Saturday, September 4, 2010

Langerhans cell histiocytosis and related tumors

Langerhans cell histiocytosis
Proliferative disorder of monocytes
Variants include:
Hand-Schuller-Christian disease
-Skull lesions
-Exophthalmos
-Diabetes insipidus
-Mostly in patients older than 2

Letterer-Siwe syndrome
-Hepatosplenomegaly
-Anemia/hemorrhage
-Patients usually under 2

Histiocytosis X
-solitary eosinophilic granuloma
-bone lesions
-older children (over 2)
-may be observed if asymptomatic
-RT given for lesions recurrent after surgery or refractory to steroids/chemo
-10Gy gives good local control, over 80%

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