Abstract
BackgroundMalignant peripheral nerve sheath tumor is a rare and aggressive form of sarcoma that arises from a peripheral nerve, mostly in association with neurofibromatosis type 1. Half of the cases were reported in the extremities, with the lungs being the most common site of metastasis. We report a rare case of successful limb salvage surgery performed for a large exophytic malignant peripheral nerve sheath tumor of the right forearm with wide surgical margins followed by split-thickness skin graft and later a flexor carpi radialis tendon transfer to extensor digitorum communis tendon.Case presentationA 51-year-old Bedouin Arabic man presented to our institution with an incompletely excised, large, fungating, malignant peripheral nerve sheath tumor occupying most of his right forearm. Staging imaging showed multiple lung nodules. He underwent wide local excision followed by skin graft and tendon transfer as a palliative measure to preserve the function of his dominant limb. The operation was performed without any complications, and the patient had an excellent postoperative result. Afterward, he was started on multiple lines of chemotherapy that failed because of disease progression, and the patient died 7 months after the operation.ConclusionClinicians must consider the possibility of soft tissue sarcoma even in a patient with a small, slow-growing, superficial mass. Furthermore, a wrong open biopsy or nononcological surgical procedure may lead to possible contamination and ultimately a more radical procedure than would have originally been necessary, where this can be prevented by an early referral to a highly specialized sarcoma center.
Highlights
Malignant peripheral nerve sheath tumor (MPNST) accounts for 5–10% of all soft tissue sarcomas [1]
It is well documented that patients with neurofibromatosis type 1 (NF1) have a greater lifetime risk of developing MPNSTs (8–13%) and worse prognosis in comparison with sporadic cases, with 5-year disease-specific survival of 16–38% versus 42– 57%, respectively [2, 5,6,7,8,9,10]
We present a rare case of a solitary, slow-growing mass that persisted for 18 months, which is a nontypical feature of sarcomas, which have very rapid and aggressive behavior
Summary
This case report summarizes the surgical forearm preservation of a rare presentation of MPNST where a solitary, nonspecific, small, superficial, slow-growing mass encountered in the limbs should alert physicians to consider the possibility of sarcoma and use a careful approach. In the setting of low- to intermediate-income countries with limited resources, patients are frequently referred to tertiary cancer centers when the disease has already rapidly progressed locally, most likely due to possible contamination by a wrong open biopsy or nononcological surgical procedure. We believe that, sharing and publishing our experience in managing challenging sarcoma cases will better enhance the understanding of how to approach and treat similar cases
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