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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 10  |  Issue : 2  |  Page : 105-107

Presternal bronchogenic cyst: A rare presentation


1 Department of General Surgery, JNMC, DMIMS (DU), Wardha, Maharashtra, India
2 Department of Pathology, JNMC, DMIMS (DU), Wardha, Maharashtra, India
3 Department of Internal Medicine, JNMC, DMIMS (DU), Wardha, Maharashtra, India

Date of Submission24-Nov-2018
Date of Decision09-Apr-2019
Date of Acceptance17-Mar-2019
Date of Web Publication24-May-2019

Correspondence Address:
Dr. Samarth Shukla
Department of Pathology, JNMC, DMIMS (DU), Sawangi (Meghe), Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/INJMS.INJMS_32_18

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  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


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

How to cite this URL:
Jajoo S, Shukla S, Acharya S. Presternal bronchogenic cyst: A rare presentation. Indian J Med Spec [serial online] 2019 [cited 2019 Jun 26];10:105-7. Available from: http://www.ijms.in/text.asp?2019/10/2/105/258989




  Introduction Top


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.


  Case Report Top


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.
Figure 1: Patient presenting with substernal swelling (arrow)

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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 the cyst oozed out. The cyst was uniloculated with smooth glistening internal wall.
Figure 2: Gross specimen of the substernal swelling, well-circumscribed round to oval mass, firm to cystic in consistency

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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 [Figure 3] and [Figure 4].
Figure 3: H and E stained histopathology slide ×10 view shows cystic tissue lined by pseudostratified columnar epithelium, deeper tissue showing unremarkable fibrocollagenous tissue

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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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The patient was discharged the same day; postoperatively, he has had an unremarkable clinical course since then.


  Discussion Top


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 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 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.

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 Top


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.



 
  References Top

1.
Farag M, Naguib N, Izzidien A. Bronchogenic cyst, case report and review of literature. West Lond Med J 2010;2:1-4.  Back to cited text no. 1
    
2.
Zvulunov A, Amichai B, Grunwald MH, Avinoach I, Halevy S. Cutaneous bronchogenic cyst: Delineation of a poorly recognized lesion. Pediatr Dermatol 1998;15:277-81.  Back to cited text no. 2
    
3.
Carden C, Dykes E, Pollock AM. An unusual midline swelling – Case report of a cutaneous bronchogenic cyst. Eur J Pediatr Surg 2008;18:345-6.  Back to cited text no. 3
    
4.
Hameed SM, Chan WK, Neuman GD, Urschel JD. Presternal bronchogenic cyst in an adult. Plast Reconstr Surg 1993;92:763-4.  Back to cited text no. 4
    
5.
Fraga S, Helwig EB, Rosen SH. Bronchogenic cysts in the skin and subcutaneous tissue. Am J Clin Pathol 1971;56:230-8.  Back to cited text no. 5
    
6.
Casagrande A, Pederiva F. Association between congenital lung malformations and lung tumors in children and adults: A systematic review. J Thorac Oncol 2016;11:1837-45.  Back to cited text no. 6
    
7.
Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J Thorac Cardiovasc Surg 1995;109:1003-10.  Back to cited text no. 7
    
8.
Limaïem F, Ayadi-Kaddour A, Djilani H, Kilani T, El Mezni F. Pulmonary and mediastinal bronchogenic cysts: A clinicopathologic study of 33 cases. Lung 2008;186:55-61.  Back to cited text no. 8
    
9.
Jiang JH, Yen SL, Lee SY, Chuang JH. Differences in the distribution and presentation of bronchogenic cysts between adults and children. J Pediatr Surg 2015;50:399-401.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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