A rare case of a retroperitoneal enterogenous cyst with in-situ adenocarcinoma
© Lordan et al. 2007
Received: 05 June 2007
Accepted: 10 October 2007
Published: 10 October 2007
Retroperitoneal enterogenous cysts are uncommon and adenocarcinoma within such cysts is a rare complication.
We present the third described case of a retroperitoneal enterogenous cyst with adenocarcinomatous changes and only the second reported case whereby the cyst was not arising from any anatomical structure.
This case demonstrates the difficulties in making a diagnosis as well as the importance of a multi-disciplinary approach, and raises further questions regarding post-operative treatment with chemotherapy.
Four years later, he re-presented with a 5-month history of intermittent dull right sided abdominal pain and a right sided palpable abdominal mass was palpated. Liver function tests revealed a bilirubin of 29 but no other abnormalities. His α-feto-protein was 3 and CA 19.9 was 22129.
The repeat CT scan concurred with the previous scan, reporting a large benign cyst, with a maximum diameter of 16 cm. Figure 1 demonstrates the CT with the cyst, and the marker is the measure of the maximum diameter. Endoscopic ultrasound demonstrated a frond-like mucosal prominence within the cyst wall lining.
At laparotomy, the cyst was found to be retroperitoneal lateral to the right psoas muscle, displacing the pancreas and duodenum medially, the liver superiorly and the right kidney and ureter inferiorly. It did not appear to be arising from any particular organ. An attempt at complete excision was successful but there was some spillage of viscous cyst fluid. The abdominal cavity was thoroughly lavaged with water, followed by saline.
This conclusion was not entirely satisfactory due to the lack of specificity of CA 19-9 as a mucinous tumour marker. Also, the CT scans and operative findings were reviewed retrospectively and it was confirmed that the cyst was not involved with the pancreatic or biliary tree. It was thus concluded that this was adenocarcinomatous change within an enterogenous cyst as there was smooth muscle lining the walls with cytokeratins CK20 and CK7 both positive, suggesting a fore-gut origin. Figure 2 is a picture of the histological slide. Also, there was clear carcinomatous change in a plaque within the cyst wall with no penetration from the outer lining described, suggesting no external origin of the malignancy. The patient was to be given Fluoropyrimidine and oxaliplatin following recovery from surgery.
Enterogenous cysts (duplication cysts) are rare. Their presence have been described in the pericardium , central nervous system, testis  and mediastinum . They can also occur anywhere within the gastro-intestinal tract, although they are most commonly found in the small intestine . They tend to present in infancy or early childhood [4 6 9].
Dardik et al.,  described pathological criteria for the diagnosis of gastrointestinal enterogenous cysts. These include the presence of alimentary mucous membrane lining, a smooth muscle coat and intimate attachment to some part of the gastro-intestinal tract. However, cysts with respiratory epithelial lining and no attachment to the gastro-intestinal tract have been described [4 10]. The current article demonstrates a retroperitoneal enterogenous cyst that also does not conform to Dardik's original criteria. Therefore, these criteria should be amended to include that such cysts need not arise from any particular organ.
The embryogenesis of enterogenous cysts have included intra-uterine volvulus resulting in ischaemia and infarction, persistence of an intra-uterine diverticulum and failure of complete vacuolisation of the solid alimentary tract [2–4]. This case represents the third report in the literature of a retroperitoneal enterogenous cyst with adenocarcinomatous changes [4 5], and only the second of such a cyst not arising from any anatomical structure.
The two CT scans (the first in 2002, the second in 2006) initially reported an intimate relationship of the cyst with the liver. Initially, radiographic and clinical signs suggested a simple benign liver cyst resulting in the patient's discharge. However, at surgery 4 years later, the cyst did not appear to be arising from any organ and retrospective review of the CT scans concurred with this finding. Previously, enterogenous cysts have been defined as arising from a gastro-intestinal organ, although one report has described such a cyst with no relationship to another organ[4 10], which may alter this definition.
Duplication cysts of the gastro-intestinal tract, specifically arising from the rectum, have been described with in-situ adenocarcinoma [11–14]. In these cases many have been successfully excised surgically. However, only one case in the literature has been described arising from no organ. Published cases re-iterate the lack of understanding with regard to adjuvant chemo- or radio-therapy. Also, the cases in the literature do not demonstrate the aggressive malignant potential seen in the current article.
This case illustrates the difficulties in making the diagnosis and the dangers of leaving such cysts untreated.
Histology revealed invasive in-situ adenocarcinoma, which has been previously described . Immunohistochemistry of the epithelium showed that cytokeratins 7 (CK7) and 20 (CK20) were both positive. Classically, adenocarcinoma of the lung, breast ovary and omentum are CK7 positive and CK20 negative, while adenocarcinoma of colorectal origin tends to have the opposite (CK7 negative and CK20 positive) [4 15].
In view of the differential cytokeratin profile in this case (CK 7 and 20 positive) suggesting a fore gut origin, an adjuvant chemotherapy regimen comprising a fluoropyrimidine and oxaliplatin was recommended. Due to the rarity of such cases no direct evidence regarding adjuvant systemic therapy could be applied. Unfortunately, the condition of this patient deteriorated rapidly and he did not receive chemotherapy.
The aggressive nature of this type of cyst has not previously been described. The cyst had remained dormant for at least 4 years. The high levels of CA19.9 related to the cyst may be a marker of its aggressive nature. The authors do not know if tumour markers should be measured routinely in cases similar to this.
Following surgical excision the patient developed rapidly progressive metastatic disease. Although the patient died before receiving chemotherapy, the decision to treat with adjuvant therapy was made since the cyst had ruptured during surgery. Although its role remains to be defined the aggressive malignant potential demonstrated in this article suggests that post-surgical adjuvant therapy should be considered.
This represents the third case in the literature of adenocarcinoma arising within a retroperitoneal enterogenous cyst. It is also the second case of such a cyst not associated with any anatomical structures and resulted in death due to aggressive uncontrollable malignant disease following excision. Cases like these must be reported in order to build a profile of clinical and pathological presentations of these rare cysts. This case demonstrates the difficulties in make a diagnosis, the importance of a multi-disciplinary approach and raises further questions regarding post-operative treatment with chemotherapy.
Written consent was obtained from the patient for publication of this case report.
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