Langerhans' cell histiocytosis: Pathology, imaging and treatment of skeletal involvement

E. Michel Azouz, Gaurav Saigal, Maria Rodriguez, Antonello Podda

Research output: Contribution to journalArticle

220 Citations (Scopus)

Abstract

Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a "button sequestrum." Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.

Original languageEnglish
Pages (from-to)103-115
Number of pages13
JournalPediatric Radiology
Volume35
Issue number2
DOIs
StatePublished - Feb 1 2005

Fingerprint

Langerhans Cell Histiocytosis
Pathology
Bone and Bones
Skull
Radionuclide Imaging
Therapeutics
Spine
Bone Marrow
Eosinophilic Granuloma
Histiocytes
Temazepam
Langerhans Cells
Temporal Bone
Bone Diseases
Ribs
Quality Improvement
Pelvis
Mandible
Catalytic Domain
Age Groups

Keywords

  • Eosinophilic granuloma
  • Histiocytosis
  • Imaging
  • Langerhans' cell
  • Pathology
  • Pediatric

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Pediatrics, Perinatology, and Child Health
  • Radiological and Ultrasound Technology

Cite this

Langerhans' cell histiocytosis : Pathology, imaging and treatment of skeletal involvement. / Azouz, E. Michel; Saigal, Gaurav; Rodriguez, Maria; Podda, Antonello.

In: Pediatric Radiology, Vol. 35, No. 2, 01.02.2005, p. 103-115.

Research output: Contribution to journalArticle

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