A ciliated cyst in the posterior mediastinum compatible with a paravertebral

Mullerian cyst
Adrian P. Businger, Harald Frick, Martin Sailer and Markus Furrer
Eur J Cardiothorac Surg 2008;33:133-136
DOI: 10.1016/j.ejcts.2007.09.036

This information is current as of February 23, 2011

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://ejcts.ctsnetjournals.org/cgi/content/full/33/1/133

The European Journal of Cardio-thoracic Surgery is the official Journal of the European Association
for Cardio-thoracic Surgery and the European Society of Thoracic Surgeons. Copyright © 2008 by
European Association for Cardio-Thoracic Surgery. Published by Elsevier. All rights reserved. Print
ISSN: 1010-7940.

Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011

 European Journal of Cardio-thoracic Surgery 33 (2008) 133—136
www.elsevier.com/locate/ejcts

Case report

A ciliated cyst in the posterior mediastinum compatible

with a paravertebral Mullerian cyst
Adrian P. Businger a, Harald Frick b, Martin Sailer a, Markus Furrer a,*
a
Department of Surgery, Thoracic and Vascular Surgical Unit, Kantonsspital Graubunden, Loestrasse 170, CH-7000 Chur, Switzerland
b
Department of Pathology, Kantonsspital Graubunden, Loestrasse 170, CH-7000 Chur, Switzerland

Received 11 June 2007; received in revised form 5 September 2007; accepted 27 September 2007; Available online 31 October 2007

Abstract
Several types of cysts in the mediastinum have been described, such as bronchogenic or thymic cysts and esophageal duplications, most of
which arise in the middle or posterior mediastinum close to the tracheobronchial system or the esophagus. We present herein the rare case of a
ciliated cyst anatomically distant to the genitourinary organs. An abnormal formation in the posterior mediastinum was incidentally detected on
chest X-ray during the preoperative evaluation for a herniorrhaphy in a 54-year-old woman who had a negative medical history. The patient
exhibited no clinical signs or symptoms associated with the mass. MRI revealed hypointense signals in T1-weighted scans and homogeneous
hyperintense signals in T2-weighted scans. The lesion was resected thoracoscopically and histologic and immunohistochemical stainings showed a
ciliated cyst of probable Mullerian origin. Because of their uncertain biological behavior, cystic lesions in the posterior mediastinum should be
removed surgically to allow definitive histologic diagnosis.
# 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Keywords: Mediastinal cyst; Biological behavior; Mullerian cyst

1. Introduction findings and laboratory tests were normal. Chest MRI

Mediastinal cysts have a broad range of etiologies, such as
bronchogenic, thymic, and neuroenteric cysts, or esophageal
duplication [1]. Neuroenteric cysts may develop through
abnormal separation of the embryonic germ cell layer [2]. In
2005, Hattori [3] described the first case of a ciliated cyst of
probable Mullerian origin arising in the mediastinum, which is
a very rare entity. Herein we report a patient with a ciliated
cyst in the posterior mediastinum.
2. Case presentation
A 54-year-old, non-smoking woman under hormone
replacement therapy with estrogen and gestagene (Trise-
quens) was found to have an abnormal shadow on chest X-ray
(Fig. 1a and b) obtained during a preoperative medical
examination for an inguinal herniorrhaphy in 2006. The
patient did not complain of respiratory problems or fevers.
No anatomic abnormalities were detected. She had no history
of weight loss, persistent cough, inhalation trauma, or
overexposure to ambient or industrial agents. Physical
* Corresponding author. Tel.: +41 81 256 62 21; fax: +41 81 256 62 79.
E-mail address: markus.furrer@ksgr.ch (M. Furrer).
1010-7940/$ — see front matter # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
doi:10.1016/j.ejcts.2007.09.036
demonstrated a lesion, 28 mm  40 mm  45 mm in size,
located in the posterior mediastinum, dorsal to the aorta.
There was no direct communication between the lesion and
the spinal cord, the tracheobronchial system, or the
esophagus. The T2-weighted MRI scans showed a homo-
geneous hyperintense lesion (Fig. 1c); the T1-weighted scans
showed a hypointense lesion (Fig. 1d). The left upper lobe
was slightly displaced by the lesion. Of note, the lesion with
the same dimensions existed on a chest X-ray that had been
obtained in 1999. Surgical removal of the cyst was performed
thoracoscopically, consistent with the radiologic diagnosis of
a neuroenteric cyst. During the operation, the cyst was
opened and an opaque, yellowish fluid was evacuated. The
cyst had an extraordinarily thin wall with a smooth interior,
and an exterior that was white and pale brown in color. The
cyst was in close proximity to the vagus nerve.
Histologic examination of the specimen revealed a thin-
walled cyst lined by regular cuboidal or cylindrical ciliated
epithelium without cellular atypia (Fig. 2a—c). Adjacent to the
epithelium, there was an almost uninterrupted layer of smooth
muscle, some slightly ectatic capillaries, fibrolipomatous
stroma, and small peripheral nerves (Fig. 2c). The van Gieson
stain (Fig. 2d) highlighted the smooth muscle layer, as did
immunohistochemical staining with actin (Fig. 2e). The
outside of the cyst was coated with a calretinin-positive
mesothelium. Neither cartilage nor glands were detected.

Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011

134 A.P. Businger et al. / European Journal of Cardio-thoracic Surgery 33 (2008) 133—136

Fig. 1. (a and b) Chest X-ray shows an abnormal shadow. (c) T2-weighted scans reveal hyperintense signals with (*) slight suppression of the vertebral body. (d) T1-
weighted scans showed a hypointense lesion in the posterior medistinum (coronal view).

Additional immunohistochemical stainings were per-
formed. As shown in Fig. 2f, the inner luminal epithelial
layer showed a strong positive reaction against cytokeratin 7
(mouse K72; Cell Marque), the oncoprotein bcl-2 (mouse bcl-
2/100/D5; Novocastra), and focal positivity against human
epithelial antigen (mouse BerEp-4; Cell Marque). There was
nearly 100% nuclear positivity for both estrogen receptor-
alpha (ER; mouse 6F11; Novocastra) and progesterone
receptor (PR; mouse PGR 312; Novocastra). None of the
sections exhibited a positive reaction against cytokeratin 20
(mouse KS 20.8; Novocastra), the lung epithelial marker TTF1
(mouse 8G7 G3/1; Cell Marque), or the intestinal transcrip-
tion factor CDX2 (mouse AMT 28; Novocastra). Based on Ki67
staining (mouse MM1; Novocastra), the proliferation rate of
the cyst was estimated to be below 1%; p53 (mouse DO-7;
Novocastra) showed weak nuclear positivity in about 30% of

Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011

 A.P. Businger et al. / European Journal of Cardio-thoracic Surgery 33 (2008) 133—136 135

Fig. 2. (a and b) The cyst epithelium showed strong nuclear positivity against estrogen receptor alpha, as well as against the progesterone receptor. The nuclei of the
stromal cells, especially the smooth muscle cells, were completely negative for both ER and PR (ER alpha and PR; original magnification 200). (c) The cyst almost had
an extraordinarily thin wall with an almost completely circumferential smooth muscle layer (short arrows). With a higher magnification (insert HE; original
magnification 630) the ciliated epithelium is shown with regular round-to-oval nuclei without atypia and homogeneous cytoplasm of the cells. Focal formation of
short pseudo-papillae is evident (long arrow) (H&E; original magnification  200). (d) The smooth muscle layer is much more obvious and highlighted yellow with a van
Gieson stain; as well as fibrous tissue stains red. Occasionally, the muscle layer is accentuated (arrow) with slight atrophy of the overlaying cuboidal epithelium
(vG; original magnification 200). (e) Immunohistochemistry underlines the circumferential smooth muscle layer; the walls of the small vessels (short arrow) stain
positive as an internal positive control (Actin; original magnification 200). (f) The luminal cyst epithelium was also strongly positive for cytokeratin 7 (CK7; original
magnification 400).

Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011

136 A.P. Businger et al. / European Journal of Cardio-thoracic Surgery 33 (2008) 133—136
the epithelial cells. All incubations were performed on an
automated Bond maX (Vision Biosystems, Novocastra) using
ready-to-use pre-diluted antibodies. Together, these findings
were compatible with a paravertebral cyst of probable
Mullerian origin [4].
Postoperatively, the patient recovered well and was
discharged from the hospital after 3 days and resumed her
regular professional life after 2 weeks.
3. Comment
Mullerian cysts anatomically distant to the genitourinary
organs or the pelvis are extremely rare disease entities
[5—7]. Some authors contend that mediastinal paraverteb-
ral Mullerian cysts are more frequent than generally
assumed and propose the establishment of mediastinal
paravertebral cysts as an entity [4]. In 2005, Hattori [3]
described the first case of a ciliated cyst of probable
Mullerian origin arising in the mediastinum. The probable
mechanism giving rise to Mullerian cysts in the mediastinum
has not been elucidated. It is assumed that Mullerian cysts
in the mediastinum arise during embryonic development
vis-a-vis dispersed Mullerian epithelia or by metaplasia.
Both radiologically and intraoperatively, the lesion in our
case was diagnosed as a (neuro-) enterogenous cyst, or an
esophagus duplication of a foregut cyst [8]. The expression
of ER and PR and the histologic similarity to fallopian tubes
with the ciliated cuboidal epithelium followed by a smooth-
muscular layer revealed the Mullerian origin. Both enter-
ogenous and bronchogenic cysts are lined by ciliated
columnar epithelium with abutting smooth muscle. In
contrast, the wall of bronchogenic cysts contains cartilage
and glands and the wall of enterogenous cysts contains
gastrointestinal tissue. It seems that mediastinal cysts are
often misdiagnosed preoperatively as bronchogenic or
neurogenic lesions, but when bronchogenic cysts are
diagnosed based on the presence of cartilage and the
hormone receptors are specified, this could be avoided.
There are no systematic immunohistochemical studies
pertaining to misdiagnosed Mullerian cysts.
Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011
4. Conclusion
The preoperative differentiation of lesions in the posterior
mediastinum is challenging and asymptomatic cystic lesions in
this location are often only detected incidentally. Since
asymptomatic patients with mediastinal cysts may later
develop malignant transformation and since the clinical course
is highly unpredictable [1,9,10], we suggest resection of any
lesion within the posterior mediastinum; a definitive histologic
diagnosis can only be established following surgical removal.
Acknowledgment
The authors would like to acknowledge the effort put into
this report by Thomas Boehm, MD and for providing the MRI
pictures.
References
[1] Takeda S, Miyoshi S, Minami M, Ohta M, Masaoka A, Matsuda H. Clinical
spectrum of mediastinal cysts. Chest 2003;124:125—32.
[2] Sirivella S, Ford WB, Zikria EA, Miller WH, Samadani SR, Sullivan ME.
Foregut cysts of the medistinum. Results in 20 consecutive surgically
treated cases. J Thorac Cardiovasc Surg 1985;90:776—82.
[3] Hattori H. Ciliated cyst of probable mullerian origin arising in the poster-
ior mediastinum. Virchows Arch 2005;446:82—4.
[4] Hattori H. High prevalence of estrogen and progesterone receptor expres-
sion in mediastinal cysts situated in the posterior mediastinum. Chest
2005;128:3388—90.
[5] Farmer ER, Helwig EB. Cutaneous siliated cysts. Arch Dermatol 1978;114:
70—3.
[6] Konishi E, Nakashima Y, Iwasaki T. Immunohistochemical analysis of
retroperitoneal Mullerian cyst. Hum Pathol 2003;34:194—8.
[7] Chong SJ, Kim SY, Kim HS, Kim GM, Kim SY, Jung JH. Cutaneous ciliated
cyst in a 16-year-old girl. J Am Acad Dermatol 2007;56:159—60.
[8] Nobuhara KK, Gorski YC, La Quaglia MP, Shamberger RC. Bronchogenic
cysts and esophageal duplications: common origins and treatment. J
Pediatr Surg 1997;32:1408—13.
[9] Kouerinis IA, Zografos GC, Exarchos DN, Silimingas NT, Argiriou ME,
Manoussaridis JT, Misiakos EP, Fotiadis CI, Bellenis IP. A huge poster-
omedial mediastinal cyst complicated with vertebral dislodgment. World
J Surg Oncol 2006;4:56.
[10] Bernheim J, Griffel B, Versano S, Bruderman I. Mediastinal leiomyosar-
coma in the wall of a bronchial cyst. Arch Pathol Lab Med 1980;104:221.
 A ciliated cyst in the posterior mediastinum compatible with a paravertebral
Mullerian cyst
Adrian P. Businger, Harald Frick, Martin Sailer and Markus Furrer
Eur J Cardiothorac Surg 2008;33:133-136
DOI: 10.1016/j.ejcts.2007.09.036
This information is current as of February 23, 2011

Updated Information including high-resolution figures, can be found at:

& Services http://ejcts.ctsnetjournals.org/cgi/content/full/33/1/133
References This article cites 10 articles, 2 of which you can access for free at:
http://ejcts.ctsnetjournals.org/cgi/content/full/33/1/133#BIBL
Subspecialty Collections This article, along with others on similar topics, appears in the
following collection(s):
Mediastinum
http://ejcts.ctsnetjournals.org/cgi/collection/mediastinum
Permissions & Licensing Information about reproducing this article in parts (figures, tables)
or in its entirety can be found online at:
http://ejcts.ctsnetjournals.org/misc/Permissions.shtml
Reprints Information about ordering reprints can be found online:
http://ejcts.ctsnetjournals.org/misc/reprints.shtml

Downloaded from ejcts.ctsnetjournals.org by on February 23, 2011