A 9 year old boy presenting with lytic lesion on midshaft tibia. On HPE, there are grooved nuclei, giant cells eosinophilic cytoplasm and fibroblastic proliferation. Xray is as shown below. What is the likely diagnosis?
High-Yield Explanation
The grooved nuclei, lytic lesion in first decade suggests Langerhan's cell histiocytosis. LANGERHANS CELL HISTIOCYTOSIS (LCH): DE!STRUCTION DUE TO HISTIOCYTES, IDIOPATHIC1. Letterer-Siwe disease: a fulminant systemic disease, age group < 3 years, fatal2. Hand-Schuller-Christian disease: Triad of skull lesions (lytic lesion), Exophthalmos and diabetes insipidus. 3. Eosinophilic Granuloma: Solitary lesion of bone or lung (Pulmonary histiocytosis X) 1st decade of life. Skull is the most common site in skeletal system - Bevelled edge lytic lesion is seen in skull (double contour).Biopsy: Gold standard (cells with Birbeck's granules (tennis racket appearance) under election microscopy) grooved nuclei are seen. TreatmentSpontaneous resolution. Highly radiosensitive and Excision + Curettage for resistant cases.