ARTICLE
Auteur(s) :, Manabu HAMADA1,*, Hiromaro
KIRYU2, Tomoko OHTA3, Masutaka
FURUE1
1Department of Dermatology, Graduate School of
Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku,
Fukuoka, Japan
2Division of Dermatology, Kitakyushu Municipal Medical
Center, Kitakyushu, Japan
3Ohta Clinic of Dermatology, Kitakyushu, Japan
*Manabu Hamada. Fax: (+81)-92-642-5600. E-mail:
moemio@dermatol.med.kyushu-u.ac.jp
accepté le 1 Mars 2004
Cases of cutaneous cysts lined by ciliated epithelium are rarely
documented. Only a few cases of such cysts in the vulvar vestibulae
have been reported [1-6]. The origin of the cysts is controversial
because the location is so anatomically specific. In this report,
we describe an 11-year-old girl with a ciliated cyst of the vulva
and discuss the entity of ciliated epithelium.
CASE REPORT
An 11-year-old Japanese girl with a three-week history of a lump in
the vulva was transferred to the Dermatologic Clinic of Kitakyushu
Municipal Medical Center for further investigation and treatment.
The 1.1 cm in diameter lump was tender and located on the
superior portion of the right labium minus (( Fig. 1 )). It had a
smooth surface and was pink in color. Aspiration of the lump
yielded clear fluid indicating its cystic contour. The patient had
a history of atopic dermatitis that had been treated with topical
corticosteroids. The laboratory investigations showed no abnormal
hematologic results.
Under local anesthesia, the patient underwent a complete
resection of the lump. The specimen was a submucosal dilated cyst
that was lined by a single layer of cuboidal to columnar epithelium
with ciliated cells, pseudostratified columnar cells and papillary
projections, resembling müllerian epithelium (Fig. 2, 3). No
smooth muscle was identified in the supporting tissues beneath the
lining epithelium. The stroma around the cyst was devoid of cells
with the characteristics of endometrial tissue. The epithelium was
positive for Periodic acid-Schiff (PAS) stain even after diastase
digestion. However, alcian blue staining (pH2.5) failed to reveal
the presence of mucin in the epithelial cells.
Immunohistochemically, the lining epithelium was positive for
epithelial membrane antigen (EMA) and negative for S-100 protein,
carcinoembryoantigen (CEA), and estrogen receptor (ER). The patient
was diagnosed as having a ciliated cyst of the vulva.
Discussion
Cysts are only rarely found in vulvar vestibulae, especially those
lined with nonsquamous epithelium. Embryologically, müllerian ducts
give rise to the fallopian tubes, uterus, uterine cervix, and the
upper portion of the vagina. The lower two-thirds of the vagina is
derived from the urogenital sinus. The nonkeratinized squamous
epithelium of the vestibulae merges with the transitional
epithelium of the urethral meatus, and the orifices of the
paraurethral glands, Bartholin’s glands, and the minor vestibular
glands. Bartholin’s gland is usually lined with mucous-secreting
cells. Robboy reported that Bartholin’s glands lined by mucinous or
ciliated epithelium are normal constituents of the vulvar
vestibulae and are of urogenital sinus origin [2], whereas the
cervix is of müllerian derivation. Therefore it may be difficult to
make a correct diagnosis of the origin in the vulvar cysts to the
nonsquamous epithelium. Kurban has written a clinicopathological
review of a variety of cutaneous cysts with a nonsquamous
epithelial lining [7].
Histopathologically, ciliated cyst of the vulva is a rare
anomaly that is lined by columnar epithelium resembling müllerian
epithelium [8]. Paramesonephric mucinous cysts of the vulva have
been previously reported [5]. The lining of the cyst is a single
layer of columnar epithelium containing abundant mucin in the
cytoplasm. Ciliated cells are occasionally present in the lining
epithelium. Recently, both ciliated cyst of the vulva and
paramesonephric cyst have come to be regarded as the same entity
[8].
The cutaneous ciliated cyst is usually found on the lower
extremities in young women [9]. The cyst wall is composed of
columnar ciliated epithelium with occasional papillary projections.
No mucin secretion is observed. The cyst is hypothesized to be an
estrogen dependent müllerian heterotopia during embryogenesis.
Cutaneous ciliated cyst is observed also in male patients, derived
from müllerian remnants in the male [10, 11]. Leonforte reported a
case of cutaneous ciliated cystadenoma with columnar ciliated cells
and apical caps in the heel of a male that was thought to have
originated as a sweat gland metaplasia [10]. Kang also described a
case of ciliated cyst occurring in the labium minus, with a lining
of cuboidal cells, ciliated cells, apical caps and decapitation
secretion. However, its apocrine origin seemed less likely after
immunohistochemical study [1]. The mesonephric-like cyst, or
Wolfian-like duct cyst that is usually seen on the lateral sites of
the vulva, contains a clear fluid and is lined by thin-walled
cuboidal to columnar epithelium with mural smooth muscle in the
submucosa [12]. The lining epithelium lacks both cilia and mucin.
The Bartholin gland cyst occurs near the opening of the ducts of
Bartholin’s glands in the vulvar vestibulae [12] and is lined by
mucin-secreting cells and squamous to transitional cells. The
pathogenesis of the cyst is thought to be associated with occlusion
of the duct, especially by chronic bacterial infection. A müllerian
cyst occurs anywhere within the vagina [12]. The lining epithelium
of the cyst consists of müllerian duct epithelium, including
mucinous endocervical, endometrial, and ciliated cells with
occasional columnar mucin-secreting cells. The cyst is thought to
be derived from islands of adenosis. Ciliated cysts have been also
reported within the vulvar vestibule in a woman with chronic
inflammation of the vestibule associated with Stevens-Johnson
syndrome [3], as well as in a woman with a history of extensive
laser or topical 5-FU therapy of the vagina [4]. The cyst is
described as a ciliated cyst of the vulvar vestibule [13] and
Sedlacek has suggested that the müllerian epithelium is not
necessary for the development of ectopic glandular epithelium.
Cases of either ciliated cysts of the vulva or paramesonephric
cysts are reported in the 25-35 year-old age group of young women
and are occasionally associated with pregnancy or exogenous
progesterone. Clinically, the cyst that contains fluid occurs on
the superior portion of labium minus and measures 1.0 to
3.0 cm. No cases of either multifocal lesions or recurrences
have been reported. The excision of the lesion should be curative.
The etiology is unknown. However, heterotopia (sequestration and
migration of müllerian tissue during embryogenesis), dysontogenesis
(defective embryonic development), prosoplasia (abnormal
development resulting in organization), and metaplasia have been
suggested as mechanisms for the origin of the cyst. Of these
hypotheses, müllerian heterotopia has been most commonly proposed
to be the basis of the similar histopathologic features of the
epithelium [5, 14]. Reports in the dermatology journals have been
limited to only one case of ciliated cyst of the vulva and three
cases of paramesonephric cysts. Sidoni reported a case of ciliated
cyst of the perineal skin in male [15]. Newland insisted that
mucinous cysts of the vulva are derived from the entodermally
derived urogenital sinus epithelium [6]. The differential diagnosis
between ciliated cyst of the vulva and cutaneous ciliated cyst
based on histology can be impossible and diagnosis was previously
made only by the location of the tumor. Epithelial cells in cases
of cutaneous ciliated cyst demonstrated nuclear staining for
estrogen receptor [16-18]. Our patient is younger than patients in
previous reports of ciliated cysts of the vulva. Although
ciliogenesis produced by administration of estrogen has been
reported in some animals [19], there were no signs of pregnancy, no
history of either estrogen or progesterone treatment, and no
evidence for ER by immunohistochemical analysis of tissue in our
case (Table I( Table I )). The
origin of the ciliated epithelium, as related to embryonic
müllerian remnants or urogenital sinus, is still unknown. However,
chronic inflammation of the vulvar vestibulae may contribute to the
pathogenesis of ciliated cyst of the vulva.
Table I Immunohistochemical analysis for ciliated
cysts
|
Cyst
|
EMA
|
S-100
|
CEA
|
ER
|
Reference
|
|
Cutaneous ciliated cyst
|
+
|
ND
|
—
|
+
|
1994, [16]
|
|
Cutaneous ciliated cyst
|
+
|
—
|
—
|
ND
|
1995, [11]
|
|
Cutaneous ciliated cyst
|
ND
|
ND
|
+
|
ND
|
1997, [15]
|
|
Cutaneous ciliated cyst
|
+
|
+ (<10%)
|
—
|
+ (<10%)
|
1999, [17]
|
|
Cutaneous ciliated cyst
|
+
|
—
|
—
|
+
|
2002, [18]
|
|
Ciliated cyst of the vulva
|
+
|
—
|
—
|
ND
|
1995, [1]
|
|
Ciliated cyst of the vulva (our case)
|
+
|
—
|
—
|
—
|
|
References
1 Kang , Kim , Choi Ciliated cyst of the vulva J Am Acad
Dermatol 32 1995 514-515
2 Robboy , Ross , Prat , Keh , et-al. Urogenital
sinus origin of mucinous and ciliated cysts of the vulva Obstet and
Gynecol 51 1977 347-351
3 Marquette , Su , Woodruff Introital adenosis associated
with Stevens-Johnson Syndrome Obstet and Gynecol 66 1985
143-145
4 Sedlacek , Riva , Magen , Mangan , et-al.
Vaginal and vulvar adenosis. An unsuspected side effect of
CO2 laser vaporization J Reprod Med 35 1990 995-1001
5 Hart Paramesonephric mucinous cysts of the vulva Am J
Obstet Gynecol 107 1970 1079-1084
6 Newland , Fusaro Mucinous cyst of the vulva Nebraska
Med J 76 1991 307-310
7 Kurban , Bhawan Cutaneous cysts lined by nonsquamous
epithelium Am J Dermatopathol 13 1991 509-517
8 Scott Cutaneous cysts and related lesions Barnhill ,
Crowson , Busam , Granter Textbook of Dermatology 1997
McGraw-Hill New York 489-
9 Farmer , Helwig Cutaneous ciliated cysts Arch Dermatol
114 1978 70-73
10 Leonforte Cutaneous ciliated cystadenoma in a man Arch
Dermatol 118 1982 1010-1012
11 Ashton Cutaneous ciliated cyst of the lower limb in a
male Histopathology 26 1995 467-469
12 Zaino Cysts 4th ednKurman Blaustein’s Pathology of the
Female Genital Tract 2001 Springer-Verlag New York 150-152
13 Wilkinson Cysts 4th ednKurman Blaustein’s Pathology of
the Female Genital Tract 2001 Springer-Verlag New York 65-66
14 True , Golitz Ciliated plantar cyst Arch Dermatol 116
1980 1066-1067
15 Sidoni , Bucciarelli Ciliated cyst of perineal skin Am
J Dermatopathol 19 1997 93-96
16 Sickel Cutaneous ciliated cyst of the scalp: A case
report with immunohistochemical evidence for estrogen and
progesterone receptors Am J Dermatopathol 16 1994 76-79
17 Yokozaki , Yanagawa , Harada , Tahara Cutaneous
ciliated cyst of the right lower leg Pathol Int 49 1999 354-357
18 Fontaine , Lau , Murray , Fraser , Wright Cutaneous
ciliated cyst of the abdominal wall Am J Dermatopathol 24 2002
63-66
19 Anderson , Hein Estrogen dependent ciliogenesis in the
chick oviduct Cell Tiss Res 171 1976 459-466
|
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