Advertisement

Journal of Neuro-Oncology

, Volume 109, Issue 1, pp 205–211 | Cite as

A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion

  • Bing Yan
  • Xianze Meng
  • Bian Shi
  • Jun Shi
  • Zhifeng Qin
  • Pinkang Wei
Case Report

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are usually located in the trunk, extremities, head, or neck, and most occur with neurofibromatosis type 1 (NF1; von Recklinghausen’s disease). No biomarkers have previously been found to be associated with their progression. Retroperitoneal NF1-independent MPNSTs are rare; they are considered to be less aggressive and to have better prognoses compared to NF1-related tumors. Currently, en bloc excision is the only consensus treatment approach. In a 27-year-old male with a giant retroperitoneal MPNST and no stigmata or family history of neurofibromatosis type-1 (NF1), a remarkable elevation of serum CA125 was detected. The high-grade tumor displayed a striking progression: the primary lesion, 25 cm in diameter, recurred in its previous site as a 17-cm MPNST less than 50 days after total excision. Subsequent treatment with microwave ablation and huachansu, a traditional Chinese medication, proved ineffective, and the patient died within 3 months. Our case suggests that retroperitoneal MPNSTs can deteriorate rapidly even if NF1 independent, that aggressive treatment may not benefit large high-grade MPNSTs, and that novel and effective treatment is urgently needed. Our case also suggests the possibility of using serum tumor markers in the early detection and monitoring of MPNSTs.

Keywords

Malignant peripheral nerve sheath tumor Retroperitoneum Aggressive treatment Prognosis CA 125 

Notes

Acknowledgments

We thank Dr. Guan Yu of the Shanghai Changzheng Hospital for radiological suggestions, Prof. Chenguang Bai of the Shanghai Changhai Hospital for pathological analysis, and Dr. Lyn Lowry of the Center for Integrative Medicine, University of Maryland School of Medicine for her editorial support.

Conflict of interest

The authors have no potential conflict of interest.

References

  1. 1.
    Hajdu SI (1993) Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 72:3549–3552PubMedCrossRefGoogle Scholar
  2. 2.
    Wong WW, Hirose T, Scheithauer BW et al (1998) Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 42:351–360PubMedCrossRefGoogle Scholar
  3. 3.
    Evans DG, Baser ME, McGaughran J et al (2002) Malignant peripheral nerve sheath tumors in neurofibromatosis 1. J Med Genet 39:311–314PubMedCrossRefGoogle Scholar
  4. 4.
    Carli M, Ferrari A, Mattke A et al (2005) Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23:8422–8430PubMedCrossRefGoogle Scholar
  5. 5.
    Ducatman BS, Scheithaller BW, Piepgras DG et al (1986) Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 57:2006–2021PubMedCrossRefGoogle Scholar
  6. 6.
    Kar M, Deo SV, Shukla NK et al (2006) Malignant peripheral nerve sheath tumors (MPNST)-clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol 4:55PubMedCrossRefGoogle Scholar
  7. 7.
    Hirsch FR, Mok TS, Borges V et al (2010) Molecularly targeted therapy: when to stop and when to continue? Lancet Oncol 11:709–711PubMedCrossRefGoogle Scholar
  8. 8.
    Meng Z, Yang P, Shen Y et al (2009) Pilot study of huachansu in patients with hepatocellular carcinoma, nonsmall-cell lung cancer, or pancreatic cancer. Cancer 115:5309–5318PubMedCrossRefGoogle Scholar
  9. 9.
    Hagel C, Zils U, Peiper M et al (2007) Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors. J Neurooncol 82:187–192PubMedCrossRefGoogle Scholar
  10. 10.
    Williams VC, Lucas J, Babcock MA et al (2009) Neurofibromatosis type 1 revisited. Pediatrics 123:124–133PubMedCrossRefGoogle Scholar
  11. 11.
    Frahm S, Mautner VF, Brems H et al (2004) Genetic and phenotypic characterization of tumor cells derived from malignant peripheral nerve sheath tumors of neurofibromatosis type 1 patients. Neurobiol Dis 16:85–91PubMedCrossRefGoogle Scholar
  12. 12.
    Niwa T, Aida N, Fujita K et al (2008) Diffusion-weighted imaging of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. Magn Reson Med Sci 7:49–53PubMedCrossRefGoogle Scholar
  13. 13.
    Lee WH, Kim TH, You SS et al (2008) Benign schwannoma of the liver: a case report. J Korean Med Sci 23:727–730PubMedCrossRefGoogle Scholar
  14. 14.
    Zhou H, Coffin CM, Perkins SL et al (2003) Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions. Am J Surg Pathol 27:1337–1345PubMedCrossRefGoogle Scholar
  15. 15.
    Halling KC, Scheithauer BW, Halling AC et al (1996) P53 expression in neurofibroma and malignant peripheral nerve sheath tumor. An immunohistochemical study of sporadic and NF1-associated tumors. Am J Clin Pathol 106:282–288PubMedGoogle Scholar
  16. 16.
    Miller SJ, Rangwala F, Williams J et al (2006) Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res 66:2584–2591PubMedCrossRefGoogle Scholar
  17. 17.
    Liapis H, Marley EF, Lin Y et al (1999) P53 and Ki-67 proliferating cell nuclear antigen in benign and malignant peripheral nerve sheath tumors in children. Pediatr Dev Pathol 2:377–384PubMedCrossRefGoogle Scholar
  18. 18.
    Kroep JR, Ouali M, Gelderblom H et al (2011) First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study. Ann Oncol 22:207–214PubMedCrossRefGoogle Scholar
  19. 19.
    McMenamin ME, Fletcher CD (2001) Expanding the spectrum of malignant change in schwannomas: epithelioid malignant change, epithelioid malignant peripheral nerve sheath tumor, and epithelioid angiosarcoma: a study of 17 cases. Am J Surg Pathol 25:13–25PubMedCrossRefGoogle Scholar
  20. 20.
    Nixon AJ, Neuberg D, Hayes DF et al (1994) Relationship of patient age to pathologic features of the tumor and prognosis for patients with stage I or II breast cancer. J Clin Oncol 12:888–894PubMedGoogle Scholar
  21. 21.
    Moriguchi S, Maehara Y, Korenaga D et al (1993) Relationship between age and the time of surgery and prognosis after gastrectomy for gastric. J Surg Oncol 52:119–123PubMedCrossRefGoogle Scholar
  22. 22.
    Moon SJ, Lee JK, Seo BR et al. (2008). An intraosseous malignant peripheral nerve sheath tumor of the cervical spine: a case report and review of the literature. Spine (Phila Pa 1976) 33: E712-716Google Scholar
  23. 23.
    Huang XY, Huang ZL, Wang L et al (2010) Herbal compound “Songyou Yin” reinforced the ability of interferon-alfa to inhibit the enhanced metastatic potential induced by palliative resection of hepatocellular carcinoma in nude mice. BMC Cancer 10:580PubMedCrossRefGoogle Scholar
  24. 24.
    Okada K, Hasegawa T, Tajino T et al (2007) Clinical relevance of pathological grades of malignant peripheral nerve sheath tumor: a multi-institution TMTS study of 56 cases in Northern Japan. Ann Surg Oncol 14:597–604PubMedCrossRefGoogle Scholar
  25. 25.
    Li T, Zeng ZC, Wang L et al (2011) Radiation enhances long-term metastasis potential of residual hepatocellular carcinoma in nude mice through TMPRSS4-induced epithelial-mesenchymal transition. Cancer Gene Ther 18:617–626PubMedCrossRefGoogle Scholar
  26. 26.
    Hayden EC (2009) Cutting off cancer’s supply lines. Nature 458:686–687PubMedCrossRefGoogle Scholar
  27. 27.
    Mahller YY, Vaikunth SS, Currier MA et al (2007) Oncolytic HSV and erlotinib inhibit tumor growth and angiogenesis in a novel malignant peripheral nerve sheath tumor xenograft model. Mol Ther 15:279–286PubMedCrossRefGoogle Scholar
  28. 28.
    Tawbi H, Thomas D, Lucas DR et al (2008) Epidermal growth factor receptor expression and mutational analysis in synovial sarcomas and malignant peripheral nerve sheath tumors. Oncologist 13:459–466PubMedCrossRefGoogle Scholar
  29. 29.
    Ellis LM, Reardon DA (2010) Is there really a yin and yang to VEGF-targeted therapies? Lancet Oncol 11:809–811PubMedCrossRefGoogle Scholar
  30. 30.
    Zou CY, Smith KD, Zhu QS et al (2009) Dual targeting of AKT and mammalian target of rapamycin: a potential therapeutic approach for malignant peripheral nerve sheath tumor. Mol Cancer Ther 8:1157–1168PubMedCrossRefGoogle Scholar
  31. 31.
    Wojtkowiak JW, Fouad F, LaLonde DT et al (2008) Induction of apoptosis in neurofibromatosis type 1 malignant peripheral nerve sheath tumor cell lines by a combination of novel farnesyl transferase inhibitors and lovastatin. J Pharmacol Exp Ther 326:1–11PubMedCrossRefGoogle Scholar
  32. 32.
    Byer SJ, Eckert JM, Brossier NM et al (2011) Tamoxifen inhibits malignant peripheral nerve sheath tumor growth in an estrogen receptor-independent manner. Neuro Oncol 13:28–41PubMedCrossRefGoogle Scholar
  33. 33.
    Jordan CT, Guzman ML, Noble M et al (2006) Cancer stem cells. N Engl J Med 355:1253–1261PubMedCrossRefGoogle Scholar
  34. 34.
    Takaishi S, Okumura T, Tu S et al (2009) Identification of gastric cancer stem cells using the cell surface marker CD44. Stem Cells 27:1006–1020PubMedCrossRefGoogle Scholar
  35. 35.
    Visvader JE, Lindeman GJ (2008) Cancer stem cells in solid tumours: accumulating evidence and unresolved questions. Nat Rev Cancer 8:755–768PubMedCrossRefGoogle Scholar
  36. 36.
    Beug H (2009) Breast cancer stem cells: eradication by differentiation therapy? Cell 138:623–625PubMedCrossRefGoogle Scholar
  37. 37.
    Su YJ, Lai HM, Chang YW et al (2011) Direct reprogramming of stem cell properties in colon cancer cells by CD44. EMBO J 30:3186–3199PubMedCrossRefGoogle Scholar
  38. 38.
    Su W, Sin M, Darrow A et al (2003) Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. Glia 42:350–358PubMedCrossRefGoogle Scholar
  39. 39.
    Ramanathan RC, Thomas JM (1999) Malignant peripheral nerve sheath tumours associated with von Recklinghausen’s neurofibromatosis. Eur J Surg Oncol 25:190–193PubMedCrossRefGoogle Scholar
  40. 40.
    Kabawat SE, Bast RC, Bhan AK et al (1983) Tissue distribution of a coelomic epithelium related antigen recognized by the monoclonal antibody OC 125. Int J Gynecol Pathol 2:275–285PubMedCrossRefGoogle Scholar
  41. 41.
    Sevinc A, Camci C, Turk HM et al (2003) How to interpret serum CA 125 levels in patients with serosal involvement? A clinical dilemma. Oncology 65:1–6PubMedCrossRefGoogle Scholar
  42. 42.
    Lee CS, Huh JS, Chang JW et al (2010) The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic resonance imaging. J Korean Neurosurg Soc 47:51–54PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2012

Authors and Affiliations

  • Bing Yan
    • 1
  • Xianze Meng
    • 1
  • Bian Shi
    • 1
  • Jun Shi
    • 1
  • Zhifeng Qin
    • 1
  • Pinkang Wei
    • 1
  1. 1.Department of Traditional Chinese Medicine, Shanghai Changzheng HospitalSecond Military Medical UniversityShanghaiChina

Personalised recommendations