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

Abbas N, George K, Dardis R: Primary amyloidoma of the thoracic spine causing paraparesis. British Journal of Neurosurgery 2008; 22 (2): 286-288.

Arnesen M, Manivel JC.: Plasmacytoma of the thoracic spine with intracellular amyloid and massive extracellular amyloid deposition. Ultrastruct Pathol.

1993 May-Aug;17(3-4):447-53.

Belber CJ, Graham DL: Multiple myeloma-associated solitary epidural amyloidoma of C2-C3 without bony connection or myelopathy: case report and

review of the literature. Surg Neurol 2004;62:506-509; discussion 509.

Bruninx G, Nubourgh Y, Cornut P, Fumiere E, Vanderkelen B, Delcour C: Isolated idiopathic amyloid tumor of the sacrum. An important differential

diagnosis. J Radiol 2001;82:495-497.

Cloft HJ, Quint DJ, Markert JM, Iannettoni MD, Papadopoulos SM: Primary osseous amyloidoma causing spinal cord compression. AJNR Am J Neuroradiol

1995;16:1152-1154.

Dee CH, Missirian RJ, Chernoff IJ: Primary amyloidoma of the spine. A case report and review of the literature. Spine 1998;23:497-500.

Dickman CA, Sonntag VK, Johnson P, Medina M: Amyloidoma of the cervical spine: a case report. Neurosurgery 1988;22:419-422.

Haridas A, Basu S, King A, Pollock J: Primary isolated amyloidoma of the lumbar spine causing neurological compromise: case report and literature review.

Neurosurgery 2005;57:E196; discussion E196.

Hsu CW, Wu MS, Leu ML: Dialysis-related cervical amyloidoma presenting with quadriplegia. Ren Fail 2001;23:135-138.

Hwang SS, Park YH, Kim JY, Jung SL, Ahn MI, Park CK, et al.: Primary amyloidoma of the cervical spine. AJNR Am J Neuroradiol 2000;21:601-603.

Iplikcioglu AC, Bek S, Gokduman CA, Cosar M, Sav A: Primary solitary cervical amyloidosis: case report and review of the literature. Spine 2007;32:E45-

47.

Leeson MC, Rechtine GR, Makley JT, Carter JR: Primary amyloidoma of the spine. A case report and review of the literature. Spine 1985;10:303-306.

References

Mathew JM, Rajshekhar V: Primary amyloidoma of the thoracic spine. Br J Neurosurg 1998;12:448-451.

McKechnie S, Yang F, Harper CG, McGee-Collett M, Henderson CJ, Liepnieks JJ, et al.: Amyloidoma of a spinal root. Neurology 2003;61:834-836.

Meyers SP, Mullins KJ, Kazee AM: Unifocal primary amyloidoma of the spine causing compression of the cervical spinal cord: MR findings. J Comput Assist

Tomogr 1996;20:592-593.

Mizuno J, Nakagawa H, Tsuji Y, Yamada T: Primary amyloidoma of the thoracic spine presenting with acute paraplegia. Surg Neurol 2001;55:378-382.

Moonis G, Savolaine ER, Anvar SA, Khan A: MRI findings of isolated beta-2 microglobulin amyloidosis presenting as a cervical spine mass. Case report and

review of literature. Clin Imaging 1999;23:11-14.

Mulleman D, Flipo RM, Assaker R, Maurage CA, Chastanet P, Ducoulombier V, et al.: Primary amyloidoma of the axis and acute spinal cord compression: a

case report. Eur Spine J 2004;13:244-248.

Omura, Kikuo; Hukuda, Sinsuke; MD, PhD; Matsumoto, Keiji; MD, PhD; Katsuura, Akitomo; Nishioka, Junichi; MD, PhD; Imai, Shinji.. Cervical Myelopathy

Caused by Calcium Pyrophosphate Dihydrate Crystal Deposition in Facet Joints: A Case Report. Spine. 21(20):2372-2375, October 15, 1996.

Suri VS, Tatke M, Kumar S, Gupta V.: Amyloidoma of the thoracic spine. Case report. J Neurosurg. 2001 Apr;94(2 Suppl):299-301.

Tanja Staub-Zähner, Daniela Garzoni, Christian Fretz, Christoph Lampert, Christian Öhlschlegel, Rudolf P Wüthrich and Thomas Fehr. Pseudotumor of gout

in the patella of a kidney transplant recipient .Nature Clinical Practice Nephrology (2007) 3, 345-349

Unal A, Sütlap PN, Kýyýk M.: Primary solitary amyloidoma of thoracic spine:a case report and review of the literature. Clin Neurol Neurosurg. 2003

Jul;105(3):167-9.

Volkan Aydin M, Sen O, Bolat F, Tufan K, Kizilkilic O, Altinors N.: Primary amyloidoma of the thoracic spine. J Spinal Disord Tech. 2006 Apr;19(2):145-7.

6/15/10

Comments by Dr. Brian Summers

6/15/10

EQUINE SKIN MASS 2010-2

CR CR

Polarized light