Primary Intra-osseous Malignant Peripheral Nerve Sheath Tumor of Tibia: A Case Report and Review of Literature

Primary intra-osseous malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare tumors either associated with neurofibromatosis type 1 (NF1) or arise spontaneously from a peripheral nerve or nerve sheath-associated cells. We report a case of primary MPNST involving the proximal tibia in a 53-year-old gentleman who presented with pain and swelling near the right knee joint. These tumors pose a distinct challenge to the clinician from diagnosis to management. Immunohistochemistry is pivotal in making the appropriate diagnosis. Radical resection remains the mainstay of treatment, while currently the role of adjuvant therapies has not been clearly established.