Eosinophilic granuloma (EG), a benign osseous variant of Langerhans cell histiocytosis, is rarely observed in cervical spineand being in axis (C2) is even more infrequent. We present here an unusual case of EG of the axis vertebra with neurologicalimpairment due to cervical cord compression, where the patient underwent decompression, C1–C2 spinal stabilization and steroidtherapy. Cervical vertebrae involvement of EG with cord compression and neurological deficit is very unlikely, and this case reportcontributes to the clinical experience of this rare but highly prodigious disease. The benefit of surgical treatment with total removalof the tumor and spinal stabilization to prevent irreversible neurological deficit is emphasized

Rudrappa, Satish

Som, R S Chitra

English

Atharva Scientific Publications (E-Jounals)

Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 244
Case Report
A case report of eosinophilic granuloma in axis (C2) vertebra causing spinal 
cord compression
R S Chitra Som1, Satish Rudrappa2
From 1Department of Neurosurgery, 2Department of Spine Surgery, Manipal Hospital, Bengaluru, Karnataka, India
Correspondence to: Dr. R S Chitra Som, Department of Neurosurgery, Manipal Hospital, No. 98, Hal Airport Road, Bengaluru - 560 017, 
Karnataka, India. E-mail: chitramarine@yahoo.com
Received - 27 June 2017 Initial Review - 20 July 2017  Published Online - 08 October 2017
Eosinophilic granuloma (EG) is a solitary, nonneoplastic proliferation of histiocytes localized in bone or lung. It comes under the spectrum of the autoimmune disorder, 
Langerhans’s cell histiocytosis (LCH) [1]. Despite EG’s high 
percentage of bone involvement, the incidence of vertebral 
involvement ranges from only 7.8% to 25%. EG involving 
the vertebral bodies show a predilection for the thoracic spine 
followed by the lumbar spine [2]. The location at the level of the 
cervical spine is very rare, and even that correlated to symptoms 
of spinal cord compression is still rarer [3]. The diverse treatments 
of EG of cervical spine include immobilization, chemotherapy, 
radiation therapy, steroid injection, and surgery depending on 
the disease symptoms [3-5]. However, the adequate disease 
management modality is still a subject of controversy. We report 
the case of axis vertebra EG with neurologic deficit from an 
11-year-old boy, issuing a displacement of the spinal cervical 
cord and compression, whose diagnosis was established after the 
radiological investigation and also by the lesion histopathology. 
We underline the rarity of the case, the excellent response to the 
surgical treatment, and the disease’s good prognosis after 2 years 
of follow-up.
CASE REPORT
An 11-year-old male was presented to us with a history of neck 
pain and stiffness of a month’s duration. The patient had restricted 
range of neck movements with torticollis toward the right side. 
Neurologic examination showed limb weakness of Grade 2. His 
laboratory data were unremarkable. Radiography of the cervical 
spine showed focal osteolysis of the axis (C2) vertebra (Fig. 1). 
Magnetic resonance imaging (MRI) (T1 contrast) of the cervical 
spine showed C1–C2 enhancement and destruction. Lateral 
mass with associated soft tissue was evident. MRI (T2 contrast) 
revealed cord compression and edema (Fig. 2). Computed 
tomography (CT) scans of the craniovertebral junction (CVJ) 
revealed destruction of the dens, body, bilateral pedicles, and the 
left lamina of axis vertebra with significant left lateral subluxation 
(Fig. 3).
To avert spinal deformity and permanent neurologic deficits, 
surgical correction through posterior approach was performed. In 
prone position, the skin and soft tissues were incised in the midline 
at the region of CVJ. It was found that the posterior elements of 
C2 were destroyed significantly including the pars. A biopsy was 
taken from the destroyed site. C1–C2 posterior spinal fusion was 
carried out with the cervical polyaxial screws and interfacetal 
C1–C2 arthrodesis with the iliac autograft. The surgical wound 
was closed over a closed draining tube in anatomical layers. 
Post-operative course was uneventful with resolution of pain and 
neurological symptoms. Post-surgery, treatment with oral steroid 
was commenced, and the patient continued on the steroid drug 
prednisolone, for 6 months. The follow-up investigations showed 
resolution of the C2 body lesion, no vertebral body collapse, 
and C1–C2 fusion. The bone scan showed no uptake at C1–C2 
levels and in other sites (Fig. 4). At 2 years, the patient remains 
asymptomatic with no signs of recurrence.
Histopathologically, the section appeared as a soft tissue with 
osseous fragments composed of proliferated histiocytes mixed 
with infiltrates of eosinophils. The histiocytes had vesicular, 
ABSTRACT
Eosinophilic granuloma (EG), a benign osseous variant of Langerhans cell histiocytosis, is rarely observed in cervical spine 
and being in axis (C2) is even more infrequent. We present here an unusual case of EG of the axis vertebra with neurological 
impairment due to cervical cord compression, where the patient underwent decompression, C1–C2 spinal stabilization and steroid 
therapy. Cervical vertebrae involvement of EG with cord compression and neurological deficit is very unlikely, and this case report 
contributes to the clinical experience of this rare but highly prodigious disease. The benefit of surgical treatment with total removal 
of the tumor and spinal stabilization to prevent irreversible neurological deficit is emphasized.
Key words: Cervical eosinophilic granuloma, Spinal cord compression, Spinal stabilization
Som and Rudrappa Eosinophilic granuloma of cervical spine
Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 245
grooved nuclei, and moderate to abundant cytoplasm. The 
immunohistochemistry study stained positive for S100. The 
findings were suggestive of LCH. Lack of nuclear atypia and 
atypical mitoses confirmed the pathology as EG and not the other 
variants of LCH.
DISCUSSION
EG, the benign form of LCH is characterized by unifocal 
involvement of bone or lung. The pathogenesis of EG remains 
inconclusive and it has been proposed that basic immune or 
genetic defects elicit proliferation of Langerhans cells. EG is 
identified by the presence of Langerhans cells in a characteristic 
milieu which includes histiocytes, eosinophiles, and neutrophiles. 
EG is differentiated from the other variants of LCH by 
the lack of nuclear atypia and atypical mitosis as observed 
histopathologically [6]. The cervical lesion is often represented 
as a lytic lesion involving the vertebral body with preserved disc 
spaces; however, rarely, it causes erosion or destruction of pedicles 
and posterior elements [7]. Despite the frequent reporting of 
vertebral involvement of EG, reports on spinal cord involvement 
and compression are scanty. Our patient endured neurologic 
deficit due to the spinal cord compression correlated with the 
osteolytic lesion at the axis vertebral level. Such neurological 
complications are reported to arise due to vertebral collapse or 
due to soft tissue involvement [8].
Plain radiography remains the mainstay of diagnosis in 
patients with EG. CT and MRI help to evaluate the vertebral lesion 
extension and soft tissue involvement, respectively, and assist with 
planning a biopsy or a surgical excision [9]. Radionuclide bone 
imaging is a complementary technique in the initial diagnosis of 
Figure 1: Osteolysis of the axis vertebra
Figure 2: Cord compression and edema
Figure 3: Destruction of vertebral body with significant lateral 
subluxation
Figure 4: Post-surgery bone scan
Som and Rudrappa Eosinophilic granuloma of cervical spine
Vol 3 | Issue 4 | Oct - Dec 2017 Indian J Case Reports 246
bone lesions including EG. However, recurrences are identified 
more readily with fewer false-negative findings with radionuclide 
bone scans. The radionuclide bone scans’ ability to exhibit 
lesional activity in addition to lesion localization is crucial for the 
follow-up of EG [9]. The mechanism of bone scan is based on the 
function of osteoblastic activity and changes in bone metabolism 
such as growing or healing lesions [9]. This diagnostic modality 
proved to be helpful in our patient in the follow-up, evaluation of 
response and also helped in the decision of cessation of treatment 
with prednisolone.
The disease management of vertebral EG has been extremely 
variable and controversial. Even now, there is no gold-standard 
treatment accepted for this condition. Considering the self-
limiting nature of the lesion, which is inclined to a spontaneous 
resolution, different therapeutic approaches have been proposed. 
The goals of treatment are the preservation of spinal stability 
and neurological function. Cures have been reported even with 
conservative treatments like cervical immobilization in patients 
with no neurological deficits [10]. However, when the integrity 
of the spinal cord or of the roots is threatened, it is necessary to 
undertake more aggressive treatment as in our case. Our patient 
had progressive neurological deficit caused by cord compression 
which necessitated decompression. The patient endured the 
surgery without complications and gained complete clinical 
benefit early after surgery.
Steroids are recommended in patients of EG, considering the 
high bone regenerative potential in younger patients [4]. Post-
surgery, our patient was given treatment with the oral steroid, 
prednisolone and he continued on the medication for 6 months, 
thereafter. Some authors suggest radiotherapy as a treatment 
choice [3], but there are also reports that warn about the risk 
of malignant transformation of the lesions after radiotherapy, 
especially in the pediatric age group [2].
CONCLUSION
The results of our case suggest that although there is no 
standard treatment regimen for axis EG, decompression, and 
surgical stabilization of the cervical spine are both essential in 
treating patients with neurological impairment involving cord 
compression. Surgical correction followed by treatment with oral 
steroids drastically reduces the chances of disease recurrence. 
Radionuclide bone scan proves to be a reliable diagnostic tool 
for the treatment response follow-up assessment in patients with 
EG.
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Funding: None; Conflict of Interest: None Stated.
How to cite this article: Som RSC, Rudrappa S. A case report of eosinophilic 
granuloma in axis (C2) vertebra causing spinal cord compression. Indian J 
Case Reports. 2017;3(4):244-246.


A case report of eosinophilic granuloma in axis (C2) vertebra causing spinal cord compression

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A case report of eosinophilic granuloma in axis (C2) vertebra causing spinal cord compression

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