Orbital Xanthogranuloma – A Rare and Challenging Orbital Disease

398 AIOC 2009 PROCEEDINGS
Orbital Xanthogranuloma – A Rare and Challenging Orbital
Disease
Dr. E. Ravindra Mohan, Dr. Moupia Goswami, Dr. Malay Verma, Dr. Bipasha
Mukherjee, Dr. Kunal Kumar, Dr. Jotirmay Biswas, Dr. Krishna Kumar,
Dr. Nirmala Subramanian
(Presenting Author: Dr. Moupia Goswami)
Orbital xanthogranuloma (XG) is a rare
proliferative lesion of unknown etiology.It
is classified under non – Langerhans cell
histiocytoses (class 2) according to the current
classification by Writing Group of the Histiocyte
Society. 1 The orbital XG may present as a solitary
lesion or as a part of disseminated xanthogranulomatous
infiltration of various organs and long
bones. 2 We present the clinical and radiological
features and treatment outcome of 7 patients
with orbital XG seen at Sankara Nethralaya
during the period Jan 1991 to May 2008.
Materials and Methods
This retrospective non comparative
interventional case series includes 7 patients with
histopathologically confirmed orbital XG .The
clinical, radiological and histopathological
features and treatment outcome were reviewed.
In all patients, hematological and radiological
tests were done to rule out systemic involvement.
Case 1
A 35 year old lady was examined for painless ,
progressive protrusion of both eyes of 5 years
duration. She had been treated with oral steroids
off and on with dramatic improvement in
symptom , and recurrence on stopping the same.
On examination , axial proptosis was noted with
firm palpable masses in both orbits. .Slit lamp
examination revealed reduced tear meniscus,
superficial punctate keratopathy and posterior
subcapsular cataract in both the eyes. Bilateral
parotid enlargement was noted. Thus the
possibility of Sjogren’s syndrome with
underlying autoimmune disease was considered.
CT scan revealed bilateral diffuse illdefined extra
and intraconal soft tissue with clumps of
calcification. Transeptal orbital biopsy was done.
Histopathological examination showed multiple
scattered foamy histiocytes and Toutan giant
cells suggestive of xanthogranulomatous
inflammation. In view of side effect of long term
steroids, patient was started on oral anti –
inflammatory drugs and steroid sparing
immunosuppressive therapy (Cyclophosphamide,
Endoxan 50 mg daily), with biweekly
monitoring of WBC and platelet counts. Surgical
debulking was not considered in view of
potential risks.The patient was symptomatically
better.There was considerable decrease in
proptosis The patient underwent uneventful
phacoemulsification with foldable intraocular
lens implantation in the right eye for steroid
induced cataract.
Case 2
A 30 year old man was examined for recurrent
painless right upper lid swelling of 7 years
duration, resolving partially with oral steroids
and recurring on stopping the same. On
examination, a diffuse swelling with central firm
nodularity was noted in the right upper lid.CT
scan showed diffuse preseptal swelling.
Histopathological examination of biopsy
specimen suggested xanthogranulomatous
inflammation. Complete blood count,
erythrocyte sedimentation rate, serum lipid
profile, liver and kidney function tests , X-ray of
long bones and ultrasonography abdomen was
normal. Patient was started on tapering dose of
oral steroids.Recurrence occurred after stopping
steroids and debulking had to be done twice
during the followup of 5 years. There was
significant reduction in swelling during last
followup.
Case 3
A 46 year old man came with complaints of
gradually increasing, painless , lower lid swelling
in both eyes since last 4-5 years. On examination,
nontender, firm, nodular well defined soft tissue
mass was palpable along the lower lid.
Xanthelesmata were present superonasally on
both upper eyelids. CT showed diffuse illdefined
soft tissue thickening in the lower eyelids
.Histopathological examination of incisional
biopsy specimen revealed features of

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xanthogranuloma. Serum cholesterol was
elevated. Serum protein electrophoresis and bone
marrow examination was normal. Patient was
again seen two years later with complaints of
increase in size of the same lower lid swelling
,when further debulking was done.There was no
further recurrence till last follow up.
Case 4
A 48 year old woman was seen with complaints
of painless swelling in the periocular area of both
eyes since 3 years. On examination, firm
nontender mass was palpable in the periocular
area. CT scan revealed diffuse preseptal and
anterior orbital swelling. Debulking was done
through anterior orbitotomy. Histopathological
examination revealed features of orbital
xanthogranuloma.There has been no recurrence
during the follow up period of three years.
Case 5
A 57 year old woman was examined for left
upper lid swelling of 1 year duration. On
examination , yellowish discolouration of upper
lid with underlying firm, nodular swelling was
found. Xanthelasmata of eyelid was present. CT
scan revealed diffuse lesion in extraconal,
superior and superotemporal region of left orbit.
Histopathology examination of incisional biopsy
specimen showed features of xanthogranuloma.
There was resolution on oral steroids, but
recurrence on stopping the same , during the
followup of 3 years.
Case 6
A 62 – year old gentleman presented to us with
gradually increasing painless swelling of the
right eye since one year. History was suggestive
of non improvement with oral steroids. On
examination, firm non tender upper and lower
lid mass with proptosis of 6 mm was noted in
the right eye. Motility was minimally restricted
in all directions. There was increased resistance
to retropulsion. CT scan showed homogenous,
periocular soft tissue lesion extending
extraconally in the right orbit with minimal
calcification in the lacrimal gland area.
Histopathological examination of the biopsy
specimen was suggestive of orbital
xanthogranuloma. As adequate debulking was
done intraoperatively, patient was kept under
observation . There was no recurrence during
follow up of 6 months.
Case 7
A 62 year old gentleman was examined for very
slowly progressive periocular swelling in both
eyes of two years duration. On examination , non
tender, firm mass was palpable in both upper
lids. Xanthelasma was seen in upper lid. CT scan
showed bilateral diffuse enlargement of lacrimal
gland, thickening of superior muscle complex
with periglandular and perimuscular fat
stranding.Histopathological examination of
incisional biopsy specimen suggested
xanthogranuloma with chronic inflammation.
Systemic and hematological workup was normal.
The patient was kept under close follow up.
Discussion
All our patients had proptosis or eyelid swelling.
Four patients had bilateral and 3 had unilateral
disease.Clinical features included proptosis and
severe extraocular motility limitation [2 patients],
xanthelasma of eyelids [3 patients] ] and dry eyes
in [1 patient].
Tumor volume and location determined the
amount of proptosis. CT scan revealed
homogenous, infiltrating soft tissue masses in the
orbital and periorbital soft tissues. Enlargement
of lacrimal gland was seen in 2 patients. No bone
destruction secondary to orbital masses was seen
in any of the cases.This finding is consistent with
the study by Shields et al. 3
Cutaneous xanthomas may occur in orbital XG
patients, with either normal or elevated
triglyceride and cholesterol levels, as seen in
our study as well as in that by Z.A. Karcioglu et
al. 2
The usual treatment modalities for XG lesions are
surgical excision combined with corticosteroids
and chemotherapeutic agents. 3,4 XG is
radioresistant.Treatment in our series included
oral steroids [2 patients], NSAID with
cyclophosphamide [1 patient] , debulking only
[2 patients] and observation [2 patients]. Mean
followup was 2.78 years. Marked resolution of
lesion was seen in the patient on
cyclophosphamide. Two patients were
unresponsive to steroids, one of whom
underwent debulking twice. The two patients
under observation only had mild disease and
remained stable during the follow up period . In

400 AIOC 2009 PROCEEDINGS
the study by Z.A . Karcioglu et al 2 also debulking
and oral steroids were the modalities of
treatment. In their study, three patients
responded to surgical excision alone, one patient
responded to debulking and in three patients the
XG recurred despite corticosteroid treatment and
EBRT.Out of the three patients who had poor
outcome, two patients had systemic
xanthogranulomatosis. The outcome of
treatment was variable as in our series.
1. Chu AC, Dangio G, Favera D.Histiocytosis
syndrome in children. Lancet 1987;1:208-9.
2. Z.A.Karcioglu, N.Sharara, A. Boles, A. M. Nasr.
Orbital Xanthogranuloma, clinical and
morphological features in eight patients. Ophthal
Plastic and Reconstructive Surgery. 19:372-81.
3. Shields JA et al. Orbital and eyelid involvement
with Erdheim – Chester disease:a report of two
References
Treatment of orbital XG needs to be tailored and
titrated to the disease severity and patient. Since
it often tends to relapse and recur, long term
followup is needed as well as monitoring to
assess for complications of orbital disease like
ocular motility restriction or complications of
therapy, like steroid induced cataract. The
prognosis is variable and depends on the
underlying disease and its severity.
cases. Arch Ophthalmol 1991;109:850-4.
4. Alper MG, Zimmmerman LE et al.Orbital
manifestations of Eedheim – Chester Disease. Trans
Am Ophthalmol Soc 1983;81:64-85.
5. Valerie L.Vick et al. Orbital and eyelid
manifestations of xanthogranulomatous diseases.
Orbit, 2006;25:221-5.