orbital tumors

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The International Journal on Orbital Disorders, Oculoplastic and
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Erdheim-Chester disease with orbital involvement:
Case report and ophthalmic literature review
Helen Merritt, Margaret L. Pfeiffer, Karina Richani & Margaret E. Phillips
To cite this article: Helen Merritt, Margaret L. Pfeiffer, Karina Richani & Margaret E. Phillips
(2016): Erdheim-Chester disease with orbital involvement: Case report and ophthalmic
literature review, Orbit, DOI: 10.1080/01676830.2016.1176211
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2. REVIEW ARTICLE
Erdheim-Chester disease with orbital involvement: Case report and ophthalmic
literature review
Helen Merritta
, Margaret L. Pfeiffera
, Karina Richania,b
, and Margaret E. Phillipsa,b
a
Ruiz Department of Ophthalmology and Visual Science, The University of Texas Health Science Center at Houston, Houston, Texas, USA;
b
Robert Cizik Eye Clinic, Houston, Texas, USA
ABSTRACT
Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disease in which orbital involve-
ment can have devastating outcomes. Through a case report and review of the ophthalmic
literature, we explore orbital findings, disease progression, and treatment options. Cases of orbital
involvement in Erdheim-Chester disease were identified in the ophthalmic literature with a
PubMed query and review of cited references. A total of 14 publications reporting 19 separate
cases that included ophthalmic examination data were identified. Patient ages ranged from 26–77
years with a mean age of 50 years. Seventy-four percent (14/19) were men. Vision progression to
no light perception was found in 32% (6/19) of the patients. Reviewed cases reported a variety of
medical and surgical treatment approaches, however, only 53% reported cases (10/19) demon-
strated disease improvement or stabilization. Erdheim–Chester disease with orbital involvement is
a devastating disease with a poor prognosis. Awareness of this entity by the ophthalmologist is
important as orbital signs and symptoms may manifest early, and orbital biopsy is often crucial to
the definitive diagnosis.
ARTICLE HISTORY
Received 9 November 2015
Accepted 4 April 2016
KEYWORDS
Erdheim-Chester; orbit;
xanthogranulomatous
Introduction
Erdheim-Chester disease (ECD) is a systemic xanthogra-
nulomatous disease that most commonly affects the long
bones and visceral organs but can also present with orbital
involvement. We present a case to exemplify the profound
ophthalmic and visual sequelae that can result from orbital
involvement of this rare disease. Additionally, we review
disease characteristics, treatment options, and outcomes of
similar cases reported in the ophthalmic literature.
Case
A 71-year-old woman presented to Memorial Hermann
Hospital–Texas Medical Center for management of
severe bilateral proptosis, vision loss, and eye pain in
June of 2014. Her past medical history was significant
for coronary artery disease, hydronephrosis, diabetes
insipidus, chronic lymphedema, papillary thyroid can-
cer, and breast cancer. A presumptive diagnosis of
thyroid eye disease had been given prior to presentation
to our facility, and she had been given a high-dose oral
steroid taper and 20 Gy of orbital radiation therapy for
compressive optic neuropathy. She had continued to
worsen with significant periocular swelling and was
transferred to our facility for further care and surgical
intervention.
Initial examination demonstrated marked bilateral
proptosis with significant resistance to retropulsion,
periorbital edema, and bullous chemosis (Figure 1).
Visual acuity was no light perception in the right eye
and light perception in the left eye. Both pupils were
fixed. Motility examination showed complete ophthal-
moplegia in both eyes.
Both computed tomography (CT) and magnetic
resonance imaging (MRI) of the orbits revealed enhan-
cing soft tissue-density masses in the intraconal retro-
bulbar space surrounding the optic nerves with
obliteration of normal orbital fat bilaterally (Figure 2).
After our evaluation, the patient was started on intra-
venous methylprednisolone to reduce orbital conges-
tion and underwent bilateral endoscopic medial wall
decompression, orbital floor decompression, and orbi-
tal tumor biopsy and debulking. Biopsies of the firm,
yellow masses in the intraconal spaces revealed foamy
histiocytes with Touton giant cells, fibrotic and lym-
phocytic infiltrate, and positive immunostaining for
CD68 and negative for CD1A, consistent with a diag-
nosis of ECD. Systemic workup revealed findings con-
sistent with advanced ECD including pericardial and
CONTACT Margaret E. Phillips margaret.e.phillips@uth.tmc.edu Ruiz Department of Ophthalmology and Visual Science, The University of Texas
Medical School at Houston, Robert Cizik Eye Clinic, 6400 Fannin Street, 18th
Floor, Houston, TX 77030, USA.
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3. pleural effusions, involvement of the bilateral lower
extremities, and soft tissue masses in the retroperito-
neum. Her previously diagnosed diabetes insipidus,
hydronephrosis, and renal insufficiency were attributa-
ble to ECD. Despite recommendations of treatment
with interferon-alpha with possible radiotherapy for
local palliation, the patient declined further medical
treatment.
The patient was followed in our ophthalmology
clinic for further comfort care of orbital disease and
microbial keratitis. By 2 months’ follow-up, her vision
had progressed to no light perception in each eye. She
died from complications of the disease 3 months after
her initial presentation.
Methods
We wished to explore specifically the ophthalmic exam-
ination details and disease progression of cases of
Erdheim-Chester disease with orbital involvement and
therefore chose to limit our case review to only include
cases published in the ophthalmology literature.
Published cases were identified by a PubMed search
for the terms “Erdheim-Chester disease” with the
terms “orbit” and “orbital.” Cases published in
English, reported in the ophthalmic literature, and
that discussed patients with pathology-proven ECD
involving the orbits were included. Cases that lacked
ophthalmic examination data were excluded.
Results
Our PubMed search identified 13 separate publications
reporting ECD with orbital involvement in the English
language ophthalmic literature.1−13
One additional pub-
lication was identified by review of all cited references.14
These 14 total publications reported 21 separate patients
with ECD involving their orbits. Of these patients, 19/21
reported ophthalmic examination details, treatment, and
disease course and were therefore included for review
and analysis (Table 1).
Age at time of presentation ranged from 26 to 77
years with a mean age of 50 years. Of these patients, 14/
19 (74%) were men and 5/19 (26%) were women.
Average reported follow-up was 119 months. These
demographics were similar to previously published lar-
ger series in the non-ophthalmic literature.15,19
In this
review, 17/19 cases exhibited the classic presenting
symptoms of proptosis and motility disturbance with
diffuse, intraconal orbital involvement. Notable differ-
ences included one patient presenting with motility
restriction and optic disc edema and another patient
with bilateral lacrimal gland enlargement.8,9
Additionally, two patients had mass extension past the
Figure 1. External photograph showing marked bilateral proptosis and bullous chemosis.
Figure 2. CT (A) and MRI (B) showing retrobulbar intraconal masses with obliteration of normal orbital fat.
2 H. MERRITT ET AL.
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4. Table 1. Summary of 19 reported ECD cases in the ophthalmic literature.
Case Author, Year
Age
Sex Va OD* Va OS* Ophthalmic Findings Imaging
Surgical
Intervention Treatment and Outcome
Follow
Up
1 Alper et al.
19831
70
yo
M
20/25 20/40 Bilateral proptosis +
EOM limitation,
chemosis, posterior
pole striae, increased
IOP on upgaze
CT – bilateral
enhancing
retrobulbar masses.
Right mass
extending to
chiasm.
Right lateral
orbitotomy with
removal of mass
Corticosteroids with
decrease in mass size,
improved EOM. Death
from cardiac failure.
42
months
2 44
yo
F
NLP NLP Bilateral proptosis +
EOM limitation, optic
disc edema, decreased
color vision, visual
field loss, thinning of
lower lids with lid
xanthelasmas
CT – bilateral
enhancing masses
filling muscle
cones of both
orbits
Orbital biopsy,
resection of lid
lesion
Death from stroke.
Autopsy revealed orbit
masses extending to
chiasm and surrounding
pituitary gland.
29
years/
348
months
3 Shields et al.
199111
38
yo
M
NLP NLP Bilateral proptosis +
EOM limitation, lid
xanthelasmas
CT – Bilateral,
diffuse orbital
masses
Bilateral lateral
orbitotomy with
mass debulking
Corticosteroids.
Improved pain and
proptosis with surgical
debulking. Death from
renal and cardiovascular
failure.
12
months
4 77
yo
M
20/30 20/25 Left proptosis + EOM
limitation (only mild
elevation limitation,
left), lid xanthelasmas
CT – bilateral,
lobulated,
enhancing,
infiltrative
intraconal and
extraconal orbital
masses
Orbital biopsy Patient survival at 2 years
follow-up with severe
cardiovascular illness.
24
months
5 De Palma et al.
19975
61
yo
M
<20/400 <20/400 Bilateral proptosis,
total ophthalmoplegia,
optic disc edema,
posterior pole striae
CT – bilateral
intraconal
retrobulbar masses
extending to apex
Bilateral orbital
decompression of
medial wall,
lateral wall, floor
Corticosteroids with
improved motility.
Development of
keratouveitis, glaucoma
three years later.
Death from cardiac
decompensation.
52
months
6 Valmaggia
et al. 199713
55
yo
M
20/20 20/20 Bilateral proptosis +
EOM limitation
MRI - bilateral
retrobulbar
intraconal
infiltration
Transconjunctival
biopsy of left
globe
Corticosteroids.
Improvement of
proptosis and EOM. Slow
progression of optic
atrophy.
Not
reported
7 Amrith et al.
19992
35
yo
M
20/20 20/20 Bilateral proptosis +
EOM limitation, left
forniceal mass, lid
xanthelasmas
CT - bilateral
enhancing
intraconal masses
with extension into
inferior extraconal
space of left orbit
Anterior
orbitotomy with
biopsy and
debulking
Corticosteroids,
vinblastine with
improvement of
proptosis, decreased
orbital mass size. Lost to
follow-up for 4 years
with return of proptosis,
growth left orbital mass
into cavernous sinus,
preserved vision.
48
months
8 Sheidow et al.
199910
57
yo
M
NLP NLP Bilateral proptosis +
EOM limitation,
chemosis, optic disc
edema, left ptosis, lid
xanthelasmas,
CT – bilateral
enhancing
intraconal masses
None Corticosteroids,
Cladribine, EBRT (25 Gy)
without response of
masses and with
subsequent radiation
retinopathy.
12
months
9 46
yo
M
20/80 20/400 Bilateral proptosis,
chemosis, posterior
pole striae
MRI – diffuse
infiltration of both
orbits, worse on
the left
Orbital biopsy Corticosteroids, EBRT (40
Gy,) without response of
tumors, azathioprine, 20
Gy with improvement of
Va to 20/40 OS. Radiation
retinopathy with
multiple BRVOs.
Not
reported
10 Esmaeli, et al.
20016
55
yo
M
20/80 20/200 Bilateral proptosis +
EOM limitation, optic
disc edem1a
MRI – massive
infiltration of the
retrobulbar space
Orbital biopsy Corticosteroids, IFN-α
with Va improvement to
20/25 OD, 20/30 OS,
resolved pain, lesion size
decrease at 4 years.
12
months
(Continued)
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5. orbital apex to surround the pituitary gland or to
invade the cavernous sinus.1,2
Vision loss in cases of
orbital ECD was most commonly secondary to orbital
or anterior visual pathway compression of the optic
nerves, however involvement of the central nervous
system with masses in the posterior visual pathway
have resulted in homonymous hemianopia.9,20
The degree of vision loss in each described patient
varied, with severe visual impairment and vision pro-
gression to no light perception only occurring in 6/19
(32%) of our reviewed cases.1,8−12
The average time
from initial ophthalmic consultation to NLP vision in
the 5/6 of these cases reporting this information was 3
years, with a range in timing from 4 months to 10
Table 1. (Continued).
Case Author, Year
Age
Sex Va OD* Va OS* Ophthalmic Findings Imaging
Surgical
Intervention Treatment and Outcome
Follow
Up
11 Karcioglu et al.
(2) 20038
38
yo
M
LP NLP Bilateral proptosis+
EOM limitation, lid
retraction, lid
xanthelasmas
CT – bilateral
intraconal masses
Bilateral
orbitotomy with
surgical
debulking
EBRT with recurrence.
Death in 12 months from
renal failure.
12
months
12 51
yo
M
20/80 20/30 Bilateral lacrimal gland
and upper lid masses,
EOM limitation
CT – bilateral
lacrimal gland and
eyelid masses
Incomplete
surgical excision
Corticosteroids with
recurrence, EBRT without
response.
Death in 14 months from
renal failure.
14
months
13 Myra et al.
200414
45
yo
M
20/20 20/30 Bilateral proptosis +
EOM limitation,
chemosis, optic disc
edema, posterior pole
striae
CT – bilateral
intraconal masses
Anterior
orbitotomy and
biopsy
Corticosteroids,
azathrioprine,
cyclophosphamide,
etoposide, cyclosporine
without sustained
improvement. Cladribine
with improvement of Va
and proptosis.
156
months
14 Hoffmann
et al. 20047
61
yo
M
20/250 CF Bilateral proptosis +
EOM limitation, optic
disc edema, posterior
pole striae
CT – bilateral
diffuse retrobulbar
masses
Orbital biopsy,
endonasal
decompression
Corticosteroids, EBRT
with 20 Gy without
improvement, MTX.
Continued loss of visual
acuity.
Not
reported
15 Cruz 20064
28
yo
F
20/20 20/20 Left proptosis + EOM
limitation
CT – Intraconal
infiltration right
orbit. Diffuse
intraconal and
extraconal
infiltration left
orbit.
Orbital biopsy Corticosteroids,
cyclophosphamide with
resolution of proptosis
and motility deficits after
18 months.
24
months
16 Sivak-Callcott
et al. 200612
67
yo
F
NLP HM Bilateral proptosis +
EOM limitation
CT – intraconal
homogenous
enhancing masses
with smooth
margins with
obscured EOMs
and displaced optic
nerves
None Corticosteroids,
cyclophosphamide with
initial improvement then
decline.
Death 4 months after
presentation.
4
months
17 Pineles et al.
20119
26
yo
F
NLP CF EOM limitation, optic
disc edema
progressing to
atrophy, lid
xanthelasmas
MRI – bilateral
symmetric
retrobulbar soft
tissue masses
Orbital biopsy EBRT (18 Gy) with stable
disease for 1 year.
Corticosteroids,
Cladribine with decrease
in size of orbital lesions,
IFN-α without
improvement of Va.
48
months
18 32
yo
F
Not
reported
<20/400 Left proptosis, left
relative afferent
pupillary defect
CT – left orbital
infiltrative lesion
involving rectus
muscles
Left orbital
decompression
IFN-α with decreased size
of orbital lesions, stable
disease at 1 year.
24
months
19 Arora et al.
201213
66
yo
M
<20/400 20/200 Bilateral proptosis,
chemosis
CT - Multiple
bilateral intraconal
masses
MRI – Enhancing
intraorbital masses
hypointense to
muscles
Orbital biopsy Corticosteroids, Imatinib
with 20% reduction in
orbit disease over 11
months.
11
months
*Denotes worst visual acuity reported after disease progression in each case, converted to closest Snellen fraction.
NLP = No light perception; LP = Light perception; HM = Hand motion; CF = Count fingers; Va = visual acuity; EOM = Extraocular motility; IOP = intraocular
pressure; EBRT = External beam radiation therapy; CT = Computed tomography; MRI = Magnetic resonance tomography; BRVO = branch retinal vein
occlusion; IFN-α = interferon alpha; MTX = methotrexate.
4 H. MERRITT ET AL.
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6. years.1,9−12
The average follow-up time for patients that
preserved vision was 41 months. Our patient pro-
gressed to profound vision loss in both eyes despite
treatment with systemic corticosteroids, orbital radio-
therapy, and bilateral orbital decompression. There is
no standard treatment for ECD with orbital involve-
ment. Typically, treatment options involve a combina-
tion of surgical debulking, radiation, and medical
therapies including systemic corticosteroids, che-
motherapeutic agents, or immunotherapy.3,12,16
Discussion
Erdheim–Chester disease is a rare, xanthogranuloma-
tous disease characterized by diffuse infiltration of the
long bones, heart, lungs, kidneys, and retroperitoneum
by histiocytes and lipid-laden macrophages.
Histopathology classically reveals fibrosing xanthogra-
nulomas with foamy histiocytes and Touton giant cells.
Periorbital findings include xanthelasma of the eyelids
and eyelid thinning.1
ECD with orbital involvement is
less common, occurring in about 25% of these
patients,15,16
and with less than 40 total cases reported
in the English-language literature.12
In contrast to other
xanthogranulomatous diseases that may involve only
the anterior orbit, ECD orbital lesions are diffuse and
more likely to cause vision loss.12
Classic presenting
symptoms with orbital involvement include painless,
progressive, bilateral proptosis that may be associated
with decreased visual acuity. Additional ophthalmic
manifestations most often include ophthalmoplegia,
optic disc edema or atrophy, and retinal striae.1
Imaging studies most commonly reveal bilateral, enhan-
cing intraconal masses, however there may be extraconal
lesions present as well.1,2,4,8,11,17
Histopathology of orbital
lesions show diffuse xanthogranulomatous findings includ-
ing histiocytes, Touton giant cells, and fibrous tissue with
clustered plasma cells, lymphocytes, and eosinophils.
Histiocytes exhibit positive expression of CD68 and are
negative for CD1a and S-100, differentiating this disease
from Langerhans cell histiocytosis.[4]
These lesions are
infiltrative, found to completely replace the orbital fat,
and may extend into the extraocular muscles and optic
nerve.18
ECD has a very poor overall prognosis with one
case series reporting the death of 22 of 37 patients (59%)
within an average follow-up period of 32 months.16
Although there is no standard treatment for ECD with
orbital involvement, most therapeutic approaches involve a
combination of systemic corticosteroids, chemotherapeutic
agents, or immunotherapy.3,12,16
The successful use of
interferon-alpha has been reported, with one case series
of 3 patients demonstrating marked improvement of orbi-
tal and systemic disease, excellent toleration of therapy, and
duration of improvement over 3 years of maintenance
dosing.21
Another case report described a patient with
complete, dramatic resolution of proptosis and improve-
ment in visual acuity after administration of cladribine, a
purine analogue targeting monocytes, after failure of
response to several other chemotherapeutic medications.14
Although other reported patients failed to show such
impressive results with cladribine administration,9,10,14
this patient had no recurrence of orbital signs and symp-
toms at 2 years after cessation of therapy. The authors
hypothesized that this result was due to increased mono-
cyte activation in this particular patient, arguing for con-
sideration of choosing therapy tailored to distinct
histopathological or immunohistochemical features.
Treatment with external beam radiation therapy
(EBRT) has been described as a palliative treatment for
pain associated with ECD, however the duration of relief
is demonstrated to be limited.22
Our reviewed cases
included 6/19 patients receiving EBRT to the orbits for
treatment of their disease with total doses ranging from
18 to 60 Gy.7−10
All cases failed to demonstrate durable
improvement of symptoms or significant prevention of
disease progression. Notably, two of these cases resulted
in the development of marked, bilateral radiation retino-
pathy after orbital EBRT with permanent secondary loss
of vision.10
Our patient received 20 Gy of radiation
therapy to both orbits prior to transfer into our care
without improvement in her condition and with even-
tual progression of vision loss.
Surgical treatment options include mechanical debulk-
ing of intraorbital masses and orbital decompression. Of
the reported cases, 5/19 described partial or total resec-
tion of orbital masses.1,2,8,11
Three reported cases utilized
surgical decompression for alleviation of orbital mass
effect in addition to systemic therapy. 5,7,9
Our patient
also underwent bilateral orbital decompression without
significant improvement in her vision and with eventual
progression of her orbital disease. Review of the outcomes
in the 9 patients who underwent surgical debulking or
orbital decompression revealed improved pain in one
case11
but overall poor response and continued progres-
sion in 8 cases despite further attempted systemic treat-
ment. The best therapeutic outcome and symptom
improvement was found instead in the patients receiving
aggressive medical systemic treatment with corticoster-
oids, chemotherapy, or immunomodulatory therapy.3,4,6
-
6,9,14
Our patient declined further systemic treatment.
Conclusions
Erdheim-Chester disease with orbital involvement is a rare
condition with challenging management. Despite this rar-
ity, ECD should be kept in the differential diagnosis for
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7. patients presenting with bilateral proptosis and orbital
masses, particularly in the setting of associated periorbital
xanthelasmas and other common systemic manifestations.
Although not well defined in the literature, overall survival
in patients with ECD appears to be correlated to the extent
of organ involvement.13
Consequently, early diagnosis of
patients presenting with orbital manifestations may lead to
earlier intervention in hopes of slowing systemic progres-
sion. The few reviewed cases in this series with reported
orbital or systemic disease stabilization or improvement
showed no clear, discernable temporal relationship
between ophthalmic manifestations, systemic manifesta-
tions, timing of treatment, and outcome. Importantly,
patients may present with ophthalmic signs and symptoms
as the first expression of this disease, making the ophthal-
mologist an important player in disease recognition.
Our patient and 13/19 literature cases (68%) report
the definitive diagnosis of ECD as either dependent on
or greatly helped by orbital findings and biopsy.
Unfortunately, despite varied, aggressive medical and
surgical approaches, only 10/19 reported cases (53%)
demonstrated improvement or stabilization of ophthal-
mic and orbital morbidity. Although disease progres-
sion and unfavorable visual outcomes occur in many
patients, systemic treatments and surgical intervention
may still be considered to improve ophthalmic symp-
toms and quality of life.
Declaration of interest
The authors report no conflicts of interest. The authors alone
are responsible for the content and writing of the article.
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