Bobby Wirawan Hassan

Bong Ju Moon

Jung-Kil Lee

Ki-Young Choi

Sang-Deok Kim

Young-Jin Kim

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Langerhans cell histiocytosis in the adult lumbar spine: case report

Hassan et al. SpringerPlus  (2016) 5:1398 
DOI 10.1186/s40064-016-3006-7
CASE STUDY
Langerhans cell histiocytosis in the adult 
lumbar spine: case report
Bobby Wirawan Hassan, Bong Ju Moon, Young‑Jin Kim, Sang‑Deok Kim, Ki‑Young Choi and Jung‑Kil Lee*
Abstract 
Introduction: Langerhans cell histiocytosis (LCH) occurs rarely in the spine of adults. The radiological findings usu‑
ally resemble vertebral tumors. Etiology of LCH has not been clearly established yet. Therapeutic approaches are still 
controversial. We describe a case of LCH in an adult spine.
Case description: A patient who presented with low back pain had an osteolytic lesion in the L1 vertebral body 
without neurological deficits, and fluoroscopy‑guided needle biopsy of the L1 vertebral body was performed. The 
immunohistochemical diagnosis confirmed LCH. The patient was successfully treated with conservative methods.
Discussion: The choice of appropriate therapy is very important, with treatment options varying from watch‑and to 
aggressive treatment.
Conclusion: LCH is considered as a pediatric disease that is extremely rare in the spine of adults and should be 
include in the differential diagnosis of osteolytic vertebral lesions. Conservative treatment is best choice for a patient 
with LCH without neurological deficit or spinal instability.
Keywords: Langerhans cell histiocytosis, Adult spine, Osteolytic lesion, Conservative method
© 2016 The Author(s). This article is distributed under the terms of the Creative Commons Attribution 4.0 International License 
(http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, 
provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, 
and indicate if changes were made.
Background
Langerhans cell histiocytosis (LCH) is a clonal prolifera-
tive disease characterized by infiltration of single or mul-
tiple organs by specific dendritic cells that resemble the 
normal epidermal Langerhans cell. LCH is considered 
a pediatric disease and is extremely rare in the spine of 
adults. Spinal involvement occurs in 6.5–25 % of all skel-
etal lesions. In the spine, LCH mainly involves the ver-
tebral bodies, with a predilection for the thoracic spine 
(54 %) followed by the lumbar (35 %) and cervical spine 
(11  %) (Abu-Bonsrah et  al. 2016). Only a small number 
of cases of LCH in the adult lumbar spine have been 
reported in the English literature (Huang et  al. 2010; 
Demirci 2004; Bavbek et al. 2004; Bilge et al. 1995). This 
article describes the case of 35-year-old man diagnosed 
with LCH presenting as an osteolytic lesion of the L1 ver-
tebral body.
Case report
A 35-year-old man presented with a one-year history of 
low back pain, and limited movement at the waist with-
out any neurological deficit. He had no history of fever, 
night sweats, or weight loss, and was unable to recall any 
recent injury. The patient’s medical history was unre-
markable for trauma or other bone diseases. A physical 
examination showed localized tenderness and percussive 
pain over the L1 spinous process, and restricted move-
ment at the waist without any motor or sensory abnor-
malities. Laboratory tests including blood cell count, 
serum electrolytes, renal and liver function tests, eryth-
rocyte sedimentation rate (ESR), and C-reactive protein 
(CRP) did not reveal any abnormalities. Plain lumbar 
spine X-ray revealed a radiolucency at the L1 vertebral 
body (Fig.  1a). Computed tomography (CT) showed a 
defined osteolytic lesion involving the left side of the 
vertebral body and pedicle of L1 (Fig.  1b, c). Magnetic 
resonance imaging (MRI) revealed low-signal intensity 
changes on T1-weighted images and high-signal inten-
sity changes on T2-weighted images of the L1 vertebral 
body (Fig.  2). On the basis of the radiological features, 
Open Access
*Correspondence:  jkl@chonnam.ac.kr; leejk0261@hanmail.net 
Department of Neurosurgery, Chonnam National University Hospital 
and Medical School, 42, Jebong‑ro, Donggu, Gwangju 501‑757,  
Republic of Korea
Page 2 of 5Hassan et al. SpringerPlus  (2016) 5:1398 
Fig. 1 a Plain lumbar X‑ray lateral view showing radiolucency of the L1 vertebral body. Sagittal (b) and axial (c) computed tomography showing an 
osteolytic lesion of the L1 vertebral body
Fig. 2 Sagittal magnetic resonance images showing low signal intensity changes of the L1 vertebral body on T1‑weighted images (a) and high 
signal intensity changes on T2‑weighted images (b). Axial magnetic resonance images showing low signal intensity changes of the L1 vertebral 
body and pedicle on T1‑weighted images (c) and high signal intensity changes on T2‑weighted images (d)
Page 3 of 5Hassan et al. SpringerPlus  (2016) 5:1398 
there was a high possibility of a neoplastic lesion. A fluor-
oscopy-guided needle biopsy of the L1 vertebral body 
was performed. Histopathology showed proliferation 
of Langerhans cells arranged in sheets, with abundant 
eosinophilic cytoplasm and indentations on the nuclear 
membrane. Immunohistochemistry confirmed LCH with 
diffuse immunoreactivity of S-100 and CD1a (Fig. 3). No 
other lesion was found by bone scan. The patient was 
treated conservatively with nonsteroidal anti-inflamma-
tory drugs and allowed to walk. 3  months after biopsy, 
bone remodeling of a destructive lesion of the L1 verte-
bral body was detected by CT. 2 years after biopsy, he was 
free from low back pain. Notably, follow-up CT revealed 
significant bone remodeling one year after biopsy, but the 
height of the vertebral body remained stable without fur-
ther collapse, and lumbar kyphosis did not occur (Fig. 4). 
No evidence of recurrence or neurological deficit has 
been detected.
Discussion
Langerhans cell histiocytosis (LCH) is a rare, clonal pro-
liferative disease characterized by infiltration of single or 
multiple organs by specific dendritic cells that resemble 
the normal epidermal Langerhans cell. LCH comprises a 
spectrum of disorders. Localized disease is described as 
eosinophilic granuloma, while multisystem forms of LCH 
have eponymous descriptions such as Hand-Schüller-
Christian disease, Letterer-Siwe disease, and systemic 
histiocytosis X. The present case was a solitary osseous 
lesion without systemic involvement. Although LCH 
most commonly occurs in the first two decades of life, 
it may affect patients of any age, from infants to elderly 
individuals. Among children, it has an annual incidence 
of 3-5 cases per million. In adults, the disease is rare, 
with an estimated prevalence of 1–2 cases per million/
year (Azouz et al. 2005). Bone is the most common organ 
affected by LCH. The most frequent sites of the osseous 
lesions of LCH are the skull, femur, mandible, pelvis, and 
spine. LCH in the spine is reported to mainly involve the 
vertebral bodies, occurring in 6.5–25 % of cases, with the 
most frequent site being the thoracic vertebrae (54 %) in 
children, and the cervical vertebrae in adults. In the pre-
sent case, LCH occurred in the lumbar vertebral body 
in an adult spine. Well-defined osteolytic lesions can be 
seen on radiological studies, although osteoblastic lesions 
can rarely develop (Beltran et  al. 1993; Kilborn et  al. 
2003). In children, most of the vertebral body volume 
is infiltrated by LCH, causing complete collapse, with a 
characteristic “vertebra plana” appearance. In adults, a 
relatively small volume of the vertebral body is affected, 
Fig. 3 a High‑power photomicrography showing Langerhans histiocytes and mixed numerous eosinophils. b Neoplastic cells showing folded and 
convoluted nuclei with intra‑nuclear groove resembling coffee beans (arrow). Immunohistochemical staining for S‑100 protein (c) and CD‑1a (d) 
showing immunoreactivity of Langerhans cells
Page 4 of 5Hassan et al. SpringerPlus  (2016) 5:1398 
and LCH of the body is present without collapse. CT can 
show some characteristic signs such as osteolytic lesions 
with a sclerotic margin. MRI can show a hypointense or 
heterogeneous signal intensity lesion on T1-weighted 
images and a hyperintense lesion on T2-weighted images. 
In the case of our patient, plain X-ray, CT, and MRI were 
performed, and an osteolytic lesion of the L1 vertebral 
body was identified. Although characteristic clinical and 
radiological features suggest the diagnosis, these features 
cannot provide a definitive diagnosis. Histological confir-
mation is always required in adults (Huang et  al. 2010). 
Malignant tumor, osteomyelitis, aneurysmal bone cyst, 
Ewing’s sarcoma, osteoblastoma, Gaucher’s disease, and 
acute leukemia must be differentiated (Reddy et al. 2000). 
The diagnosis of LCH is confirmed by immunohisto-
chemical staining for CD-1a and S-100 protein (Azouz 
et al. 2005). Spinal LCH is self-limiting and the progno-
sis is usually good. A variety of treatment modalities for 
spinal LCH have been reported, including conservative 
management, intralesional steroid injection, radiation 
therapy, chemotherapy, and curettage with or without 
reconstructive surgery (Puigdevall et al. 2008; Garg et al. 
2003; Bertram et al. 2002). Mild, isolated involvement of 
the spine without neurological deficits or spinal insta-
bility can be managed with conservative methods such 
as simple observation, nonsteroidal anti-inflammatory 
drugs, immobilization, or casting with or without initial 
bed rest. Although radiation therapy has the potential 
for secondary malignancy and vertebral growth-plate 
damage in the skeletally immature patient, low-dose 
radiation therapy appears safe and effective for adult 
patients with progressive spinal lesions or neurological 
impairment. Chemotherapy, especially modest dose of 
ARA-C (100  mg/m2  daily for 5  days, repeated monthly 
for 6) is suggested for disseminated LCH with multiple 
bone lesions or multisystem disease (Cantu et al. 2012). 
Recent studies have been reported that administration of 
pegylated interferon-alpha can be helpful for treatment of 
LCH by inducing anti-tumoral response (Furudate et al. 
2014). A large fraction of LCH cases appear to be asso-
ciated with BRAF mutations and those could potentially 
benefit from treatment with a BRAF inhibitor (Badalian-
Very et al. 2010). Surgery should be reserved for selected 
cases with severe mechanical instability, deformity, or 
neurological deficit due to compressionBecause spinal 
LCH is self-limiting, most authors have recommended 
conservative treatment for disease without neurological 
or mechanical complications, as in our case.
Conclusion
LCH is considered a pediatric disease that is extremely 
rare in the spine of adults. However, it should be included 
in the differential diagnosis of osteolytic vertebral lesions 
in adults. Conservative treatment is a good choice for a 
patient with LCH without neurological deficits or spinal 
instability.
Authors’ contributions
BWH: Operated the patient. BJM: Wrote the first summary of the work in the 
original language and reviewed all the others. YJK, SDK and KYC: Revised the 
manuscript. JKL: Reviewed the chart and published literature. All authors read 
and approved the final manuscript.
Acknowledgements
The present work was approved by the Ethical review committee of Chonnam 
National University Hospital and Medical School. This study was supported 
Fig. 4 Sagittal (a) and axial (b) computed tomography a year after biopsy showing bone remodeling of the previous osteolytic lesion of the L1 
vertebral body
Page 5 of 5Hassan et al. SpringerPlus  (2016) 5:1398 
by a grant (CRI 13902‑21) from the Chonnam National University Hospital 
Biomedical Research Institute.
Competing interests
The authors declare that they have no competing interests.
Patient consent
The patient and guardian have consented to the submission of the case 
report. (IRB of Chonnam National University Hospital).
Received: 22 June 2016   Accepted: 5 August 2016
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Langerhans cell histiocytosis in the adult lumbar spine: case report

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