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 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 result from compression of intra-thoracic structures, thus, symptoms such as cough and dyspnoea due to compression of the major airway may occur. Hypoglycaemia has also been reported in patients with ectopic pancreatic tissue in the mediastinum [
4]. We carried out Pub med search to find all the reported cases of ectopic, heterotopic or aberrant pancreatic tissue in the mediastinum and found that in addition to the index patient, there were thirty reported cases of aberrant pancreas in the mediastinum (Table
1). Most of the cases described were in young adults, the average age was 29 years. The index patient was a two year old girl. The prevalence was higher in females (58%). Aberrant pancreas in the mediastinum is uncommon and to the best of our knowledge thirty cases have been documented in the literature (Table
1). We report an uncommon clinical case of a giant mediastinal cyst containing aberrant pancreatic tissue.
Table 1
Ectopic pancreas in the anterior mediastinum- A report of 30 patients [
6,
8]
1 | Shillitoe etal 1957 | F | 15 | 5.5 | benign |
2 | Carr et al. 1977 | F | 57 | 10 | benign |
3 | Von Schweinitz et al. 1990 | M | 5 | 5x5x5 | benign |
4 | Perez-Ordonez et al. 1996 | F | 16 | 12 | benign |
5 | Gong et al. 1997 | F | 26 | 20 × 15 | benign |
6 | Gong et al. 1997 | F | 26 | 4.3 × 1.3 | benign |
7 | Wu et al. 1998 | F | 60 | 10 × 15 | benign |
8 | Cagirici et al. 2001 | F | 45 | 10 × 8 | benign |
9 | Sentis et al. 2004 | M | 44 | 10x8x7.5 | benign |
10 | Yamato et al. 2005 | M | 39 | 10 × 8 | benign |
11 | Al-Salam et al. 2006 | M | 40 | 8x6x6 | benign |
12 | Wang et al. 2007 | M | 17 | 12x12x4 | benign |
13 | Wang et al. 2007 | F | 24 | 10x8x4 | benign |
14 | Ehricht et al. 2009 | M | 25 | 15 × 15 | benign |
15 | Chen et al. 2009 | F | 32 | 13x16x8 | benign |
16 | Fayoumi et al. 2010 | M | 51 | 10x7x5 | benign |
17 | Fayoumi et al. 2010 | M | 42 | 10 × 5 | benign |
18 | Takemura et al. 2011 | F | 21 | 3.5 × 3.5 | benign |
19 | Sandor et al. 2012 | M | 32 | 4 × 4 | benign |
20 | Byun CS et al. 2012 | F | 31 | 7x3x4 | benign |
21 | St Romain et al. 2012 | F | 66 | 11x | malignant |
22 | Rokach et al. 2013 | F | 22 | 5.1 × 3.8 × 2.3 | benign |
23 | Zhang et al. 2014 | M | 15 | 7 × 4.5 | benign |
24 | Zhang et al. 2014 | F | 16 | 6 | benign |
25 | Li et al. 2014 | M | 18 | 16x12x9 | benign |
26 | Sibel et al. 2014 | M | 23 | 6 × 8 | benign |
27 | Koh et al. 2015 | M | 17 | 7.5x7x5.5 | benign |
28 | Wu et al. 2015 | F | 45 | 7.5x7x5.5 | benign |
29 | Mansi et al. 2017 | F | 29 days | 5x4x3.5 | benign |
30 | Snak et al | F | 21 | 6.7 × 7.5 | benign |
31 | Index case | F | 2 | 20x16x3.5 | benign |