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

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.

DOI: 10.1007/s11060-012-0865-9

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Cite this paper

@article{Yan2012ARN, title={A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion}, author={Bing Yan and Xianze Meng and Bian Shi and Jun Shi and Zhifeng Qin and Pin-kang Wei}, journal={Journal of Neuro-Oncology}, year={2012}, volume={109}, pages={205-211} }