title: 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.
authors.affiliation: Department of Traditional Chinese Medicine, Shanghai Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.
authors.forename: Bing
authors.initials: B
authors.lastname: Yan
authors.forename: Xianze
authors.initials: X
authors.lastname: Meng
authors.forename: Bian
authors.initials: B
authors.lastname: Shi
authors.forename: Jun
authors.initials: J
authors.lastname: Shi
authors.forename: Zhifeng
authors.initials: Z
authors.lastname: Qin
authors.forename: Pinkang
authors.initials: P
authors.lastname: Wei
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000097:blood*; D018394:CA-125 Antigen / Q000097:blood*; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008565:Membrane Proteins / Q000097:blood*; D009364:Neoplasm Recurrence, Local / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D018317:Nerve Sheath Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000473:pathology*; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor; D018394:CA-125 Antigen; C451759:MUC16 protein, human; D008565:Membrane Proteins