Jae-Seung Hur; Hong-Sik Kim; Yong-Wook Park; Ju-Yeon Pyo; Young-Ho Lee; Ye-Soo Park

doi:10.5292/jkbjts.2013.19.2.78

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Hur, Kim, Park, Pyo, Lee, and Park: Single System Langerhans’ Cell Histiocytosis with Multifocal Bone Lesions and Pathologic Fracture: A Case Report

Case Report

J Korean Bone Joint Tumor Soc 2013;19(2):78-82.

Published online: 10 January 2013

DOI: https://doi.org/10.5292/jkbjts.2013.19.2.78

Single System Langerhans’ Cell Histiocytosis with Multifocal Bone Lesions and Pathologic Fracture: A Case Report

Jae-Seung Hur, Hong-Sik Kim, Yong-Wook Park^*, Ju-Yeon Pyo^*, Young-Ho Lee^†, Ye-Soo Park

Department of Orthopaedic Surgery, Guri, Korea

^*Department of Pathology, Guri Hospital, Hanyang University College of Medicine, Guri, Korea

^†Department of Pediatrics, Hanyang University College of Medicine, Seoul, Korea

Correspondence to: Ye-Soo Park Department of Orthopaedic Surgery, Guri Hospital, Hanyang University College of Medicine, 249-1, Kyomoon-dong, Guri 471-701, Korea TEL: +82-31-560-2317 FAX: +82-31-557-8781 E-mail: hyparkys@hanyang.ac.kr

Received 21 October 2013 Revised 27 November 2013 Accepted 29 November 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Langerhans cell histiocytosis is known as one of the diseases related to excessive proliferation of normal monocytes and has the variety of clinical courses and treatment. Especially, in cases with the spine, it shows a feature of single or multiple osteolysis. According to the location, disease progression and concomitant symptom, variety of treatments (observation, radiotherapy, chemotherapy, surgery, etc.) have been attempted, however, appropriate treatment has not been established yet. The authors introduce the case of single system Langerhans cell histiocytosis which involves cervical and lumbar vertebrae simultaneously with bone marrow destruction and pathologic fracture.

Keywords: Langerhans' cell histiocytosis, pathologic fracture

References

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Figure 1.

Initial plain radiographs show asymmetric collapse in anteroposterior view (A) and about 30% of collapse on L3 vertebral body in lateral view (B) of lumbar spine.

Figure 2.

In preoperative computerized tomography scan, axial view shows osteolytic lesion in vertebral body and right pedicle (A). Sagittal view shows pathologic fracture with osteolytic lesion in L3 vertebral body (B).

Figure 3.

(A) Contrast-enhanced T1WI MRI shows enhancing lesion in L3 vertebral body and paravertebral soft-tissue. (B) Fat suppression T1WI MRI shows high signal intensity in right superior articular process of C6 vertebra.

Figure 4.

Staging study confirms that there was no evidence of other suspicious lesion except spinal lesion. PET-CT shows high uptake of ¹8fluorodeoxyglucose (FdG) in right superior articular process of C6 vertebra (A) and in right pedicle and body of L3 vertebra (B).

Figure 5.

H&E stain (×100) shows nodular aggregation of histiocytes and variable inflammatory cells (A). Histiocytes (Langerhans cell) with oval nuclei as diagnostic hall marker are found in H & E stain (×500) (B). Immunohistochemically, the Langerhans’ cells are positive for S-100 protein (C) and CD1a (D).

Figure 6.

12 months after treatment, plain radiographs show L3 corpectomy, and placement of an expandable anterior cage and L2-L4 posterior fusion using pedicle screws.

Figure 7.

The lesions that were observed previously (A) disappeared in follow up PET-CT (B).

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