Malignant Peripheral Nerve Sheath Tumour of Cervical Plexus –⁠ a Case Report

Czech version

Authors: Z. Kadaňka Jr ¹; J. Hanák ²; B. Gál ²
Authors‘ workplace: Neurologická klinika LF MU a FN Brno ¹; ORL klinika LF MU a FN u sv. Anny v Brně ²
Published in: Cesk Slov Neurol N 2013; 76/109(6): 751-755
Category: Case Report

Overview

Malignant peripheral nerve sheath tumours are rare neoplasms, especially in the head and neck. They are often asymptomatic. We present a case of 29-years old patient with neurofibromatosis type 1 with malignant tumour of the cervical plexus. A small resistance in the upper mediastinum was diagnosed (ganglioneurinoma) in 2005 and treated surgically (total exstirpation). A small infiltration in the right supraclavicular area occurred five years later and was also managed surgically (debulking) –⁠ histologically MPNST. There was a relapse of this tumour in the same area in 2011; this was treated by en bloc resection, followed by chemotheraphy. At present, the patient is in a good clinical status, with no neurological deficit.

Key words:
malignant peripheral nerve sheath tumour –⁠ cervical plexus –⁠ malignant schwannoma –⁠ neurofibromatosis type 1

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Sources

1. Beneš V III, Kramář F, Hrabal P, Kaiser M, Buchvald P. Maligní tumor z pochvy periferního nervu –⁠ dvě kazuistiky. Cesk Slov Neurol N 2009; 72/ 105(2): 163 –⁠ 167.

2. Bludovsky D, Zidek S, Hes O, Kazakov D. Melanotic Schwannoma of the Th9Nerve Root. Case Report. World Spinal Column Journal, 2010 : 1(2). [on-line]. Available from:http://www.wscj.org.

3. Minovi A, Basten O, Hunter B, Draf W, Bockmühl U.Malignant peripherical nerve sheath tumors of the head and neck: management of 10 cases and literature review. Head Neck 2007; 29(5): 439 –⁠ 445.

4. Ducatman BS, Scheithauer BW, Piepgras DG, Reimann HM, Ilstrup DM. Malignant peripheral nerve sheat tumors: a clinicopathologic study of 120 cases. Cancer 1986; 57(10): 2006 –⁠ 2021.

5. D‘Agostino AN, Soule EH, Miller RH. Primary malignant neoplasms of nerves (Malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen‘s disease). Cancer 1963 : 16(8): 1003 –⁠ 1014.

6. Gupta G, Mammis A, Maniker A. Malignant peripheral nerve sheath tumours. Neurosurg Clin N Am 2008; 19(4): 533 –⁠ 543.

7. Rawal A, Yin Q, Roebuck M, Sinopidis C, Kalogrianitis S, Helliwell TR et al. Atypical and malignant peripheral nerve ‑⁠ sheath tumors of the brachial plexus: report of three cases and review of the literature. Microsurgery 2006; 26(2): 80 –⁠ 86.

8. Bollag G, Clapp DW, Shih S, Adler F, Zhang YY, Thompson P et al. Loss of NF1 results in activation of the Ras signaling pathway and leads to aberrant growth in haematopoietic cells. Nat Genet 1996; 12(2): 144 –⁠ 148.

9. Largaespada DA, Brannan CI, Jenkins NA, Copeland NG. NF1 deficiency causes Ras ‑⁠ mediated granulocyte/ macrophage colony stimulating factor hypersensitivity and chronic myeloid leukaemia. Nat Genet 1996; 12(2): 137 –⁠ 143.

10. Dai Ch, Santagata, S Tang Z, Shi J, Cao J, Kwon H et al. Loss of tumor suppressor NF1 activates HSF1 to promote carcinogenesis J Clin Invest 2012; 122(10): 3742 –⁠ 3754.

11. Kindblom LG, Ahldén M, Meis ‑⁠ Kindblom JM, Stenman G, Virchows. Immunohistochemical and molecular analysis of p53, MDM2, proliferating cell nuclear antigen and Ki67 in benign and malignant peripheral nerve sheath tumours. Virchows Arch 1995; 427(1): 19 –⁠ 26.

12. Berner JM, Sørlie T, Mertens F, Henriksen J, Saeter G,Mandahl N et al. Chromosome band 9p21 is frequently altered in malignant peripheral nerve sheath tumors: studies of CDKN2A and other genes of the pRB pathway. Genes Chromosomes Cancer 1999; 26(2): 151 –⁠ 160.

13. Stark AM, Buhl R, Hugo HH, Mehdorn HM. Malignant peripheral nerve sheath tumours ‑⁠ report of 8 cases and review of the literature. Acta Neurochir (Wien) 2001; 143(4): 357 –⁠ 363.

14. Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002; 39(5): 311 –⁠ 314.

15. Adamson DC, Cummings.TJ, Friedman AH. Malignant peripheral nerve sheath tumor of the spine after radiation terapie for Hodgkinʼs lymphoma. Clin Neuropathol 2004; 23(5): 245 –⁠ 255.

16. Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology 2003; 61(5): 696 –⁠ 698.

17. Ghosh BC, Ghosh L, Huvos AG, Fortner JG. Malignant schwannoma. A clinicopathologic study. Cancer 1973; 31(1): 184 –⁠ 190.

18. Furniss D, Stan MC, Morritt D, Lim J, Khanna T, Way BL et al. A 10‑year review of benign and malignant peripheral nerve sheath tumors in a single center: clinical and radiographic features can help to differentiate benign from malignant lesions. Plast Reconstr Surg 2008; 121(2): 529 –⁠ 533.

19. Friedrich RE, Kluwe L, Funsterer C, Mautner VF. Malignant peripheral nerve sheath tumors (MPNST) in neurofibromatosis type 1 (NF1) diagnostic findings on magnetic resonance images and mutational analysis of the NF1 gene. Anticancer Res 2005; 25(3A): 1699 –⁠ 1702.

20. Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumor of the buttock and lower extremity. A study of 43 cases. Cancer 1990; 66(6): 1253 –⁠ 1265.

21. Ferner RE, Lucas JD, O‘Doherty MJ, Hughes RAC, Smith MA, Cronin B et al. Evaluation of 18 fluorodeoxyglucose positron emission tomography in the detection of malignant peripheral nerve sheath tumors in neurofibromatosis 1. J Neurol Neurosurg Psychiatry 2000; 68(3): 353 –⁠ 357.

22. Perry A, Roth KA, Banerjee R, Fuller CE, Guttmann DH.NF1 deletions in S ‑⁠ 100 protein‑positive and negative cells of sporadic and neurofibromatosis 1 (NF1) –⁠ associated plexiform neurofibromatosis and malignant peripheral nerve sheath tumors. AM J Pathol 2001; 159(1): 57 –⁠ 61.

23. Wong WW, Hirose T, Scheithauer BW, Schild SE, Gunderson LL. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998; 42(2): 351 –⁠ 360.

24. Wanebo JE, Malik JM, VandenBerg SR, Wanebo HJ,Driesen N, Persing JA. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer 1993; 71(4): 1247 –⁠ 1253.

25. Vraa S, Keller J, Nielsen OS, Sneppen O, Jurik AG, Jensen OM. Prognostic factors in soft tissue sarcomas: the Aarhus experience. Eur J Cancer 1998; 34(12): 1876 –⁠ 1882.