Issue 1 • 1000905

Abstract

Purpose: To describe the squamous cell carcinoma in the orbit as a primary site through systematic review and case report. Method: A Systematic review of the literature was performed to focus on all the cases of primary squamous cell carcinoma that involves the orbit and exclude the cases that affect the ocular surface area and eye lid. Results: Primary Squamous Cell Carcinoma (PSCC) in the orbit is extremely rare. Only 10 cases are reported in the literature including 2 atypical cases involved the orbit after retinal surgery. PSCC in the orbit are presented by vague symptoms such as headaches, fascial pain, numbness, and diplopia. Also, no standard accepted treatment exists. PSCC in the orbit can involve any tissue included the extraocular muscle, the lacrimal gland and indistinct area in the orbit. Conclusion: Primary SCC at orbital site is very rare as only 10 cases are reported after systematic review of literatures and the common is secondary SCC through perineural invasion from a cutaneous lesion.

Full text

Volume 13 • Issue 1 • 1000905J Clin Exp Ophthalmol, Vol.13 Iss.1 No:1000905
OPEN ACCESS Freely available online
Journal of Clinical & Experimental
Ophthalmology Research Article
Correspondence to: Radwa El Sherif, Department of Medicine, Ain Shams University, Cairo, Egypt, Email: elshereifr@yahoo.com
Received: Accepted: Published:
Citation: El Samkary M, Eliwa T, El Sherif R (2022) Primary Squamous Cell Carcinoma, Orbital Site: A Case Study and Systematic Review. J Clin Exp
Ophthalmol. 13:905
Copyright: © 2022 El Samkary M, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
ABSTRACT
Purpose: To describe the squamous cell carcinoma in the orbit as a primary site through systematic review and case report.
Method: A Systematic review of the literature was performed to focus on all the cases of primary squamous cell carcinoma
that involves the orbit and exclude the cases that affect the ocular surface area and eye lid.
Results: Primary Squamous Cell Carcinoma (PSCC) in the orbit is extremely rare. Only 10 cases are reported in the literature
including 2 atypical cases involved the orbit after retinal surgery. PSCC in the orbit are presented by vague symptoms such as
headaches, fascial pain, numbness, and diplopia. Also, no standard accepted treatment exists. PSCC in the orbit can involve
any tissue included the extraocular muscle, the lacrimal gland and indistinct area in the orbit.
Conclusion: Primary SCC at orbital site is very rare as only 10 cases are reported after systematic review of literatures and the
common is secondary SCC through perineural invasion from a cutaneous lesion.
Keywords: Squamous cell carcinoma; Orbit; Orbital tumour; Primary; Lacrimal gland; Periorbital
Primary Squamous Cell Carcinoma, Orbital Site: A Case Study and
Systematic Review
Mahmoud El Samkary, Tamer Eliwa, Radwa El sherif*
Department of Medicine, Ain Shams University, Cairo, Egypt
INTRODUCTION
Myopia Squamous Cell Carcinoma (SCC) is an invasive epithelial
malignancy showing keratinocytic differentiation in the ocular
and periocular region; it can affect the conjunctiva, cornea, and
eyelid skin. It is a rare disease in the conjunctiva with an incidence
of 0.13 to 1.9 per 100,000 inhabitants, mainly affecting individuals
between 50 and 75 years of age [1]. While the incidence of eyelid
SCC has been reported to be between 0.09 and 2.42 cases per
100,000 population [2]. Squamous Cell Carcinoma (SCC) of
the orbit is almost uniformly the result of local invasion from a
cutaneous primary extension by perineural invasion, or the result
of metastasis; in our knowledge of histopathology, no squamous
epithelial cells in the orbit so primary squamous cell carcinoma
in the orbit is very rare. This highlights the rareness of primary
squamous cell carcinoma in orbital site cases.
LITERATURE REVIEW
Systematic reviews and meta-analysis guidelines were followed and
the research on PubMed, MEDLINE and Web of science were
conducted for all the articles that published from September
1991, the year of the first article had talked about the squamous
cell carcinoma in the orbit, to the last article published in January
2021.The research “squamous cell carcinoma” combined with
following terms “orbit”, “orbital”, “lacrimal gland”, “periorbital”,
“adnexal”, “conjunctiva”, “eyelid” and “ocular surface area”. All
articles and their references are viewed and analyzed. The case
studies and reports that documented squamous cell carcinoma in
the orbit as a primary lesion are included. Secondary squamous
cell carcinoma, conjunctiva, ocular surface, and eyelid squamous
cell carcinoma are excluded from the analysis after a complete
review. The articles with inclusion criteria were reviewed and
analyzed for: publication year, age, sex, laterality, presentation,
signs, orbital location, systemic disorder, treatment, and outcome.
Ethical Conduct for Research Involving Humans. Research
adhered to the tenents of the Declaration of Helsinki as amended
in 2008. Institutional Review Board approval was not required for
the case report as per the Tri-Council Policy Statement.
CASE REPORT
A 99-years old, Egyptian female patient, presented with an
enormous left eyelid mass extended with hall upper and lower lid,
of 2 years duration and complained of severe headache, frontal
January 04, 2022; January 18, 2022; January 24, 2022

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J Clin Exp Ophthalmol, Vol.13 Iss.1 No:1000905
numbness, and loss of vision. The history was recorded as the
patient and her relevant stated that the lesion began with puffiness
in the lateral site of eyelid with early proptotic eye since two years,
but the patient refused to do any investigation and neglected
the symptoms of 2 years until the numbness and the headaches
increased and the giant mass appeared so the surrounding people
started to be worried. On examination, A giant mass in the left
upper lid extended to lower lid, with skin eruption, vesicle and over
stretched on the mass, the eye globe can’t be assist, complete loss
of vision and also the ocular motility was lost completely (Figure
1). On investigation, the CT orbit performed and shows that the
mass from lacrimal gland extended to all orbital cavities, pushing
the eyeball down and invading it. Also the mass extended to the
whole upper and lower eyelids (Figure 2). Further investigations
such as MRI scan were difficult to carry as the general condition
of patient did not help. Our strategy to manage this patient was
to do excision biopsy with preserving the eye globe if is it healthy.
The patient and her relevant consent were taken to perform the
surgery. During the surgery, skin incision at site of anatomical
skin crease was firstly performed then dissection of the mass
until the eye globe was reached. However, we found that the eye
globe was completely invaded, and the extraocular muscle was
difficult to distinguish from the invading tissue so the decision
of complete orbital exenteration was decided. The large part of
skin was excised as well as a complete removal of orbital tissue,
periosteum, eye globe and extraocular muscle. The orbit irrigated
with broad spectrum antibiotic after fine cautery and followed
by closure of the upper and lower skin, total tarsorrhaphy,
completely (Figure 3). Postoperative, systemic antibiotic and
topical ointment dressed the wound. Unfortunately, the patient
cut the sutures and opened the eyelid after 3 weeks due to her
age which was not helpful; however the orbit was clear so the
ulcerated epidermal covering overlying infiltrating tumor tissue
formed groups of moderately pleomorphic malignant squamous
cells grade II. There is a focal necrosis. The tumor infiltrating the
eye globe and surrounding orbital tissue. The patient was referred
to the oncology department, full-body PET-CT surveillance was
performed, and detected that no lymph node involvement or
evidence of any metastasis in the body and the oncologist decided
that there is no need to perform systemic chemotherapy or
radiotherapy. 6 months have passed since the original diagnosis
and the patient is in good health.
RESULTS OF SYSTEMATIC REVIEW
Systematic reviews and meta-analysis on PubMed, MEDLINE,
and Web of Science databases, with reference searching and
google scholar, were performed and 479 non-duplicate results of
which 10 articles were included representing 10 patients (Figure 5).
Figure 1: Left orbital giant mass.
Figure 2: Axial CT orbit.
Figure 3: At the end of orbital exenteration, total tarsorrhaphy.
Figure : At the end of orbital exenteration, total tarsorrhaphy.
artificial prosthesis can be done (Figure 4). On histopathology,
4

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DISCUSSION
Both primary and secondary Squamous Cell Carcinoma
(SCC) of the orbit are rare entities, though cystic SCC is even
more, so it may provide a significant diagnostic conundrum to
oculoplastic surgeons [3].The systematic review in the literatures
detect that only 10 cases were reported with primary SCC
in orbital site [4-7] but only 2 reported cases were non-apical
primary SCC in the orbit [8,9]; it occurs after retinal surgery with
proposed transplanted conjunctival epithelium and subsequent
malignant transformation of a conjunctival cyst [10]. All special
manifestation about all the reported cases is mentioned in Table
1. The symptoms of numbness, intermittent pain, puffiness of lid
and mass in the orbit increase in size within 1-2 years duration are
similar to our reported case, the only differences are in the size
of mass and eye globe symptoms as in our reported case the huge
size is very characteristic to a degree that covers completely the eye
globe and obscured the all-eye globe signs. The occult cutaneous
lesion that producing perineural invasion and so secondary SCC
in the orbit are excluded by history as no right facial lesions and
none were seen on examination also by dermatology records,
dermatopathology reports and with the histopathology of tumor
reported no neural invasion. All cases reported were between
the age of 50-78 years old while the patient in our study is 99
years old which is not common nowadays so it may increase the
incidence of the unusual cancer [11].
Figure 5: Systematic reviews and meta-analysis.
Table 1: Literature data collection for case study.
Author/Year Age Sex Characteristic
symptoms Investigation Site of lesion Management
Ruff et al. 1985 53 F Left fascial pain CT face Inferotemporal
orbit
Gallium scan,
orbital radiation
Saha et al. 2011 56 F
Right forehead,
cheek numbness,
diplopia,
right eye pain,
blurry vision
MRI brain,
orbit sinus
(Initially negative),
repeat
MRI orbit a year
later
Orbital apex Primary orbital
radiation therapy
Peckinpaugh 2012
43 F A common
symptom: Diplopia
Full-body CT/PET
scan Orbital apex
Orbital
radiotherapy,
adjuvant
chemotherapy
63 M Ophthalmoplegia
Forehead numbness
67 M
Hromas and Sokol,
2014 43 M
Left decrease vision
limited ocular
motility
MRI orbital, lateral
orbitotomy with
excisional biopsy
Full body CT/PET
scan
Inferior interconal
space between
inferior rectus and
optic nerve
Left orbital
exenteration
adjuvant orbital
radiation

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J Clin Exp Ophthalmol, Vol.13 Iss.1 No:1000905
Choi et al. 2014 74 F
Left upper eyelid
swelling,
eye pain,
double vision,
left forehead,
cheek numbness
MRI orbit Superomedial obit
Left orbital
exenteration
adjuvant orbital
radiation
Arbulu et al. 2017 73 F
Headache,
right periorbital
pain,
parenthesis,
right ptosis,
diplopia
CT orbit
MRI orbit
Incisional biopsy
Full body CT/PET
scan
Superior orbit
abutting
Craniofacial
resection with
orbital exenteration
with adjuvant
orbital radiation
Blandford et al.
2018 63 M
Right forehead,
numbness,
right ptosis,
diplopia
MRI brain
MRI orbit
Incisional biopsy
Full body CT/PET
Superomedial orbit
Right orbitotomy
with excisional
biopsy adjuvant
chemotherapy
Orbital radiation
Zho et al. 2021 78 F Right painless mass,
Puffiness eye lid CT orbit Superolateral orbit
Removal of lacrimal
gland bloc with
adjuvant orbital
radiation
Elsamkary 2022
(our study) 99 F
Left puffiness
eyelid,
giant lid mass,
facial numbness
CT orbit/PET scan Superolateral orbit Left orbital
exenteration
As the primary SCC in site the orbit is extremely rare, the ideal
strategy for the management were unclear, in the reported
cases, the treatment ranged from primary orbital radiation and
observation to orbital exenteration with adjuvant chemotherapy
and orbital radiation. The incision biopsy of tissue with frozen
section is performed if SCC is detected and then the decision
of complete excision even exenteration with adjuvant chemo-
radiation therapy after taking the consent of the patient and
explaining the condition to the patient and his relevant is the
ideal strategy. So in our case, the decision of complete excision
with exenteration after explaining the condition and taking
consent from the patient and her relevant for performing the
surgery with photographic and video recording the operation.
The drawback, in our case that the general condition of the
patient didn’t help to perform an MRI investigation preoperative,
as done in other reports where repeated radiological MRI were
carried to help in following up the lesion, and to explain the
radiological appearance of the lesion.
CONCLUSION
Our case may aid to the idea of how to deal with a very rare
presentation of an enormous primary SCC in site the orbit
with this age group and focused on systemic review about such
cases. Primary SCC at orbital site is very rare as only 10 cases are
reported after systematic review of literatures and the common
is secondary SCC through perineural invasion from a cutaneous
lesion.
DECLARATIONS
Patient consent
The patient(s) described herein consented to publication of the
case in writing.
Disclosure statement
None of the authors have a proprietary interest in this study or
any conflicts of interest to disclose and our study aren’t supported
by any source of funding.
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