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Langerhans cell histiocytosis mimicking acute dacryocystitis
N Guàrdia-Ruiz1, I M López-Miñarro1, E Obregón-Martínez2
1Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, Spain.
Insights
Langerhans cell histiocytosis (LCH) in a child presented as lacrimal sac swelling, mimicking dacryocystitis. Diagnosis required careful evaluation due to its unusual presentation and specific molecular markers.
Area of Science:
- Oncology
- Pediatric Pathology
- Dermatopathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm involving clonal proliferation of Langerhans-type dendritic cells.
- LCH typically presents with diverse clinical manifestations, often requiring differential diagnosis.
Observation:
- An unusual pediatric case of LCH presented with significant swelling in the left lacrimal sac region.
- The clinical presentation mimicked acute dacryocystitis, a common infection of the tear sac.
Findings:
- Microscopic examination revealed intense inflammatory infiltrate and histiocytes with irregular nuclei.
- Immunohistochemistry confirmed tumor cells positive for S-100 protein, CD1a, and CD207 (langerin).
- Molecular analysis identified a BRAF V600E/E2/D mutation in exon 15.
Implications:
- This case highlights the importance of comprehensive evaluation, including imaging and histology, for pediatric orbital and periorbital masses.
- Recognizing LCH's potential to mimic benign conditions like dacryocystitis is crucial for timely and accurate diagnosis.
- The presence of the BRAF V600 mutation in this presentation may inform therapeutic strategies.
Abstract:
Langerhans cell histiocytosis (LCH) is a myeloid neoplasm characterized by clonal neoplastic proliferation of Langerhans-type dendritic cells associated with an inflammatory infiltrate predominantly composed of lymphocytes and eosinophils. In this article, we present an unusual case of LCH with significant swelling in the left lacrimal sac region in a 3-year-old child, clinically mimicking acute dacryocystitis. Microscopically, it showed intense inflammatory infiltrate and histiocytes with irregular nuclei. The tumor cells were positive for S-100 protein, CD1a, and CD207 (langerin). Molecular study was positive for the V600E/E2/D mutation (EXON 15). This case emphasizes the importance of careful clinical, radiographic, and microscopic evaluation, as some neoplasms may mimic common benign lesions.
