Case 3 -
Primary Cerebral Amyloidoma
Suzanne Z. Powell
The Methodist Hospital
Click on each slide thumbnail image for an enlarged view
A 57-year-old woman presented with complaints of impaired vision. Physical examination revealed a right homonomous hemianopsia. Preoperative CT and MRI scans showed a large left parieto-occipital intra-axial cerebral mass. Craniotomy and resection of the mass were performed.
Case 3 - Figure 1 - Pre-operative axial T2-weighted MRI without contrast. The lesion is not well demarcated or demonstrated.
Case 3 - Figure 2 - Pre-operative axial T2-weighted MRI with contrast. A parieto-occipital mass is seen.
Case 3 - Figure 3 - Pre-operative axial T2-weighted MRI with contrast. Parieto-occipital mass.
Case 3 - Figure 4 - Hyalinized, amorphous amyloid material forming the mass lesion. Focal perivascular chronic inflammatory cell infiltrates are also present (H&E; 2X)
Case 3 - Figure 5 - "Fluffy" plaque-like amyloid deposition (H&E, 40X)
Case 3 - Figure 6 - Vascular and plaque-like amyloid deposition (H&E, 40X)
Case 3 - Figure 13 - Polarized Congo red stain demonstrating "apple green birefringence" in the mass "apple green birefringence" in the mass (Congo red, 100X)
Case 3 - Figure 14 - Polarized Congo red stain demonstrating "apple green birefringence" in the mass (Congo red, 200X)
Primary Cerebral Amyloidoma
The precise nature of amyloid fibrils and their cellular origin was a mystery from the 18th
century until the 1970's. A unifying biochemical principle has emerged that "amyloid" consists of
polymerized proteins that share a common secondary structure: beta-pleated sheets. Approximately 30
proteins are susceptible to increased folding of the polypeptide backbone into an increased beta sheet
form, which leads to fibril formation, polymerization, and deposition. The clinical features of amyloid
deposition disorders depend on the cellular origin and nature of the deposited protein. The nomenclature
of the amyloid proteins has evolved to reflect the particular precursor protein that leads to the
polymerized protein deposition. The current standardized nomenclature consists of the letter "A" (representing "Amyloid") followed by an abbreviation of the name of precursor protein. For example, amyloid resulting
from the deposition of immunoglobulin light chain is designated " AL" – "A" for "Amyloid" and "L" for
"light chain." For up-to-date information on this topic, see reference number 22 (textbook) and the
The following table can be found at the above website:
Table 1. Human Amyloidoses
|Type ||Fibril Protein ||Main Clinical Settings|
|Systemic ||Immunoglobulin light chains ||Plasma cell disorders|
|Transthyretin ||Familial amyloidosis, senile cardiac amyloidosis|
|A amyloidosis ||Inflammation-associated amyloidosis, familial Mediterranean fever|
|Beta2-microglobulin ||Dialysis-associated amyloidosis|
|Immunoglobulin heavy chains ||Systemic amyloidosis|
|Hereditary ||Fibrinogen alpha chain ||Familial systemic amyloidosis|
|Apolipoprotein AI ||Familial systemic amyloidosis|
|Lysozyme ||Familial systemic amyloidosis|
|Central nervous system ||Beta protein precursor ||Alzheimer syndrome, Down syndrome, hereditary cerebral hemorrhage with amyloidosis (Dutch)|
|Prion protein ||Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, fatal familial insomnia|
|Cystatin C ||hereditary cerebral hemorrhage with amyloidosis (Icelandic)|
|Ocular ||Gelsolin ||Familial amyloidosis (Finnish)|
|Lactoferrin ||Familial corneal amyloidosis|
|Keratoepithelin ||Familial corneal dystrophies|
|Localized ||Calcitonin ||Medullary thyroid carcinoma|
|Amylin* ||Insulinoma, type 2 diabetes|
|Atrial natriuretic factor amyloidosis ||Isolated atrial amyloidosis|
|Prolactin ||Pituitary amyloid|
|Keratin ||Cutaneous amyloidosis|
|Medin ||Aortic amyloidosis in elderly people|
* Islet amyloid polypeptide amyloidosis
"Amyloidomas" are rare causes of mass lesions in the central nervous system and constitute the rarest
form of central nervous system amyloidosis. In a recent report and review of the literature, Tabatabai
et al.  analyzed 11 cases of primary cerebral amyloidoma
review included patients with an age span of 26 to 76 and whose clinical presentation ranged from
asymptomatic to seizure disorders, psychiatric symptoms and cognitive decline. The series included
several cases of amyloid deposition localized to the trigeminal (gasserian) ganglion (one cause of
trigeminal neuralgia), skull base, and, less commonly, the orbit, sacrum, soft tissues of the legs and
nasopharynx. Spine and other bony sites were also represented
18]. Several additional cases of
solitary CNS amyloidoma that were not cited in the review have been reported
20], and there are a
few cases in the radiology literature
Amyloid deposition in the central nervous system has been reported in association with multiple
myeloma . Neurologic complications of multiple myeloma are diverse and include direct compression
(radiculopathy, cord compression, or tumor at the base of the skull or cervical spine), infiltrative
lesions (peripheral neuropathy),
and even autoimmune peripheral neuropathies .
More commonly, amyloid is associated with Alzheimer's Disease (AD). Amyloid in AD is deposited in
diffuse and core plaques, and also within leptomeningeal and cortical blood vessels. Vascular amyloid
deposition can result in lobar hemorrhage. Amyloid-related lobar hemorrhage can be seen in association
with amyloid angiopathy alone or in association with AD (see table above).
The diagnosis of amyloidoma can be made cytologically by fine needle aspiration with or without touch
or "squash" preparations
20], or through histologic examination of specimens obtained by peripheral
nerve biopsy, stereotactic biopsy, craniotomy or autopsy. Patients who have been newly diagnosed with
amyloidoma should undergo evaluation for systemic amyloidosis and for multiple myeloma .
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