MRI
Axial T2
Axial T1
Axial T1
Axial FLAIR
Axial DWI
Axial ADC
Axial Gradient Echo
Axial MRA
Axial T1 C+ fat sat
Coronal T1 C+ fat sat
Sagittal T1 C+ fat sat
Axial T1 C+
Axial T1 C+
Axial T1 C+
What is the differential diagnosis based on the above imaging features?
The imaging differentials are: granulomatous: tuberculous/sarcoidosis; vasculitis- neuro-Behcet; neoplastic: lymphoma/gliomatosis cerebri; other: histiocytosis;
What are the organs that can be involved in Erdheim Chester disease?
It is a multisystem disease and can involve the bones, lung, heart, renal, skin and retroperitoneum.
What are the other disorders of non-Langerhans histiocytosis?
Juvenile xanthogranuloma, Rosai -Dorfman disease, haemophagocytic lymphohistiocytosis, histiocytic sarcoma.
What are the characteristic intracranial MR imaging features for the diagnosis of Erdheim Chester disease?
T2 hypointense mass lesions predominantly involving the hypothalamic-pituitary axis and meningeal involvement, both of which show post contrast enhancement.
T1 iso-hypointense, T2 hypointense, FLAIR hyperintense mass in the suprasellar cistern and in the region of the hypothalamus with bilateral symmetrical hyperintensities in the basal ganglia, thalamus, midbrain. DW/ADC images show no restricted diffusion. No features to suggest hemorrhage on gradient sequence. MRA shows intracranial periarterial abnormal signal intensities seen in the region of circle of Willis. CEMR MPR images show thick and nodular enhancement of the hypothalamic-pituitary axis along with pachymeningeal thickening and enhancement in the suprasellar and perimesencephalic cisterns with obstructive hydrocephalus of the lateral ventricles.