Aberrant pancreatic tissue in a giant mediastinal cyst: an uncommon entity

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(2019) 14:134

CASE REPORT

Open Access

Aberrant pancreatic tissue in a giant mediastinal cyst: an uncommon entity Josiah Miner Njem3* , Benjamin Ugwu3, John Collins1, Innocent Emmanuel2, Ojile Akpa Philip2 and John Awodi3

Abstract Background: Heterotopia of pancreatic tissue in the thorax and mediastinum is uncommon, although, a common developmental anomaly in the gastrointestinal tract where the aberrant tissue is a component of gastrointestinal duplication cysts, intralobular pulmonary sequestration or teratomas. Case presentation: We report a case of an ectopic pancreas located in a giant mediastinal cyst in a 2-year old girl who presented with severe dyspnoea due to external compression of the intra-thoracic structures, mainly the right main bronchus. Surgical resection was carried out with complete relief of symptoms. The cyst was confirmed histopathologically to contain pancreatic tissue. Literature review showed that this is an uncommon presentation hence the need to report the case. Conclusion: Heterotopia of the pancreas although, an uncommon occurrence in the thorax and mediastinum, should be considered when dealing with cystic lesions of the mediastinum and surgery plays an important role in its management. Trial registration: JUTH/DCS/ADM/127/XXV/203. Registered 2nd September 2018. Keywords: Mediastinal cyst, Heterotopic pancreatic tissue

Introduction Heterotopic pancreas, also known as aberrant or ectopic pancreas, is pancreatic tissue which has no anatomic and vascular continuity with the main body of the pancreas [1–5]. Congenital ectopia of pancreatic tissue although, a common developmental anomaly in the gastrointestinal tract, its occurrence in the thorax and mediastinum is uncommon [1–3]. ,Aberrant pancreatic tissue in the anterior mediastinum was first described pathologically by Klob in 1859 and first published by Shillitoe and Wilson in 1957 [4, 6, 8]. When found in the thorax, the pancreatic tissue is mostly a component of gastroenteric duplication cysts, intralobular pulmonary sequestration or teratomas [1, 4–8]. This anomaly has been reported in approximately 2% of autopsies and 70–90% of these were located in the gastrointestinal tract mainly in the stomach, duodenum, jejunum and ileum [1–3]. The aetiology of this anomaly is unclear, there are two theories regarding its embryogenesis [5, 6]. The first theory * Correspondence: [email protected] 3 Cardiothoracic Surgery Unit, Department of Surgery Jos University Teaching Hospital, Jos, Nigeria Full list of author information is available at the end of the article

involves abnormal differentiation of the pluripotent epithelial cells of the ventral primary foregut resulting in the formation of ectopic pancreatic tissues in the mediastinum [5–8]. This was supported by the fact that the pancreas and lower respiratory tract, share the same embryological origin from the primitive foregut. The second theory involves migration of cells from the pancreatic bud to the different sites [6]. Clinical presentations are non-specific and essentially re