Ectopic pancreatic pseudocyst and cyst presenting as a cervical and mediastinal mass - case report and review of the literature
© Rokach et al.; licensee BioMed Central Ltd. 2013
Received: 27 May 2013
Accepted: 8 October 2013
Published: 23 October 2013
Ectopic pancreas in the mediastinum is extremely rare. We are reporting on a case of a twenty two year old woman who presented to our clinic with a large cervical mass. The CT scan revealed a cystic lesion in the anterior mediastinum. The patient underwent surgical resection by cervical approach. A Cystic mass with pseudocysts, cysts and complete pancreatic tissue were found in pathology. There were no signs of pancreatitis or malignancy. No recurrence was observed after a follow up of four years. We reviewed the case reports describing this rare condition in the medical literature.
We conclude that the possibility of ectopic pancreatic tissue should be included in the differential diagnosis of anterior mediastinal cystic mass, though as a remote possibility. Surgery is probably needed for the diagnosis and treatment. Posterior mediastinal pseudocyst is a different entity associated with acute pancreatitis. In those cases surgery is not recommended. Our third conclusion is that pancreatic tissue should be actively sought, if a structure resembling a pseudocyst is found in an unexpected location.
The virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/1849369005957671
KeywordsEctopic pancreas Mediastinum Computed tomography Pseudocyst Cyst
Congenital ectopic pancreas is a known phenomena found in the gastrointestinal tract in about 2% of autopsies. An ectopic pancreas arising from the anterior mediastinum is extremely rare. To the best of our knowledge, only a few cases[2–19] have been reported in the literature, all of them arising from the anterior mediastinum. A different phenomenon are the pseudocysts arising from the posterior mediastinum as a result of acute pancreatitis[20–26]. Ectopic tissue arising from the mediastinum might contain other organs like spleen and endometrium.
The first reported case was published in 1957 by Shillitoe and Wilson. Herein we describe a case report of asymptomatic ectopic pancreas in the anterior mediastinum. In our case report pseudocysts and cysts were the main pathologic findings. We reviewed the cases published in the literature and distinguished between ectopic pancreas arising from the anterior mediastinum and ectopic pancreas arising from the posterior mediastinum.
Review of the literature
Ectopic pancreas in the anterior mediastinum- clinical features of 22 cases
Dyspnea, Night sweats
Von Schweinitz 1990
5 × 5 × 5
20 × 15
Chest pain, Cough
4.3 × 1.3
10 × 5
10 × 8
Chest pain, cough
10 × 8 × 7.5
Chest pain, Dyspnea
10 × 8
8 × 6 × 6
12 × 12 × 4
Chest pain, Dyspnea
10 × 8 × 4
Chest pain, Dyspnea
15 × 15
13 × 16 × 8
10 × 7 × 5
Chest pain, cough
10 × 5
11 × 9
3.5 × 3.5
4 × 4
Chest pain, Hemoptysis
Byun CS 2012
7 × 3 × 4
Chest pain, cough, Hemoptysis
5.1 × 3.8 × 2.3
None (Asymptomatic cervical mass)
In addition to our patient we found twenty one case reports of ectopic pancreas in the mediastinum. Fifteen case reports were written in English three in Chinese, two in German and one in Spanish. Most of the cases were described in young adults; Average age was thirty four and ages ranged from 5 to 66. The prevalence was higher in females (64% females). The clinical presentation was usually nonspecific including, chest pain, shoulder pain, shortness of breath, fever, neck swelling, night sweats, heart murmur, fatigue, chronic pulmonary infiltrate and tamponade. Four patients were asymptomatic at presentation. All the cases showed cystic lesions located in the anterior mediastinum. Most of the lesions were large ranging from 3.5 cm to 20 cm in diameter. Large lesions encompassed adjacent structures such as the great vessels. Associated findings were pleural effusions and pericardial effusions. There were no specific findings on the CT scan that could distinguish ectopic pancreatic tissue from other diagnosis. The cyst wall and the solid portion of the lesions usually showed mild to moderate enhancement with contrast material. The radiological appearance could not be distinguished from Thymoma, Lymphoma or Teratoma. In all cases the diagnosis was done only after surgery. Twenty one cases were benign and fully recovered after the operation and in a single case pancreatic carcinoma arising from the mediastinum was found. In the benign cases no recurrence was reported. The only patient with pancreatic adenocarcinoma died 15 months after the operation. This case strengthens the importance of surgery.
Ectopic pancreas in the mediastinum is a very rare condition. There are two theories on the embryogenesis of this anomalous development[4, 8]. The first theory involves pluripotent epithelial cells of the ventral primary foregut underwent abnormal differentiation that led to the formation of ectopic pancreatic tissue in the anterior mediastinum. The second theory involves migration of cells from the pancreatic bud to a different site. Ectopic pancreas can be found in other locations as well. The most common site is the gastrointestinal tract. Pancreatic tissue is found there in two percent of autopsies.
A different entity is a pseudocyst arising from the posterior mediastinum as a result of acute pancreatitis[20–26]. In those cases extension of pancreatic necrosis into the mediastinum was possible via the aorta or the esophageal hiatus, through the diaphragmatic crura, or through erosion in the diaphragm.
Ectopic tissue in the mediastinum is a rare phenomenon. The anomalous development of ectopic tissue may occur due to abnormal differentiation of pluripotent cells, migration of ectopic cells or malignant transformation. A few case reports described this rare phenomenon. Hong Li et al. described a rare liposarcoma in the superior mediastinum. The origin of this rare tumor was the Para pharyngeal region. Monika Saini et al. described intrapulmonary Teratoma attaching the medial mediastinum. Composite lymphoma in the anterior mediastinum, a rare lymphoma from two different origins, was described by Guohua Yu et al.. These cases represent malignant transformation. In our cases there were no signs of malignancy and the origin of the ectopic tissue was probably from abnormal differentiation of pluripotent cells or migration of ectopic cells.
The first reported case of ectopic mediastinal pancreas was published in 1957 by Shillitoe and Wilson. They described a fifteen year old female that had benign ectopic pancreatic tissue in the anterior mediastinum. Ectopic Pancreas in the anterior Mediastinum is extremely rare. Only twenty one case reports were described in the literature, all in young adults. The lesions were solid-cystic. The pathology and the clinical course were benign in twenty cases and malignant in one case. There were no signs of pancreatitis. Posterior mediastinal pseudocyst is a different entity associated with acute pancreatitis. In those cases surgery is not recommended.
We conclude that ectopic pancreas should be considered in the differential diagnosis of anterior mediastinal lesions. Surgery is probably needed for the diagnosis and treatment. Pancreatic tissue should be actively sought, if a structure that looks like a pseudocyst is found in an unexpected location.
Written informed consent was obtained from the patient for the publication of this report and any accompanying images.
- Lai EC, Tompkins RK: Heterotopic pancreas: review of a 26 year experience. Am J Surg. 1986, 151: 697-700. 10.1016/0002-9610(86)90045-0.View ArticlePubMedGoogle Scholar
- Shillitoe AJ, Wilson JE: Enterogenous cyst of thorax with pancreatic tissue as a constituent. J Thorac Surg. 1957, 34: 810-814.PubMedGoogle Scholar
- Carr MJT, Deiraniya AK, Judd PA: Mediastinal cyst containing mural pancreatic tissue. Thorax. 1977, 32: 512-516. 10.1136/thx.32.4.512.PubMed CentralView ArticlePubMedGoogle Scholar
- Von Schweinitz D, Wittekind C, Freihorst J: Mediastinaler sequester mit ektopem Pankreasgewebe [Mediastinal sequestration with ectopic pancreas]. Z Kinderchir. 1990, 45: 249-250. [German]PubMedGoogle Scholar
- Perez-Ordonez B, Wesson DE, Smith CR, et al.: A pancreatic cyst of the anterior mediastinum. Modern Pathol. 1996, 9: 210-214.Google Scholar
- Gong N, Fang G: Ectopic pancreas in within thorax: two case reports. Chin J Thorac Cardiovasc Surg. 1997, 13: 308-[Chinese]Google Scholar
- Wu J, Chen Y, Ni X: Ectopic pancreas in anterior mediastinal with pseudo-cyst: one case report. Chin J Thorac Cardiovasc Surg. 1998, 14: 214-10.1016/S1010-7940(98)00168-7. [Chinese]View ArticleGoogle Scholar
- Cagirici U, Ozbaran M, Veral A, et al.: Ectopic mediastinal pancreas. Eur J Cardio-Thorac. 2001, 19: 514-515. 10.1016/S1010-7940(01)00606-6.View ArticleGoogle Scholar
- Sentis MI, Sanchis JB, Garolera JMG, et al.: Mediastinal enteric cyst: unusual clinical presentation and histopathology. Arch Bronconeumol. 2004, 40: 185-187. [Spanish]Google Scholar
- Tamura Y, Takahama M, Kushibe K, et al.: Ectopic pancreas in the anterior mediastinum. Jpn J Thorac Cardiovasc Surg. 2005, 53: 498-501. 10.1007/s11748-005-0095-6.View ArticlePubMedGoogle Scholar
- Al-Salam S, Al AM: Ectopic pancreatic tissue in the anterior mediastinum. Virchows Arch. 2006, 448: 661-663. 10.1007/s00428-006-0159-8.View ArticlePubMedGoogle Scholar
- Wang W, Li KC, Qin W, et al.: Ectopic pancreas in mediastinum report of 2 cases and review of the literature. J Thorac Imag. 2007, 22: 256-258. 10.1097/01.rti.0000213587.44719.a7.View ArticleGoogle Scholar
- Ehricht A, Putzschler F, Weissmann K, et al.: Ektopes Pankreasgewebe in einer Mediastinalzyste - eine seltene klinische Manifestation. [Ectopic pancreatic tissue within a mediastinal cyst—a rare clinical manifestation]. Zentralbl Chir. 2009, 134: 178-181. 10.1055/s-2008-1076882. [German]View ArticlePubMedGoogle Scholar
- Chen ZH, Yu RS, Dong F, et al.: CT findings of an ectopic pancreas in the anterior mediastinum. Korean J Radiol. 2009, 10: 527-530. 10.3348/kjr.2009.10.5.527.PubMed CentralView ArticlePubMedGoogle Scholar
- Fayoumi S, Al-Husseini L, Jalil R, et al.: Ectopic pancreatic tissue in the thoracic cavity: report of two cases. Ann Thorac Surg. 2010, 90: 25-27. 10.1016/j.athoracsur.2010.05.059.View ArticleGoogle Scholar
- Romain PS, Muehlebach G, Damjanov I, et al.: Adenocarcinoma arising in an ectopic mediastinal pancreas. Ann Diagn Pathol. 2012, 16: 494-497. 10.1016/j.anndiagpath.2011.04.005.View ArticleGoogle Scholar
- Takemura M, Yoshida K, Morimura K: Thoracoscopic resection of thoracic esophageal duplication cyst containing ectopic pancreatic tissue in adult. J Cardiothorac Surg. 2011, 6: 118-10.1186/1749-8090-6-118.PubMed CentralView ArticlePubMedGoogle Scholar
- Szabados S, Lénárd L, Tornóczky T, et al.: Ectopic pancreas tissue appearing in mediastinal cyst. J Cardiothorac Surg. 2012, 7: 22-10.1186/1749-8090-7-22.PubMed CentralView ArticlePubMedGoogle Scholar
- Byun CS, Park IK, Kim H, et al.: Ectopic pancreas with hemorrhagic cystic change in the anterior mediastinum. Korean J Thorac Cardiovasc Surg. 2012, 45: 131-133. 10.5090/kjtcs.2012.45.2.131.PubMed CentralView ArticlePubMedGoogle Scholar
- Kirchner SG, Heller RM, Smith CW: Pancreatic pseudocyst of the mediastinum. Radiology. 1977, 123: 37-42.View ArticlePubMedGoogle Scholar
- Owens GR, Arger PH, Mulhern CB, et al.: CT evaluation of mediastinal pseudocyst. J Comput Assist Tomogr. 1980, 4: 256-259. 10.1097/00004728-198004000-00028.View ArticlePubMedGoogle Scholar
- Maier W, Roscher R, Malfertheiner P, et al.: Pancreatic pseudocyst of the mediastinum evaluated by CT. Eur J Radiol. 1986, 6: 70-72.PubMedGoogle Scholar
- Crombleholme TM, deLorimier AA, Adzick NS, et al.: Mediastinal pancreatic pseudocysts in children. J Pediatr Surg. 1990, 25: 843-845. 10.1016/0022-3468(90)90188-F.View ArticlePubMedGoogle Scholar
- Rose EA, Haider M, Yang SK, Telmos AJ: Mediastinal extension of a pancreatic pseudocyst. Gastroenterol. 2000, 95: 3638-3639.Google Scholar
- Winsett MZ, Amparo EG, Fagan CJ, et al.: MR imaging of mediastinal pseudocyst. J Comput Assist Tomogr. 1988, 12: 320-322. 10.1097/00004728-198803000-00024.View ArticlePubMedGoogle Scholar
- Karantanas AH, Sandris V, Tsikrika A, et al.: Extension of Pancreatic Pseudocysts into the Neck: CT and MR Imaging Findings. AJR. 2003, 180: 843-845. 10.2214/ajr.180.3.1800843.View ArticlePubMedGoogle Scholar
- Li H, Zhou X, Ran Q, Wang L: Parapharyngeal liposarcoma: a case report. Diagn Pathol. 2013, 42: 1596-1598.Google Scholar
- Saini ML, Krishnamurthy S, Kumar RV: Intrapulmonary mature Teratoma. Diagn Pathol. 2006, 1: 38-10.1186/1746-1596-1-38.PubMed CentralView ArticlePubMedGoogle Scholar
- Guohua Y, Lingling K, Guimei Q, et al.: Composite lymphoma in the anterior mediastinum: a case report and review of the literature. Diagn Pathol. 2011, 6: 60-10.1186/1746-1596-6-60.View ArticleGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.