Abstract
Objective
To describe differences between lipomatosis of nerve (LN) and neuromuscular choristoma (NMC) evaluated with MR spectroscopy (MRS).
Materials and methods
Eight patients were included in this prospective pilot study: three patients with LNs and five with NMCs. Single voxel PRESS MRS of the tumors were acquired with 3 T MRI. MRS data were processed with LCModel version 6.3-1J using the internal “lipid-8” basis set. From individual lipid peak and water content measurements, total fatty acid molecules (TFAM), unsaturated fatty acid molecules (UFAM), and glycerol molecules (GM) were computed and analyzed, as well as ratios of UFAM/TFAM, TFAM/GM, and a fatty-acid chain-length index (CLI).
Results
The LN group included two men and one woman (average age 58.3 years); the NMC group included two men and three women (average age 20.4 years). Lipid composition analysis showed that LN had considerably more fat than NMC: TFAM: LN = 15.29 vs NMC = 7.14; UFAM: LN = 4.48 vs NMC = 2.63; GM: LN = 5.20 vs NMC = 1.02. Both tumors had a similar fraction of unsaturated fatty acids: UFAM/TFAM: LN = 0.29 vs NMC = 0.37. LN had the usual number of FA molecules/glycerol molecule, while NMC had considerably more: TFAM/GM: LN = 2.94 vs NMC = 6.98. Finally, average FA chains were longer in NMC: CLI: LN = 17.39 vs NMC = 22.55.
Conclusion
Our analysis suggests measurable differences in the amount and composition of lipid in LN and NMC. While a larger, statistically powered study is needed, these initial findings may be helpful to properly diagnose ambiguous cases and thereby avoid surgical intervention such as biopsy.
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References
Blackburn PR, Milosevic D, Marek T, Folpe AL, Howe BM, Spinner RJ, et al. PIK3CA mutations in lipomatosis of nerve with or without nerve territory overgrowth. Mod Pathol. 2020;33(3):420–30.
Silverman TA, Enzinger FM. Fibrolipomatous hamartoma of nerve. A clinicopathologic analysis of 26 cases. Am J Surg Pathol. 1985;9(1):7–14.
Fletcher CDM, Organization WH. WHO classification of tumours of soft tissue and bone: IARC Press; 2013.
Spinner RJ, Scheithauer BW, Amrami KK, Wenger DE, Hebert-Blouin MN. Adipose lesions of nerve: the need for a modified classification. J Neurosurg. 2012;116(2):418–31.
Marek T, Spinner RJ, Syal A, Mahan MA. Strengthening the association of lipomatosis of nerve and nerve-territory overgrowth: a systematic review. J Neurosurg. 2019:1–9.
Prasad NK, Mahan MA, Howe BM, Amrami KK, Spinner RJ. A new pattern of lipomatosis of nerve: case report. J Neurosurg. 2017;126(3):933–7.
Prasad NK, Howe BM, Maus TP, Moran SL, Spinner RJ. Could congenital infiltrating lipomatosis of the face have an anatomic explanation: lipomatosis of the trigeminal nerve and nerve-territory overgrowth? Clin Anat. 2017;30(5):552–4.
Mahan MA, Howe BM, Amrami KK, Spinner RJ. Occult radiological effects of lipomatosis of the lumbosacral plexus. Skelet Radiol. 2014;43(7):963–8.
Van Dorpe J, Sciot R, De Vos R, Uyttebroeck A, Stas M, Van Damme B. Neuromuscular choristoma (hamartoma) with smooth and striated muscle component: case report with immunohistochemical and ultrastructural analysis. Am J Surg Pathol. 1997;21(9):1090–5.
Maldonado AA, Spinner RJ, Carter JM, Stone JJ, Howe BM. Clinical and magnetic resonance imaging longitudinal follow-up of neuromuscular choristomas. World Neurosurg. 2019;129:e761–6.
Marom EM, Helms CA. Fibrolipomatous hamartoma: pathognomonic on MR imaging. Skelet Radiol. 1999;28(5):260–4.
Niederhauser BD, Spinner RJ, Jentoft ME, Everist BM, Matsumoto JM, Amrami KK. Neuromuscular choristoma: characteristic magnetic resonance imaging findings and association with post-biopsy fibromatosis. Skelet Radiol. 2013;42(4):567–77.
Marek T, Spinner RJ, Syal A, Wahood W, Mahan MA. Surgical treatment of lipomatosis of nerve: a systematic review. World Neurosurg. 2019;128:587–592 e582.
Hebert-Blouin MN, Amrami KK, Spinner RJ. Addendum: evidence supports a “no-touch” approach to neuromuscular choristoma. J Neurosurg. 2013;119(1):252–4.
Lee CH, Wu JS, Goldsmith JD, Kung JW. Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor. Skelet Radiol. 2013;42(12):1751–4.
Lee CH, Wu JS, Goldsmith JD, Kung JW. Reply to the letter to the editor: in response to “Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor”. Skelet Radiol. 2014;43(2):131–2.
Niederhauser BD, Spinner RJ, Howe BM, Amrami KK. In response to “Lipomatosis of the sciatic nerve secondary to compression by a desmoid tumor”. Skelet Radiol. 2014;43(2):129.
Fayad LM, Wang X, Blakeley JO, Durand DJ, Jacobs MA, Demehri S, et al. Characterization of peripheral nerve sheath tumors with 3T proton MR spectroscopy. AJNR Am J Neuroradiol. 2014;35(5):1035–41.
Griffey RH, Eaton RP, Sibbitt RR, Sibbitt WL Jr, Bicknell JM. Diabetic neuropathy. Structural analysis of nerve hydration by magnetic resonance spectroscopy. JAMA. 1988;260(19):2872–8.
Wang CK, Li CW, Hsieh TJ, Chien SH, Liu GC, Tsai KB. Characterization of bone and soft-tissue tumors with in vivo 1H MR spectroscopy: initial results. Radiology. 2004;232(2):599–605.
Hamilton G, Schlein AN, Middleton MS, Hooker CA, Wolfson T, Gamst AC, et al. In vivo triglyceride composition of abdominal adipose tissue measured by (1) H MRS at 3T. J Magn Reson Imaging. 2017;45(5):1455–63.
Strobel K, van den Hoff J, Pietzsch J. Localized proton magnetic resonance spectroscopy of lipids in adipose tissue at high spatial resolution in mice in vivo. J Lipid Res. 2008;49(2):473–80.
Ren J, Dimitrov I, Sherry AD, Malloy CR. Composition of adipose tissue and marrow fat in humans by 1H NMR at 7 Tesla. J Lipid Res. 2008;49(9):2055–62.
Bottomley PA. Spatial localization in NMR spectroscopy in vivo. Ann N Y Acad Sci. 1987;508:333–48.
Provencher SW. Automatic quantitation of localized in vivo 1H spectra with LCModel. NMR Biomed. 2001;14(4):260–4.
Hodson L, Skeaff CM, Fielding BA. Fatty acid composition of adipose tissue and blood in humans and its use as a biomarker of dietary intake. Prog Lipid Res. 2008;47(5):348–80.
Watt MJ, Cheng Y. Triglyceride metabolism in exercising muscle. Biochim Biophys Acta Mol Cell Biol Lipids. 2017;1862(10 Pt B):1250–9.
Watt MJ, Hoy AJ. Lipid metabolism in skeletal muscle: generation of adaptive and maladaptive intracellular signals for cellular function. Am J Physiol Endocrinol Metab. 2012;302(11):E1315–28.
Manco M, Mingrone G, Greco AV, Capristo E, Gniuli D, De Gaetano A, et al. Insulin resistance directly correlates with increased saturated fatty acids in skeletal muscle triglycerides. Metabolism. 2000;49(2):220–4.
Wakil SJ, Abu-Elheiga LA. Fatty acid metabolism: target for metabolic syndrome. J Lipid Res. 2009;50(Suppl):S138–43.
Acknowledgments
The authors wish to thank Mandie Maroney for her assistance managing the MRS and imaging data for this study.
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Marek, T., Amrami, K.K., Spinner, R.J. et al. MR spectroscopy differences between lipomatosis of nerve and neuromuscular choristoma: a potential adjunctive diagnostic tool. Skeletal Radiol 49, 2051–2057 (2020). https://doi.org/10.1007/s00256-020-03479-9
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DOI: https://doi.org/10.1007/s00256-020-03479-9