Samer Abdel Al
Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive form of sarcoma that develops within a peripheral nerve with most cases reported association with neurofibromatosis type-1 (NF1). Half of the cases were reported in the extremities and with the lungs being the most common organ for metastasis. To the best of our knowledge, this is the first case of a limb salvage surgery for an excision of a large exophytic MPNST of the forearm with wide surgical margins followed by split thickness skin graft and later a flexor carpi radials (FCR) tendon transfer.
Case presentation: A 51-year-old man presented to our centre with a large fungating malignant peripheral nerve sheath tumor occupying most of his forearm for which he underwent wide local excision followed by skin graft and tendon transfer as a palliative measure, since he was found to have multiple lung nodules on staging imaging, to preserve the function of his dominant limb. The operation was performed without any complications and the patient had an excellent postoperative results. Afterwards, he was started on multiple lines of chemotherapy that failed due to disease progression and the patient passed away seven months after the operation.
Conclusion: Although rare and aggressive, complete surgical excision of an MPNST is considered the primary treatment option which can be achieved with wide negative margins. However, due to late diagnosis, early metastasis, and rapid local recurrence rate, few cases have been reported with a high 5-year disease-specific survival.
Keywords: Malignant peripheral nerve sheath tumors; Heterologous tumor; Forearm
Published Date: 2020-11-30; Received Date: 2020-11-20