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Year : 2001  |  Volume : 49  |  Issue : 4  |  Page : 267-8

Langerhans cell histiocytosis of the orbit.

Department of Ophthalmology, M S Ramaiah Medical Teaching Hospital, Bangalore, India

Correspondence Address:
S B Shetty
Department of Ophthalmology, M S Ramaiah Medical Teaching Hospital, Bangalore
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Source of Support: None, Conflict of Interest: None

PMID: 12930121

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Langerhans cell histiocytosis (LCH) accounts for less than 1% of all orbital tumours. Though rare, orbital involvement in LCH is not uncommon. Most reports so far have been in Western literature. We report here a case of LCH of orbit with intracranial extension. To the best of our knowledge, this is the first case of single system multifocal LCH reported from India.

Keywords: Child, Histiocytosis, Langerhans-Cell, pathology, radiography, Humans, Male, Orbital Diseases, pathology, radiography, Tomography, X-Ray Computed,

How to cite this article:
Shetty S B, Mehta C. Langerhans cell histiocytosis of the orbit. Indian J Ophthalmol 2001;49:267

How to cite this URL:
Shetty S B, Mehta C. Langerhans cell histiocytosis of the orbit. Indian J Ophthalmol [serial online] 2001 [cited 2021 Mar 3];49:267. Available from: https://www.ijo.in/text.asp?2001/49/4/267/14689

Langerhans cell histiocytosis (LCH) accounts for less than 1% of all orbital tumours. Though rare, orbital involvement in LCH is not uncommon. Most reports so far have been in Western literature. We report here a case of LCH of orbit with intracranial extension. To the best of our knowledge, this is the first case of single system multifocal LCH reported from India.

Eosinophilic granuloma (EG) was first described by Lichtenstein and Jaffer in 1940.[1] In 1953, Lichtenstein introduced the term Histiocystosis X for a group of diseases that included EG, Hand-Schuller Christian disease and Letterer-Siwe disease.[1] All of them share common histiogenesis and have similar morphological findings. In 1973 Nezelof et al reported that lesions of histiocytosis X were the result of inappropriate proliferation and infiltration of various tissues with abnormal histiocytotic cells that are morphologically and immunologically similar to cells of the Langerhans cell system.[2] The Histiocytosis Society suggested the term Langerhans Cell Histiocytosis to encompass all these dieseases.[3] We report here a case of LCH of orbit.

  Case report Top

A 12-year-old boy presented in August 1998 with painless progressive swelling of the right orbit of two months' duration. On examination, the swelling over the upper and lower lateral aspect of right orbit measured 3 x 5cms. It was smooth, lobulated, of variable consistency and partly adherent to the bone [Figure - 1]a. Another soft swelling measuring 2 x 2 cm with smooth surface was found over the right parietal region.

Ocular examinations including fundus, intraocular pressure (IOP) and ocular movements were normal. Vision was 6/6 in both eyes and fields were normal. CT scan showed a peripherally enhancing soft tissue mass in the superolateral aspect of the right orbit, extending posteriorly along the lateral wall with lytic destruction [Figure - 2]. The mass had intra and extra-orbital soft tissue components with infiltration of the lateral rectus, superior rectus and intraconal fat, extending up to the optic canal. It showed intracranial (extradural) extension into the right frontal lobe consequent to erosion of the greater wing of sphenoid. The right optic nerve was thickened near its entry into the optic canal. The choroid and retina were normal. Another focal expansile lytic lesion was seen in the right parietal bone adjacent to the vertex. Haematocrit, bleeding time (BT), clotting time (CT) peripheral blood picture, bone marrow aspiration, liver function tests and urine specific gravity were normal. Chest X-ray, skeletal survey and abdominal scan were also normal.

Unguided fine needle aspiration cytology (FNAC) smears from lateral wall of orbit showed large cells with abundant granular foamy cytoplasm and vesicular nucleus with distinctive longitudinal wavy grooves in the nuclear membrane (histiocytes). Many eosinophils and multinucleated giant cells were also seen.

The child was treated with 1000 rads of radiation in one week. The patient responded very well to one week of radiation, with no recurrence in one and a half years of follow-up [Figure - 1]b.

  Discussion Top

Patients with LCH fall into two groups - disease limited to either bone or soft tissue or combination of both. LCH can have a benign course or sometimes can present with a very diffuse progressive disease.[4] These can be subdivided into three clinico-pathological entities: acute disseminated LCH, unifocal and multifocal unisystem LCH, and multisystem LCH. However, there is no unanimity regarding the nomenclature.[5] Any organ may be involved. Bone involvement is seen in 80% of patients: skull, ribs, proximal long bone, vertebrae, and pelvis are the commonly involved sites. Orbital involvement in a series of 76 children with LCH was 23%.[2] The disease seems to particularly affect young children between one and 4 years, with no gender preponderance. Orbital involvement in LCH is characterised by osteolytic lesions with sclerotic margins, commonly involving the zygomatico-frontal suture, as seen in our patient. He had intra-orbital and intracranial (extradural) extension of the lesion into the right frontal lobe.

Patients with bony lesion often have soft tissue involvement in adjacent juxtaneural locations such as orbit, brain or spinal cord. This involvement or intracranial extension from skull lesions does not classify the patients in multisystem group nor worsen the overall prognosis.[4] It is important to do a thorough systemic evaluation to rule out multi-system disease. As pituitary involvement with diabetes insipidus is greater in children, the evaluation should include urine concentration ability. The distinction between single and multisystem disease is very important as management and prognosis depend on this differentiation. Our patient had another lytic lesion in the parietal bone, thus falling into category of single system multifocal disease. To our knowledge, this is the first such case to be reported from India (Medline search).

Infiltration into the orbit and cranium directed us to perform FNAC in our patient. FNAC is a rapid, useful technique for immediate diagnosis that allows concurrent institution of therapy.[5-7] The typical cytopathological picture consists of a mixture of abundant predominantly disassociated Langerhans cells accompanied by many eosinophils and a varying number of neutrophils, lymphocytes, macrophages and multinucleated giant cells with pale ill-defined eosinophilic cytoplasm and lobulated nuclei with longitudinal grooves,[6] best visualised in Papanicolaou-stained smears.[5] Based on the predominant cells, they may be further categorised as Langerhans cells predominant, eosinophil-predominant or macrophage-predominant. Our patient had eosinophil-predominant LCH.

The relative proportion of the individual constituents may vary in a cytopathologic smear of LCH, and make differentiation from an inflammatory or carcinogenic lesion (primary/metastatic) smear difficult.[5] The differential diagnoses include granulocytic sarcoma, fungal/parasitic disease, inflammatory pseudotumour and juvenile xanthogranuloma.[4] A definitive diagnosis is made by presence of Birbeck granules on electron microscopy (rod-like structures with a striated core having dilated ends giving a tennis racket appearance) or positivity for Cluster Designation (CDI) antigen determinants on cryostat sections.[4] In an appropriate clinico-radiological setting, a typical cytopathology alone can be used for effective diagnosis and definite proof of LCH.[5] Immuno-histochemistry (positive staining for adenosine triphosphatase, S-100 protein, alpha-mannosidase and peanut lectin binding), and electron microscopy should probably be reserved for cases which are atypical, as these procedures are time consuming, costly and require repeated needling.[5]

The management is controversial because of unpredictability of outcome and possibility of spontaneous healing. Modalities vary from observation, curettage, intralesional steroids, low-dose radiation, high-dose systemic corticosteroids and chemotherapy, bone marrow transplantation and antibody therapy for recalcitrant cases. Rapid onset cases require active intervention. Low dose radiation in 4-6 fractions may be used when the disease is extensive, inaccessible or if it threatens an important structure.[8] Since our patient had multifocal and extensive lesions, he was treated with radiation. He had no signs of optic nerve damage, radiation cataract, local recurrence or systemic dissemination up to one and half years of follow-up.

  References Top

Char D.H, Ablin A, Beckstead J. Histiolytic disorders of the orbit. Ann Ophthalmol 1984;16:867-73.  Back to cited text no. 1
Hidayat AA, Mafee ME, Laver NV, Noujaim S. Langerhans' cell histiocytosis and juvenile xanthogranuloma of orbit - clinicopathologic, CT and MR imaging features. Radiol Clin North Am 1998:36:1229-40.  Back to cited text no. 2
Writing Group of the Histiocyte Society. Histiocytosis syndromes in childhood. Lancet 1987;1(8526):208-9.  Back to cited text no. 3
Kramer TR, Noecker RJ, Miller JM, Clark LC. Langerhans cell histiocytosis with orbital involvement. Am J Ophthalmol 1997;124:814-24.  Back to cited text no. 4
Pohar-Marinsek Z, Us-Krasovec M. Cytology of Langrehans cell histiocytosis. Acta Cytol 1996;40:1257-64.  Back to cited text no. 5
Ayala AG, RO-JY, Famming CV, Flores JP, Yaskee AW. Core needle biopsy and fine needle aspiration in diagnosis of bone and soft tissue lesions. Hematol Oncol Clin North Am Jun 1995;9:633-51.  Back to cited text no. 6
Shabb N, Faming CV, Carrascu CH, guosq, Kutz R.L, Ayda Ag, et al. Diagnosis of eosinophilic granuloma of bone by fine needle aspiration with concurrent institution of therapy: a cytologic, histologic, clinical and radiologic study of 27 cases. Diagn Cytopathol 1993:9:3-12.  Back to cited text no. 7
Sessa S, Sommelet D, Lascombes P, Prevol J. Treatment of Langerhans cell histiocytosis in children - Experience at the children's Hospital of Nancy. J Bone Joint Surgery-Am 1994;76:1513-25.  Back to cited text no. 8


  [Figure - 1], [Figure - 2], [Figure - 3]

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