Adult onset Langerhans cell histiocytosis of the orbit - A case report

purpose To report an interesting case of adult onset Langerhans cell histiocytosis in a 41-year-old male with clinicopathological correlation. design Interventional case report. materials and methods A 41-year-old male presented with an ill-defined mass occupying the temporal quadrant of the left or...

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Bibliographic Details
Published in:Orbit (Amsterdam) 2004-06, Vol.23 (2), p.99-103
Main Authors: Subramanian, Nirmala, Krishnakumar, Subramanian, Babu, Kalpana, Mohan, Ravindra, DO, K. S. Lakshmi, Biswas, Jyotirmay
Format: Article
Language:English
Subjects:
Adult
Antigens, CD - metabolism
Antigens, Differentiation, Myelomonocytic - metabolism
Combined Modality Therapy
Diagnosis, Differential
Glucocorticoids - therapeutic use
Histiocytosis, Langerhans-Cell - diagnostic imaging
Histiocytosis, Langerhans-Cell - metabolism
Histiocytosis, Langerhans-Cell - pathology
Histiocytosis, Langerhans-Cell - therapy
Humans
Immunohistochemistry
Langerhans Cell Histiocytosis
Male
Orbital Diseases - diagnostic imaging
Orbital Diseases - metabolism
Orbital Diseases - pathology
Orbital Diseases - therapy
Orbital Tumors
Radiotherapy
Radiotherapy, Adjuvant
S100 Proteins - metabolism
Steroid Therapy
Tomography, X-Ray Computed
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Summary:purpose To report an interesting case of adult onset Langerhans cell histiocytosis in a 41-year-old male with clinicopathological correlation. design Interventional case report. materials and methods A 41-year-old male presented with an ill-defined mass occupying the temporal quadrant of the left orbit. Computerized tomography revealed an ill-defined extraconal mass involving the superolateral aspect of the left orbit with areas of osteolysis involving the lateral and superior orbital walls. The mass excised at orbitotomy showed microscopic features consistent with Langerhans cell histiocytosis. Immunohistochemistry with CD-68 macrophage marker and S-100 was positive, confirming the diagnosis. Treatment included oral steroids and radiotherapy. At 6 months follow-up, he developed a punched-out lytic lesion in the left parietal calvarium. He again received external beam radiotherapy. At 14 months follow-up, he is doing well with no recurrences. conclusions Adult onset Langerhans cell histiocytosis, though rare, should be included in the differential diagnosis of lytic lesions of the lateral orbital wall in an adult patient.
ISSN:0167-6830
1744-5108
DOI:10.1080/01676830490501550