An unusual case of retrobuibar orbital apocrine hidrocystoma

Acta M ed Kinki Univ
Vol.39, No.2 97-99, 2014
An unusual case of retrobuibar orbital apocrine
hidrocystoma
Narihiko Hirano, Shinichi Asamura, Noritaka, Isogai
Department of Plastic and Reconstructive Surgery, Kinki University Faculty of Medicine,
Osakasayama, Osaka 589-8511, Japan
Abstract
A 61-year-old woman was referred to our
department from an eye clinic with a 3 year
history of progressive proptosis of the right eye.
M agnetic resonance imaging revealed a mass
with a cystic appearance located in the retrobulbar orbital region. Histological features of the
excised cystic tumor were characteristic of
apocrine hidrocystoma (AH). It is thought to
arise from a cystic dilatation of the sweat glands
contiguous with the skin s surface. In the
Introduction
Apocrine hidrocystoma (AH) is a type of
cystic tumor that is typicallysmall and commonly located in the head and neck region, particularly in the periorbital region.
Pathohistologically,AH usuallyoriginates from a dilated
sweat gland. In this report, we describe the
clinical features of a relatively unusual case of
AH presenting in the retrobulbar orbital region,
and thecourseoftreatment. Wethen discuss our
findings with reference to previous research,and
present our hypothesis regarding the origin of
AH.
Case report
A 61-year-old woman was referred to our
department from an eye clinic with a 3 year
history of progressive proptosis of the right eye
and decreasing visual acuity. Her Hertel measurements were 17 and 21 mm on the left and
right sides, respectively. The patients were normal about preoperative light reflex and eye
operative specimens, no epidermal elements
were observed near the cystic structure. The
absence of contiguous subcutaneous tissue was a
rather unusual phenomenon. Although rare,AH
should be considered in the differential diagnosis
of a cystic tumor presenting in the retrobulbar
orbital region.
Key words: Apocrine hidrocystoma, Sweat
gland, Orbit, Cyst
movement. Magnetic resonance imaging (MRI)
with gadolinium contrast revealed a mass with a
cystic appearance located anteriorly in the
medial intraconal space of the right orbit［Figure 1］.
Treatment was based on a swinging eyelid
approach.
Using a lateral rim removal
approach,osteotomywas performed with a bone
saw at a level just above the fronto-zygomatic
suture and above the zygomatic arch, followed
by advancement to deep areas using a bone
chisel and wood hammer. Fracture was then
induced at the zygomatic bone-sphenoid bone
suture area by holding and mobilizing the bone
fragment using bone forceps, which facilitated
wide visualization ofthe deep corner area. After
the globe had been gently removed laterally,the
tumor was found to be buried within the orbital
fatty tissue. With meticulous dissection of the
fatty tissue, the tip of the tumor was detected,
allowing it to be subsequently isolated and
resected. Macroscopically, the cystic tumor was
observed to have a thin wall and contain clear
fluid with a purplish tint［Figure 2］
. The piece
Received July 11, 2014; Accepted August 8, 2014
97
N.Hirano et al.
Fig.3
Fig.1
Preoperative MRI
A : Horizontal plane, B: Coronal plane
Histological features
The arrow shows apocrine snouts which are characteristic of AH.
the cyst wall was formed by myoepithelial cells
in which the long axes ran parallel to the cyst
wall. The cells of the inner layer were mildly
columnar with occasional apocrine snouts.
Papillaryprojectionsextended from thesecretory
layer into the cyst cavity, showing decapitation
secretion. These features are all characteristic of
AH［Figure 3］
.
Discussion
Fig.2
A swinging eyelid approach
The arrow shows AH is isolated and resected.
of the bone tissue previously removed from
Fronto-zygomatic region was replaced at the
original site.
Upon completion of the procedure, proptosis
was no longer noticeable. The patient experienced a transient postoperative diplopia in the
peripheral gaze that resolved within 2 months.
The postoperative visual acuity improved 0.3 to
0.7 on the right side within 2 weeks.
Microscopic examination revealed a fibrous
tissue wall lined by epithelium composed of
double cell layers but with areas of occasional
thickening. The outer layer of cells comprising
98
Hidrocystomas can be subdivided into eccrine
and apocrine types. Although the eccrine type
is more frequently associated with multiple
lesions, the apocrine type (i.e., AH) typically
presents as a solitary benign cystic tumor.
Attributed to adenomatous cystic proliferation
of the apocrine glands and characterized by
cylindrical epithelia,AH is generallythought to
arise from a cystic dilatation of the sweat glands
contiguous with the skin s surface. In the operative specimens described here, no epidermal
elements were observed near the cystic structure.
From the viewpoint of histogenesis, the absence
of contiguous subcutaneous tissue is a rather
unusual phenomenon.
AHs are benign cysts that arise from the apocrinesecretoryglands ofMoll and arecommonly
located in the head and neck region, Although
they have been observed in other areas of the
body. Similar lesions have been reported on
the outer surface of the eyelids, canthal areas,
and the orbital rim.
Shields emphasized that
AHs typically develop around the medial canthus of the eyelid, but rarely within the orbit.
The content of the orbital cavity is composed
An unusual case of retrobuibar orbital apocrine hidrocystoma
ofthe eyeball,extraocular muscles,nerves,blood
vessels, lacrimal glands, and orbital fat tissue.
The origins of their histogenesis are the neuroectoderm,surface ectoderm,and mesoderm that
exists between the surface and neuroectoderm.
The anterior portion of the ocular structures,
including the eyelids, corneal epithelium, and
lenses,is derived from the surface ectoderm. On
the other hand, the posterior portion of the
ocular structures, including the retina, optic
nerves, extraocular muscles, and orbital fat tissue, is derived from the neuroectoderm and
mesoderm. Regarding the histogenesis of the
present case of AH, it is extremely difficult to
clarifyits origins from an embryological perspective.
Although thepathogenesisofAH isnot entirely clear, Epidemiological research indicates that
these lesions often appear between 30 and 70
years ofage and then grow slowly. None ofthe
hypotheses regarding the etiology of the cyst
origin,such as a trauma or orbital bone remodeling during childhood,has been proven scientifically. We hypothesize that AH arises from
tissue originating from the surface ectoderm
during embryological development. While this
tissue evanesces on further ocular development
under normal circumstances,It doesnot evanesce
for known reasons under unusual circumstances,
providing the conditions for cyst from the embryonal remnant tissue many years later. We
cannot propose any other rational explanation
for the oncogenesis of AH.
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