Langerhans cell histiocytosis of the orbit -: diagnosis, management and follow-up in a child and two adults

Background: Langerhans cell histiocytosis usually affects children. Systemic Langerhans cell histiocytosis may be present in 37% of affected children. Ten per cent of all children die of the disease. Case reports: Patient 1. A 9 year-old boy presented with painful upper lid swelling OD. Ultrasonography revealed a space-occupying lesion adjacent to a lytic defect: of the frontal bone as shown on coronal CT. MRI showed marked contrast enhancement of the mass which came close to the intracranial fossa. The vascularized tumor was excised using an anterior orbitotomy with upper lid crease incision. No evidence of local or systemic recurrence was present three years postoperatively. Patient 2. A 19-year-old male presented with foreign body sensation, double vision OS and numbness of the left face of six weeks' duration. The ocular findings revealed a sixth nerve palsy OS. Otherwise, the eyes were unremarkable. Sensitivity of cranial nerve V2 was decreased, CT revealed an osteolytic process in the lateral orbital wall and sphenoid wing. MRI demonstrated a space-occupying lesion involving the temporalis muscle laterally, the pterygopalatine fossa medially with intracranial extension into the temporal lobe. Surgical excision was performed using a transcranial approach. Signs and symtoms had completly resolved six months after surgery with no evidence of local or systemic recurrence. Patient 3. A 30-year-old male presented with right-sided headache, painful upper lid swelling and vertical diplopia. Coronal CT scan revealed a lytic defect in the frontal bone laterally. Curettage and complete excision were performed using an infrabrow incision. Follow-up examination four years later revealed an intact ocular motility with no evidence of local or systemic recurrence. In all three cases the tumor showed characteristic light-microscopy and immunohistochemical features of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis of the orbit is an important differential diagnosis of osteolytic and/or space-occupying lesions not only in children, but also in adults. A diagnostic biopsy followed by surgical excision and/or local and systemic corticoid- and chemotherapy are essential regarding the prognosis of local and systemic disease.