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Year : 2012  |  Volume : 8  |  Issue : 2  |  Page : 286-288

Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study

1 EKO CT and MRI Scan Centre at Medical College and Hospitals, Campus 88-College Street, Kolkata, India
2 Department of Radiology, Darbhanga Medical College and Hospitals, Darbhanga, India
3 Department of Radiology, Medical College and Hospitals, Kolkata, India
4 Vivekanand Institute of Medical Sciences, Kolkata, India

Date of Web Publication26-Jul-2012

Correspondence Address:
Mohammad A Hashmi
EKO CT and MRI Scan Centre at Medical College and Hospitals, Campus 88-College Street, Kolkata
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.98991

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 > Abstract 

Langerhans cell histiocytosis (LCH) is a relatively rare disease affecting the reticuloendothelial system in the pediatric age group. It can affect bones, lung, liver, spleen, lymph nodes and skin. MR imaging is particularly informative in diagnosis and management of bone LCH. In this report, we present the initial and 23 months post-treatment MR images of a femoral LCH lesion in a 12-year-old child to describe the role of MRI in bone LCH.

Keywords: Langerhans cell histiocytosis, magnetic resonance imaging, eosinophilic granuloma

How to cite this article:
Hashmi MA, Haque N, Chatterjee A, Guha S. Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study. J Can Res Ther 2012;8:286-8

How to cite this URL:
Hashmi MA, Haque N, Chatterjee A, Guha S. Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study. J Can Res Ther [serial online] 2012 [cited 2022 May 20];8:286-8. Available from: https://www.cancerjournal.net/text.asp?2012/8/2/286/98991

 > Introduction Top

Langerhans cell histiocytosis (LCH) of the bone is an uncommon disease of the pediatric age group. It is characterized by clonal proliferation of histiocytes. Although the X-ray appearance of this disease is extensively described in literature, it is MR imaging that is particularly helpful for not only characterizing the lesion but also delineation of the local and systemic extent of the disease and follow-up of the patient. In this report, we describe a case of Langerhans cell histiocytosis of the femur in a 12-year-old child with description of MR appearances at the initial diagnosis and after 23 months of follow-up after chemotherapy and review the existing literature on MR imaging in bone LCH.

 > Case Report Top

A 12-year old female patient presented with a swelling over the lower part of right thigh. X-ray of lower end of right femur showed a small cortical lytic lesion in lower diaphyses without any evidence of sclerosis [Figure 1] and [Figure 2]. It was associated with focal endosteal scalloping and medullary expansion. There was mild linear periosteal reaction. Fat planes especially in anterior and medial aspect were partly obscured. MRI of right thigh showed heterogeneous hyperintense signals involving lower femur on STIR and T2 weighted GRE images. Cortical thinning and periosteal reaction were detected. An associated large extraosseus soft tissue component was seen [Figure 3] and [Figure 4]. Focal breach of cortex was also noted. Marrow edema was present in almost the entire length of the femur. Lymphadenopathy was also noted in right inguinal region. The MRI appearance suggested a bony mass in lower end of right femur with extraosseus involvement. Biopsy taken from the bony lesion showed a lesion composed of sheets of histiocytes, a large number of eosinophils admixed with lymphocytes, plasma cells and neutrophils. There was presence of multinucleated giant cells. A histological diagnosis of eosinophilic granuloma was made and biopsy from the right inguinal lymph node corroborated with the diagnosis. Considering the gross soft tissue involvement, the patient was classified as group B Langerhans cell histiocytosis and was started on combination chemotherapy. Review MRI was done almost after 23 months, which showed considerable diminution of bony and soft tissue edema [Figure 5] and [Figure 6]. The lesion also appeared less heterogeneously hyperintense as compared to earlier images [Figure 7].
Figure 1: X-ray AP view showing lytic lesion in lower diaphysis without any evidence of sclerosis

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Figure 2: X-ray lateral view showing endosteal scalloping. Fat planes in anterior aspect are obliterated

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Figure 3: T2 weighted STIR images showing focal breach of cortex. Marrow edema is seen in almost entire length of the femur, with extraosseus soft tissue component

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Figure 4: T2 weighted GRE images in axial plane showing extraosseus soft tissue component

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Figure 5: Post treatment STIR images showing marked resolution of the lesion, soft tissues and bony edema

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Figure 6: T1 weighted images after treatment showing marked resolution of the lesion and periosteal reaction at the lesion site

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Figure 7: T2 weighted GRE images showing resolution of lesion and soft tissue edema

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 > Discussion Top

Langerhans cell histiocytosis (LCH) is primarily a disease of childhood. It is characterized by clonal proliferation of histiocytic cells with highly variable biologic behavior. It predominantly affects the reticuloendothelial system of the bone marrow, liver, spleen, lymph node and the lung. Its variable forms were regarded in the past as different entities, viz. eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease, having increasing severity respectively. But essentially these are the parts of a spectrum of the same disease. For the purpose of chemotherapy, LCH can be divided into three risk groups: those with multifocal bone disease (group A), those with soft-tissue involvement without any organ dysfunction (group B) and those with organ dysfunction (group C). [1]

More than 50% cases of eosinophilic granuloma involve the skull, spine, pelvis, ribs and mandible. Long bone involvement is typically diaphyseal and most commonly it involves the femur. [2] The X-ray appearance of bony lesions is that of a medullary lytic lesion with a sharp, scalloped endosteal margin. It is usually non-expansile but apparent expansion may be there due to linear periosteal reaction. Geographic pattern of bone destruction is found in calverial lesions. Vertebrae plana is another characteristic lesion in LCH, especially involving the thoracic region in children. Sclerosis may be present in the healing stage. [3]

MR is most suited for delineating marrow extent and soft-tissue involvement in LCH of the bone. [4] Some authors suggested that whole body MRI is more useful than conventional radiography and scintigraphy in not only locating more skeletal lesions, but in identifying extraskeletal lesions also. [5] MRI findings in LCH of the bone are non-specific. A focal lesion with extensive soft tissue and marrow edema is most commonly found as hypointese areas in T1W images and hyperintense area on T2W and STIR images. [6] The peritumoral edema is less extensive than that of Ewing's sarcoma and osteomyelitis, two most common differentials of LCH. An endosteal rim of low signal intensity can be found in LCH, which may be an early sign of healing. This feature along with limited peritumor edema is indicative of underlying LCH. [7] MRI findings can be correlated with the histologic stages of the disease. Lesion with high cellularity show high signal on T2w and contrast enhanced T1W images, where lesions with fibrosis show low signal on both image sequences. [8] In multilevel spinal lesions, MRI is useful in guiding biopsy by identifying the active from the inactive lesions. [9]

 > Conclusion Top

MRI is presently the most informative imaging tool in the management of bone LCH. Its ability to accurately delineate the marrow and soft tissue extent of a lesion and characterize the activity of a lesion makes it a particularly powerful modality in the staging, treatment and follow-up of bone LCH.

 > References Top

1.Gadner H, Heitger A, Grois N, Gatterer-Menz I, Ladisch S. Treatment strategy for disseminated langerhans cell histiocytosis. DAL HX-83 Study Group. Med Pediatr Oncol 1994;23:72-80.  Back to cited text no. 1
2.Stoker DJ, Saifuddin A. Myeloproliferative and similar disorders. In Grainger and Allison's Diagnostic Radiology. 4 th ed. vol. 3. p. 1901-23.  Back to cited text no. 2
3.Cobby M, Watt I. Disorders of the lymphoreticular system and other haematopoietic disorders: Textbook of Radiology and Imaging. 6 th ed. vol. 1. p. 223-6.   Back to cited text no. 3
4.Azouz EM, Saigal G, Rodriguez MM, Podda A. Langerhans' cell histiocytosis: Pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005;35:103-15.   Back to cited text no. 4
5.Goo H W, Yang DH, Ra YS, Song JS, Im HJ, Seo JJ, et al. Whole-body MRI of Langerhans cell histiocytosis: Comparison with radiography and bone scintigraphy. Pediatr Radiol 2006;36:1019-31.  Back to cited text no. 5
6.Beltran J, Aparisi F, Bonmati LM, Rosenberg ZS, Present D, Steiner GC. Eosinophilic granuloma: MRI manifestations. Skeletal Radiol 1993;22:157-61.  Back to cited text no. 6
7.Davies AM, Pikoulasb C, Griffitha J. MRI of eosinophilic granuloma. Eur J Radiol 1994;18:205-9.  Back to cited text no. 7
8.Moon TY, Lee J, Lee IS, Choi KU, Chae JM, Kim J, et al. MRI and Histopathologic Classification of Langerhans Cell Histiocytosis. Curr Med Imaging Rev 2009;5:14-8.  Back to cited text no. 8
9.Kaplan GR, Saifuddin A, Pringle JA, Noordeen MH, Mehta MH. Langerhans' cell histiocytosis of the spine: Use of MRI in guiding biopsy. Skeletal Radiol 1998;27:673-6.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]

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