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 Table of Contents  
CASE REPORTS
Year : 2021  |  Volume : 4  |  Issue : 2  |  Page : 218-222

Eosinophilic granuloma of the thoracolumbar junction in the adult spine: A case report and review of literature


1 Head, Department of Spine Surgery, Kothari Medical Centre, Kolkata, West Bengal, India
2 Department of Orthopaedics, D.Y. Patil Hospital & Research Centre, Navi Mumbai, Maharashtra, India
3 Consultant Spine Surgeon, Shalby Hospital, Mohali, Chandigarh, India
4 Consultant Spine Surgeon, Shalby Hospital, Ahmedabad, Gujarat, India

Date of Submission09-Mar-2020
Date of Decision07-Jul-2020
Date of Acceptance11-Sep-2020
Date of Web Publication24-May-2021

Correspondence Address:
Rohan Bharat Gala
D.Y. Patil Hospital & Research Centre, Sector 5, Nerul, Navi Mumbai 400706, Maharashtra.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/isj.isj_17_20

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  Abstract 

Langerhans cell histiocytosis (LCH) is a common disease in the pediatric population with a peak incidence between 5 and 10 years of age. Eosinophilic granuloma (EG) accounts for less than 1% of all primary bone tumors primarily affecting the skull, pelvis, ribs and metaphyses of long bones and is a benign localized form of LCH. It is extremely rare in adults, more so in the spine, with a predilection for the thoracic spine than the lumbar and cervical region. A 35-year-old gentleman presented with transitional mid back pain for two months radiating to the right flank which worsened at night. There was no history of fever or trauma. Physical examination revealed right dorsolumbar tenderness without any neurological deficit. Serological parameters were normal and C-reactive protein (CRP) was negative. X-ray showed radiolucency in the right D12 pedicle with CT scan showing a lytic lesion in D12 body extending into the right pedicle. Magnetic resonance imaging (MRI) showed a hypointense lesion on T1 and hyperintense lesion on T2. Transpedicular biopsy was done and histopathology revealed Langerhans cell with abundant basophilic cytoplasm and a centrally placed nucleus with a groove. On further confirmation with IHC, a diagnosis of LCH was made. He then received radiotherapy for 12 days. PET CT done one year after diagnosis revealed near-total metabolic response of the tumor after comparing the PET CT done prior to diagnosis. The patient has reported complete relief since 2 years with recent radiological evidence of no recurrence. A precise diagnosis of LCH largely relies on biopsy and histopathology and once definitive diagnosis is established, EG can be managed by chemotherapy and/or radiotherapy in patients with no neurological deficit. Despite its rarity, EG should be considered as an important differential for solitary osteolytic lesions at the thoracolumbar junction in the adult spine.

Keywords: Adults, eosinophilic granuloma, radiotherapy, spine


How to cite this article:
Basu S, Gala RB, Banta A, Solanki AM. Eosinophilic granuloma of the thoracolumbar junction in the adult spine: A case report and review of literature. Indian Spine J 2021;4:218-22

How to cite this URL:
Basu S, Gala RB, Banta A, Solanki AM. Eosinophilic granuloma of the thoracolumbar junction in the adult spine: A case report and review of literature. Indian Spine J [serial online] 2021 [cited 2021 Aug 4];4:218-22. Available from: https://www.isjonline.com/text.asp?2021/4/2/218/316660




  Introduction Top


Eosinophilic granuloma (EG) accounts for less than 1% of all primary bone tumors primarily affecting the skull, pelvis, ribs and metaphysis of long bones.[1],[2],[3] It is a benign and localized form of Langerhans cell histiocytosis (LCH) and is usually present as an osteolytic lesion originating from the reticuloendiothelial system.[4] The disease predominantly affects children and adolescents with nearly 80% presenting before the age of 10.[5] Involvement of the spine is rare with less than 10% of cases reported with a predilection for the thoracic spine.[6] The lesions are usually solitary, and the vertebral bodies are the main structures affected presenting as collapse in children. It is considered as a pediatric disease and is extremely rare in adults. This article describes the case of a 35-year-old man diagnosed with LCH presenting as an osteolytic lesion of the D12 vertebral body.


  Case Report Top


A 35-year-old gentleman presented with localized mid back pain, radiating to the right flank since 2 months. His symptoms were aggravated at night and on changing posture. The patient denied history of any constitutional symptoms, recent trauma or any symptom suggestive of metabolic bone disease. He had tenderness at the dorsolumbar junction more so on the right side. There was no neurological deficit. On investigation, his serological parameters like total blood counts were within normal limits and CRP was negative. X-ray of the dorsolumbar spine showed radiolucency in the right D12 pedicle with widening on anteroposterior view [Figure 1]. Computed Tomography (CT) scan showed a well defined expansile osteolytic lesion extending from the right pedicle into the right posterolateral corner of the D12 vertebra [Figure 2]. The lesion was hypointense on T1 and hyperintense on T2 MRI images with preservation of disc spaces and without neural compression [Figure 3]. He underwent a percutaneous transpedicular biopsy and the histopathology reports revealed langerhans cells with abundant basophilic cytoplasm with a centrally placed nucleus with a groove. On further confirmation with IHC studies the sample tested positive for CD68, CD1a and S100 and hence a diagnosis of LCH was made [Figure 4]. After a detailed discussion with the oncologist, he was further managed with radiotherapy. He received radiotherapy for 12 days with a daily dose of 24 Gy. His symptoms significantly subsided after radiotherapy. PET CT done one year after diagnosis revealed near-total metabolic response of the tumor after comparing the PET CT done prior to diagnosis. The patient has reported complete relief since two years with no evidence of recurrence on CT and MRI scans [Figure 5].
Figure 1: Anteroposterior and lateral views of the thoracolumbar spine showing widening of the right D12 pedicle and radiolucency, respectively

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Figure 2: CT scan of the thoracolumbar spine showing lytic lesion of the right pedicle of the D12 vertebra extending into the posterolateral corner of the body

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Figure 3: T2 axial MRI scan at the level of the pedicle of the D12 vertebral body showing hyperintense lesion in the right pedicle extending into the body

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Figure 4: (A) Langerhans cells with eosinophils seen on histopathology. (B) Positive immunohistochemistry stain for CD1a. (C) Positive immunohistochemistry stain for CD 68. (D) Positive immunohistochemistry stain for S 100

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Figure 5: (A) T2-weighted axial MRI scan at the level of the pedicle of the D12 vertebral body showing resolution of the lesion in the right pedicle. (B) Axial CT scan at the level of the pedicle of the D12 vertebral body showing resolution of the lesion in the right pedicle. (C) Coronal CT scan showing resolution of the lesion at the right posterolateral corner of the D12 vertebral body

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  Discussion Top


LCH is a clonal proliferative disease characterized by the infiltration of single or multiple organs by specific dendritic cells that resemble the normal epidermal Langerhans cell. It is a spectrum of disorders and localized disease is described as EG. It is rare in adults with a prevalence of 1–2 cases per million/ year and bone is the most common organ affected by LCH.[7],[8] Spinal involvement accounts for 6.5% to 25% of all skeletal cases of LCH. It results in “vertebra plana” in children due to a collapse of the affected vertebral body. In adults however, it affects a small volume of the vertebral body causing minimal instability and rarely any neurological deterioration. The most common location of the tumor in adults is in the cervical spine with very few cases reported at the thoracolumbar junction. In the largest case series comprising 30 cases of adult EG by Huang et al.,[8] 70% of the tumors were located in the cervical spine with only one case located at the thoracolumbar junction. They also concluded that adults rarely present with vertebra plana, instead, they have an asymmetric collapse of the vertebra with instability. Pain is the most common symptom in these patients with the duration being at least a few weeks to 3 months prior to diagnosis. Neurological deficits have been reported in the literature due to rapidly progressive lesions causing collapse and subsequent impingement on the neural elements.[9] Bilge et al.[10] reported a case of adult granuloma in the lumbar vertebra leading to persistent unilateral radiculopathy eventually managed with surgical resection whereas Bavbek et al.[11] reported a case of combined cervical and lumbar EG with the latter undergoing spontaneous resolution over time concluding that the decision concerning surgery must be based on neurological findings when multiple bone sites are involved.

These lesions are mostly osteolytic with sclerosis on radiographs which are difficult to pick at times and require further CT scan and MRI to document bone destruction. CT scans show osteolysis, irregular margins with or without a sclerotic margin. The lesion can erode both the vertebral body and posterior elements such as transverse process, pedicle, or even paravertebral soft tissue. The MRI images of the lesions are characterized with iso or low- intense signals on T1-weighted images; high-intense signals on T2-weighted images, with a high or moderate-signal change in the adjacent intervertebral discs. In our patient radiology was similar to the one described above. In adults, neither the clinical manifestations are characteristic nor the radiological findings pathognomonic of EG. The differential diagnosis includes vertebral osteomyelitis, aneurysmal bone cysts, Gaucher’s disease, metastatic tumor disease, and Ewing’s sarcoma.[12]

Hence, the diagnosis of this entity relies mainly on histopathology (HP) and immunohistochemistry (IHC) reports.[8] Histopathology shows proliferation of Langerhans cells arranged in sheets, with abundant eosinophilic cytoplasm and indentations on the nuclear membrane. Immunohistochemistry confirms LCH with diffuse immunoreactivity of S-100 and CD1a.[13]

Treatment is dependent upon the location and extent of disease, presence or absence of instability, and preservation/restoration of neurological function. Various treatment options include observation and immobilization, indomethacin administration, methylprednisolone injections, radiofrequency ablation, local excision and curettage with or without bone grafting, chemotherapy, and irradiation. The results of these modalities have a reported recurrence rate of less than 20%.[5],[14],[15],[16],[17] In patients with moderate pain and mild neurological deficits from solitary EGs of the spine, immobilization, and low-dose radiation therapy is advocated to be effective in the healing of lytic lesions and limiting disease progression.[18] Other modalities like surgery are reserved for cases that have neurological deterioration or increased collapse amounting to instability or those who have failed to respond to conservative treatment. Our patient was therefore managed with radiotherapy and responded to the same. Not only did he have subsidence of symptoms and regression of the metabolic response, but he also shows no evidence of recurrence at 3-year follow-up.

To conclude LCH is considered a pediatric disease that is extremely rare in the spine of adults. A precise diagnosis of LCH largely relies on biopsy and histopathology and once definitive diagnosis is established, LCH can be managed by chemotherapy and/or radiotherapy in patients with no or minimal neurological deficit. Surgical intervention can effectively treat a solitary lesion and maintain spinal alignment, which is an option for existing instability or deformity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mickelson MR, Bonfiglio M. Eosinophilic granuloma and its variations. Orthop Clin North Am 1977;8:933-45.  Back to cited text no. 1
    
2.
Schajowicz F, Slullitel J. Eosinophilic granuloma of bone and its relationship to Hand-Schüller-Christian and Letterer-Siwe syndromes. J Bone Joint Surg Br 1973;55:545-65.  Back to cited text no. 2
    
3.
Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet 1987;1:208-9.  Back to cited text no. 3
    
4.
Seimon LP. Eosinophil granuloma of the spine. J Pediatr Orthop 1981;1:371-6.  Back to cited text no. 4
    
5.
Brown CW, Jarvis JG, Letts M, Carpenter B. Treatment and outcome of vertebral langerhans cell histiocytosis at the children’s hospital of eastern Ontario. Can J Surg 2005;48:230-6.  Back to cited text no. 5
    
6.
Bertram C, Madert J, Eggers C. Eosinophilic granuloma of the cervical spine. Spine (Phila Pa 1976) 2002;27:1408-13.  Back to cited text no. 6
    
7.
Greenlee JD, Fenoy AJ, Donovan KA, Menezes AH. Eosinophilic granuloma in the pediatric spine. Pediatr Neurosurg 2007;43: 285-92.  Back to cited text no. 7
    
8.
Huang W, Yang X, Cao D, Xiao J, Yang M, Feng D, et al. Eosinophilic granuloma of spine in adults: A report of 30 cases and outcome. Acta Neurochir (Wien) 2010;152:1129-37.  Back to cited text no. 8
    
9.
Davidson RI, Shillito J Jr. Eosinophilic granuloma of the cervical spine in children. Pediatrics 1970;45:746-52.  Back to cited text no. 9
    
10.
Bilge T, Barut S, Yaymaci Y, Alatli C. Solitary eosinophilic granuloma of the lumbar spine in an adult. Case report. Paraplegia 1995;33:485-7.  Back to cited text no. 10
    
11.
Bavbek M, Atalay B, Altinörs N, Caner H. Spontaneous resolution of lumbar vertebral eosinophilic granuloma. Acta Neurochir (Wien) 2004;146:165-7.  Back to cited text no. 11
    
12.
Reddy PK, Vannemreddy PS, Nanda A. Eosinophilic granuloma of spine in adults: A case report and review of literature. Spinal Cord 2000;38:766-8.  Back to cited text no. 12
    
13.
Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, et al; Euro Histio Network. Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013;60:175-84.  Back to cited text no. 13
    
14.
Sweasey TA, Dauser RC. Eosinophilic granuloma of the cervicothoracic junction. Case report. J Neurosurg 1989;71:942-4.  Back to cited text no. 14
    
15.
Scarpinati M, Artico M, Artizzu S. Spinal cord compression by eosinophilic granuloma of the cervical spine. Case report and review of the literature. Neurosurg Rev 1995;18:209-12.  Back to cited text no. 15
    
16.
Egeler RM, Thompson RC Jr, Voûte PA, Nesbit ME Jr. Intralesional infiltration of corticosteroids in localized Langerhans’ cell histiocytosis. J Pediatr Orthop 1992;12:811-4.  Back to cited text no. 16
    
17.
Sessa S, Sommelet D, Lascombes P, Prévot J. Treatment of langerhans-cell histiocytosis in children. Experience at the children’s hospital of nancy. J Bone Joint Surg Am 1994;76:1513-25.  Back to cited text no. 17
    
18.
Osenbach RK, Youngblood LA, Menezes AH. Atlanto-axial instability secondary to solitary eosinophilic granuloma of C2 in a 12-year-old girl. J Spinal Disord 1990;3:408-12.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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