Desmoid Fibromatosis in the Brachial Plexus Mimicking an Ulnar Nerve Entrapment

Emelie Styring^1, , Marie Ahlström², Pehr Rissler³, Fredrik Mertens⁴, Kasim Abul-Kasim⁵ and Lars B. Dahlin^{6, 7}

¹Lund University, Skane University Hospital, Department of Orthopedics, 221 85 Lund, Sweden

²Lund University, Skane University Hospital, Department of Hematology, Oncology and Radiophysics, 221 85 Lund, Sweden

³Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Pathology, 221 85 Lund, Sweden

⁴Lund University, Skane University Hospital, Division of clinical genetics, 221 85 Lund, Sweden

⁵Department of Radiology, Division of Neuroradiology, Lund University and Skane University hospital, 205 01 Malmö, Sweden

⁶Department of Translational Medicine – Hand Surgery, Lund University, 205 01 Malmö, Sweden

⁷Department of Hand Surgery, Skane university Hospital, 205 01 Malmö, Sweden

Cite this paper

Emelie Styring, Marie Ahlström, Pehr Rissler, Fredrik Mertens, Kasim Abul-Kasim and Lars B. Dahlin. Desmoid Fibromatosis in the Brachial Plexus Mimicking an Ulnar Nerve Entrapment. American Journal of Medical Case Reports. 2019; 7(3):36-40. doi: 10.12691/AJMCR-7-3-2

Abstract

Introduction: Ulnar nerve entrapment is a common cause of sensory disturbance and weakness in the upper extremity, especially in patients with diabetes mellitus. However, if the symptoms are atypical and the patient has severe pain other differential diagnoses should be considered. Case report: A 37-year-old man with type 1 diabetes mellitus was referred to the hand surgery unit due to increasing pain, numbness and weakness in his right arm developing over more than one year. An ulnar nerve neurography was inconclusive and the patient had a frozen shoulder on the right side. Due to the pain, the patient required high doses of opioids. At examination, the clinical presentation did not correspond to an ulnar nerve entrapment why other causes were considered. A chest X-ray revealed a lesion in the apical part of the right lung. Consequtive CT scan, MRI and fine and core needle biopsies led to the diagnosis of a desmoid tumor. Surgery was deemed to be too mutilating and treatment was initiated with doxorubicin. Due to tumor and symptom progression the therapy was changed to PegIntron, then antiestrogen and NSAID and finally tyrosine kinase inhibitor (sorafenib) resulting in tumor shrinkage. Conclusion: Although nerve entrapment is a common cause of discomfort and impaired function in the upper extremities different etiologies, including various tumors, have to be considered when the symptoms are atypical and if the patient has severe pain. For these patients, the diagnostic work-up has to be broadened.

Keywords

ulnar entrapment, desmoid, radiating pain, paresthesia

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Mondelli M, Giannini F, Ballerini M, Ginanneschi F, Martorelli E. Incidence of ulnar neuropathy at the elbow in the province of Siena (Italy). J Neurol Sci. 2005; 234(1-2): 5-10.
[2]	Anker IZ, M. Andersson, G.A. Jacobsson, H. Dahlin, L.B. Outcome and predictors in simple decompression of ulnar nerve entrapment at the elbow. Hand and Microsurgery. 2018; 7(1): 24-32.
[3]	Thomsen NO, Mojaddidi M, Malik RA, Dahlin LB. Reduced myelinated nerve fibre and endoneurial capillary densities in the forearm of diabetic and non-diabetic patients with carpal tunnel syndrome. Acta Neuropathol. 2009; 118(6): 785-91.
[4]	Dahlin LB, Scherman P, Besjakov J, Lindberg E, Solomon DA, Horvai AE, et al. Intraneural glomus tumor of "uncertain malignant potential" and with BRAF mutation in the median nerve - an unusual case. Clin Neuropathol. 2017; 36(4): 164-70.
[5]	Wadhwa V, Salaria SN, Chhabra A. Granular cell tumor of the ulnar nerve: MR neurography characterization. J Radiol Case Rep. 2014; 8(6): 11-7.
[6]	Krisht KM, Karsy M, Shah LM, Schmidt MH, Dailey AT. Unusual brachial plexus lesion: Hematoma masquerading as a peripheral nerve sheath tumor. Surg Neurol Int. 2016; 7(Suppl 3): S64-6.
[7]	Benevello C, Sommacale D, Palladino E, Bildea A, Tres I, Volpin E. A rare case of malignant schwannoma of the brachial plexus. World J Surg Proced. 2013; 3(1): 1-3.
[8]	Assmus H, Antoniadis G, Bischoff C, Hoffmann R, Martini AK, Preissler P, et al. Cubital tunnel syndrome - a review and management guidelines. Cent Eur Neurosurg. 2011; 72(2): 90-8.
[9]	Rydholm A. Management of patients with soft-tissue tumors. Strategy developed at a regional oncology center. Acta Orthop Scand Suppl. 1983; 203: 13-77.
[10]	Orege J, Koech F, Ndiangui F, N.M. B, Mbaruku N. Desmoid Tumour of the Brachial Plexus. Case Reports in Surgery. 2013; 2013.
[11]	Go MH, Kim SH, Cho KH. Brachial plexus tumors in a consecutive series of twenty one patients. J Korean Neurosurg Soc. 2012; 52(2): 138-43.
[12]	Dinauer PA, Brixey CJ, Moncur JT, Fanburg-Smith JC, Murphey MD. Pathologic and MR imaging features of benign fibrous soft-tissue tumors in adults. Radiographics: a review publication of the Radiological Society of North America, Inc. 2007; 27(1): 173-87.
[13]	Mertens F, Willen H, Rydholm A, Brosjo O, Carlen B, Mitelman F, et al. Trisomy 20 is a primary chromosome aberration in desmoid tumors. Int J Cancer. 1995; 63(4): 527-9.
[14]	Crago AM, Chmielecki J, Rosenberg M, O'Connor R, Byrne C, Wilder FG, et al. Near universal detection of alterations in CTNNB1 and Wnt pathway regulators in desmoid-type fibromatosis by whole-exome sequencing and genomic analysis. Genes Chromosomes Cancer. 2015; 54(10): 606-15.
[15]	Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017; 29(4): 268-74.
[16]	Sivanesan E, Gitlin MC. Desmoid Tumors: A Review of the Literature and Pharmacologic Management. J Pain Palliat Care Pharmacother. 2016; 30(2): 99-105.