Case report of a primary ectopic extradural and extraspinal meningioma of the brachial plexus.

ABSTRACT

Primary ectopic extradural and extraspinal meningiomas are rare. We present a unique case of this type of meningioma in the brachial plexus. A 25-year-old man consulted us because of neuropathic supraclavicular pain and the appearance of a supraclavicular mass whose volume had increased. Clinical examination found paresis of the deltoid, biceps brachii and brachialis muscles rated as M4 (MRC) and a strong Tinel sign at the supraclavicular fossa, over the palpable mass. There was no sign pointing towards central nervous system involvement or altered general condition. MRI revealed a mass measuring 53 × 24 mm invading the C5-C6 plexus roots and the primary upper trunk, but not the bone or spinal area. This lesion was hyperintense on DWI/ADC, hyperintense on T2 with hypointense spots, and hypointense on T1 with intense heterogeneous gadolinium enhancement. Excisional biopsy was done 6 months after symptoms started. The tumor had developed at the C5 root, which was fibrous and at the C6 root, which was grossly normal. Anatomical pathology confirmed the WHO grade 1 meningioma, meningothelial and psammomatous histological subtypes. At 6 months, a follow-up MRI found no postoperative tumor remnants or recurrence. During the postoperative course, persistent paralysis of the deltoid muscle at 5 months justified a nerve transfer. This is a rare case of ectopic extraspinal and extradural meningioma of the brachial plexus. The diagnosis of an ectopic meningioma must be considered when a patient presents with a brachial plexus tumor causing neurological deficits. The extradural nature is not sufficient to rule out this diagnosis.