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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 6 | Page : 1361-1363
Atypical presentation of primary spinal amyloidoma mimicking a giant cell tumor
Devi P Patra1, Harsimrat Bir Singh Sodhi1, Rajesh Chhabra1, Bishan D Radotra2
1 Department of Neurosurgery, PGIMER, Chandigarh, India
2 Department of Pathology, PGIMER, Chandigarh, India
|Date of Web Publication||11-Nov-2016|
Harsimrat Bir Singh Sodhi
Department of Neurosurgery, PGIMER, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Patra DP, Sodhi HB, Chhabra R, Radotra BD. Atypical presentation of primary spinal amyloidoma mimicking a giant cell tumor. Neurol India 2016;64:1361-3
Amyloidoma is a localized accumulation of amyloid in the extracellular spaces of bone and soft tissues giving rise to a tumor like mass. It is classified as primary, in the absence of any plasma cell dyscrasias with the presence of normal serum proteins. Primary spinal amyloidoma is a rare entity, and so far, there are only a few, anecdotal case reports.,,,,, We report a case of primary thoracic spinal amyloidoma mimicking a giant cell tumor on radiological and cytological evaluation.
A 43-year-female patient presented with dull aching, gradually progressive backache of 6 month duration. At the initial presentation, the patient did not have any neurological deficits. The patient was investigated with a gadolinium enhanced magnetic resonance imaging (Gd-MRI) of the dorsolumbar spine, which revealed an expansile lesion involving the 11th dorsal (D11) vertebral body, with a heterogenous signal intensity on T1-weighted (T1W) and T2-weighted (T2W) images, and peripheral contrast enhancement [Figure 1]a-d]. The lesion had significant extra-osseous extension into the pre- and para-vertebral regions along with extension into the spinal canal. Needle cytology from the lesion revealed the presence of spindle cells with occasional mitosis, suggestive of a giant cell tumor. Whole body positron emission tomography (PET) computerized tomography showed a fluoro-2-deoxy-D-glucose avid, sclerotic lytic lesion at D11, and another small lesion at L4. Digital subtraction angiography was suggestive of hypervascularity, with a single feeding vessel into the lesion, which was also suggestive of giant cell tumor.
|Figure 1: Computed tomogram, sagittal reconstruction, shows the lytic lesion in the 11th dorsal vertebral body (a). Magnetic resonance imaging T1-weighted axial (b), T2-weighted sagittal (c), and T1-weighted-contrast (d), showing a heterogenously enhancing lesion involving the vertebral body with extension into the spinal canal compressing the cord. Histopathology images showing nodular deposits of amyloid surrounded by lymphoplasmacytic infiltrate (e) with apple-green bifringence under polarized light (f). Postoperative follow-up gadolinium enhanced magnetic resonance imaging showing the decompressed cord with implant in situ (g and h)|
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Endovascular angio-embolization of the lesion was done as a primary treatment modality in view of the giant cell tumor pathology. The patient presented one month later with increasing back pain, spastic paraparesis (Grade 4/5) with sensory involvement. A repeat Gd-MRI dorsolumbar spine MRI imaging showed an increase in the size of the lesion with a significant increase in soft tissue mass that was causing cord compression. The patient underwent a D11 corpectomy with cage placement and pedicle screw fixation through a postero-lateral approach. Intraoperatively, the vertebral body was destroyed, and there was a yellowish gritty avascular mass, with preservation of posterior elements. There was extradural and paravertebral necrotic collection (probably secondary to embolization and ischemic necrosis). Histopathological examination of the lesion revealed nodular deposits with apple green bifringence under polarized light suggestive of amyloid [Figure 1]e and [Figure 1]f. Further, workup for systemic amyloidosis was negative.
Although the postoperative course was morbid, the patient had improvement in neurological status. She developed collapse of the lower zone of the left lung that later on led to pneumonitis but recovered well with antibiotics and intensive chest physiotherapy. At present, she is doing fine at a follow up of 24 months, and has no neurological deficits. The follow-up Gd-MRI image of the dorsolumbar spine was suggestive of a small residual lesion at D11 level with no major uptake on PET imaging, and the lesion at L4 level was persistent, with no change in size or appearance [Figure 1]g and [Figure 1]h.
Amyloidoma is a nodular mass of amyloid, which is an extracellular deposit of beta-pleated proteins. Due to its tumor like morphology and clinical behavior, it is difficult to diagnose this entity on imaging studies. A correct histological diagnosis and an adequate resection of the lesion is associated with excellent results with complete cure. Amyloidoma in the spine usually arises from the marrow spaces sparing the disc space. Primary spinal amyloidoma is a rare benign lesion with a predilection for the thoracic spine. It is a slow growing lesion but can be associated with significant local tissue destruction, making the spine prone to developing pathological fractures. The lesion may cause a paravertebral soft tissue involvement with a variable amount of calcification. Most of the patients become symptomatic because of neurological deficits and associated localized pain. The majority of the patients have a slow and progressive deterioration, but acute presentation with sudden onset paraplegia and urinary incontinence has also been reported. At radiology, the lesions appear as lytic lesions associated with bony destruction and adjacent soft tissue mass. Due to the ectopic ossification and calcification, it shows an intense uptake of bone radiotracers. The noncalcified part of the lesion may show moderate contrast enhancement. On Gd-MRI, they are hypo to isointense on T1W images, iso- to hyperintense on T2W images and with a variable contrast enhancement on gadolinium injection. Overall, radiologically it may mimic the presence of an infection, bony neoplasm, and metabolic diseases.
Due to the tumor like appearance, a definitive diagnosis based on radiology is extremely difficult to establish, and histology is invariably needed. Amyloids form beta-pleated sheets, which can be identified with a polarized microscope as characteristic apple green bifringence with Congo red staining. Electron microscopy and immunohistochemical studies are useful for further characterization of the amyloid deposits. Patients with solitary amyloidoma usually have a good prognosis after adequate resection, and there is no report of recurrence till date., Even 50–75% reduction in the lesion size is sufficient for regression of the amyloid deposit. Progression to systemic amyloidosis is exceptionally rare even in the presence of detectable M proteins or abnormal light chain ratio. However, all patients should be systemically evaluated and periodically followed up because failure to detect the co-existing plasma cell dyscrasias may later on lead to the development of generalized amyloidosis. Postoperative complete evaluation for systemic amyloidosis or myelomatosis is necessary before adjuvant radiotherapy and chemotherapy is instituted. Chemotherapy can be given in patients with amyloidosis with the aim to suppress the underlying monoclonal plasma cell dyscrasias. There is no role of radiotherapy.
Spinal amyloidoma, although rare, should be considered as an important differential diagnosis while dealing with lytic mass lesions in the spine with calcification. Fine-needle cytology can sometimes be misleading, as in our case, and adequate tissue biopsy is required in such lesions. Extreme caution should be exercised in these patients because safe surgical excision may be the best option, especially when the spinal lesion is associated with spinal cord compression.
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