Spotty skin pigmentation and multiple blue naevi as cutaneous markers for spinal melanotic schwannoma

Spinal melanotic schwannomas are rare spinal tumours with a very poor prognosis in terms of mortality due to difficulty in complete resection and local recurrences. A 67-year-old man presented with acute onset progressive paraparesis for 2 weeks. The patient also had spotty skin pigmentations (blue naevi) in his right lateral thigh. MRI revealed an intradural extramedullary enhancing lesion in the lower thoracic cord level. With a preoperative diagnosis of simple nerve sheath tumour excision was planned. At laminotomy, an infiltrating "en plaque" like lesion reaching up to mid and upper thoracic cord level was identified and excision was carried out. Postoperative histopathology was unique with the identification of melanin and presence of epitheloid cells, with the additional detection of psammoma bodies and adipose-like cells. Melanotic schwannomas though rare and carry poor prognosis must be borne in mind in patients with spotty skin pigmentation and acute onset limb weakness.