Contributed by Orna O’Toole1, Alan O’Hare2, Liam Grogan3, Ciaran Bolger4, Francesca M. Brett1
Departments of 1Neuropathology, 2Neuroradiology, 4Neurosurgery and 3Oncology, Beaumont Hospital, Dublin 9. Ireland
A 20 year old woman attended her general practitioner with right upper limb pain and intermittent paraesthesias for a 4 month period. She had no neck pain or systemic symptoms and was a non smoker. There was no family history of note. She was commenced on pregabalin for pain and an MRI of cervical spine was ordered. The MRI revealed a right-sided intradural extramedullary mass extending from C7-T1 that was displacing the spinal cord to the left. She was reviewed by neurosurgery and was now complaining of paraesthesias in the right lower limb also. She had no bowel or bladder symptoms. Her examination revealed reduced sensation in the right upper limb but normal tone, power, coordination and reflexes. Cranial nerves, the left upper limb and bilateral lower limb examination were documented as normal. Imaging revealed that the lesion now extended from C5 to T3 and was causing significant cord compression at C7-T1. An enhancing extradural soft tissue mass centered in and expanding the exit foramina and indenting the thecal sac (arrow) is shown by axial T1 MRI post-contrast (Figure 1). On the T2 Sagittal MRI (Figure 2) the low signal soft tissue mass is demonstrated in the right exit foramina at four spinal levels(small arrows), normal high signal fat is seen in the foramen below (larger arrow). (Figure 2) The patient underwent emergency resection of the lesion. A large rubbery, tan piece of tissue measuring 1.5 x 0.8 x 0.5 cms and further multiple pieces of cream grey tissue measuring 3.5 x 3 x up to 0.3 cm in aggregate were removed.
Paraffin sections showed a nodular and diffuse cellular infiltrate with intervening fibrous bands. The cellular areas contained sheets of histiocytoid cells with scattered large pleomorphic cells with prominent eosinophilic nucleoli (Figure 3). Admixed with these were eosinophils and plasma cells. No classic Reed Sternberg cells were identified. Immunocytochemistry for CD30 and CD15 were positive in the larger cells (Figure 4). These cells were negative for CD21, CD23 and ALK.