Open Journal of Ophthalmology, 2012, 2, 37-39 Published Online May 2012 ( 37
A Rapidly Progressive Proptosis, a Case Report
Afshin Tajdini, Mazen Sinjab, Somer Hassan
Department of Ophthalmology, Al-Mouasat Hospital, Damascus, Syria.
Received January 10th, 2012; revised February 16th, 2012; accepted March 8th, 2012
In contrast to intraocular metastases, orbital metastases are a rare manifestation of systemic malignancies; such orbital
metastases account for only 1% - 13% of all orbital tumors [1]. Such tumors grow rapidly; they may undergo necrosis,
as the rate of tumor angiogenesis cannot keep pace with the rapidly expanding mass. Here, we report a rare case of an
orbital metastasis from a nasopharyngeal carcinoma.
Keywords: Proptosis; Orbital Metastasis; Nasopharyngeal Carcinom a; Undifferentiat ed C a rci noma
1. Case History
A 45-year-old heavy smoker male presented with a rap-
idly expanding left proptosis, diminished vision and re-
stricted eye movements, which he had first noticed one
month prior to presentation. The Left eye Examination
revealed severe proptosis (grade IV), restricted eye
movement in all directions (frozen eye), severe con-
gested conjunctiva and clear medias. Tonometry was
normal. The visual acuity was hand motion with no im-
provement on pinhole or correction. The fundus on the
affected side showed normal disc, generalized RPE atro-
phy and macula showed dull foveal reflex. Retinal arter-
ies were normal, while veins were congested, but there
were no hemorrhages or exudates. There was a history of
nasopharyngeal carcinoma (NPC) with metastasis to 3rd
and 4th cervical spine and to the root of common carotid
artery one year ago. He had received chemotherapy and
radiotherapy treatment at the time of diagnosis. The last
radiotherapy session was about 5 month before.
An orbital CT scan of the left orbit, that was done one
month before, showed a large soft tissue mass (5 × 3 cm)
was extended from lateral wall of the orbit to the optic
foramen and to the cranium, in order to lie between the
frontal and temporal lobes. The mass pushed the glob to
the medial and optic nerve to lateral. (Figure 1)
An orbital MRI of the left orbit showed a two-part
large soft tissue mass (5 × 3 cm & 2 × 3 cm) that each
part was connected to the other. The first bigger part (5 ×
3 cm) was extended from lateral wall of the orbit to the
optic foramen and to the cranium, in order to lie between
the frontal and temporal lobes. This part pushed the glob
to the medial and optic nerve to lateral. The second part
was smaller in size (2 × 3 cm), extracranial, close to the
lacrimal bone, with infiltration to temporal and masseter
bone (Figure 2). MRI revealed the same T1 and T2 sig-
nal intensity with intermediate gadolinium enhancement,
without any obvious invasion or pathological enhance-
ment on brain parenchyma (Figure 3).
The tumoral cells on immunohistochemical staining
were positive for cytokeratin (CK) (Figure 4), and nega-
tive for Leucocyte-Common-Antigen (LCA) (Figure 5),
that were consistent with invasive, poorly differentiated,
non-keratinizing Squamous cell carcinoma. The patient
referred to an oncology center for receiving radiotherapy.
2. Discussion
Although nasopharyngeal carcinoma (NPC) commonly
metastasizes to cervical lymph nodes, orbital metastasis
Figure 1. CT scan shows an orbital soft tissue mass, causing
proptosis in a 45 years old man.
Copyright © 2012 SciRes. OJOph
A Rapidly Progressive Proptosis, a Case Report
Figure 2. MRI shows a two-part large soft tissue mass that
are connected togethe r.
Figure 3. T2 imaging shows intermediate gadolinium en-
hancement, without obvious invasion on brain parenchyma.
are rare. Most cases of orbital involvement are through
direct invasion typically via the cavernous sinus into the
apex causing proptosis and m uscle paralysis [2].
The mean age at time of presentation is 55 years. The
primary tumor is most often a carcinoma involving the
breast (40%), lung (11%), or prostate (8%), followed by
nasopharyngeal carcinoma. The most common manifes-
tations of orbital metastases are diplopia, exophthalmos,
inflammation, decreased visual acuity, pain, chemosis,
and eyelid swelling, depending on the site affected. The
symptoms usually occur acutely and progress rapidly,
over the course of weeks to months [3,4].
Figure 4. The tumoral cells on immunohistochemical stain-
ing were positive for cytokeratin (CK).
Figure 5. The tumoral cells on immunohistochemical stain-
ing were negative for Leucocy te-Common-Antigen (LCA).
The World Health Organization (WHO) has classified
NPC into 3 categories:
WHO-1 is defined as well-to-moderately differenti-
ated squamous or transitional cell carcinoma with
keratin production.
WHO-2 is nonkeratinizing carcinoma.
WHO-3 is undifferentiated carcinoma.
Most cases of NPC seen in Southeast Asia belong to
WHO-3 (undifferentiated carcinoma). In southern China,
the most common primary cancer that metastasizes to the
orbit is nasopharyngeal carcinoma (30.34%), followed by
lung cancer and liver cancer [5]. There is no exact infor-
mation about the most commo n type of NPC in Syria.
Although NPC commonly metastasizes to cervical
lymph nodes, orbital metastases are rare. Most cases of
orbital involvement are through direct invasion typically
Copyright © 2012 SciRes. OJOph
A Rapidly Progressive Proptosis, a Case Report
Copyright © 2012 SciRes. OJOph
via the cavernous sinus into the apex causing proptosis
and muscle paralysis. [2] less commonly, the direct
spread may be via the nasolacrimal duct and in such
cases epiphora is common [6].
The relationship between NPC and Epstein-Barr virus
was first observed in 1966 , when the sera of patients with
the cancer were found to show precipitating antibodies
against cells infected with the virus [7]. Later studies
revealed elevated levels of IgG and IgA antibodies di-
rected against components of Epstein-Barr virus in pa-
tients with NPC [8]. The high antibody titres to EBV
antigens in NPC patients can serve as a marker for the
diagnosis of NPC especially if the patients have signs of
NPC but no NPC is seen during endoscopic examination.
However, elevated EBV titers may also be associated
with other disease entities, such as sinonasal undifferen-
tiated carcinoma (SNUC), sinonasal lymphoma, and
tongue cancer.(MAL) Circulating EBV-DNA has been
shown to improve prognostication and monitoring of
NPC patients [9] .
Orbital metastases, even with newer diagnostic tech-
niques can be difficult to diagnose. [10] in a study,
showed that either CT or MRI provide essential info rma-
tion in documen ting orbital invasion and de termining the
pathway of tumor spre ad [11].
An immunohistochemical panel of poorly differenti-
ated orbital metastases is helpful in the identification of
the primary tumor site. In a study, Immunohistochemis-
try was performed to detect cytokeratin (CK) 7, CK 20,
thyroid transcription factor-1 (TTF-1), BRST1, BRST2,
carcinoembryonic antigen (CEA) and prostate-specific
antigen (PSA) in seven cases of poorly differentiated
orbital metastases. And the association of seven markers
with the patient’s clinical histor y allowed for the positive
identification of the primary tumor in the majority of
these cases [12].
The aim in treating orbital metastases is to relieve dis-
comfort. Radiotherapy is the mainstay treatment for the
disease [9]. Surgical removal of the mass is not recom-
mended. Prognosis is poor, because these patients are
usually at an advanced stage of the disease. In one report,
the median survival time was 1.3 years, and the two-year
survival rate is 27% [13].
3. Conclusions
Although NPC, rarely metastases to the orbit, but should
be considered in every rapidly progressing proptosis,
especially in elderly.
Imaging and immunohistochemichal panel can be
helpful in differentiating and rule out of other diagnoses.
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