Adult-Onset Langerhans Cell Histiocytosis With Calvarial and Hypothalamic Involvement: Two Cases.
Chiaki Shinohara, Takuya Yonemochi, Miyu Kikuchi et al.
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Case in brief
This report details two cases of adult-onset Langerhans cell histiocytosis (LCH). The first case involved a 36-year-old woman with a solitary osteolytic lesion in the calvarium, treated with surgical resection and bisphosphonates, resulting in stability. The second case featured a 26-year-old man with hypothalamic involvement, presenting with cognitive impairment and endocrine dysfunction. Despite partial tumor regression with chemotherapy, cognitive dysfunction persisted, underscoring the impact of CNS involvement on outcomes.
Diagnostic / clinical pearl
Clinicians should be aware that LCH can present with varying neurological symptoms based on the site of involvement, and CNS lesions may lead to significant long-term cognitive impairment despite treatment.
Why it matters
The contrasting outcomes in these cases highlight the importance of early diagnosis and comprehensive evaluation in LCH, particularly when the central nervous system is involved, as it can significantly affect treatment strategies and patient prognosis.