Multiple Langerhans cell histiocytosis with spinal involvement.

Area of Science:

• Oncology
• Pediatric Oncology
• Histiocytosis

Background:

• Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disorder.
• Spinal involvement in LCH, particularly multifocal disease, presents unique challenges in prognosis and management.
• Understanding long-term outcomes is essential for optimizing patient care.

Purpose of the Study:

• To determine the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) affecting the spine.
• To identify risk factors associated with progression-free survival (PFS) in this patient cohort.

Main Methods:

• Retrospective analysis of 28 patients with multifocal spinal LCH treated between 2009 and 2021.
• Kaplan-Meier methods for estimating overall survival (OS) and PFS.
• Univariate Cox regression analysis to identify prognostic variables for PFS.

Main Results:

• Spinal LCH represented 15.4% of all spinal LCH cases, predominantly affecting thoracic and lumbar regions.
• Pain and neurological dysfunction were the most common symptoms; osteolytic bone destruction and paravertebral masses were frequent findings.
• Median PFS was 50.5 months; stage (HR 4.324) and chemotherapy (HR 0.203) were significant prognostic factors for PFS.

Conclusions:

• Spinal LCH can lead to significant pain and neurological deficits due to bone destruction and instability.
• Chemotherapy significantly improves PFS, while radiotherapy aids in local control.
• Long-term follow-up and timely intervention are critical due to the potential for disease progression and multi-system involvement.