Malignant Peripheral Nerve She ath Tumor – Two Case Reports


Authors: V. Beneš Iii 1;  F. Kramář 2;  P. Hrabal 3;  M. Kaiser 1;  P. Buchvald 1
Authors‘ workplace: Neurochirurgické oddělení, Krajská nemocnice Liberec, 2Neurochirurgická klinika 1. LF UK, IPVZ a ÚVN Praha, 3Oddělení patologie ÚVN Praha 1
Published in: Cesk Slov Neurol N 2009; 72/105(2): 163-167
Category: Case Report

Overview

Malignant peripheral nerve she ath tumor (MPNST) is a highly malignant tumor of the peripheral nervo us system, which is likely to reccur locally or metastatize. Its incidence is higher in pati ents with ne urofibromatosis type 1 (NF‑1). We present two case reports of pati ents with this rare tumor. First pati ent, 74- ye ar old woman, underwent ro utine chest exam which disclosed an expansive lesi on of the left chest cavity. Magnetic resonance imaging appe arance suggested a Th3 schwannoma. However, histopathological exam after parti al resecti on disclosed MPNST with proliferative potenti al of 25 %. The pati ent di ed six months later due to tumor progressi on. Second pati ent, 35- ye ar old woman with NF‑1 noticed enlargement of a known lump of the right calf. Histopathological exam after parti al resecti on disclosed ne urofibroma with signs of MPNST. Control magnetic resonance imaging reve aled massive tumor progressi on; the pati ent underwent limb amputati on. The pati ent has been in remissi on during 20 months follow‑up. Apart from these case reports the paper revi ews the literature as well as clinical and therape utical aspects of this tumor.

Key words:
malignant peripheral nerve she ath tumor –  malignant schwannoma –  ne urofibromatosis –  soft tissue sarcoma


Sources

1. Wo odruff JM, Ko ure a HP, Lo uis DN, Scheitha uer BW. Malignant peripheral nerve she ath tumo ur (MPNST). In: Kleihues P, Cavenee WK (eds). Pathology and genetics of tumo urs of the nervo us system. Lyon: IARCPress 2000: 172– 174.

2. Stark AM, Buhl R, Hugo HH, Mehdorn HM. Malignant peripheral nerve she ath tumo urs –  report of 8 cases and revi ew of the literature. Acta Ne urochir (Wi en) 2001; 143(4): 357– 364.

3. Ducatman BS, Scheitha uer BW, Pi epgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve she ath tumors. A clinicopathologic study of 120 cases. Cancer 1986; 57(10): 2006– 2021.

4. Bludovský D, Hes O, Choc M, Michal M. Intracerebrální supratentori ální maligní schwannom: Kazuistika. Cesk Slov Ne urol N 2001; 68/ 101(2): 120– 123.

5. Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve she ath tumor: the clinical spectrum and o utcome of tre atment. Ne urology 2003; 61(5): 696– 698.

6. Seppälä MT, Halti a MJ. Spinal malignant nerve- she ath tumor or cellular schwannoma? A striking difference in prognosis. J Ne urosurg 1993; 79(4): 528– 532.

7. Hruban RH, Shi u MH, Seni e RT, Wo odruff JM. Malignant peripheral nerve she ath tumors of the buttock and lower extremity. A study of 43 cases. Cancer 1990; 66(6): 1253– 1265.

8. Anghileri M, Miceli R, Fi ore M, Mari ani L, Ferrari A, Mussi C et al. Malignant peripheral nerve she ath tumors: prognostic factors and survival in a seri es of pati ents tre ated at a single instituti on. Cancer 2006; 107(5): 1065– 1074.

9. Tucker T, Wolkenstein P, Revuz J, Zeller J, Fri edman JM. Associ ati on between benign and malignant peripheral nerve she ath tumors in NF1. Ne urology 2005; 65(2): 205– 211.

10. Huson SM, Harper PS, Compston DA. Von Recklingha usen ne urofibromatosis. A clinical and populati on study in so uth- e ast Wales. Brain 1988; 111(6): 1355– 1381.

11. Schwarz J, Belzberg AJ. Malignant peripheral nerve she ath tumors in the setting of segmental ne urofibromatosis. Case report. J Ne urosurg 2000; 92(2): 342– 346.

12. Knudson AG jr. Mutati on and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci USA 1971; 68(4): 820– 823.

13. Kawai A, Noguchi M, Beppu Y, Yokoyama R, Mukai K, Hirohashi S et al. Nucle ar immunore acti on of p53 protein in soft tissue sarcomas. A possible prognostic factor. Cancer 1994; 73(10): 2499– 2505.

14. Cance WG, Brennan MF, Dudas ME, Hu ang CM, Cordon- Cardo C. Altered expressi on of the retinoblastoma gene product in human sarcomas. N Engl J Med 1990; 323(21): 1457– 1462.

15. Cordon- Cardo C, Latres E, Drobnjak M, Oliva MR, Pollack D, Wo odruff JM et al. Molecular abnormaliti es of mdm2 and p53 genes in adult soft tissue sarcomas. Cancer Res 1994; 54(3): 794– 799.

16. Halling KC, Scheitha uer BW, Halling AC, Nascimento AG, Zi esmer SC, Roche PC et al. p53 expressi on in ne urofibroma and malignant peripheral nerve she ath tumor. An immunohistochemical study of sporadic and NF1‑associ ated tumors. Am J Clin Pathol 1996; 106(3): 282– 288.

17. Kindblom LG, Ahldén M, Meis- Kindblom JM, Stenman G. Immunohistochemical and molecular analysis of p53, MDM2, proliferating cell nucle ar antigen and Ki67 in benign and malignant peripheral nerve she ath tumo urs. Virchows Arch 1995; 427(1): 19– 26.

18. Legi us E, Di erick H, Wu R, Hall BK, Marynen P, Cassiman JJ et al. TP53 mutati ons are frequent in malignant NF1 tumors. Genes Chromosomes Cancer 1994; 10(4): 250– 255.

19. Wong WW, Hirose T, Scheitha uer BW, Schild SE, Gunderson LL. Malignant peripheral nerve she ath tumor: Analysis of tre atment o utcome. Int J Radi ati on Oncology Bi ol Phys 1998; 42(2): 351– 360.

20. Ferner RE, Lucas JD, O’Doherty MJ, Hughes RAC, Smith MA, Cronin BF et al. Evalu ati on of (18)fluorode oxyglucose positron emissi on tomography ((18)FDG PET) in the detecti on of malignant peripheral nerve she ath tumo urs arising from within plexiform ne urofibromas in ne urofibromatosis 1. J Ne urol Ne urosurg Psychi atry 2000; 68(3): 353– 357.

21. Ferner RE, Gutmann DH. Internati onal consensus statement on malignant peripheral nerve she ath tumors in ne urofibromatosis. Cancer Res 2002; 62(5): 1573– 1577.

22. Evans DGR, Baser ME, McGa ughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve she ath tumo urs in ne urofibromatosis 1. J Med Genet 2002; 39(5): 311– 314.

23. Ducatman BS, Scheitha uer BW. Postirradi ati on ne urofibrosarcoma. Cancer 1983; 51(6): 1028– 1033.

24. Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB et al. Malignant schwannoma –  clinical characteristics, survival and response to therapy. Cancer 1981; 47(10): 2503– 2509.

25. Kozler P, Kramář F. Famili ární nádorové syndromy postihující nervový systém. In: Kozler P et al (eds). Intrakrani ální nádory. 1st ed. Praha: Galén, Karolinum 2007: 175– 182.

26. Bhargava R, Parham DM, Lasater OE, Chari RS, Chen G,Fletcher BD. MR imaging differenti ati on of benign and malignant peripheral nerve she ath tumors: use of the target sign. Pedi atr Radi ol 1997; 27(2): 124– 129.

27. Ko ure a HP, Bilsky MH, Le ung DH, Lewis JJ, Wo odruff JM. Subdi aphragmatic and intrathoracic paraspinal malignant peripheral nerve she ath tumors. A clinicopathological study of 25 pati ents and 26 tumors. Cancer 1998; 82(11): 2191– 2203.

28. Taxy JB, Battifora H, Trujillo Y, Dorfman HD. Electron microscopy in the di agnosis of malignant schwannoma. Cancer 1981; 48(6): 1381– 1391.

29. Perrin RG, Guha A. Malignant peripheral nerve she ath tumors. Ne urosurg Clin N Am 2004; 15(2): 203– 216.

30. Meis JM, Enziger FM, Martz KL, Ne al JA. Malignant peripheral nerve she ath tumors (Malignant schwannomas) in children. Am J Surg Pathol 1992; 16(7): 694– 707.

31. Wanebo JE, Malik JM, VandenBerg SR, Wanebo HJ, Dri esen N, Persing JA. Malignant peripheral nerve she ath tumors. A clinicopathological study of 28 cases. Cancer 1993; 71(4): 1247– 1253.

32. Vege DS, Chinoy RF, Ganesh B, Parikh DM. Malignant peripheral nerve she ath tumors of the he ad and neck: A clinicopathological study. J Surg Oncol 1994; 55(2): 100– 103.

33. Wo odruff JM. Pathology of tumors of the perifpheral nerve she ath in type 1 ne urofibromatosis. Am J Med Genet 1999; 89(1): 23– 30.

34. Das Gupta TK, Brasfi eld RD. Solitary malignant schwannoma. Ann Surg 1970; 171(3): 419– 428.

35. Kim DH, Murovic JA, Ti el RL, Moes G, Kline DG. A seri es of 397 peripheral ne ural she ath tumors: 30- ye ar experi ence at Lo uisi ana State University He alth Sci ences Center. J Ne urosurg 2005; 102(2): 246– 255.

36. Bojsen- Møller M, Myhre‑Jensen O. A consecutive seri es of 30 malignant schwannomas. Survival in relati on to clinico- pathological parameters and tre atment. Acta Path Microbi ol Immunol Scand (A) 1984; 92(3): 147– 155.

37. Storm FK, Eilber FR, Mirra J, Morton DL. Ne urofibrosarcoma. Cancer 1980; 45(1): 126– 129.

38. Foley KM, Wo odruff JM, Ellis FT, Posner JB. Radi ati on‑induced malignant and atypical peripheral nerve she ath tumors. Ann Ne urol 1980; 7(4): 311– 318.

39. Fein DA, Lee WR, Lanci ano RM, Corn BW, Herbert SH, Hanlon AL et al. Management of extremity soft tissue sarcomas with limb- sparing surgery and postoperative irradi ati on: do total dose, overall tre atment time, and the surgery- radi otherapy interval impact on local control? Int J Radi at Oncol Bi ol Phys 1995; 32(4): 969– 976.

40. Cashen DV, Parisi en RC, Raskin K, Hornicek FJ, Gebhardt MC, Mankin HJ. Survival data for pati ents with malignant schwannoma. Clin Orthop Rel Res 2004; 426: 69– 73.

Labels
Paediatric neurology Neurosurgery Neurology
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account