2025 Poster Presentations
P174: CHARACTERIZATION OF LANGERHANS CELL HISTIOCYTOSIS: ANALYSIS OF A CASE SERIES.
Grace Toro, MD1; Camilo Montaña, MD2; Homero Sariego, Otolaryngologist3; Matías Gómez, Otolaryngologist3; 1Hospital del Salvador; 2Hospital Roberto del Río; 3Instituto de neurocirugía Dr. Alfonso Asenjo
Background: Histiocytic disorders of childhood represent a wide spectrum of alterations that have as a common histological characteristic the existence of activated or transformed “histiocytes.” Langerhans cell histiocytosis (LCH) is the most common, with an incidence of approximately 5 per million. LCH represents a diagnostic challenge given the nonspecific signs; it can also mimic other more common childhood diseases, which is why it is important to consider it as a differential diagnosis. Bone lesions are the most common type of LCH, occurring in approximately 75% of patients. LCH can affect any bone, but the most common sites are the skull, long bones, ribs, pelvis, and vertebrae.
Otological involvement occurs in 15% to 61% of patients, manifesting as otitis, cerebellar syndrome, facial paralysis and/or hearing loss.
Treatment of LCH depends on the extent of the disease. Systemic treatment including corticosteroids and cytostatic drugs for at least one year has improved the prognosis of multisystem LCH and represents the current standard of care.
Objective: To describe 5 cases of LCH in a pediatric patient, with different clinical presentation, evolution and treatment.
Results: During the study period, 6 cases were reported. With a 50/50 distribution between women and men.
The average age was 8 years and 8 months, with the youngest patient being 3 years and 10 months and the oldest being 17 years at the time of diagnosis.
The reason for consultation was diverse: ataxia, facial paralysis, headache, earache, retroauricular pain and paralysis of the abducens nerve.
The most frequent location was the mastoid 33.3%, followed by sphenoid 16,67%, petroclival 16,67% apex petrosum 16.67% and cranial base 16.67%.
Only one of the patients presented lytic lesion in a secondary location, which was observed by means of radiography in the distal end of the humerus.
An incisional biopsy was performed in all patients, 33.33% by mastoidectomy, 50% by transsphenoidal approach and 16.67% retrolabyrinthine.
Only in one of the approaches was intratumoral administration of corticosteroids performed concomitant to the biopsy.
By means of deferred biopsy a diagnosis of Langerhans cell histiocytosis was made in 100% of the patients.
Therapeutic management indicated 50% chemotherapy, 33.3% indomethacin and 16.67% intratumoral administration of corticosteroids in a second surgical stage.
Conclusion: LCH is a low frequency pathology, capable of simulating multiple pathologies. There are no previous descriptions of its incidence in the Chilean literature, so this is an approach to the reality of the country. Further studies including other health centers throughout the country are required to estimate the real incidence of this pathology in the Chilean population.
Keywords: Langerhans cell histiocytosis, children