Histiocytic tumour

Summary

	Langerhan cell Histiocytosis	Erdheim Chester disease (house)	Rosai-Dorfman disease	Juvenile xanthogranuloma	Histiocytic sarcoma
Aetiology	Abnormal immune response	Nil	Nil	Nil	Radiation
Localization	• Craniofacial and skull base • Hypothalamic pituitary region • Meninges • Choroid plexus • Basal ganglia • Dentate nucleus	• Brain (preferentially the cerebellum and brain stem), • Spinal cord	• Dural>Parenchymal • Cerebral convexity, • Cranial base, • Cavernous sinuses • Neck	• Brain (53%), • Intradural extramedullary spine (13%) • Nerve roots (15%)	• Brain parenchyma • Meninges • Cavernous sinus
5 yr survival	88%	Nil	Nil	Nil	Nil
Genetic profile	BRAF V600E MAP2K1	BRAF V600E mutations 50% NRAS 17% PIK3CA 12%	Nil	Nil	Lack IGH or T cell receptor gene clonality
Macroscopic	Yellow/white Dural base Parenchymal infiltrates	Infiltrative parenchymal lesions (44%), dural thickening / a meningioma-like mass (37%) A combination {19%)	Firm Lobulated Yellow-greyish white dural mass	Fragmented soft yellow to tan-pink	Destructive Soft Fleshy white Yellow necrotic foci
Clinical features	• Neurogenic diabetes insipidus • Psychomotor deterioration and ataxia • Obstruction hydrocephalus	• Cerebellar signs (41%) • Pyramidal syndromes (45%) • Diabetes insipidus (47%)	• H/a • N/V • Dizzy • Hypopit and DI	• Seizure • DI • Visual disturbances	• Variable • Progressive
Average Age	2yrs	53 yrs	21 yrs	2 yrs	52 yrs
Infiltrate composition Special cell/s(x)	• Langerhans cells, • Macrophages, • Lymphocytes, • Plasma cells, • Variable eosinophils Touton giant cell	Lipid-laden histiocytes with small nuclei Touton-type giant cells, scant lymphocytes, rare eosinophils, and variable fibrosis or gliosis.	• Large pale histiocytes, • (n) lymphocytes • (n) plasma cells, • Variable fibrosis. • Emperipolesis	• Composed of rounded to spindled, variably vacuolated histiocytes, • Scattered Touton and foreign body-type giant cells, • Lymphocytes, • Eosinophils	Highly cellular, mitotically active histiocytes with abundant eosinophilic cytoplasm
Imaging	Lack of T1 hyper intensity in post pit but there is enhancement of infundibulum	Resemble meningioma Delayed gadolinium enhancement	Resemble meningioma	Nil	Nil
Prognosis	Good for Unifocal disease	Good esp having BRAF V600E	Nil	Nil	Nil
Immunophenotype +	CD1a Langerin CD207 S100	CD68	• CD11c • CD68 • L1 antigen (clone MAC387) • S100 protein	• CD11c • CD68 • Factor XII	• Histiocytic markers (e.g. CD68, CD163, lysozyme, CD11c, and CD14)

No result found