|Year : 2007 | Volume
| Issue : 2 | Page : 156-158
Isolated giant xanthogranuloma of the orbit
Ramesh Murthy1, Santosh G Honavar1, Geeta K Vemuganti2, Milind Naik1, Sanghamitra Burman3
1 The Ocular Oncology Service, LV Prasad Eye Institute, Hyderabad, India
2 The Ophthalmic Pathology Service, LV Prasad Eye Institute, Hyderabad, India
3 The Ocular Immunology Service, LV Prasad Eye Institute, Hyderabad, India
|Date of Submission||16-Jul-2004|
|Date of Acceptance||26-Dec-2005|
Santosh G Honavar
Ocular Oncology Service, LV Prasad Eye Institute, LV Prasad Marg, Banjara Hills, Hyderabad - 500 034
Source of Support: None, Conflict of Interest: None
Xanthogranuloma is an uncommon tumor in the orbit and is usually associated with systemic diseases or blood abnormalities. We report an extremely rare presentation of isolated orbital xanthogranuloma unassociated with any systemic disease, hematological or biochemical abnormalities. A 47-year-old physician presented with proptosis of the left eye of three years duration with yellowish skin plaques. The CT scan revealed a well-defined heterogeneous mass in the medial orbit. There was no evidence of systemic, serum or biochemical abnormalities. The mass was removed by a medial orbitotomy. Histopathology confirmed the diagnosis based on the presence of inflammatory infiltrates, histiocytes and Touton giant cells.
Keywords: Orbit, proptosis, xanthogranuloma
|How to cite this article:|
Murthy R, Honavar SG, Vemuganti GK, Naik M, Burman S. Isolated giant xanthogranuloma of the orbit. Indian J Ophthalmol 2007;55:156-8
|How to cite this URL:|
Murthy R, Honavar SG, Vemuganti GK, Naik M, Burman S. Isolated giant xanthogranuloma of the orbit. Indian J Ophthalmol [serial online] 2007 [cited 2019 Aug 20];55:156-8. Available from: http://www.ijo.in/text.asp?2007/55/2/156/30718
Periorbital xanthogranuloma is a rare entity, which usually presents as bilateral diffuse yellow-orange placoid or nodular lesions in the eyelid or in the orbit. They may be associated with hepatosplenomegaly and systemic diseases such as hyperlipidemias, diabetes mellitus, blood dyscrasias, lymphoproliferative malignancies or asthma and therefore necessitate a thorough systemic evaluation. Herein we report a case of a unilateral giant xanthogranuloma of the orbit in an adult without any systemic manifestations.
| Case Report|| |
A 47-year-old physician presented to us with complaints of gradually progressive, painless protrusion of the left eye of three years duration with the formation of yellowish plaques on the eyelid skin. On examination the best corrected visual acuity was 20/20 in both eyes and intraocular pressure was 16 mmHg in each eye. The left eye was displaced inferolaterally with 6 mm proptosis and a downward displacement of 9 mm [Figure - 1]. On palpation there was a large, firm, immobile, nontender mass in the medial orbit. There were two yellow preseptal placoid lesions, firm in consistency, on the skin of the eyelids. On palpation they were felt as anterior extensions of the orbital mass [Figure - 1]. Adduction in the left eye was severely restricted. No other ocular abnormality was noted. Computerized tomography (CT) scan revealed a well-defined, heterogeneously dense mass in the medial orbit, predominantly extraconal in location extending into the intraconal space displacing the optic nerve medially [Figure - 2]a and b. There was no significant contrast enhancement. Systemic examination did not reveal any organomegaly or lymphadenopathy. Serum lipids, blood counts, complement levels and erythrocyte sedimentation rates were normal. Serum immune protein electrophoresis and serum assay of IgG, IgM and IgA did not reveal any abnormality. Electrocardiography, chest X-ray and ultrasound abdomen were within normal limits. There were no signs of sclerosis of the long bones on X-ray examination.
The patient underwent orbitotomy by the medial transcaruncular approach. A well-defined yellow lobulated mass measuring 40 x 36 mm in size was removed from the medial orbit [Figure - 3]. Cut section showed streaks of yellowish areas interspersed with dense fibrous tissue. Histopathology revealed dense collagenous tissue with inflammatory infiltrates and histiocytes within collagen bundles. A few areas showed Touton type multinucleated giant cells with wreath-like arrangement of nuclei and cytoplasmic vacuoles around the nuclei [Figure - 4]. There was no evidence of necrobiosis. Based on these features, a diagnosis of orbital xanthogranuloma was made.
The patient did not show any signs of recurrence or evidence of systemic disease at 30 months of follow-up.
| Discussion|| |
Xanthogranulomas are histiocytic tumors in which the histiocytes contain lipid deposits. The appearance of xanthogranulomas on histopathology is characteristic. There is a proliferation of histiocytes, plasma cells and lymphocytes with Touton giant cells that stain positive for lipid. The Touton giant cells are multinucleate cells with the nuclei arranged in a wreath around a nidus of eosinophilic cytoplasm and separated from the cell membrane by a rim of translucent foamy cytoplasm.
An immunologic basis has been suggested for the etiopathogenesis. Bullock suggested that serum immunoglobulins were complexed with lipids and were deposited in the skin, eliciting a giant cell foreign body reaction.
Xanthogranulomas are grouped into three categories - juvenile xanthogranulomas, Erdheim-Chester disease and adult xanthogranulomas, some with features of necrobiosis.
Juvenile xanthogranulomas usually present with skin or intraocular lesions and orbital involvement which is rare, occurs almost exclusively in children. Erdheim-Chester disease is the association of adult onset xanthogranulomas with systemic disease, especially with diffuse sclerosis of the diaphysis and metaphysis of long bones and sometimes with hepatosplenomegaly and hepatic adenomas. Ocular involvement is usually rare though cases of proptosis have been reported.
Patchy areas of collagen destruction characterize necrobiotic xanthogranulomas. Kossard and Winkelmann first described the association of necrobiotic xanthogranuloma with paraproteinemia in 1980. Since then there have been many more reports of the association of necrobiotic xanthogranulomas with paraproteinemia. The associated entities include IgG monoclonal gammopathy, plasmacytosis, cryoglobulinemia, complement deficiency and leucopenia. Multiple myeloma and non-Hodgkin's lymphoma have also been reported to be associated with necrobiotic xanthogranuloma.,
About 50 cases of orbital xanthogranulomas have been reported in literature, most being necrobiotic xanthogranulomas. Jakobiec et al . reported six cases associated with adult onset asthma without features of necrobiosis at presentation. Out of the three patients of adult onset xanthogranuloma described by Rose et al ., two had features of necrobiosis. Miszkiel et al . described eight adult cases of xanthogranuloma, all of whom had associated systemic disease. Hammond et al . reported a case of adult onset xanthogranuloma without any systemic associations except asthma. Nearly all of these cases reported in the literature have either abnormal blood tests and/ or associated systemic disease. Our case was unique because it was a massive, unilateral tumor without any abnormality in the blood tests or associated systemic disease or asthma.
Various treatment modalities have been tried including local excision, radiotherapy, periocular and systemic steroids, chemotherapy with chlorambucil, nitrogen mustard, cyclophosphamide and melphalan and even plasmapheresis., We resorted to a complete excision of the mass as it was well defined. We did not plan adjuvant therapy following excision because there was no residual mass.
It has to be emphasized that the diagnosis of xanthogranuloma may be considered in a patient with proptosis with associated periocular yellowish cutaneous plaques. Patients with xanthogranulomas can develop plasma cell dyscrasias and lymphoproliferative malignancies especially in the presence of necrobiosis, hence a thorough baseline systemic evaluation and long-term follow-up is mandatory.
| References|| |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]