Clinical spectrum and management outcomes of Langerhans cell histiocytosis of the orbit

Abstract

Purpose: To describe the clinical spectrum and management outcomes of Langerhans cell histiocytosis (LCH) involving the orbit. Methods: Retrospective review of patients with orbital LCH presenting at the Sankara Nethralaya, Chennai, India, over the past 15 years. Demographic details, presenting features, radiology, histopathology, immunohistochemistry, and management outcomes were analyzed. Results: Nine patients were reviewed. The mean age of presentation was 10.12 ± 14.31 years (range: 6 weeks to 35 years). Eyelid swelling was the most common presenting feature (4, 44.4%), followed by proptosis (3, 33.3%). The mean duration of the presentation was 2.21 ± 2.77 months. Radiological investigations revealed orbital roof osteolytic defects in six (66.6%) patients. Six patients underwent near-complete excision of the mass while three underwent incisional biopsy. Histopathology revealed histiocytes with nuclear grooving and numerous eosinophils characteristic of LCH. The cells were positive for CD1a and S 100 antigens. None of the patients had any systemic involvement. Three received systemic steroids and four received systemic chemotherapy. At a mean follow-up of 17.85 ± 23.46 months, all had complete remission without any signs of recurrence. One patient was lost to follow-up after near-complete excision while one adult patient with a mass in the intraconal space had no recurrence after near-complete excision. Conclusion: LCH is a rare disorder of the orbit that commonly occurs in children and should be considered a differential for osteolytic lesions involving the orbit. All patients should undergo a systemic evaluation to rule out multifocal disease. The treatment depends upon disease extent and risk factors.

Successful treatment of orbital Langerhans cell histiocytosis with stereotactic radiosurgery: A case report and literature review

“…Literature review in PubMed yielded 68 results using the terms “langerhans cell histiocytosis and orbit” of which there are 15 applicable results outlined below. There were no complications with treatment 1,9–22 …”

“…The presence of langerin (CD207+) or cluster of differentiation 1a (CD1a) on immunohistochemistry or Birbeck granules on electron microscopy are specific for a definitive diagnosis of LCH 1,6 . The presence of S‐100, adenosine deaminase, alpha‐mannosidase, and peanut lectin binding are also common markers and two or more of these markers meet criteria from the International Histiocyte Society for a diagnosis of LCH 1 . Langerhans cell histiocytosis preferentially affects the bone, skin, lungs, and pituitary gland and can have variable presentation 14 .…”

“…6,24 These orbital LCH lesions are not associated with increased risk of developing CNS lesions. 1 A diagnosis of LCH can be made through immunohistochemistry or electron microscopy. The presence of langerin (CD207+) or cluster of differentiation 1a (CD1a) on immunohistochemistry or Birbeck granules on electron microscopy are specific for a definitive diagnosis of LCH.…”

“…1,5,6 Langerhans cell histiocytosis is an umbrella term for a group of diseases previously called Histiocytosis X. 1,2 LCH can be categorized several different ways: historic reports use two main categories, single system or multisystem LCH. 7 The current literature categorizes using three main clinical syndromes; localized or unifocal LCH (eosinophilic granuloma), chronic recurring LCH (Hand-Shullar-Christian disease), and acute disseminated LCH (Letterer-Siwe disease) which presents as a multisystem disease.…”

“…1,3,4 Though it is more common in children, adult cases are well documented in the literature with evidence of an increased incidence in males over women across all populations. 1,5,6 Langerhans cell histiocytosis is an umbrella term for a group of diseases previously called Histiocytosis X. 1,2 LCH can be categorized several different ways: historic reports use two main categories, single system or multisystem LCH.…”