Central Nervous System and Head and Neck Histiocytoses: A Comprehensive Review on the Spectrum of Imaging Findings

Area of Science:

• Radiology
• Pathology
• Oncology

Background:

• Histiocytoses encompass rare disorders involving abnormal proliferation of macrophages and dendritic cells.
• These conditions present with diverse manifestations, particularly affecting the central nervous system (CNS) and head and neck regions.

Purpose of the Study:

• To review the imaging findings associated with CNS and head and neck manifestations of histiocytoses.
• To enhance understanding of the pathophysiology, clinical presentation, and imaging characteristics of common histiocytoses.
• To aid in differentiating these disorders based on typical presentations and imaging features.

Main Methods:

• A comprehensive literature review was conducted to identify sites of disease involvement.
• Demographics, histopathologic criteria, and imaging features were analyzed for various histiocytoses.
• Manifestations in specific anatomical locations (CNS, calvaria, skull base, orbits, etc.) were described.

Main Results:

• Histiocytoses demonstrate variable imaging appearances in the CNS and head and neck.
• Key histiocytoses reviewed include Rosai-Dorfman disease, Erdheim Chester disease, Langerhans cell histiocytosis, histiocytic sarcoma, and juvenile xanthogranuloma.
• Detailed imaging features for intra-axial, extra-axial intracranial, calvarial, skull base, hypothalamopituitary axis, orbital, sinonasal, spinal, and head and neck regions are presented.

Conclusions:

• Radiologists must be aware of the spectrum of imaging findings in histiocytoses.
• Recognizing these variable appearances is crucial to avoid misdiagnosis and ensure appropriate patient management.
• This review provides a resource for understanding the diverse imaging spectrum of histiocytoses in the CNS and head and neck.