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 system ic process or plasma cell dyscrasia. We present the second reported ca se of a cervical spine amyloidoma and discuss its presentation and man agement. METHODS This 58-year-old man presented with a 1-year history of intermittent chest pain that would radiate into both legs precipita ted by valsalva maneuvers. A magnetic resonance imaging (MRI) of the c ervical spine revealed a homogenously enhancing lesion focally involvi ng the C-7 vertebral level with significant spinal cord compression. H e underwent combined anterior and posterior decompressive procedures w ith instrumentation for spinal stabilization. Histopathology revealed amyloid deposits and a systemic work-up was negative for amyloidosis. RESULTS The patient is free of any tumor recurrence at 24 months and h as a stable spine construct. CONCLUSIONS Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloido sis, has an excellent prognosis with local resection. Diagnosis requir es special stains and therefore a degree of suspicion for the disease. Management of vertebral amyloidoma involves aggressive local resectio n of the tumor when feasible and stabilization of the spine as mandate d by the degree of tumor involvement. Complete evaluation for the diag nosis of systemic amyloidosis is essential for the management and prog nostication of each case. (C) 1997 by Elsevier Science Inc.