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51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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.

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51st ANNUAL AANP DIAGNOSTIC SLIDE SESSION

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

  2. 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.

  3. 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

  4. 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

  5. Sagittal T1 Mass in dorsal longitudinal ligament

  6. Coronal T1:Spinal cord compression and extension of the mass

  7. Cortical bone and amorphous eosinophilic deposits admixed with multinucleated giant cells, histiocytes, lymphocytes, and plasma cells Hematoxylin-eosin

  8. 20x Hematoxylin-eosin

  9. Polarization Hematoxylin-eosin

  10. Differential Diagnosis Gout (Monosodium urate) Pseudogout (Ca⁺²pyrophosphate) Hydroxyapatite crystal disease Amyloid deposition

  11. GOUT l

  12. CPPD

  13. Highlights the amorphous material Congo red stain

  14. Apple-green birefringence Polarization

  15. 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

  16. Primary Solitary Amyloidoma Reported in multiple sites Extremely rare in the vertebral column Predilection for the thoracic region 2:1 Male predominance

  17. 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

  18. 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

  19. 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

  20. 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.

  21. 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.

  22. Comments by Dr. Brian Summers

  23. EQUINE SKIN MASS 2010-2 CR CR Polarized light

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