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.

Malignant Peripheral Nerve Sheath Tumor in the Lesser Sac Masquerading as a Gastrointestinal Stromal Tumor of the Stomach: A Case Report

“…An MPNST arising in the abdomen is very rare, and to the best of our knowledge, only a few cases of retroperitoneal MPNSTs have been reported (7,8). Various neoplastic diseases develop in the lesser sac.…”

Percutaneous Radiofrequency Ablation for the Treatment of Peripheral Nerve Sheath Tumors: A Case Report and Review of the Literature

“…Percutaneous treatment, as used in this case, provides a viable but underutilized option. We reviewed the literature and found prior reports of percutaneous treatment of PNSTs in only seven patients [ 4 , 6 - 9 ].…”

“…A second patient had a benign, non-operated retroperitoneal schwannoma that also decreased in size and was stable for 27 months [ 4 ]. Yan et al [ 9 ] utilized microwave ablation as a salvage procedure in a patient with a large abdominal malignant peripheral nerve sheath tumor (MPNST) that recurred within two months of surgical resection. Although imaging studies immediately post-ablation showed necrosis in the tumor, the patient died within three months of the procedure [ 9 ].…”

New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications

“…In addition to expected management with routine imaging and surgical resection, possible alternative treatment options for non-cutaneous PNSTs include cryoablation, radiosurgery, microwave ablation and radiofrequency ablation. There are only single reports of percutaneous treatment of PNSTs (42)(43)(44)(45)(46)(47). The treatment objective for BPNSTs within main peripheral nerves should be lesion control and not complete ablation, since most normal fibers travel within the capsule of the tumor.…”