Case reportMalignant peripheral nerve sheath tumor in the cervical spine with multiple nevus cell nevi
Introduction
Malignant peripheral nerve sheath tumor (MPNST) is the diagnostic term for malignant neoplasms that arises from a peripheral nerve or that incurs nerve sheath differentiation [1], [2]. Intra- and extradural MPNST in the upper cervical spine are extremely rare. Wide resection with a negative surgical margin is the most effective treatment [3], [4]; however it is often not possible to achieve in cases of MPNST in the cervical spine because of the close proximity to vital structures such as the spinal cord and vertebral artery. We experienced a rare case of MPNST in the cervical spine with dermatological findings. The patient had distinctive multiple nevus cell nevi over his left upper extremity and trunk on the same side as the tumor. The patient underwent gross total resection of the tumor with postoperative radiotherapy, and there was significant neurological improvement after surgery. Therefore, we present the clinical and neurological manifestations, as well as the radiological and pathological findings, of an intra- and extradural cervical spinal MPNST with multiple nevus cell nevi.
Section snippets
Presentation
The patient was a 45-year-old male from the Philippines with no medical history of neoplastic disease. He had more than a hundred black eruptions with the size ranging from 1 to 5 mm in diameter at the left side of his back and upper extremities (Fig. 1). Progressive posterior cervical pain and left hemiparesis was observed (manual muscle test 3/5). The patient was unable to walk by himself. He had severe bladder and bowel dysfunction. Magnetic resonance imaging (MRI) showed a dumbbell-shaped
Discussion
MPNST has been classified as a soft tissue sarcoma and constitutes only 3%–10% of all soft tissue sarcomas [3], [5]. The World Health Organization (WHO) coined the term “MPNST” to indicate that the lesions are malignant lesions of neurogenic origin with similar biological behavior [6]. MPNST can arise either de novo or from a sarcomatous degeneration within a pre-existing plexiform neurofibroma [5]. They have also been shown to arise within a field of previous irradiation [7], [8]. A definitive
Conflict of interest
The authors declare that they have no conflict of interest.
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