gms | German Medical Science

Objective: Plasmocytomas (PC) of the cranio-cervical junction (CCJ) are rare. They may occur as solitary lesion or as part of multiple myeloma (MM). Due to their destructive growth they may induce spinal instability and harbor the risk of sudden death. Although the primary treatment of plasmocytomas is radiotherapy, their location at the CCJ requires special consideration. So far there exist no guidelines. We proposed to review the management of personal cases and to perform a review of the literature to work out an effective management algorithm.

Method: Retrospective clinical study. We reviewed the electronical pathological database of a tertiary care center from 2004-2012. Patients harboring a PC or MM of the CCJ (C0-C2) were identified. Clinical charts, imaging data, operative reports and follow-up information concerning patient management were analyzed. A thorough review of the English literature was performed.

Results: The series comprised 4 patients (1 woman and 3 men, mean age 58 years). There was one solitary plasmocytoma (axis) and three multiple myelomas (atlas, n=1, axis, n=2). Two MM patients received vertebroplasty (C1 and C2, respectively) and radiotherapy as primary management. However, in both secondary instability of the CCJ occurred after 12 months and 5 months, respectively. The patient with axis vertebroplasty developed spinal kinking with myelopathy and tetraplegia and finally died. The patient with atlas vertebroplasty developed a pathological fracture with cement dislocation and basilar impression. Clinically, he suffered from progressive neck pain. He became symptom-free after occipito-cervical stabilization (OCS). Patient #3 was emergently operated because of a sudden apnoe due to brain-stem compression by a pathological C2 fracture. The outcome was good after vertebroplasty and OCS. Patient #4 had neck pain. An early vertebroplasty and OCS were performed and resulted in a favorable outcome. In total, for three patients an OCS was performed resulting in good pain control and prevention of neurological deficits.

Conclusions: PC are highly radiosensitive. As a matter of fact life-threatening instability may occur at the CCJ despite radiotherapy and vertebroplasty. Based on personal experience and a literature review we favor early occipito-cervical stabilization. It should be part of an interdisciplinary and multi-modal therapy. This results in sustainable spinal stability and good clinical outcome.