by-count":25,"title":["Strengthening the association of lipomatosis of nerve and nerve-territory overgrowth: a systematic review"],"prefix":"10.3171","volume":"132","author":[{"given":"Tomas","family":"Marek","sequence":"first","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Spinner","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akshay","family":"Syal","sequence":"additional","affiliation":[{"name":"New York Medical College School of Medicine, Valhalla, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark A.","family":"Mahan","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery, University of Utah, Salt Lake City, Utah; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Ophthal Plast Reconstr Surg","doi-asserted-by":"publisher","first-page":"e79","DOI":"10.1097\/IOP.0b013e318272f32b","article-title":"Fibrolipoma of the orbit","volume":"29","author":"Ali SF","year":"2013","unstructured":"Ali SF, Farber M, Meyer DR: Fibrolipoma of the orbit. 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