J Korean Cleft Palate-Craniofac Assoc.  2009 Apr;10(1):37-39.

A Case Report of Langerhans Cell Histiocytosis of Frontal Area

• Yang HW
• Kang MG
• Chang CH

Abstract

PURPOSE: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includeseosinophilic granuloma, Letterer-Siwe diseases, and Hand- Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area.
METHODS
A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a 33 x 25 mm sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof.
RESULTS
Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment.
CONCLUSION
The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

Keyword

Langerhans cell histiocytosis; Eosinophilic granuloma; Frontal bone

MeSH Terms

Adolescent
Eosinophilic Granuloma
Eosinophils
Frontal Bone
Granuloma
Histiocytes
Histiocytosis
Histiocytosis, Langerhans-Cell
Humans
Male
Meninges
Orbit
Prognosis
S100 Proteins
Skull
Temporal Bone
S100 Proteins