Primary solitary amyloidosis of the spine: a case report and review of the literature.

Background: Amyloidosis is a term that encompasses a group of disorders that have as their common feature the intercellular deposition of the amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. We present the second reported case of a cervical spine amyloidoma and discuss its presentation and management.

Methods: This 58-year-old man presented with a 1-year history of intermittent chest pain that would radiate into both legs precipitated by valsalva maneuvers. A magnetic resonance imaging (MRI) of the cervical spine revealed a homogenously enhancing lesion focally involving the C-7 vertebral level with significant spinal cord compression. He underwent combined anterior and posterior decompressive procedures with instrumentation for spinal stabilization. Histopathology revealed amyloid deposits and a systemic work-up was negative for amyloidosis.

Results: The patients is free of any tumor recurrence at 24 months and has a stable spine construct.

Conclusions: Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and therefore a degree of suspicion for the disease. Management of vertebral amyloidoma involves aggressive local resection of the tumor when feasible and stabilization of the spine as mandated by the degree of tumor involvement. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication of each case.