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Table 1 Summary of cervical spine amyloidoma cases within the literature

From: Cervical amyloidoma of transthyretin type: a case report and review of literature

Reference

Age

Sex

Clinical Symptoms

Imaging

Treatment

Outcome

Histological Description

CTS Hx

Spinal Stenosis

Dickman 1998 [4]

74

M

Upper cervical pain radiating to occiput and shoulders.

CT: C2 mass with bubbly-appearing cortical shell and complete central lucency.

Open biopsy and tumor resection via midline posterior approach. Second surgery involved fusion of C1-C3 with iliac bone graft.

Postoperatively exhibited no new neurological deficits. Patients died 3-months postoperative due to sudden myocardial infarction.

Demonstrated waxy-appearing Congo red-positive substance with green birefringence to polarized light.

Not reported.

Not reported.

Mullins 1997 [5]

58

M

Chest discomfort in midsternal/epigastric region precipitated by coughing and valsava.

MRI T1: Abnormal enhancement of the C7 vertebral body and posterior elements.

C6-C7 laminectomy and corpectomies completed with iliac bone graft and C4-T2 fusion.

12-month follow up revealed stable spine construct and no evidence of recurrence.

Stains revealed Congo red stain for amyloid, and green birefringence under polarized light.

Not reported.

Not reported.

Porchet 1998 [6]

73

M

6-year history of progressive numbness and spasticity in all limbs, predominantly the right side, dragging right leg, and neck pain.

1991 MRI: partially enhancing C1-C2 mass with odontoid erosion.

1995 MRI: enlargement of enhancing mass found from the clivus-C2.

1991: laminectomy from C1-C4.

1995: Trans-oral odontoidectomy with resection; C1-C2 transarticular screw fixation.

1991: Decreased right hand numbness, increase strength and coordination in all extremities, resuming to normal activity.

1995: At 7-months postop, able to ambulate half a mile, numbness resolved, and right lower limb strength returned to normal.

Positive Congo red stain with apple-green birefringence under polarized light. Immuno-histochemical staining was positive for β2M.

Bilateral carpal tunnel release in 1981, although this did not improve symptoms.

Not reported.

Moonis 1999 [7]

79

M

Progressive dysphagia, weakness in his arms and legs, rapid weight loss, and neck pain.

MRI: Peri-odontoid 3 × 2 cm hypointense mass.

Anterior microdissection of the tumor and posterior fusion from occiput to C3.

Not reported.

Stained positive for amyloid using Congo red stain. β2M antibodies revealed intense staining.

Carpal tunnel release 2 years prior.

Not reported.

Hwang 2000 [8]

45

F

Paraparesis, urinary incontinence, and 3-month long neck pain.

MRI: Inhomogeneous hypointense mass at C7 with partial collapse of the bony anatomy.

Decompressive corpectomy with anterior fusion from C6-T1.

After the operation, the patient’s paraparesis and urinary incontinence resolved completely. 3-year follow-up reported no specific symptoms.

Positive Congo red stain, with green birefringence to polarized light.

Not reported.

Not reported.

Mulleman 2004 [9]

79

F

Acute cervical pain and spastic tetraparesia occurring after a fall.

MRI: Peripheral enhancing hypointense retro-odontoid mass with compression at C1-C2.

Transoral approach for removal of mass. Underwent C1-C2 transarticular screw fixation 3-weeks postop.

Improved sphincter tone and strength in all limbs. Improved neurological function post rehabilitation.

Slightly positive for prealbumin/ATTR subtype.

Yes.

Not reported.

Shenoy 2004 [10]

58

M

Neck pain, progressive weakness of the limbs, and dysphagia for 2 months.

MRI T1: hypointense mass with bony destruction at C1-C2.

Intubated and given ventilatory support due to rapid decline in respiratory function.

Progressively worsened and developed bleeding diathesis and died.

Post-death transoral biopsy revealed amyloid deposits under Congo red stain with apple-green birefringence under polarized light.

Not reported.

Not reported.

Belber 2004 [11]

72

M

Acute non-radicular left arm pain, followed by mild right arm and leg pain.

MRI: C1-C3 hypointense lesion.

Bilateral C2 decompression and partial C1 laminectomy.

Discharged home day 10 postop fully ambulatory. Died 3 months later during management of MM.

Exhibited typical apple green birefringence under polarized light on Congo Red stain.

Not reported.

Not reported.

Samandouras 2006 [12]

75

F

Progressive lower limb stiffness over 16 years.

MRI T2: C2 hyperintense pannus with a cystic lesion. Mild erosion present on posterior cortical margin of C2.

C1-C2 laminectomy with excision of the cystic mass.

Not reported.

Positive for Congo red stain. Presumed to be AL, immune-histochemistry studies included AA and ATTR types.

Not reported.

Not reported.

Iplikcioglu 2007 [13]

72

M

4-year history of progressive weakness and numbness in both upper limbs, and neck pain.

MRI: Soft tissue isointense mass at C6-C7 with vertebral body destruction.

Anterior approach for resection of the C6-C7 mass with acrylic vertebroplasty.

Uneventful postoperative course and quadriparesis was decreased.

Tissue stained with Congo red under polarized light revealed yellow-green birefringence with deposits of primary (AL) amyloid.

Not reported.

Not reported.

Vignes 2007 [14]

50

F

Paresthesia in the hand and shoulder with progressive cervical pain.

MRI T1: hypointense C2-C3 lateral mass

C2-C3 laminectomy and tumor excision.

Improvement of neuralgia syndrome and neuropathic pain; however, required a cervical collar for 3 months.

Positive Congo red stain and birefringent under polarized light. Immuno- histochemistry revealed presence of β2M.

History of bilateral carpal tunnel operation with recurrent syndrome.

Not reported.

Oruckaptan 2009 [15]

47

M

Headache

MRI T1: hyperintense C1-C2 lesion with hypointense center.

Complete resection of the lesion and concomitant acrylic cranioplasty.

Uneventful postoperative course.

Congo red and crystal violet dyes verified diagnosis of amyloidoma. Protein electrophoresis showed β2M.

Not reported.

Not reported.

Farrell 2011 [16]

75

M

Right leg weakness and paresthesia in all four limbs followed by acute quadriparesis.

MRI: Paravertebral isointense soft tissue mass from C6-C7.

Radiotherapy, surgical debulking, Bortezomib, and Dexamethasone.

Cervical amyloidoma diagnosed in 1993, with symptoms returning in 2006. Final treatment in 2009 led to gradual improvement in leg power and normal arm power over 8-months.

Congo red staining demonstrated apple-green birefringence. Noted to express lambda-restricted immunoglobulin.

Not reported.

Not reported.

Hsu 2011 [17]

65

M

Progressive lower leg weakness and numbness over 2 years followed by quadriplegia.

MRI T2: C5-C6 hyperintense lesion with C6 vertebral body destruction.

Laminectomy with resection.

Dramatic improvement in muscle strength, at the time of the report the patient would walk without limitation.

Positive Congo red stain with a previous biopsy revealing β2M deposits.

Developed bilateral carpal tunnel syndrome after 8-years of regular hemodialysis.

Not reported.

Sueyoshi 2011 [18]

71

M

Urinary incontinence, sensory disturbances in the arms, and became unable to ambulate.

MRI: Contrast-enhancing extradural mass at C3-C4 with severe osteolysis of the vertebral body.

C3-C7 laminoplasty, bilateral C5 foraminotomy, and lumbar spinal fusion.

Upper limb weakness moderately improved; other symptoms partially improved and did not worsen.

Positive for amyloid deposits. Detected ATTRwt using mass spectrometric analysis.

No; however, nerve conduction study revealed prolonged sensory latency of the median nerve consistent with CTS.

MRI showed spinal canal stenosis due to osteophytes at L3-L4.

Takeshima 2012 [19]

51

F

Progressive headaches.

MRI T2: Solid extradural hypointense lesion at C2

Right sided hemilaminectomies at C1-C2, subtotal resection and right C2 nerve root decompression.

Discharged on day 14 postop with no neurological manifestations. MRI completed at 10-months postop revealed no recurrence.

Histological examination revealed amyloid deposits.

Not reported.

Not reported.

Hayashi 2012 [20]

75

F

Progressive numbness in upper limbs, fine motor disturbances in fingers bilaterally, and gait disturbance.

MRI: Large hypointense circumferentially enhanced mass in the epidural space from C1-C3.

C2-C4 laminectomy and resection of the mass.

Patient gained increased strength and coordination in extremities, decreased numbness, and was fully ambulatory 2-months postop.

Positive Congo red stain with immune-histochemical testing indicating non-AA amyloid.

Not reported.

Not reported.

Werner 2013 [21]

77

F

Worsening syncope and altered mental status, acute weight loss, gradual weakness in the upper and lower limbs bilaterally.

MRI: non enhancing mass at C1-C2 with erosive bony changes.

C1-C3 decompression with partial resection, followed by fusion from occiput-C5 using iliac bone graft.

At week 6, regained strength bilaterally in all extremities and became ambulatory. At 2-year follow-up reported intact strength, sensation, and ambulation without aids.

Congo red stained tissue featured yellow-green birefringence under polarized light.

Not reported.

Not reported.

Nitta 2015 [22]

66

M

1 week of reported dizziness, urinary retention, blunted sensation below the chest, and paraplegia 2-days prior.

MRI T1: Hypointense epidural mass at C7.

Laminectomy and tumor resection.

Paraplegia and urinary retention resolved acutely postoperatively with improved sensation.

Congo red positive tissue featured apple-green birefringence under polarized light. Immunostaining was positive for β2M amyloid.

No evidence of carpal tunnel.

Not reported.

Smitherman 2015 [23]

46

F

Lower extremity paralysis, lower body hypoesthesia, and worsening bowel/bladder incontinence.

MRI T1: Intradural extramedullary enhancing lesion at C4-T4.

C4-T4 laminectomy, resection, and posterior spinal fusion

Unchanged neurological examination with no progression at 1-year follow-up.

Congo red stained positively and displayed apple green birefringence under polarized light.

Not reported.

Not reported.

Shinkino 2016 [24]

57

M

Progressive neck pain, quadriplegia, and numbness of limbs.

MRI T2: C1-C2 hypointense mass

C2-C7 laminoplasty.

At 1-month follow-up, patient exhibited markedly improved symptoms compared to preoperative status.

Amyloid fibrils were densely enhanced with direct fast scarlet staining and showed green birefringence under polarized light. Immunohistochemistry demonstrated a positive finding for β2M.

Carpal tunnel surgery completed 9-years prior.

Thickened LF and PLL.

Dalolio 2017 [25]

63

M

Acute urinary retention, sensory disturbances in all 4 limbs followed by severe tetraparesis.

MRI T2: intradural extramedullary enhancing lesion at C4-C7.

C4-C7 laminectomy and resection. Underwent Revision surgery consisting of laminoatherectomy at C5-C6 and C6-C7 levels and removal of the lesion.

Discharged day 10 with persistent paraplegia, urinary incontinence, and lower limb sensory deficits with slight improvement in preoperative neurologic symptoms.

Amyloid deposits were confirmed on histological examination.

Not reported.

Thickened LF.

Rezania 2017 [26]

86

M

Progressive weakness, gait deterioration, falls, and urinary incontinence.

MRI T1: Peripherally enhanced extra-axial mass extending from the clivus to C2.

None, biopsy only.

Died due to complications from metastatic cancer one year later.

Mass spectrometry was performed on Congo red-positive areas which detected transthyretin-related (ATTR) amyloidosis.

Yes.

Yes.

Schneider 2018 [27]

84

F

Progressive ataxia and dysphagia.

MRI: Non-enhancing soft-tissue mass from the retro-clivus to C2 posteriorly.

Endoscopic trans-nasal resection and posterior stabilization via arthrodesis from occiput-C5.

Discharged 2-weeks postop with improved neuro-motor exam. 2-year follow-up revealed complete resolution of the mass.

Positive Congo red stain and apple green birefringence to polarized light. Presumed AA, negative for kappa and lambda AL.

Not reported.

Not reported.

Rotter 2019 [28]

58

M

Neck pain radiating to left arm, bilateral upper limb weakness over several months.

MRI T2: hypointense, contrast-enhancing mass at C1-C2.

C2 laminectomy and gross total resection.

One-month postop assessment revealed improved strength in upper and lower extremities.

Congo red showed green birefringence under cross-polarized light. Liquid chromatography tandem mass spectrometry detected an AL-kappa amyloid peptide profile.

Not reported.

Not reported.

Giorgi 2021 [29]

57

F

Progressive loss of upper and lower limb strength and sensitivity in addition to headaches.

MRI: Hypointense solid mass at C1-C2.

C1 laminectomy, followed by occiput-C5 fixation.

Neurological deficits improved immediately after surgery; 1-year follow-up revealed no signs of myelopathy progression.

Positive Congo red reaction and were positive for β2M immunostaining.

Not reported.

Not reported.

  1. CTS Hx Carpal Tunnel Syndrome history, β2M beta-2-microglobulin, ATTR Transthyretin, MM Multiple Myeloma, AL Amyloid Light Chain, AA Serum Amyloid A, ATTRwt Transthyretin wild type, LF Ligamentum Flavum, PLL Posterior Longitudinal Ligament