An Aggressive Form of Langerhan Cell Histiocytosis in an Adult:Therapeutic Challenges

Langerhans cell histiocytosis (LCH) is rare in adults. Regular follow-up is mandatory due to reoccurrence. A 35-year-old malewith an incidental left iliac bone lesion was diagnosed with LCH. He later became symptomatic with hip pain and spread of thedisease. Despite excision of the symptomatic iliac lesion, he had progression while on cytarabine and nivolumab, evidenced byincreased bone pain and involvement of other bones on imaging. He underwent excision of the jaw lesion followed by vinblastine;he was pain free and had stable disease on PETimaging after 3 months. LCH is an uncommon neoplasia. Treatment is reserved forsymptomatic patients while asymptomatic patients are observed. Follow-up is imperative due to the risk of reoccurrence. Despitesurgical treatment together with one of the front-line agents for refractory disease, in this case cytarabine, he still had progressionof the disease. Furthermore, the trial of nivolumab was of no bene:t. )is case highlights good response to vinblastine which ispreviously reported to have good success. No trials are published, and the optimal strategy has yet to be de:ned. LCH withmultiple bony involvement can be aggressive and therapeutically challenging.