Histiocytic Disorder Mimicking a Brain Tumor: A Report of 2 Rare Cases

16 August 2022: Articles

Challenging differential diagnosis, Diagnostic / therapeutic accidents, Management of emergency care, Rare disease

Frances Xiuyan Feng ^B , Negin Farsi ^B , Min Dai ^B , Areli K. Cuevas-Ocampo ^A , Diana M. Veillon ^A , James D. Cotelingam ^A*

DOI: 10.12659/AJCR.935885

Am J Case Rep 2022; 23:e935885

Table 2. Comparison of CNS involvement of three most common histiocytic lesions.

	LCD	ECD	RDD
Epidemiology	Mostly occur in childhood and male predominant	Mean patient age at diagnosis is 55–60 year, with male predilection. Pediatric cases are reported as well	Most cases occur in adolescent and young adult with African American predilection
Most common location of CNS involvement	Hypothalamic-pituitary axis	Hypothalamic-pituitary axis and meningeal involvement, cranial lesion	Dural based lesions
Clinical features	Diabetes insipidus followed cranial involvement. Skull and mandibular involvement are common	Diabetic insipidus and severe neurodegenerative cerebellar disease	Mimic meningioma
Morphological features	Neoplastic histocytes containing kidney shaped nuclei with grooves and reactive eosinophils in the background. Birbeck granules are typical ultrastructural features	Neoplastic foamy histocytes and Touton cells. Reactive inflammatory and fibrosis may present	Lesional histocytes with emperipolesis and reactive abundant mature plasma cells. Dutcher bodies may be prominent
Immunophenotype	S100+, CD1a+, Langerin +; CD68+	CD68+, S100-; CD1a-, Langerin-	CD68+; S100+, CD1a-, Langerin-
Genetic profiles	~50% cases demonstrate mutation	~50% cases demonstrate	No evidence of clonality