april hendryx d.o. musc june 12, 2010 case # 2 51st annual aanp diagnostic slide session

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April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Page 1: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

April Hendryx D.O.MUSC

June 12, 2010Case # 2

51st ANNUAL AANPDIAGNOSTIC SLIDE SESSION

Page 2: 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.

Page 3: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 4: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 5: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

Sagittal T1

Mass in dorsal longitudinal ligament

Page 6: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

Coronal T1: Spinal cord compression and extension of the mass

Page 7: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Hematoxylin-eosin

Cortical bone and amorphous eosinophilic deposits admixed with multinucleated giant cells, histiocytes, lymphocytes, and plasma cells

Page 8: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Hematoxylin-eosin

20x

Page 9: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Hematoxylin-eosin

Polarization

Page 10: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

Differential Diagnosis

Gout (Monosodium urate)

Pseudogout (Ca ²pyrophosphate) ⁺

Hydroxyapatite crystal disease

Amyloid deposition

Page 11: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

GOUT

l

Page 12: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

CPPD

Page 13: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Congo red stain

Highlights the amorphous material

Page 14: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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Polarization

Apple-green birefringence

Page 15: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 16: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

Primary Solitary Amyloidoma

Reported in multiple sites

Extremely rare in the vertebral column

Predilection for the thoracic region

2:1 Male predominance

Page 17: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 18: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 19: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

Page 20: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

ReferencesAbbas 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.

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ReferencesMathew 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.

Page 22: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

6/15/10

Comments by Dr. Brian Summers

Page 23: April Hendryx D.O. MUSC June 12, 2010 Case # 2 51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

6/15/10

EQUINE SKIN MASS 2010-2

CR CR

Polarized light