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Langerhans Cell Histiocytosis Presenting As A Pre-Tibial Mass In A Child

Robert S. Young1,Jeffrey E. Martus2,Melissa A. Hilmes1,J. Herman Kan1

1Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, CCC-1121 MCN, Nashville, TN 37232.
2Department of Orthopaedics and Rehabilitation, Vanderbilt Children’s Hospital, 2200 Children’s Way, 4202 DOT, Nashville, TN 37232-9565.

Address for Correspondence:
Robert S. Young
Department of Radiology and Radiological Sciences
Vanderbilt University Medical Center
CCC-1121 MCN, Nashville, TN 37232.

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We present a 4 month old male who presented with a pre-tibial soft tissue mass with cortical destruction found in the juxtapyseal metaphysis found to represent Langerhans cell histiocytosis (LCH).  Eccentric, cortically based LCH may be under-appreciated unless they occur in regions with little overlying soft tissue, such as the calvarium and anterior calf. Pre-tibial LCH should be included in the differential diagnosis of a pre-tibial mass, and cross-sectional imaging is critical in distinguishing LCH from the more common entities such as pre-tibial subcutaneous granuloma annulare.

J.Orthopaedics 2010;7(1)e2


Langerhans Cell Histiocytosis, Magnetic resonance imaging, tibia, Pediatric imaging, Radiography


The most common pathologically proven pre-tibial mass in a child is subcutaneous granuloma annulare. We present an unusual case of pre-tibial Langerhans cell histiocytosis (LCH). Eccentric LCH may be under-appreciated unless they occur in areas with little overlying soft tissue such as the calvarium and anterior calf, and cross-sectional imaging is critical in distinguishing LCH from subcutaneous granuloma annulare and other tumor and tumor-like conditions in children affecting the anterior tibia. 

Case Report:

A 4-month-old male presented to his pediatrician after the parents noticed a pre-tibial mass over the left proximal tibia.  The mass was nonmobile and without erythema, warmth, or tenderness.  Initial workup included radiographs, which were normal.  This was followed by an ultrasound, which demonstrated a hypoechoic, soft tissue mass with cortical destruction (figure 1).  CT imaging demonstrated a 5mm eccentrically located lucent lesion in the proximal tibial metaphysis with cortical destruction and a soft tissue mass (figure 2).  MR imaging demonstrated an intraosseous lesion with a large anterior exophytic soft tissue component within the juxtaphyseal metaphysis with undercutting of the physeal equivalent region of the tibial tuberosity.  The lesion was isointense on T1W images and hyerintense on T2W images with subtle enhancement as well as anterior periosteal rim enhancement (figures 3). Open biopsy with frozen section analysis, and subsequent curettage of the lesion, showed histiocytes with grooved, folded, indented nuclei and thin nuclear membranes without atypia in a background of scattered small lymphocytes, macrophages, eosinophils, and a few giant cells (figure 4).

Figure 1: Grey Scale with Color Doppler Longitudinal Ultrasound image demonstrates a hypoechoic pre-tibial soft tissue lesion (white arrowheads) with central color flow (T – tibial shaft, E – tibial epiphysis).

Figure 2: Axial CT image through the proximal tibia demonstrates anterior lytic lesion with minor intramedullary extension (white arrow) with significant pretibial soft tissue component (white arrowheads).

Figure 3:  Axial fat saturated T1 post-contrast images demonstrate subtle central enhancement and anterior rim enhancement of the pre-tibial mass.

Figure 4:  Hematoxylin and eosin (H & E) stain of the cortical lesion shows multiple abnormal histiocytes (white arrows) with grooved, folded, indented nuclei and thin nuclear membranes without atypia in a background of eosinophils (black arrows).

Immunohistochemical staining demonstrated multiple S-100 positive cells, which are characteristic of Langerhans cells, and therefore LCH was the diagnosis.   CT imaging of the chest and abdomen as well as bone scintigraphy demonstrated no other foci of disease.  Curettage was considered therapeutic, and the patient remains asymptomatic without evidence of new lesions or recurrence.  The patient has been disease free for approximately 3 months at the time this report was written.

Discussion :

Langerhans’ cell histiocytosis (LCH) which was formally known as Histiocytosis X, is a rare disorder which may manifest with either local or systemic effects, most commonly affecting male (2:1) children younger than 15.1  Histology consists of unusual monocyte-like cells called Langerhans cells, which have vesicular, grooved nuclei.  These cells are usually seen in a background of eosinophils and this combination is unusual in pathologic specimens other than LCH.

Up to 80% of the LCH lesions that occur in children are isolated to bone.1,2   Local pain is the primary symptom and sometimes a palpable tender mass may be present. The osseous manifestations of LCH present more than 50% of the time as solitary, lytic lesions that are moderately destructive and involve the flat bones of the skull, mandible, ribs, and pelvis and most commonly affect children 1-3 years of age.2,4 Less commonly the long bones of the appendicular skeleton are involved, with lesions most commonly occurring in the diaphysis (58%), followed by the metyphysis.5  Physeal and epiphyseal involvement is rare. The lesions of the appendicular skeleton most commonly present as a centric lesion with endosteal scalloping.5 Eccentric, cortically based lesions may be a more common finding than has been reported in the literature.  When LCH involves cortex where there is little overlying soft tissue, such as the calvarium, or the anterior tibia, as seen in our case, they are probably detected at an earlier stage because they can be readily palpated compared with osseous lesions surrounded by more substantive soft tissue, such as the mid-diaphysis of the femur.

The top differential clinical consideration of a pre-tibial mass in a child was subcutaneous granuloma annulare, but this was excluded as a consideration given the presence of cortical destruction of the anterior tibia. The alternative differential diagnostic considerations for this patient’s pre-tibial mass given the imaging findings of anterior cortical destruction included subacute osteomyelitis with mass-like granulation tissue, an unusual manifestation of a solid osteofibrous dysplasia or adamantinoma with anterior cortical breech, Ewing’s sarcoma, leukemia/lymphoma, and cortically based solid aneurysmal bone cyst. Cross sectional imaging work-up in this patient was helpful in defining the lesion epicenter and the extent of cortical and intramedullary destruction, planning the biopsy and operative treatment, and excluding diagnostic considerations such as cortical aneurysmal bone cyst or an unusual manifestation of infection.

Treatment of patients with isolated LCH of the appendicular skeleton typically consists of curettage of the affected site with possible use of allograft bone if there is concern for pathologic fracture at the site of curettage.  Asymptomatic lesions may be managed conservatively as the lesions commonly regress and heal with time.  Systemic involvement may require treatment with chemotherapy and/or radiation.2,4  Our patient underwent curettage of the lesion and is currently asymptomatic with no other lesions identified.

In summary, cortically based LCH presenting with a palpable soft tissue mass is an unusual manifestation in long bones, although a frequent finding affecting the calvarium. Cortically based LCH may in fact be more common than what has been reported in the literature and may represent an early manifestation of osseous LCH. Cortically based LCH lesions of the long bones are under-appreciated unless they occur in areas with little overlying subcutaneous tissue, such as the anterior calf.  In the proper clinical context and patient age, LCH should be a diagnostic consideration in children presenting with a pre-tibial mass, which ultimately requires biopsy to differentiate from other neoplastic and tumor-like cortically based lesions in children.

Reference :

  1. Azouz MA, Saigal G, Rodriquez MM, Podda A. Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatric Radiology 2005; 35:103-115.

  2. Hoover KB, Rosenthal DI, Mankin H.  Langerhans cell histiocystosis. Skeletal Radiol 2007; 36:95-104.

  3. David R, Oria RA, Kumar R, et al.  Radiologic Features of Eosinophilic Granuloma of Bone. AJR Am J Roentgenol 1989; 153:1021-1026. 

  4. Meyer JS, Harty MP, Mahboubi S, et al.  Langerhans cell histiocytosis: presentation and evolution of radiologic findings with clinical correlation. RadioGraphics 1995; 15:1135-1146.

  5. Levine SM, Lambiase RE, Petchprapa CN.  Cortical lesions of the tibia: characteristic appearances at conventional radiography. RadioGraphics 2003; 23:157-177.


This is a peer reviewed paper 

Please cite as: Robert S. Young: Langerhans Cell Histiocytosis Presenting As A Pre-Tibial Mass In A Child

J.Orthopaedics 2010;7(1)e2





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