Chromosomal imbalances and NF2 mutational analysis in a series of 10 spinal nerve sheath myxomas

Abstract

Although these 10 nerve sheath myxomas exhibited Schwann cell differentiation and frequently showed loss of chromosome 22q typically encountered in peripheral nerve tumours, only two cases demonstrated mutations of the NF2 gene. This may indicate involvement of other tumour suppressor genes on 22q in nerve sheath myxomas and shows that they are more closely related at the molecular level to sporadic schwannomas, underscoring the presumption that they are true nerve sheath tumours.

Spinal Nerve Sheath Myxoma

“…Although nerve sheath myxomas are often classified as dermal tumors, some have been reported to occur in the spinal canal [5,6]. The most common presenting symptoms of a spinal nerve sheath myxoma in adults are sciatic pain, muscle weakness, and paranesthesia [7]. Differential diagnoses on MRI include meningioma, schwannoma, neurofibroma, paraganglioma, and leptomeningeal metastases.…”

“…Myxomas of the intraspinal nerve sheath are rare. Only eight cases of intraspinal nerve sheath myxoma have been reported to date, according to our knowledge [5][6][7][8][9][10][11]. However, no cases of nerve sheath myxoma in the cervical spinal canal have been reported.…”

Intradural extramedullary myxoma at the L1 level

“…Клиническая картина зависела от топогра-фоанатомического расположения опухоли. Так, опухоль чаще манифестировала с болевого синдро-ма, чувствительных и двигательных расстройств [5]. Дифференциальная диагностика, по данным МРТ, затруднительна ввиду того, что рентгенологическая картина сходна с менингиомами, невриномами, нейрофибромами и нейротекомами [6].…”