Journal of the Egyptian National Cancer Institute 29 (2017) 159–161

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Journal of the Egyptian National Cancer Institute

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Case report

Unilateral proptosis as the initial manifestation of malignancy

K. Rakul Nambiar a,⇑, P.S. Ajith b, Asha Arjunan c
a
Medical Oncology Resident, Dept of Medical Oncology, Regional Cancer Centre, Trivandrum, Kerala, India
b
Radiation Oncology Resident, Dept of Radiation Oncology, Regional Cancer Centre, Trivandrum, Kerala, India
c
Associate Professor, Dept of Radiation Oncology, Regional Cancer Centre, Trivandrum, Kerala, India

a r t i c l e i n f o a b s t r a c t

Article history: Proptosis, a common sign with a broad differential diagnosis, is defined as anterior displacement and pro-
Received 3 April 2017 trusion of one or both orbital globes. Patients can present with varying degrees of chronicity, visual loss
Received in revised form 20 May 2017 and associated symptoms. The etiology of acquired unilateral proptosis is diverse, ranging from benign to
Accepted 22 May 2017
life-threatening. The causes of unilateral proptosis include traumatic, vascular, endocrine, inflammatory,
Available online 30 June 2017
infective and malignant. Breast carcinoma is the most common metastatic cause of proptosis; however,
proptosis has never been reported as the initial manifestation of breast carcinoma. Our patient presented
Keywords:
with unilateral proptosis secondary to an intraorbital lesion and histopathology of orbital lesion was sug-
Unilateral proptosis
Malignancy
gestive of metastatic breast adenocarcinoma. She was later diagnosed to have primary breast carcinoma.
Metastasis We present this unusual case of a 56-year-old woman who presented with proptosis as the initial man-
Breast ifestation of a metastatic breast malignancy.
Ó 2017 National Cancer Institute, Cairo University. Production and hosting by Elsevier B.V. This is an open
access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction in all directions. There was no associated pupillary abnormality

Proptosis is a common clinical manifestations of orbit pathol-
ogy. The causes of unilateral proptosis include trauma followed
by endocrine ophthalmopathy, orbital haemangioma, malignant
lymphomas, pseudotumour, lacrimal gland epithelial tumours,
meningioma, lymphangioma, glioma of optic nerve, metastatic
malignant tumours [1]. Orbital metastates constitute between
2.3% and 7% of orbital tumours [2,3]. Metastatic lesions are pre-
dominantly bilateral and breast carcinoma is the most common
primary cancer to cause metastases to the orbits [4]. Breast carci-
noma with proptosis has always been reported in patients with
an established diagnosis of prior breast carcinoma. Proptosis as
the initial presenting manifestation due to breast malignancy
metastases has never been reported.
Case report
A 56-year-old woman presented with symptoms of left eye
proptosis, which had been slowly progressing over the past
6 months. Ophthalmologic examination findings confirmed pro-
found left proptosis associated with limitation of ocular movement
Peer review under responsibility of The National Cancer Institute, Cairo University.
⇑ Corresponding author.
E-mail addresses: rakulnambiar@yahoo.com (K. Rakul Nambiar), ajithps@gmail.
com (P.S. Ajith), ashaarjunan@yahoo.com (A. Arjunan).
and rest of the neurologic examination was otherwise normal.
The past history and the family history were not contributory. This
patient underwent magnetic resonance (MR) imaging of the orbits
to enable further evaluation. MRI of the orbits showed an expansile
mass with hemorrhages in the superolateral aspect of the left orbit,
greater wing of sphenoid, infiltrating the lateral rectus and extra-
axial extension into the middle cranial fossa (Figs. 1 and 2). Possi-
bility of malignant hemangiopericytoma was suggested by the
radiologist and hence left fronto-temporal craniotomy with super-
olateral orbitotomy was done with gross total excision of the
lesion. Histopathological examination revealed metastatic adeno-
carcinoma and immunohistochemical stains showed CK7, ER and
HER-2/neu protein positivity, with PR and TTF1 – negative (Figs. 3–
5). Possibility of metastatic breast carcinoma was considered;
however our patient did not have any symptoms of breast disease.
A repeat physical examination revealed a breast lump of sixe
2  2 cm in right breast and mammogram demonstrated an irreg-
ular lesion in right breast with microcalcification suggestive of
malignancy. Breast Imaging Reporting and Data System (BIRADS)
score was 4. Trucut biopsy from the breast lesion was confirmatory
of infiltrating ductal adenocarcinoma. Bone scan with Tc-99m
MDP/F-18 revealed extensive bone metastases involving skull, ribs,
vertebra and pelvis. The final diagnosis was metastatic breast car-
cinoma presenting with unilateral proptosis due to intraorbital
metastases. She was treated with letrozole and zolendronic acid.
Anti- Her-2 therapy was not started in view of financial

http://dx.doi.org/10.1016/j.jnci.2017.05.004
1110-0362/Ó 2017 National Cancer Institute, Cairo University. Production and hosting by Elsevier B.V.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
160 K. Rakul Nambiar et al. / Journal of the Egyptian National Cancer Institute 29 (2017) 159–161

Fig. 3. H&E stain (400x) showed infiltrating ductal carcinoma of breast.

Fig. 1. MR imaging of the orbits showed an expansile mass in the superolateral

aspect of the left orbit.

Fig. 4. Immunohistochemical staining showing ER positivity of tumor cells.

Fig. 2. MR imaging of the orbits showed an expansile mass in the lateral aspect of
the left orbit with infiltration of the lateral rectus.

constraints. She has been on follow-up for the past seven months.
Her proptosis has normalized and she is symptomatically better.
Fig. 5. Immunohistochemical staining showing HER-2/neu protein positivity of
tumor cells.
Discussion

Orbital metastases occur in 2–3% of patients with cancer [5]. tumours and breast carcinoma is the commonest primary malig-
Typical manifestations of orbital metastases include proptosis, nancy to cause orbital metastases [4]. The incidence of orbit metas-
pain, inflammation, bone involvement, chemosis, and eyelid swel- tases among patients with breast carcinoma range from 8% to 10%
ling. Orbital metastases have been described for a variety of solid [6] and the frequency of breast carcinoma in orbital metastases
 K. Rakul Nambiar et al. / Journal of the Egyptian National Cancer Institute 29 (2017) 159–161
varied from 29% to 51%. Usually, the primary tumor is diagnosed
before symptoms of orbital disease manifest, and the average time
between the primary diagnosis and orbital metastases varies from
4.5 to 6.5 years. Patients presenting with symptoms of orbital
metastases are usually in the 6th decade of life and are mostly
females [6]. The prognosis is poor with mean survival duration of
breast cancer being 22 months after the diagnosis of orbital metas-
tasis [7].
Orbital metastases from breast cancer have a tendency to infil-
trate the extraocular muscle and orbital fat, causing ocular motility
deficits. Bilateral orbital involvement has been reported in 20% of
orbital metastases due to breast malignancy, which exceeds the
average incidence of other primary neoplasms causing bilateral
metastases [8].
Recognition of orbital metastatic disease and early treatment
are important to improve the quality of life. The aim of treating
orbital metastases is improvement of patient’s quality of life and
restoration or preservation of visual function. Thus, the treatment
of orbital metastases is usually palliative and may include radio-
therapy, hormonal therapy, systemic chemotherapy, or surgery.
Radiotherapy is effective in treatment of orbital metastases with
objective response rates up to 79% [9]. Radiotherapy improves
symptoms in 80% of cases and restores vision in some cases [10].
Novel targeted systemic treatments are being increasingly used
in addition to chemotherapy. Sytemic therapy with Trastuzumab
and vinorelbine has been reported to have shown a dramatic local
response of a unilateral choroidal metastasis in Her2/neu-positive
breast cancer [11].
Extensive orbital surgery is not recommended for removal of
the lesion because surgery is not curative and can be associated
with significant ocular morbidity. Enucleation and other radical
measures do not offer any advantages and may be used only in
cases of unmanageable local hygiene or intractable ocular pain.
The only appropriate role of surgical intervention for orbital metas-
tases is a biopsy for establish the diagnosis.
161
Conclusion
The etiology of acquired unilateral proptosis is diverse, ranging
from benign to life-threatening. Hence, a thorough physical exami-
nation with radiological and pathological analysis is required to
arrive at a correct diagnosis. Thus, the differentials can be signifi-
cantly narrowed with targeted investigations. Although rare, breast
cancer patients can develop metastases to the orbital and ocular
region and can present with orbital symptoms as the initial
manifestation
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