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Lipomatosis of Nerve

MedGen UID:
277510
•Concept ID:
C1335389
•
Disease or Syndrome
Synonyms: Fibrolipomatous Hamartoma of Peripheral Nerve; Neural Fibrolipoma; Peripheral Nerve Fibrolipomatous Hamartoma

Definition

A tumor composed of mature adipocytes and fibrous tissue infiltrating the epineurium and peripheral nerves. It is often seen at birth or during childhood and may be associated with macrodactyly. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLipomatosis of Nerve

Professional guidelines

PubMed

Clinical characteristics and surgical management of facial infiltrating lipomatosis: a single center experience.
Chen H, Sun B, Xia W, Qiu Y, Gao W, Hua C, Lin X
Head Face Med 2024 Feb 20;20(1):13. doi: 10.1186/s13005-024-00412-6. PMID: 38378686Free PMC Article
Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods, classification and management.
Hansson E, Svensson H, Brorson H
Orphanet J Rare Dis 2012 Apr 30;7:23. doi: 10.1186/1750-1172-7-23. PMID: 22546240Free PMC Article

Recent clinical studies

Etiology

Bilateral Nerve Involvement in Lipomatosis of Nerve.
Marek T, Amrami KK, Spinner RJ, Mahan MA
J Surg Orthop Adv 2021 Spring;30(1):44-49. PMID: 33851914
Surgical Treatment of Lipomatosis of Nerve: A Systematic Review.
Marek T, Spinner RJ, Syal A, Wahood W, Mahan MA
World Neurosurg 2019 Aug;128:587-592.e2. Epub 2019 Apr 19 doi: 10.1016/j.wneu.2019.04.110. PMID: 31009789
Intraneural lipomas: institutional and literature review.
Marek T, Amrami KK, Mahan MA, Spinner RJ
Acta Neurochir (Wien) 2018 Nov;160(11):2209-2218. Epub 2018 Sep 21 doi: 10.1007/s00701-018-3677-7. PMID: 30242496
Fat-containing soft-tissue masses in children.
Sheybani EF, Eutsler EP, Navarro OM
Pediatr Radiol 2016 Dec;46(13):1760-1773. Epub 2016 Nov 19 doi: 10.1007/s00247-016-3690-z. PMID: 27866258
Long-term progression of lipomatosis of nerve.
Mahan MA, Niederhauser BD, Amrami KK, Spinner RJ
World Neurosurg 2014 Sep-Oct;82(3-4):492-9. Epub 2013 Sep 10 doi: 10.1016/j.wneu.2013.09.005. PMID: 24036342

Diagnosis

Ultrasound and MR imaging of peripheral nerve tumors: the state of the art.
Lefebvre G, Le Corroller T
Skeletal Radiol 2023 Mar;52(3):405-419. Epub 2022 Jun 17 doi: 10.1007/s00256-022-04087-5. PMID: 35713690
Ultrasonographic Diagnosis of Lipomatosis of Nerve: A Review of Ultrasonographic Finding for 8 Cases.
Xu P, Lu B, Deng HP, Mi YR, Yin CX, Ding Y, Shao XZ, Zhang GS, Xiu XF
World Neurosurg 2020 Jul;139:e730-e736. Epub 2020 Apr 25 doi: 10.1016/j.wneu.2020.04.116. PMID: 32344138
Surgical Treatment of Lipomatosis of Nerve: A Systematic Review.
Marek T, Spinner RJ, Syal A, Wahood W, Mahan MA
World Neurosurg 2019 Aug;128:587-592.e2. Epub 2019 Apr 19 doi: 10.1016/j.wneu.2019.04.110. PMID: 31009789
A new pattern of lipomatosis of nerve: case report.
Prasad NK, Mahan MA, Howe BM, Amrami KK, Spinner RJ
J Neurosurg 2017 Mar;126(3):933-937. Epub 2016 May 6 doi: 10.3171/2016.2.JNS151051. PMID: 27153174
Fat-containing soft-tissue masses in children.
Sheybani EF, Eutsler EP, Navarro OM
Pediatr Radiol 2016 Dec;46(13):1760-1773. Epub 2016 Nov 19 doi: 10.1007/s00247-016-3690-z. PMID: 27866258

Therapy

Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor.
Lee CH, Wu JS, Goldsmith JD, Kung JW
Skeletal Radiol 2013 Dec;42(12):1751-4. Epub 2013 Jun 26 doi: 10.1007/s00256-013-1671-y. PMID: 23801100
Fibroproliferative neuromas may occur after iatrogenic injury for lipomatosis of nerve.
Mahan MA, Amrami KK, Spinner RJ
Neurosurgery 2013 Aug;73(2):271-81; discussion 281. doi: 10.1227/01.neu.0000430289.93304.e5. PMID: 23615087

Prognosis

Neural Fibrolipoma of the Ankle: A Case Report and Review of the Literature.
Nodelman LO, Silverman TJ, Theodoulou MH
J Foot Ankle Surg 2016 Sep-Oct;55(5):1063-6. Epub 2016 Feb 20 doi: 10.1053/j.jfas.2015.12.012. PMID: 26905251

Clinical prediction guides

Expanding the phenotypic spectrum of lipomatosis of the sciatic nerve: Early-onset colonic diverticular disease.
Marek T, Mahan MA, Amrami KK, Blackburn PR, Caffes PL, Carter JM, Camilleri M, Spinner RJ
Neurogastroenterol Motil 2020 Oct;32(10):e13917. Epub 2020 Jun 21 doi: 10.1111/nmo.13917. PMID: 32567170
The first radiographic image of a peripheral nerve disorder? Lipomatous macrodactyly (unrecognized lipomatosis of nerve).
Mahan MA, Prasad N, Spinner RJ
Acta Neurochir (Wien) 2015 Jun;157(6):1059-62; discussion 1062. Epub 2015 Apr 12 doi: 10.1007/s00701-015-2416-6. PMID: 25862172

Recent systematic reviews

Surgical Treatment of Lipomatosis of Nerve: A Systematic Review.
Marek T, Spinner RJ, Syal A, Wahood W, Mahan MA
World Neurosurg 2019 Aug;128:587-592.e2. Epub 2019 Apr 19 doi: 10.1016/j.wneu.2019.04.110. PMID: 31009789

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