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Histiocytosis: a review focusing on neuroimaging findings
Larissa Barcessat Gabbay1, Cláudia da Costa Leite2, Ranieli Saraiva Andriola1
1Instituto de Radiologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
Insights
This study reviews neuroimaging in Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (NLCH). Recognizing characteristic patterns is key for accurate diagnosis of these systemic diseases affecting the central nervous system.
Area of Science:
- Neurology
- Radiology
- Pathology
Background:
- Histiocytosis encompasses systemic diseases often involving the central nervous system.
- Langerhans cell histiocytosis (LCH) is the most common form.
- Non-Langerhans cell histiocytosis (NLCH) includes diverse entities like hemophagocytic syndrome, Erdheim-Chester disease, and Rosai-Dorfman disease.
Purpose of the Study:
- To delineate the neuroimaging characteristics of LCH and NLCH.
- To aid in the differential diagnosis of histiocytic disorders affecting the CNS.
Main Methods:
- Comprehensive literature review.
- Analysis of pathologically confirmed illustrative cases.
Main Results:
- LCH neuroimaging commonly shows osseous lesions (craniofacial/skull base), intracranial extra-axial and intra-axial changes (white/gray matter), and atrophy.
- NLCH diagnosis relies on clinical and laboratory correlation, with presentations including intraparenchymal, meningeal, orbital, and paranasal sinus involvement.
Conclusions:
- Recognizing distinct neuroimaging patterns is crucial for including LCH and NLCH in the differential diagnosis.
- Accurate identification of histiocytosis subtypes on imaging facilitates timely and appropriate patient management.
Objective:
Histiocytosis is a systemic disease that usually affects the central nervous system. The aim of this study is to discuss the neuroimaging characteristics of Langerhans cell histiocytosis (LCH), the most common of these diseases; and the non-Langerhans cells histiocytosis (NLCH), which includes entities such as hemophagocytic syndrome, Erdheim-Chester and Rosai-Dorfman diseases.
Method:
Literature review and illustrative cases with pathologic confirmation.
Results:
In LCH, the most common findings are 1) osseous lesions in the craniofacial bones and/or skull base; 2) intracranial, extra-axial changes; 3) intra-axial parenchymal changes (white and gray matter); 4) atrophy. Among the NLCH, diagnosis usually requires correlation with clinical and laboratory criteria. The spectrum of presentation includes intraparenchymal involvement, meningeal lesions, orbits and paranasal sinus involvement.
Conclusion:
It is important the recognition of the most common imaging patterns, in order to include LCH and NLCH in the differential diagnosis, whenever pertinent.
