Malignant peripheral nerve sheath tumour – A long story: Case report

•Segmental neurofibromatosis with MPNST showing multiple recurrences has not been reported in literature.•Studies were not conducted regarding the survival rate of such patients due to its extreme low incidence.•Being considered as a highly aggressive sarcoma, our patient with MPNST survived for a long period without distant metastasis.•Maintenance of old records helped to carry out review workup effectively. We present a rare case of Malignant Peripheral Nerve Sheath Tumour (MPNST) of the upper limb, which was excised thirteen times in thirteen years and ultimately ended in above elbow amputation. A 48 year old female presented initially with a localised swelling of 2 cms diameter in the front of the left elbow in 2007, which was excised. It recurred repeatedly and was excised. In the earlier presentations, the swellings were firm, mobile and not fixed to bone. In the last stage alone, bone fixity was identified. All the fourteen surgeries were performed by the primary author from 2007 to 2020, as the patient was particular. had been neurofibroma and fibrosarcoma. There was no evidence of distant metastasis all these years. She did not respond to radiation or chemotherapy. Initially it was single, but later multiple. She had no clinical features of Neurofibromatosis 1 (NF1) or any family history. As the history progressed, the swellings became muscle deep and later encircled the radial nerve. The radial nerve was salvaged on three occasions. On the last three occasions, the tumour had to be shaved off from the humerus. The final amputation specimen showed a single tumour infiltrating the humerus and x-ray revealed bone destruction and tumour calcification. Final diagnosis was aided by immunohistochemistry (IHC) and cytogenetic study (FISH). The case is presented for the rarity of the presentation and the trust and dependence of the patient on her personal surgeon.