ABSTRACT
Introduction:
Leptomeningeal melanocytomas are rare tumours originating from
neural crest derived
melanocytes. They are usually solitary and presentation with multifocal meningeal melanocytoma is very rare and indicative of potentially more aggressive behaviour. This case
report and scoping
review sought to evaluate the presentation, and key radiological features that can help differentiate multifocal meningeal melanocytoma from other differentials and provide a discussion of the key management and prognostic points once these tumours are diagnosed. Case presentation A 26 year old
male presented with
neck pain radiating to both
shoulders and subjective weakness in left
shoulder movement. MRI demonstrated a large enhancing C2-C3 intradural-extramedullary lesion with further lesions at the T7/T8 level, left
cerebellopontine angle and midline suprachiasmatic region. Whilst the imaging appearances were initially
thought be indicative of a
phacomatosis such as NF2-related schwannomatosis, surgical excision of the cervical tumour confirmed a melanocytic tumour of leptomeningeal origin, consistent with multifocal meningeal melanocytoma.
Patient made a good post-operative recovery and remains under half yearly
radiological surveillance, with repeat MRI 6 months after
surgery demonstrating subtle
growth of the untreated intracranial and spinal lesions.
Literature review and
conclusions:
This is the first description, to our
knowledge, of a multifocal meningeal melanocytoma associated with both
cerebellopontine angle and suprasellar lesions. This case and included scoping
review highlight the need to consider this rare
diagnosis whenever multifocal craniospinal lesions are encountered, and the need to consider aggressive management through surgical resection and adjuvant craniospinal
radiotherapy once these tumours are diagnosed.