Extraosseous Langerhans Cell Histiocytosis in Children

Abstract

Langerhans cell histiocytosis, a rare disease that occurs mainly in children, may produce a broad range of manifestations, from a single osseous lesion to multiple lesions involving more than one organ or system. The clinical course varies widely in relation to the patient's age. Multisystem disease may demonstrate especially aggressive behavior in very young children, with the outcome depending largely on the stage of disease and the degree of related organ dysfunction at the time of diagnosis. Extraosseous manifestations are less commonly seen than osseous ones and may be more difficult to identify. To accurately detect extraosseous Langerhans cell histiocytosis at an early stage, radiologists must recognize the significance of individual clinical and laboratory findings as well as the relevance of imaging features for the differential diagnosis. The pattern and severity of pulmonary, thymic, hepatobiliary, splenic, gastrointestinal, neurologic, mucocutaneous, soft-tissue (head and neck), and salivary involvement in Langerhans cell histiocytosis generally are well depicted with conventional radiography, ultrasonography, computed tomography, and magnetic resonance imaging. However, the imaging features are not pathognomonic, and a biopsy usually is necessary to establish a definitive diagnosis.

Central diabetes insipidus due to Langerhans’ cell histiocytosis in an adolecent nigerian girl

“…Axillary lymph node enlargement has been observed in 48.5% of cases (14). The involvement of the bone marrow, lymph node and the posterior pituitary gland is suggestive of a high-risk multisystem disease (15), which is associated with long-term complications (39). The presence of anaemia (haemoglobin concentration < 10g/dl) is indicative of the bone marrow, which was present in our patient (25).…”

“…The definitive diagnosis is usually based on a positive staining of the cells from the lesion with CD1a and/or Langerin (CD207). A protein that is expressed in Birbeck granules, cytoplasmic organelles typically found in Langerhans cells (15,32,33). However, in a recent review by Badalian-Very et al has stated that the demonstration of Birbeck granules by electron microscopy is no longer required for diagnosis due to the remarkable similarity of the histopathological features that are present regardless of the site of the lesion (15,32).…”

Cardiothoracic manifestations of primary histiocytoses

“…Advanced parenchymal destruction may subsequently lead to pulmonary hypertension (Table 1). 8 Mediastinal involvement is also rarely possible. The thymus is rarely involved in cases of LCH; however, its true incidence is likely underestimated.…”

“…MRI shows areas of high T 1 weighted signal intensity, which may represent fatty replacement due to fibroxanthomatous reaction. 8 Other mediastinal manifestations of systemic LCH include diffuse lymphadenopathy and posterior mediastinal infiltration (Figure 2). Effects of mediastinal involvement may include tracheal compression, pericardial effusion, superior vena cava thrombosis and erosion of the sternum and vertebral bodies.…”

Langerhans Cell Histiocytosis with Atypical Intervertebral Disc and Sacroiliac Joint Involvement Mimicking Osteoarticular Tuberculosis in an Adult

“…Bone and skin lesions are the most common manifestations of disease in children (2); in adults, the most commonly affected sites are bone and the lungs (3). A few articles have described unusual skeletal and soft tissue manifestations of LCH (4)(5)(6)(7)(8).…”