gms | German Medical Science

Objective: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of dendritic cells. Literature is limited, especially on LCH in adults. As the disease is more common in children, guidelines were established in pediatric oncology. In contrast, there is no comprehensive analysis of differences between adults and children and diagnostic and therapeutic approaches in adults are derived from the pediatric literature. Aim of this study was to analyze the role of neurosurgery in LCH, with focus on pediatric versus adult patients.

Methods: Patients with histologically proven LCH treated between 2000 and 2017 were identified and relevant demographic, medical and radiological variables retrieved. Patients were stratified according to age at diagnosis (≤17 years versus >17 years) and descriptive statistics were performed.

Results: Twenty patients (10 female, 10 male) were stratified into pediatric (N = 12, mean age 7 years, range 1-17 years) or adult (N = 8, mean age 25 years, range 18-40 years). The presenting symptoms in children versus adults were localized pain in 50% versus 42% and palpable swelling in 25% versus 33%, respectively. Imaging revealed a skull lesion in 75% and 88%, with dural invasion in 33% and 38%. Two children presented with spinal lesions and one adult with a suprasellar lesion. Multi-organ involvement was found in two children and one adult. Whole body magnetic resonance imaging was performed in 92% of children, but only in 38% of adults. Neurosurgical intervention was resection in 75% of children and 88% of adults and biopsy in the remaining patients, followed by chemotherapy in 50% of children and 25% of adults. Within a mean follow-up of 6 years (range 1-17 years), complete remission was achieved in 92% and 88% of patients, respectively. No disease related mortality occurred.

Conclusion: Cranial and spinal LCH mainly affects bone and rarely presents with dural or intradural involvement. Multi-organ disease occurs at comparable rates, with 17% of children and 13% of adults being affected. Of note, whole body imaging was performed in almost all children, but only in one-third of adults. This might reflect stringent adherence to guidelines in pediatric oncology, or better clinical guidance on pediatric LCH. Despite a lower proportion of chemotherapy in adults, outcome in both cohorts was favourable. Our study highlights potential areas for further research, which would be best performed in a multi-center approach.