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Presternal bronchogenic cyst: A rare presentation

Article in Indian Journal of Medical Specialities · January 2019

DOI: 10.4103/INJMS.INJMS_32_18

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Case Report

Presternal Bronchogenic Cyst: A Rare Presentation

Suhas Jajoo, Samarth Shukla1, Sourya Acharya2
Departments of General Surgery, Pathology and 2Internal Medicine, JNMC, DMIMS (DU), Wardha, Maharashtra, India
1

Abstract
Among the various congenital anomalies of the respiratory system, the extrapulmonary bronchogenic cyst is a rare pathologic lesion. The
bronchogenic cyst is a developmental anomaly, occurring due to malformation of ventral foregut during the first 6 weeks of the intrauterine
life. The cystic abnormality presents in infancy, children, and often in young adults. A case of 27‑year‑old male presented with subcutaneous
swelling in the presternal area. The nodular swelling was excised and subsequently sent for surgical pathology examination. The cystic swelling
was diagnosed on histopathology as a cutaneous bronchogenic cyst.

Keywords: Bronchogenic cyst, extrapulmonary, presternal, ventral foregut

Introduction Case Report

Bronchogenic cysts are rare developmental anomalies which
occur due to abnormal budding of the tracheobronchial tree
derived from the ventral foregut defects (embryonic); these are
types of duplication cysts. Although the defect occurs during
the 4th–6th week of intra‑gestation life, the bronchogenic cysts
are more common in males, and bronchogenic cysts can occur
at any time from infancy to young adults though often manifest
in the early adult life.[1]
Since the diagnosis of bronchogenic cyst is a rare finding, array
of differentials is kept in mind by the clinicians. Differential
diagnosis usually considered for such cysts depends on the
site of lesion; differentials include thyroglossal cyst, epidermal
cyst, branchial cyst, trichilemmal cyst, inclusion cyst, dermoid
cyst, and adnexal tumors. The closest differential being
the branchial cyst.[2] The cutaneous bronchogenic cysts are
extremely rare with not more than 70 cases reported till date.[3]
From being clinically silent and asymptomatic to being
symptomatic, bronchogenic cysts may have variable
presentations. During infancy, the bronchogenic cyst
malformations may present with respiratory distress to severe
infections among children. Rarely, the cyst may present with
local compression of neighboring structures and even undergo
malignant transformation. The bronchogenic cyst when
diagnosed is usually a histopathological delight, much less
expected clinically.
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A 27‑year‑old male presented with a swelling in the presternal
area [Figure 1], which he came across as a chance finding
around 6 months back. Since then the swelling has gradually
increased in size. The patient has no history of any local injury
or external trauma. The patient has had no major illness in the
past 1 year. He has no significant history of drug intake.
On physical examination, the swelling was freely mobile
and not fixed to the underlying structures; the overlying
skin covering the swelling appeared unremarkable; with no
ulceration or discharging sinus. The size of the cystic swelling
on clinical examination was 8 cm × 6 cm × 3 cm. The swelling
was firm to cystic in consistency, nontender.
The swelling was surgically excised and sent for histopathological
examination to the surgical pathology section in the department
of pathology at a rural tertiary care hospital. On pathological
examination, grossly, the swelling was brownish‑red in color,
with a size of 6 cm × 4 cm × 2 cm [Figure 2], round to oval in
shape with firm to cystic consistency, external surface of the
swelling was unremarkable, on cut opening serous fluid from
Address for correspondence: Dr. Samarth Shukla,
Department of Pathology, JNMC, DMIMS (DU), Sawangi (Meghe),
Wardha, Maharashtra, India.
E‑mail: samarth21174@gmail.com
Received: 24‑11‑2018 Revised: 09-04-2019
Accepted: 17-03-2019 Published Online: 24-05-2019
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For reprints contact: reprints@medknow.com

DOI:
10.4103/INJMS.INJMS_32_18 How to cite this article: Jajoo S, Shukla S, Acharya S. Presternal
bronchogenic cyst: A rare presentation. Indian J Med Spec 2019;10:105-7.

© 2019 Indian Journal of Medical Specialities | Published by Wolters Kluwer - Medknow 105
[Downloaded free from http://www.ijms.in on Tuesday, May 28, 2019, IP: 10.232.74.22]
Jajoo, et al.: Presternal bronchogenic cyst
the cyst oozed out. The cyst was uniloculated with smooth
glistening internal wall.
On microscopic examination, the cystic tissue was lined by
ciliated pseudostratified columnar lining epithelium, deeper
tissue showed unremarkable fibrocollagenous tissue with
minimal nonspecific chronic inflammatory infiltrate, and
pericystic areas showed increased fibrocollagenous tissue. The
final diagnosis of the cyst on histopathology was suggestive of
extrapulmonary bronchogenic cyst [Figures 3 and 4].
The patient was discharged the same day; postoperatively, he
has had an unremarkable clinical course since then.
Discussion
Although bronchogenic cysts are developmental anomaly; they
are unusually diagnosed in young adults.[4] Among the many
cystic lesions occurring in the respiratory tract, bronchogenic cysts
constitute around 20%; out of which majority are intrapulmonary
and around 10%–15% being extrapulmonary in origin. The
bronchogenic cysts in extrapulmonary locations are seen in the
pericardium and intra‑atrial area, diaphragmatic, abdominal,
retroperitoneal, and subcutaneous locations. The bronchogenic
cysts of extrapulmonary origin are not connected to the primary
Figure 1: Patient presenting with substernal swelling (arrow)
Figure 3: H and E stained histopathology slide ×10 view shows cystic
tissue lined by pseudostratified columnar epithelium, deeper tissue
showing unremarkable fibrocollagenous tissue
106 Indian Journal of Medical Specialities ¦ Volume 10 ¦ Issue 2 ¦ April-June 2019
pulmonary tissue in majority of the cases. Around the chest area,
sternum often has the development of bronchogenic cysts, the
most common site being substernal area followed with presternal,
scapular, neck, and abdominal wall as other rare sites.[5]
Commonly, the surgical pathological features of bronchogenic
cysts include grossly multiloculated or uniloculated
cysts filled with serous fluid and proteinaceous debris;
microscopically, the cysts are lined by columnar lining
epithelium (pseudostratified ciliated), at times the lining
epithelia consist of submucosal gland, goblet cells, smooth
muscles, and deeper tissue showing cartilaginous tissue as well.
The histopathological differentials for bronchogenic cysts are
the branchial cysts, cutaneous cysts, and thyroglossal cysts.
The branchial cysts form one of the closest differentials of
bronchogenic cysts. The branchial cysts on histopathology are
often surrounded by lymphoid tissue lined by columnar epithelium
albeit nonciliated and/or stratified squamous epithelium. The other
close differential is the simple cutaneous cysts which are lined by
Figure 2: Gross specimen of the substernal swelling, well‑circumscribed
round to oval mass, firm to cystic in consistency
Figure 4: H and E stained histopathology slide ×40 view, shows
characteristic ciliated pseudostratified columnar epithelium, features
characteristic of bronchogenic cyst
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Jajoo, et al.: Presternal bronchogenic cyst
squamous or cuboidal/columnar lining epithelium; usually, the
lining epithelium is simple but may be stratified with papillary
fronds and projections into the cystic lumen. Finally, a cystic lesion
in the area of the head and neck which needs to be addressed as a
differential diagnosis is thyroglossal cyst. The thyroglossal duct
cysts on histopathology may be lined with stratified squamous
epithelium, cuboidal, columnar, rarely the columnar lining maybe
ciliated. The main characteristic histologic feature of thyroglossal
duct cyst which differentiates it from bronchogenic cysts is the
presence of low cuboidal cells forming thyroid follicles filled with
colloid which appears as homogeneous proteinaceous material
filling the cyst.
The clinical spectrum of bronchogenic cysts is extremely
variable; from the patients being completely asymptomatic
to malignant transformation, all have been reported.
Symptoms also depend on the location of the cyst. Among
infants, respiratory distress is common, some cases present
with compression symptoms (superior vena cava syndrome,
dysphagia, and dyspnea) depending on the location, whether
the esophagus or trachea. Respiratory symptoms can result
from localized abscess to recurrent infection, pneumonia and
pleurisy and dyspnea due to tracheal compression.
On occasions, bronchogenic cysts tend to form discharging
sinus, which are connected with small opening toward the
outside, discharge can be serous or transudate. Children often
develop recurrent abscess and infection in the bronchogenic
cysts. Occasionally, patients may present with malignant
changes within the bronchogenic cyst.
In a systematic review carried out by Casagrande and
Pederiva, bronchogenic cysts occurring in adults as pulmonary
developmental anomaly were found to develop bronchogenic
carcinoma.[6] Ribet et al. have reported that around 70% of
symptomatic bronchogenic cysts in children were due to the fact
that most of the cysts were located in the critical location that
is rests in and around the carina. Similarly, 60% of adults who
were symptomatic had the mediastinal cysts near the carina.[7]
In a series of a study carried out by Limaïem et al., of 33 cases
reported maximum cases had respiratory system involved by
bronchogenic cysts leading to dyspnea, cough, chest pain, and
hemoptysis.[8] Jiang et al. carried out a study in children being
diagnosed with bronchogenic cysts, of 16 cases, eight cases of
bronchogenic cysts occurred in the neck and cutaneous area and
were asymptomatic except one. Of the remaining eight cases,
all were present in the mediastinum except one which had a
pulmonary location, 75% of these children were asymptomatic.
In contrast, of 23 cases in adults of bronchogenic cysts located
in the mediastinum, only 53% of cases were asymptomatic.[9]
Clinicians need to take cognizance of the fact that though
the bronchogenic cysts clinically maybe deceptive and be a
diagnosis of chance, there are varied pathologies which can
occur in a bronchogenic cyst. The management of the cyst
depends on the histopathological diagnosis of the cyst and
the accompanying pathology within the same, which could
extend along the spectrum from an asymptomatic simple cyst
to infective abscess, rarely malignancy.
Indian Journal of Medical Specialities ¦ Volume 10 ¦ Issue 2 ¦ April-June 2019
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In case of diagnostics being carried out during the antenatal
period, the fetal ultrasound helps to locate the lesion, besides
the ultrasound, simple chest X‑rays can help in diagnosis
depending on the site or location of the lesion. Contrast
computed tomography maybe helpful in diagnosis of the same,
in case of preoperative workup and localization.
However, the final diagnosis is with confirmed histopathological
diagnosis, where ciliated pseudostratified columnar epithelium
is confirmatory for diagnosis, and virtually helps rule out other
close clinical, radiological diagnosis such as branchial cyst,
inclusion cyst, and thyroglossal cyst.
Conclusion
Bronchogenic cysts are extremely rare in extrapulmonary
locations, cutaneous presternal bronchogenic cyst being
exceptional. The clinician needs to understand the propensity
of development of such cysts, as well as the varied clinical
symptomatology presented by the patients, need to be taken
into consideration. Histopathological examination not only
helps in putting up final diagnosis but also helps to rule out
any associated pathology with the cyst.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
None.
Conflicts of interest
There are no conflicts of interest.
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