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


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.


Malignant peripheral nerve sheath tumor Retroperitoneum Aggressive treatment Prognosis CA 125 



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.


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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

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