Spinal amyloidoma

• Calcified Spinal Amyloidoma Simulating Chondrosarcoma at Imaging
• Amyloidoma Presenting as Compressive Myelopathy
• Cervical amyloidoma of transthyretin type: a case report and review of literature
• Spinal cord compression in dialysis-related upper cervical amyloidoma - a case report
• Management and Outcome of Solitary Spinal Amyloidoma-A Systematic Literature Review
• 18F-FDG Uptake in a T2 Hypointense Paraspinal Mass
• Primary cervical spine AL-kappa amyloidoma: A case report and review of the literature
• Primary solitary retro-clival amyloidoma

Primary amyloidoma of the spine is very rare and has a predilection for the thoracic region. The tumor-like appearance and behavior make it difficult to diagnose on imaging studies. Despite its rarity and nonspecific radiologic findings, primary spinal amyloidoma should be included in the differential diagnosis of an osteolytic and calcified mass of the spine ¹⁾.

A 59 year old lady presented with progressive numbness of both feet and abdomen, walking difficulties for which the initial evaluation did not yield any result. The work up at our facility showed collapsed D3 vertebra, prevertebral collection and cord compression. She was tentatively started on antituberculous treatment, based on the radiologist opinion. Two months later, the patient had further progression of symptoms and had to undergo D3 laminectomy and surgical decompression. She also developed hypertension and diabetes in the meantime. Histopathology did not favour tuberculous process, but showed amyloid deposition. Antituberculous treatment was halted and she was evaluated for plasma cell dyscrasias. The serum protein electrophoresis with immunofixation was in favour of IgG Kappa light chain monoclonal gammopathy. She was further subjected to bone marrow aspiration and biopsy at a Cancer institute. The result was negative for plasma cell malignancy, and was suggestive of only reactive lymphocytosis. On the advice of oncologist, antituberculous treatment was started again and continued as RH- two drug regimen for 9 months along with supportive care. She improved symptomatically, able to walk independently and attended regular follow up. Three years later, a repeat MRI scan showed enlargement of the lesion to involve the posterior mediastinum. The oncology service decided to irradiate the lesion, only if she develops new symptoms ²⁾

A female patient with high-level paraparesis and lumbar stenosis in L2-L3 with combined spondylolisthesis (ASIA Impairemet Scale C). Paraparesis increased shortly after lumbar osteosynthesis. Contrast-enhanced MRI of the thoracic spine revealed medullary compression at the D5 level due to an epidural and paraspinal mass with concomitant bone infiltration. Operative decompression followed. Histopathological examination initially revealed amyloidoma. Finally the lesion was classified as a plasma cell myeloma.

Multiple myeloma may rarely present as a solitary amyloidoma in the initial pathological examination with the potential to cause spinal cord compression associated to osteolytic lesions of the spine ³⁾.

Case Report: Pathological Dorsal Spinal Fracture (Th5) due to Multiple Myeloma

Patient History: - 64-year-old male, under hematology follow-up for multiple myeloma (MM). Diagnosis Details MM IgG Kappa

This means the myeloma cells produce an abnormal IgG-type monoclonal protein with kappa light chains. IgG myeloma is the most common subtype (~50% of cases). The presence of monoclonal IgG Kappa protein is detected in serum protein electrophoresis (SPEP) and immunofixation. ISS II (International Staging System - Stage II)

ISS is based on serum beta-2 microglobulin (β2M) and albumin. Stage II: Intermediate prognosis, with β2M levels between 3.5-5.5 mg/L. Reflects a moderate tumor burden. R-ISS II (Revised ISS - Stage II)

This is an updated version of ISS that includes genetic risk markers and LDH. Stage II: Indicates an intermediate prognosis with no high-risk genetic abnormalities (e.g., del17p, t(4;14)), but possible elevated LDH. Hyperdiploidy

A favorable cytogenetic feature where there are extra copies of certain chromosomes (3, 5, 7, 9, 11, 15, 19, 21). Associated with better prognosis compared to high-risk genetic abnormalities. Previous Treatment 1. VRD (Bortezomib, Lenalidomide, Dexamethasone) – 4 cycles Bortezomib (Velcade): Proteasome inhibitor. Lenalidomide (Revlimid): Immunomodulator. Dexamethasone: Corticosteroid. Outcome: Progression of disease (PD) → This means the myeloma did not respond adequately to treatment, requiring a change in therapy. 2. Isa-KD (Isatuximab, Carfilzomib, Dexamethasone) with Radiotherapy – 3 cycles Isatuximab (Isa): Monoclonal antibody targeting CD38 on myeloma cells. Carfilzomib (K): A second-generation proteasome inhibitor. Dexamethasone (D): Anti-inflammatory and anti-myeloma effect. Radiotherapy: Used for local control of bone lesions or pain relief. Outcome: Serological and clinical response achieved → This means there was a decrease in M-protein levels and improvement in symptoms. Transplantation & Maintenance Therapy 1. TASPE (Autologous Stem Cell Transplant - ASCT) TASPE (Trasplante Autólogo de Progenitores Hematopoyéticos) is the Spanish term for Autologous Stem Cell Transplant (ASCT). High-dose chemotherapy (e.g., Melphalan) followed by stem cell reinfusion. Goal: Deepen the response and prolong remission. 2. Maintenance Therapy The patient is currently on maintenance therapy with:

Velcade (Bortezomib): Proteasome inhibitor to prevent relapse. Lenalidomide (Revlimid): Enhances immune response and prolongs remission. Summary Diagnosis: IgG Kappa MM, ISS II, R-ISS II, hyperdiploidy (intermediate risk). Initial treatment (VRD, 4 cycles): Disease progression. Second-line therapy (Isa-KD + Radiotherapy, 3 cycles): Achieved response. Underwent ASCT (TASPE). Currently on maintenance (Velcade + Lenalidomide).

- Comorbidities: Hypertension, gout, history of SARS-CoV-2 pneumonia, ex-smoker.

- Medication: Antihypertensive, anticoagulants, colchicine, antiviral, and prophylactic antibiotics.

Clinical Presentation:

The patient was discharged after surgery for pathological vertebral fracture (Th5) due to spinal amyloidoma with spinal cord compression. He presented to the emergency department for evaluation of anemia (Hb: 7.1 g/dL) and a progressively enlarging mass at the surgical wound site, accompanied by generalized weakness, dyspnea, and persistent dorsolumbar pain.

Physical Examination: - Vitals: BP 113/78 mmHg, HR 84 bpm, Temp 36.5°C, SpO2 100%. - Conscious, oriented, pale, and in regular general condition. - Mild bibasilar lung crepitations, normal cardiovascular auscultation. - Abdominal exam unremarkable. - No peripheral edema or focal neurological deficits. - Dorsal surgical site swollen, erythematous, tender, warm, and draining purulent material.

Investigations: - Hematology: Anemia (Hb 6.9 g/dL), thrombocytopenia, mild leukocytosis. - Biochemistry: Elevated inflammatory markers (PCR 34.56 mg/dL), mild liver enzyme elevation. - Microbiology:

1. Blood cultures: Negative.
2. Wound cultures: Staphylococcus capitis, Enterococcus faecalis, Haemophilus influenzae.
3. Serology: Positive for past CMV, Parvovirus B19 IgM positive.
4. Viral PCR: Metapneumovirus and Rhinovirus detected.

- Imaging:

1. CT Spine: Post-surgical changes with extensive dorsal collection (7x6x7.5 cm), concerning for abscess.
2. MRI Spine: Abscess at D6, worsening contrast enhancement of perimedullary structures, worsening vertebral collapse (Th5-Th6).
3. CT/MRI Brain: No acute pathology, but cortical atrophy beyond expected for age.

Surgical and Medical Management: - Surgery (Urgent Wound Debridement and Drainage):

1. Incision reopened with drainage of purulent collection extending to deep tissues.
2. Wound irrigated with betadine, saline, and vancomycin solution.
3. Closure by layers.
4. Samples sent for microbiology.

- Hospital Course:

1. Persistent neurological symptoms (hallucinations, sudden hearing loss, agitation) warranted neurology, psychiatry, and ENT evaluations.
2. No evidence of meningitis on lumbar puncture.

The patient remained afebrile after targeted IV antibiotic therapy. - Discharge Plan:

1. Continued IV antibiotics at home under specialized care.
2. Rehabilitation and close outpatient monitoring.

Conclusion: This case highlights the complexity of spinal infections in MM patients post-radiotherapy and surgical intervention. The presence of multiple pathogens, systemic involvement, and neurological symptoms warranted a multidisciplinary approach. Close follow-up is essential to monitor recovery and prevent further complications.