Advances in Oral and Maxillofacial Surgery 8 (2022) 100329

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Advances in Oral and Maxillofacial Surgery

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Case report

Extraglandular orbital adenoid cystic carcinoma

Sami Elhamid a, *, Bahaa Razem a, Faiçal Slimani a, b
a
Oral and Maxillofacial Surgery Department, CHUN Ibn Rochd: Centre Hospitalier Universitaire Ibn Rochd, Casablanca, Morocco
b
Hassan II University of Casablanca, Casablanca, Morocco

A R T I C L E I N F O A B S T R A C T

Keywords: Adenoid Cystic Carcinoma (ACC) is an uncommon malignancy and it’s orbital primary presentation with no
Orbital glandular involvement is exceedingly rare. However, ACC must be considered in the diagnosis of an orbital
Adenoid cystic carcinoma tumor, since complications such as intracranial spread and recurrence are often described. We present a case of
Follow-up
primary orbital ACC treated with surgical excision and the outcome of a long term follow-up in the light of a
Primary
litterature review.

1. Introduction seven years follow up showed no signs of local recurrence or systemic

Adenoid Cystic Carcinoma (ACC) is an uncommon malignancy, ac­
counting for 1–2% of all head and neck cancers and 10% of all salivary
neoplasms [1]. It is a slow growing, locally invasive tumor of epithelial
origin associated with a high risk of recurrence [2]. Its occurrence in the
orbit is often secondarily from the sinus, nasopharynx, or lacrimal gland
[1]. Adenoid cystic carcinoma of the orbit without an alternative iden­
tifiable primary source is exceedingly rare; we hence present a case of a
primary adenoid cystic carcinoma of the inferior rectus muscle treated
with simple excision.
2. Case report
A 34 years old female with no significant medical or surgical back­
ground who presented to the oral and maxillofacial department for a left
exophtalmia beginning 15 months prior to the admission.
On the admission, the exam revealed a left exophtalmia with partial
ophtalmoplegia and inferior eyelid ectropion. No decreased visual
acuity, pain or hypoaesthesia was reported by the patient.
Because of MRI unavailability at the admission, a CT scan was per­
formed showing a 1.9 × 0.8 cm mass of the left inferior rectus muscle
with no apparent eyeball invasion (Fig. 1).
The patient underwent an excisional biopsy and the resected spec­
imen was sent for histopathological diagnosis. The microscopical ex­
amination revealed a tubular form of adenoid cystic carcinoma with
clear margins.
The patient refused to undergo exenteration and adjuvant radio­
therapy, and was discharged in good health at day 3 postoperative. The
spread, with a persistent ophtalmoplegia, superior eyelid ptosis, inferior
eyelid ectropion, but no loss of visual acuity (Fig. 2).
3. Discussion
“Wolf in sheep’s clothing” is often used describing ACC, as the term it
relates to its slow but relentless growth and dissemination. The tumor
shows a female predilection with a peak of incidence in the fifth and
sixth decades of life, ranging from 10 to 96 years [3]. It most commonly
arises within major and minor salivary glands of the oral cavity (50%
and 35%, respectively) [4], the hard palate being the most common site
of origin [5]. Extraoral occurrences are extremely rare and often due to
locoregional or distant dissemination, other sites including the external
ear, pharyngeal spaces, trachea, breast, skin, and lower female genital
tract have been reported [6].
ACC is the most common malignant tumor of the lacrimal gland [1].
However, orbital occurrence with no lacrimal gland involvement is
excessively rare.
Such cases in the literature are best explained by an occult tumor
existing within the lacrimal gland, or from another region in the head
and neck spreading to the involved region via perineural extension, or
by hematogenous spreading occurring from an unidentified distant site
[2,7]. However, after 7 years of follow-up, no alternative local or distant
source was identified.
Ectopic lacrimal gland tissue can occur in any location of the ocular
adnexa such as conjunctiva, orbit, or even in an intraocular region [8,9].
For that reason, orbital ectopic lacrimal gland tissue has been proposed
to give rise to benign cysts, inflammatory reactions, and

* Corresponding author.
E-mail address: elhamidsami91@gmail.com (S. Elhamid).

https://doi.org/10.1016/j.adoms.2022.100329
Received 16 July 2022; Accepted 18 July 2022
Available online 21 July 2022
2667-1476/© 2022 The Authors. Published by Elsevier Ltd on behalf of British Association of Oral and Maxillofacial Surgeons. This is an open access article under
the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
S. Elhamid et al.
Fig. 1. CT scan showing the left inferior rectus muscle mass.
Fig. 2. Year 7 follow-up picture of the patient showing no exophtalmia, but a
persistent ophtalmoplegia, superior eyelid ptosis, inferior eyelid ectropion.
adenocarcinoma within the orbit. Green and Zimmerman, described a
case with microscopic focus of adenocarcinoma discovered on post­
mortem examination in inflamed ectopic lacrimal gland tissue [4].
ACC typically presents as a slowly enlarging mass in the head and
neck. Patients may remain asymptomatic until the tumor has invaded
local structures causing varying symptoms depending on the location
[6]. Consequently, exophtalmia is the most common presentation in
orbital ACC. Other signs include diplopia and decreased visual acuity.
Pain and hypoesthesia in the region of nerve distribution are the most
common clinical manifestations of early neural infiltration, causing
periocular pain or ptosis [2,8,10].
Magnetic resonance imaging is the modality of choice for exploring
soft tissues involvement, specifically on cases involving the orbit.
However, the relatively difficult access to this type of imaging made us
rely on CT scan only.
Accurate histopathological finding are necessary to assess the diag­
nosis of ACC. Three morphological patterns: cribriform, tubular, and
solid are described [3]. The most common and characteristic is the
cribriform comprising 44% of all lesions, while the solid pattern,
considered a high-grade lesion with a reported recurrence of up to
100%, accounts for 21% of the lesions [5].
Because of the anatomic characteristic, this kind of ACC is easily
intracranial spread. Aggressive treatment is required including orbital
exenteration with ostectomy, and usually adjuvant radiation therapy to
eradicate potential residual cancer cells [11,12].
Plaque radiotherapy has also been suggested as an alternative to
exenteration and external radiation in certain patients [12].
ACC is one of the most aggressive salivary gland tumors. While 5 year
survival rates are favorable (64–91%), long term survival is considered
poor, with 10 year and 20 years survival of 37–65% and 32–40%
respectively [13,14]. Distant metastasis occurs in 20–64%, and it is a
leading cause of reduced survival in patients with AC. It most often
occurs in the lung. No consensus exists, however, on the type or fre­
quency of chest imaging that is needed [14–16]. ACC is known to recur
at both local and distant sites years later, 53% of patients develop a local
recurrence at the primary site 63 months after treatment, this rate in­
creases to 83% at 20 years, and up to 100% at 30 years after further
extrapolation [15]. Cervical lymph node metastasis is less common
(6–23%) [17]. Perineural invasion, increasing stage, and solid growth
pattern have been found to be markers of poor prognosis [18]. One study
found the mean 5-year survival rate to be 78.5% [15].
2
Advances in Oral and Maxillofacial Surgery 8 (2022) 100329
4. Conclusion
This case report highlights the fact that ACC can rarely occur as a
primary tumor of the orbit in the absence of lacrimal gland involvement.
As such, the lack of gross or microscopic involvement of the lacrimal
gland does not exclude a diagnosis of ACC in the evaluation of an orbital
mass lesion. But given the aggressive nature of this tumor with pro­
pensity for intracranial spread, and late distant metastasis and recur­
rence, one must maintain a high index of suspicion for ACC when
evaluating orbital tumors.
Consent for publication
Written informed consent was obtained from the patient for publi­
cation of this case report and any accompanying images. A copy of the
written consent is available for review by the Editor-in-Chief of this
journal.
Declaration of competing interest
The authors declare that they have no known competing financial
interests or personal relationships that could have appeared to influence
the work reported in this paper.
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