Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma

Abstract

Abstract Objective Malignant peripheral nerve sheath tumor (MPNST) arises from nerve sheaths, mostly seen in peripheral nerves but rare in craniospinal nerves. The information available in the literature to build up treatment strategy and improve clinical outcomes is scarce. We are reviewing cases from our institute, with emphasis on radiological features for early differentiation from its benign variants. Methods We analyzed pathologically diagnosed cases retrospectively from January 2007 to December 2018 at our institute. Clinicoradiological details and treatment parameters were collected from medical records for evaluation. Each case was contacted telephonically for final clinical follow-up at the time of writing the manuscript. Results A total of seven cases of MPNST were diagnosed in the last 10 years. It included four intracranial and three spinal cases. The mean age for the cohort was 34.3 years, with five females. We could achieve gross total resection (GTR) and subtotal resection in four (57.1%) and two (28.6%) cases, respectively. We could achieve an overall survival of 57.1% in the average follow-up of 28.2 months (range: 8–84 months). Conclusion MPNST is a rare tumor with a bad prognosis. Radical surgical resection is the mainstay of the treatment, but it is not always possible to achieve it because of the inaccessible location and large size of lesions. Preoperative diagnosis is challenging; however, few radiological findings may give a clue toward it. As a disease entity overall, it has a poor outcome with a high rate of fatality.