Plasmacytoma of C1 and C2 in an elderly female: The impediments in proper management in a Rural Tertiary Care Hospital

Joe M Das; Rashmi Sapkota; Binjura Shrestha

doi:10.4103/EJSS.EJSS_11_17

CASE REPORT

Year : 2017 | Volume : 1 | Issue : 2 | Page : 40-43

Plasmacytoma of C1 and C2 in an elderly female: The impediments in proper management in a Rural Tertiary Care Hospital

Joe M Das, Rashmi Sapkota, Binjura Shrestha
Department of Neurosurgery, College of Medical Sciences - Teaching Hospital, Bharatpur, Chitwan, Nepal

Date of Web Publication

15-Sep-2017

Correspondence Address:
Joe M Das
Department of Neurosurgery, College of Medical Sciences - Teaching Hospital, Bharatpur, P. O. Box: 23, Chitwan
Nepal

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/EJSS.EJSS_11_17

Abstract

Solitary plasmacytoma arising as a primary bone tumor at the craniovertebral junction is very rare, with only a few cases reported in the literature. Here, we present a 62-year-old female who presented with complaints of neck pain and restricted neck movements of short duration. She had features of upper motor neuron involvement in all the limbs, but the motor power was preserved with no bladder symptoms. The magnetic resonance imaging of the cervical spine revealed an expansile lytic lesion at the craniovertebral junction, involving the C2 vertebral body and posterior elements of C1 and C2. The bulk of the mass was extending posteriorly and compressing the cord. Since there was a chance of neurological deterioration due to the extensive anterior and posterior column involvement, we went ahead with surgical decompression of the lesion posteriorly. The posterior component was near totally decompressed, and the spine was stabilized with occiput-C3 fixation and fusion using rib grafts and steel wires due to financial constraints. The postoperative period was uneventful, and she was followed up for 2 months during which there was no neurological deterioration. Subsequently, she was lost to follow-up. She did not undergo radiation therapy or myeloma workup due to financial limitation. The histopathological examination of the lesion revealed plasmacytoma.

Keywords: Atlantoaxial joint, craniovertebral junction, occipitocervical fusion, plasmacytoma, rib graft

How to cite this article:
Das JM, Sapkota R, Shrestha B. Plasmacytoma of C1 and C2 in an elderly female: The impediments in proper management in a Rural Tertiary Care Hospital. J Spinal Stud Surg 2017;1:40-3

How to cite this URL:
Das JM, Sapkota R, Shrestha B. Plasmacytoma of C1 and C2 in an elderly female: The impediments in proper management in a Rural Tertiary Care Hospital. J Spinal Stud Surg [serial online] 2017 [cited 2023 May 28];1:40-3. Available from: https://www.jsss-journal.com/text.asp?2017/1/2/40/214884

Introduction

The estimated incidence of primary malignancies of the spine is 2.5–8.5/100,000 population per year.^[1] Neoplasms of the craniocervical junction (CCJ) make up only 0.5% of all spinal malignancies, with metastases and primary tumors distributed equally. Nearly, half of all solitary osseous plasmacytomas (SOPs) convert to multiple myeloma (MM) within 2 years of diagnosis.^[2] Both are very rare malignancies at the CCJ. As per our knowledge, only 53 cases of plasma cell neoplasm of CCJ have been reported.^[3] Solitary plasmacytoma (SP), which constitutes <5% of all plasma cell neoplasms, is the clinical condition characterized by the localized proliferation of clonal plasma cells. Here, we describe a case of SOP occurring at the Atlas ^{More Details} and axis and its subsequent surgical management by posterior decompression and fusion.

Case Report

A 62-year-old female presented with complaints of pain and restricted movements of the neck of 2 weeks' duration. There was no history of trauma, fever, weakness of limbs, bowel or bladder symptoms. She had no history of tuberculosis. She was neither a diabetic nor a hypertensive. Clinical examination revealed spasticity in all limbs, but she had grade 5 power (medical research council grading) in all the limbs. All the deep tendon reflexes were exaggerated and bilateral plantar reflexes were upgoing. Clinically, a soft to firm fixed lump was palpable just below the occiput in the posterior midline around 4 cm × 3 cm × 3 cm.

Computerized tomogram of the cervical spine [Figure 1] revealed a lytic lesion involving the odontoid, posterior arch of the atlas, and C2 spinous process. Magnetic resonance imaging (MRI) of the cervical spine and craniovertebral junction [Figure 2], [Figure 3], [Figure 4] revealed lytic lesion involving the C2 vertebral body including dens and the posterior elements of C1, associated with large soft tissue mass component compressing and constricting the cord at C1 and C2 levels. No intrinsic cord signal changes were noted. The bulk of the mass was seen to extend posteriorly between the occiput and C3 spine. The mass was homogeneous and showing mild peripheral enhancement on contrast. The rest of the vertebrae did not show any lytic lesions. The radiological impression was metastasis.

Figure 1: Midsagittal plain computerized tomography scan of the cervical spine showing lytic lesion involving the atlas and axis vertebrae.

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Figure 2: Midsagittal contrast-enhanced magnetic resonance imaging scan of the cervical spine showing isointense lesion with peripheral contrast enhancement involving the atlas and axis with bulk of the mass posteriorly.

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Figure 3: Midsagittal T2-weighted magnetic resonance imaging scan of the cervical spine showing the hypointense lesion with cord compression.

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Figure 4: Axial contrast-enhanced magnetic resonance imaging scan of the cervical spine at the level of axis showing the tumor compressing the cord posteriorly.

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There was no anemia, renal impairment, or hypercalcemia. The metastatic workup, including chest X-ray and ultrasound abdomen, was negative. The patient was planned for surgical decompression of the lesion.

In the prone position, inion to C4 was exposed through a midline vertical incision. The tumor was seen bulging posteriorly (3 cm × 2.5 cm × 2 cm) obscuring and partially destroying the posterior arch of the atlas and spinous process and laminae of axis. The tumor [Figure 5] was grayish red in color, highly vascular, firm in consistency, and maintaining clear plane with dura, vertebral artery, and paravertebral muscles. The vertebral artery was visualized on the left side. The tumor was internally decompressed with the help of suction and sharp dissection up to the lateral masses of axis [Figure 6]. The part of the tumor extending anterior to the lateral masses and lying anterior to the cord was left behind. The dura was lax at the end of the decompression with no dural tear. For stabilization, the seventh rib on the right side was harvested through a posterior oblique incision. Steel wires were placed on either side of the occipital keel by drill holes and fixed with rib pieces kept on either side. The lower ends of the rib pieces were fixed to C3 through steel wires passed in a sublaminar fashion [Figure 7]. The wounds were closed in a layered fashion.

Figure 5: Intraoperative picture showing the grayish-red lesion – preexcision.

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Figure 6: Intraoperative picture showing the postexcision status.

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Figure 7: Intraoperative picture showing the status after occipitocervical fusion with rib grafts and steel wires.

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Postoperatively, she made an uneventful recovery. She had grade 4+ power in all limbs and was ambulant without support. Philadelphia collar was applied for a period of 6 weeks. She was able to void urine normally. Postoperative X-ray of the cervical spine [Figure 8] revealed stable fixation. The relatives were not keen for further workup for MM, and the patient did not undergo radiation therapy. She was lost to follow-up after 2 months till which there was no neurological deterioration.

Figure 8: Postoperative X-ray cervical spine showing the rib grafts and steel wires in situ.

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Histopathological examination of the specimen revealed sheets of uniform plasma cells having large nuclei and occasional cells which were multinucleated. Prominent nucleoli were seen in most cells. Russell bodies, hyaline inclusions, and vacuoles were seen occasionally [Figure 9].

Figure 9: Photomicrogram (×400) showing the uniform arrangement of plasma cells in sheets.

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Discussion

Both MM and SP are malignant primary tumors of the spine. MM is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure.^[4] SP includes two categories of tumors: SOP and solitary extraosseous plasmacytoma, where the plasma cell clone generally arises from lymphoid tissues away from the bone marrow microenvironment that normally hosts these cells. SP is more common in males (70%), and the median age at diagnosis varies from 55 to 60 years.^[5]

Diagnostic criteria for solitary osseous plasmacytomas ^[6],[7]

Single area of bone destruction due to clonal plasma cells
Bone marrow plasma cell infiltration not exceeding 5% of all nucleated cells ^[6]
The absence of osteolytic bone lesions or other tissue involvement (no evidence of myeloma)
The absence of anemia, hypercalcemia, or renal impairment attributable to myeloma
Low, if present, concentrations of serum or urine monoclonal protein
Preserved levels of uninvolved immunoglobulins.^[7]

MRI findings of plasmacytoma are relatively nonspecific (low-signal intensity on T1-weighted images and high-signal intensities on T2-weighted or short tau inversion recovery images). The “minibrain” sign is the only pathognomonic finding.^[8]

As per the NCCN guidelines, the treatment of SOP is radiation to involved field at a dose of 40–50 Gy ± surgery.^[4] The indication of surgical treatment is not well established in skull base SOP. It is because SOP at the skull base is usually diagnosed postoperatively only on histopathological examination.

The rationale for surgical treatment in our case

The patient had already developed myelopathy
Cord compression was already present at C1-C2 levels
The anterior and posterior elements of axis were destroyed, making the spine unstable, with a chance of quadriplegia at any point of time (Spinal Instability Neoplastic Score = 12)
Tissue can be obtained for histological confirmation of diagnosis
The tissue bulk can be reduced so that adjuvant therapy could be more effective
There is a chance for reduction in spasticity with subsequent restoration of the neurological function
Since the bulk of the tumor was present posteriorly, we went ahead with a posterior decompression and fusion in the initial sitting and planned for anterior decompression and fixation in the next sitting.

Impediments in management

As we work in a tertiary care hospital located in a relatively rural area, the main impediment to treatment would be the financial constraints of the patient and relatives. We faced the following difficulties in managing our case in an ideal fashion:

The relatives were not affording costly instrumentation or even titanium wire for posterior fixation. Hence, a steel wire had to be used for the fixation, thereby making a follow-up MRI impossible
Second-stage procedure and anterior decompression could not be done
Radiotherapy and workup of MM were refused by the relatives
The immunohistochemical characterization of the tissue was not possible.

The general prognosis of SOP is comparatively better than plasmacytoma, with a 5-year survival rate of about 70% and median overall survival period of 7.5–12 years. Patients, especially those with plasmacytoma of the spine, will probably eventually develop MM in the future, with a median delay of 2–4 years.^[9]

Conclusion

SOP is a very rare malignancy occurring at the skull base with involvement of the atlas and axis, with only 53 cases reported to date. Most often, the diagnosis can be made on histopathological examination only as most of the intraoperative and imaging findings are deceptive. The patient requires workup for myeloma and has to be treated by adjuvant radiation if the lesion is found to be solitary.

Acknowledgment

Dr. Anil Kumar Peethambaran, Professor and Head, Department of Neurosurgery, Government Medical College, Thiruvananthapuram, Kerala, India, for the expert advice regarding the fusion with rib graft
Dr. Gita Sayami, Consultant Pathologist, NRL Chitwan, First floor, Hospital Road, Bharatpur -10, Chitwan, Nepal – for histopathological examination of the specimen and providing photomicrograph of the same.

The Department of Radiology, College of Medical Sciences - Teaching Hospital, Bharatpur, Chitwan, Nepal - for providing the magnetic resonance scan images.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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