Systemic Amyloidosis with Predominant Spine Involvement: A Case Report

Abstract

Purpose: Primary systemic amyloidosis with main bone involvement is a rare disease. Clinical symptoms and radiographic findings are usually nonspecific and may be confused with primary bone tumor, metastatic disease, metabolic disorders or infections. The occurrence of an amyloidoma in the spine is rare. Methods: We describe the case of a 57-year old man presenting with a pathological fracture of T7 in presence of a large, soft tissue mass narrowing the spinal canal, responsible for a worsening paraplegia. Diagnosis of systemic amyloidosis was made after surgical treatment. Pathological examination showed an amorphous eosinophilic material, positive staining with Congo red, birefringence under polarized light relating to amyloid, with the presence of rare plasma cells. Results: After surgical procedure patient’s symptoms improved but with incomplete neurological recovery. PET-CT scan revealed multiple bone locations without, at the beginning, extra-skeletal involvement. The patient underwent oncological and surgical treatment with progression of the disease and visceral involvement. He died two years after the diagnosis. Conclusions: Bone involvement during systemic amyloidosis is rare and often underestimated, it had predominantly visceral involvement (kidney, heart, liver, gastrointestinal tract, lung) and unfavorable clinical course if not treated. Appropriate histopathologic studies are an essential step to define diagnosis and treatment of these patients. Treatment consists of chemotherapy, steroids, autologous blood stem cell transplantation and biologic antiinflammatory drugs. Spine localization can bring to nerves compression or pathological fracture and, in these cases, surgical treatment has a role to improve patient’s quality of life.