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Langerhans cell histiocytosis
Kevin Bradford Hoover1, Daniel I Rosenthal, Henry Mankin
1Musculoskeletal Radiology, Massachusetts General Hospital, Yawkey 6E 55 Fruit Street, Boston, MA 02114, USA. kbhoover@partners.org
Insights
Langerhans cell histiocytosis (LCH) is a rare disease with three syndromes sharing similar histology. This review covers LCH history, diagnosis, and treatment, emphasizing imaging for management.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Langerhans cell histiocytosis (LCH) is a complex neoplastic proliferation of Langerhans cells.
- It presents as three distinct clinical syndromes: eosinophilic granuloma, Hand-Schûller-Christian's disease, and Letterer-Siwe's disease, all with indistinguishable histology.
- These syndromes vary in severity and organ involvement, from localized bone or lung lesions to multi-systemic disease.
Purpose of the Study:
- To provide a comprehensive review of Langerhans cell histiocytosis (LCH).
- To discuss the historical context, histological features, potential causes, and therapeutic strategies for LCH.
- To highlight the critical role of radiographic findings and imaging techniques in diagnosing and managing LCH.
Main Methods:
- Literature review of Langerhans cell histiocytosis (LCH).
- Synthesis of information on LCH history, histology, etiology, and treatment.
- Focus on radiographic manifestations and imaging modalities for LCH diagnosis and management.
Main Results:
- LCH encompasses three syndromes with unified histology but diverse clinical presentations.
- Radiographic findings and specific imaging modalities are crucial for accurate LCH diagnosis.
- Understanding the spectrum of LCH is essential for effective patient management.
Conclusions:
- Langerhans cell histiocytosis (LCH) requires a multidisciplinary approach integrating clinical, histological, and radiological data.
- Effective management of LCH relies on accurate diagnosis through advanced imaging techniques.
- Further research into LCH etiology and treatment optimization remains important.
Abstract:
Langerhans cell histiocytosis (LCH) is a complex disease entity comprised of three distinct clinical syndromes that demonstrate indistinguishable histology. These syndromes are: eosinophilic granuloma, which is predominantly osseous or pulmonary; Hand-Schûller-Christian's disease, which involves multiple organ systems and, most typically, the skull base; and Letterer-Siwe's disease, the most severe disease manifestation, which typically involves the abdominal viscera. This article reviews our current understanding of Langerhans cell histiocytosis by discussing the history, histology, etiology, and treatment of the disease. It focuses on the radiographic findings and imaging modalities that are the most useful in disease diagnosis and management.