CASE REPORT

The Clinical Respiratory Journal

Ectopic pancreas in a giant mediastinal cyst Wilson W. Li1, Wim Jan van Boven1, Roy R. Jurhill2, Peter I. Bonta3, Jouke T. Annema3 and Bas A. de Mol1 1 Department of Cardiothoracic Surgery, University of Amsterdam, Amsterdam, the Netherlands 2 Department of Pathology, University of Amsterdam, Amsterdam, the Netherlands 3 Department of Respiratory Medicine, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

Abstract Ectopic pancreas located in the mediastium is an extremely rare anomaly. We present a case of an ectopic pancreas located in a giant mediastinal cyst in an 18-year-old man. He presented with symptoms of dyspnea due to external compression of the cyst on the left main bronchus. Complete surgical resection was performed through median sternotomy, with relief of the bronchial compression postoperatively. Literature review showed 20 previously reported cases. These masses were usually large (>10 cm), almost exclusively located in the anterior mediastinum, predominately cystic in nature and generally benign. Surgical resection was performed in all reported cases with a favorable prognosis. Due to the size of these masses, operative treatment can be challenging and should be carefully planned, with specific considerations regarding anesthetic and surgical management.

Please cite this paper as: Li WW, van Boven WJ, Jurhill RR, Bonta PI, Annema JT and de Mol BA. Ectopic pancreas pancreas in in aa giant giant mediastinal mediastinal cyst. cyst.Clin ClinRespir RespirJJ2016; 2014;10: ••: 125–128. DOI:10.1111/crj.12176. ••–••. DOI:10.1111/crj.12176.

Ethics This work was created in accordance with the ethical standards of the Declaration of Helsinki.

Conflicts of interest The authors have stated explicitly that there are no conflicts of interest in connection with this article.

Key words ectopic pancreas – perioperative management – mediastinal tumor – thoracic surgery Correspondence Wilson W. Li, MD, Department of Cardiothoracic Surgery, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands. Tel: +31 20 566 9111 Fax: +31 20 596 2289 email: [email protected] Received: 1 February 2014 Revision requested: 25 May 2014 Accepted: 22 June 2014 DOI:10.1111/crj.12176 Authorship and contributorship Dr. Li, Dr. van Boven, Dr. Bonta and Professor Annema contributed in the diagnostic work-up and treatment of the patient and in designing and writing the manuscript. Dr. Jurhill performed the pathological investigations and contributed in writing the pathological features of this paper. Professor de Mol contributed in writing and in the final revisions of this article.

Introduction

Case report

Ectopic pancreas is relatively uncommon, with a reported detection frequency of 0.5% during laparotomy and 1.7% at autopsy (1), mostly occurring in the gastrointestinal tract (2). Ectopic pancreas in the mediastinum is extremely rare. In this paper, we report a case of ectopic pancreatic tissue in a giant mediastinal cyst causing bronchial obstruction. Furthermore, a brief review of the available literature on this subject is presented.

An 18-year-old man with an unremarkable medical history was referred to our tertiary referral center with a large mediastinal mass. The lesion was discovered on chest radiography, which was performed by the primary physician because of persistent dyspnea after an episode of the flu. The patient also observed some passing complaints of stridor. There were no symptoms indicative of superior vena cava syndrome. Computed tomography (CT) demonstrated a giant cystic

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mass in the anterior mediastinum, measuring 16 × 12 × 9 cm, with compression of the superior vena cava (Fig. 1). Furthermore, a 50% stenosis of the left main bronchus was present because of the external compression of the tumor. Preoperative provisional diagnosis was thymoma or thymic cyst. Surgical resection was performed through median sternotomy, with stand by extracorporeal circulation. Bronchoscopy after induction of anesthesia showed aggravation of the main bronchial stenosis, with a passable but near total bronchial obstruction (Fig. 2A). Intraoperatively, a large, extrapleural and extrapericardial cystic mass was identified. There were firm adhesions to the direct surroundings, without evidence of invasive growth. Due to dense adhesions to the pericardium, en-bloc partial pericardiectomy was performed. A radical resection could be performed without intraoperative hemodynamic or respiratory incidents. Bronchoscopy after resection of the lesion showed total relief of the bronchial compression (Fig. 2B). Postoperative course was uneventful, and the patient was discharged on postoperative day 4. Gross pathological examination showed a large cystic lesion (13 × 11 × 7 cm) with a smooth outer and inner surface with two solid areas (6 × 6 × 2 cm and 3 × 3 × 2 cm) (Fig. 3). Microscopically, the cyst wall consisted of fibrous connective tissue. One solid area (6 × 6 × 2 cm) showed pancreatic tissue consisting of both exocrine and endocrine components, both nonneoplastic (Fig. 4A). The second solid area (3 × 3 × 2 cm) consisted of normal thymic tissue without features of thymoma (Fig. 4B). These findings were consistent with cystic pancreatic ectopia with engulf-

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(A)

(B)

Figure 2. Intraoperative bronchoscopy (after induction of anesthesia) demonstrating a passable but near total obstruction of the left main bronchus (arrow) (A), and a relief of the left main bronchus compression (arrow) with normal lumen diameter after resection of the lesion (B).

ment of pre-existing thymic tissue. Alternatively, a mediastinal teratoma was considered. However, no other components indicating a teratoma was found.

Discussion

Figure 1. Computer tomography showing a giant mediastinal cystic mass with compression of the superior vena cava (narrow arrow) and 50% obstruction of the left main bronchus (broad arrow).

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The presence of pancreatic tissue in the mediastinum can occur in the context of a pancreatic pseudocyst (3), a gastroenteric duplication cyst with entry into the mediastinum (4), or can arise in a mediastinal teratoma. Alternatively, isolated ectopic pancreas without anatomical or vascular connections to the normal pancreas is extremely rare. To the best of our knowledge, only 20 cases have been reported previously (5–8) since the first description of this entity in the literature by Shillitoe and Wilson (9). Clinical presentation is

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Mediastinal Mediastinal pancreatic pancreatic ectopia ectopia

Figure 3. Gross examination of the tumor showing a cystic mass with two solid areas, one proven to be pancreatic tissue histologically (6 × 6 × 2 cm, sections 2–6), and a second area exhibiting thymic tissue (3 × 3 × 2 cm, sections 8–10).

(A)

(B)

Figure 4. Histological examination revealing endocrine islets of Langerhans surrounded by exocrine pancreatic acini (A) in one of the solid areas. This tissue is indistinguishable from normal non-ectopic pancreatic tissue. The second solid area showed normal thymic tissue without features of thymoma (B).

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variable, with atypical chest pain and dyspnea most frequently reported. Additionally, acute presentations have been described, including pericardial tamponade after cyst rupture (9) and symptoms mimicking lobar pneumonia (10). In our case, dyspnea was the chief complaint, presumably resulting from external compression of the left main bronchus by the giant mediastinal mass. Chest CT with intravenous contrast is the preferred initial imaging modality. These lesions are almost exclusively found in the anterior part of the mediastinum, are mostly cystic in nature and are generally benign. However, adenocarcinoma arising from a mediastinal ectopic pancreas has been reported (5). Differential diagnoses of anterior mediastinal cystic lesions include thymic cyst, cystic teratoma, lymphangioma, pericardial and bronchogenic cyst (11). Regardless of the definite pathological diagnosis, surgical resection is indicated in nearly all patients. These cystic lesions are usually large (>10 cm) at presentation. Consequently, they offer challenges concerning operative therapy due to the complex anatomy of the vicinity and compression of vital structures. Therefore, surgical resection should be carefully planned, with specific considerations regarding anesthetic and surgical management (12). Due to the possible involvement or compression of the superior vena cava in large mediastinal masses, large-bore venous access should be secured in the lower extremity (preferably the femoral vein) rather than the upper extremity in all patients (12). Furthermore, from the surgeon’s point of view, we prefer double-lumen endobronchial intubation whenever feasible, as intentional unilateral pulmonary collapse could greatly facilitate tumor dissection in most cases of large 127 3

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mediastinal masses. The potential need for extracorporeal circulation support should be discussed beforehand between the surgeon and anesthesiologist. In the case under discussion, the left main bronchus was compressed with a remaining lumen of less than 50% of normal, which is predictive for perioperative respiratory complications under general anesthesia (12). Moreover, intraoperative bronchoscopy after induction of anesthesia demonstrated an important aggravation of the compression, confirming the adverse effects of anesthesia on inspiratory muscle tone and bronchial smooth muscles relaxation (12). Regarding surgical approach, most of the documented cases have been operated through a median sternotomy, although resection through thoracotomy has also been reported. Especially in cases with large cysts, dense adhesions can often be found with involvement of adjacent mediastinal structures (5), sometimes necessitating additional surgical resection. Following radical resection, prognosis is favorable. In the reported cases, no signs of recurrence were found during follow-up (up to 8 years), except for a single case with pancreatic adenocarcinoma arising in the ectopic pancreas, with sternal metastases 6 months after primary resection (5). The exact histogenesis of this entity remains unclear. From an embryological point of view, the pancreas and lower respiratory tract share a common origin, the primitive foregut. During embryonic development, the development of the pancreas begins with the formation of ventral and dorsal buds. Fusion of the lateral walls forming the tracheoesophageal septum begins caudally. Theoretically, if a pancreatic bud on the foregut becomes entrapped during this fusion process, it can be displaced into the mediastinum by the downward growth of the lungs and may thus give rise to mediastinal ectopic pancreatic tissue (13). Other theories include abnormal migration of cells from the pancreatic bud and abnormal differentiation of pluripotent epithelial cells of the ventral primary foregut. In conclusion, ectopic pancreas in the mediastinum is an extremely rare anomaly, with only 20 previously reported cases. We report another case, presenting as a giant mediastinal cyst causing bronchial compression

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as documented by bronchoscopy and CT, with total relief of the compression after surgical resection.

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The Clinical Clinical Respiratory Respiratory Journal Journal (2014) (2016) •• ISSN ISSN 1752-6981 1752-6981 The C 2014 John Wiley & Sons Ltd V © 2014 John Wiley & Sons Ltd

Ectopic pancreas in a giant mediastinal cyst.

Ectopic pancreas located in the mediastium is an extremely rare anomaly. We present a case of an ectopic pancreas located in a giant mediastinal cyst ...
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