primary solitary amyloidoma
TRANSCRIPT
April Hendryx D.O.
MUSC
June 12, 2010
Case # 2
51st ANNUAL AANP
DIAGNOSTIC SLIDE SESSION
Case # 2
68 year old male
Pain in the mid-thorax radiating to the right;
ataxic gait
Past medical history: Ulnar neuropathy,
tendonitis, degenerative joint disease, gout.
Case # 2
Neuro Exam: T6 sensory level deficit, spastic paraparesis
Thoracic MRI: Erosive extradural lesion involving the
right 6th rib and vertebra; Compression of the spinal cord
Thoracic laminectomy and spinal decompression
Frozen section analysis: CPPD crystalline disease vs.
hydroxyapatite deposition
Decompression was completed without further mass
removal to preserve neurological function. Postoperative
improvement in gait and balance
Case # 2
1.5 years later: Return of symptoms
MRI: Progression of the bony involvement. New
epidural component
Encasing and compressing the spinal cord
Extending into the right paraspinal tissues
Radical excision of the entire process
Sagittal T1
Mass in dorsal longitudinal ligament
Coronal T1: Spinal cord compression and
extension of the mass
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Hematoxylin-eosin
Cortical bone
and amorphous
eosinophilic
deposits
admixed with
multinucleated
giant cells,
histiocytes,
lymphocytes,
and plasma
cells
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Hematoxylin-eosin
20x
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Hematoxylin-eosin
Polarization
Differential Diagnosis
Gout (Monosodium urate)
Pseudogout (Ca⁺²pyrophosphate)
Hydroxyapatite crystal disease
Amyloid deposition
GOUT
l
CPPD
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Congo red stain
Highlights
the
amorphous
material
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Polarization
Apple-green
birefringence
Diagnosis: Primary Solitary Amyloidoma
Rare subset of amyloidosis
Deposition is focal and idiopathic
Deposition not secondary to a systemic
process or plasma cell dyscrasia
Benign lesions
No associated risk of plasmacytoma related
diseases
Primary Solitary Amyloidoma
Reported in multiple sites
Extremely rare in the vertebral column
Predilection for the thoracic region
2:1 Male predominance
Primary Solitary Amyloidoma
Tumor like appearance and behavior make it
difficult to diagnose on imaging
The lesions grow slowly and can produce
significant local destruction
Bony destruction and can mimic crystalline
deposition diseases
Etiology
Product of local immunoglobulin production and
amyloid formation within a “burned out”
neoplasm
Usually AL fibrils from immunoglobulin light
chains (primary)
AA fibrils secondary to inflammatory conditions
and β-2 microglobulin in dialysis patients has also
been described
Treatment and Prognosis
Surgical excision and spinal stabilization
Complete removal of the mass:
Relieve local compression
Stops the production of amyloid and associated
infiltrative neuropathy
Low recurrence rate and cure with complete
excision
References
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6/15/10
Comments by Dr. Brian Summers
6/15/10
EQUINE SKIN MASS 2010-2
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