Case Report
 
Primary retroperitoneal benign mucinous cystadenoma in a male
Malek Barka1, Faouzi Mallat2, Mohamed Ben Mabrouk1, Chaima Mrad2, Khaled Ben Ahmed2, Wissem Hmida2, Ghassen Tlili2, SidiyaOuld Chavey3, Sarra Mestiri4, Sonia Ziadi4, Faouzi Mosbah5
1MD, General Surgery Department, Sahloul Hospital, Sousse, Tunisia.
2MD, Urology Department, Sahloul Hospital, Sousse, Tunisia.
3MD, Radiology Department, Sahloul Hospital, Sousse, Tunisia.
4MD, Pathology Department, Farhat Hached Hospital, Sousse, Tunisia.
5Professor, Urology Department, Sahloul Hospital, Sousse, Tunisia.

doi:10.5348/ijcri-201533-CR-10494

Address correspondence to:
Dr. Malek Barka
Department of General Surgery, Hospital of Sahloul
Sousse
Tunisia
Phone: 0021697887258

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How to cite this article
Barka M, Mallat F, Mabrouk MB, Mrad C, Ahmed KB, Hmida W, Tlili G, Chavey SO, Ziadi SMS, Mosbah F. Primary retroperitoneal benign mucinous cystadenoma in a male. Int J Case Rep Images 2015;6(4):198–202.


Abstract
Introduction: Retroperitoneal mucinous cystadenoma is extremely rare, prevailing specifically in female gender, with unclear histogenesis. Only three cases concerning male patients have been reported.
Case Report: We report a case of a 75-year-old male presented with right flank pain and a palpable mass. An abdominal computed tomography scan revealed a retroperitoneal tumor resulting after the histological examination to a primary retroperitoneal mucinous cystadenoma.
Conclusion: This is the fourth case of primary retroperitoneal mucinous cystadenoma of benign type in a male reported in literature. Several hypotheses may explain the histogenesis of this pathological process. Radical resection is the treatment of choice.

Keywords: Cystadenoma, Male, Mucinous, Retroperitoneal


Introduction

Retroperitoneal tumors account for less than 0.2% of all neoplasm [1]. Primary retroperitoneal tumors of mucinous type is an extremely rare entity that affects, almost exclusively women [2] and can be subdivided into benign, borderline or cystadenocarcinoma [3]. Since 1965, only 51 cases have been reported in literature [4]. We report a case of a 78-year-old male presented with primary retroperitoneal mucinous cystadenoma of benign type. In our knowledge, this is the fourth case reported in literature.


Case Report

A 75-year-old male admitted to our hospital complaining of right flank pain and a palpable mass that was constantly growing up during the last three months. There were no associated features, and no aggravating or relieving factors. His past medical history included a lung hydatid cyst operated three years ago. Abdominal examination revealed a firm, sizeable and hard mass at the right abdomen extending from the inferior ribs to the right iliac crest. Biological analyses were normal.

Ultrasonography of the abdomen demonstrated a 20-cm well defined cystic mass. Abdominal computed tomography (CT) scan revealed a large cystic lesion occupying the right retroperitoneal space and measuring 20×14 cm (Figure 1).

The patient underwent laparotomy and a tumoral exeresis has been performed. The patient made an uncomplicated postoperative recovery. Microscopic examination reported a retroperitoneal mucinous cystadenoma of benign type (Figure 2).


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Figure 1: Axial computed tomography images showing the size of the tumor and its mass effect to the right kidney and the small intestine.



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Figure 2: (A, B) Microscopic findings: cystic wall lined by a monostratified mucinous type epithelium without cytologic atypia (H&E stain, x100, x200).



Discussion

This is the 23rd reported case of a benign primary retroperitoneal mucinous cystadenoma in literature (Table 1) and only the fourth primary retroperitoneal mucinous cystadenoma (PRMC) diagnosed in a male [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25] [26].

This entity was first described by Handfield-Jones in 1924 in his study on retroperitoneal cysts [26]. Only three cases of pure PRMC in a male patient were reported in literature. The retroperitoneal location of these tumors is rare because of the non-existence of epithelial cells in this area so the histogenesis of PRMC remains unclear; one theory involves that that these tumors may arise from a coelomic epithelium which normally during the embryogenesis converts to peritoneal mesothelium and ovarian germinal epithelium but that become trapped in the retroperitoneum [27] [28]. Some immunohistochemical and ultrastructural similarity with ovarian mucinous tumors as positive match to cytokeratin 7 and cytokeratin 20 antibodies supports this hypothesis [29]. A second hypothesis involves the seeding of ectopic ovarian tissue in the retroperitoneum [30]. Another histogenesis suggests the proliferation of mucinous epithelium in retroperitoneal teratoma [31]. Clinical signs and symptoms are non-specific including predominantly an abdominal mass, chronic abdominal pain or both. Radiologically, this entity presents a cystic formation repressing the organs around and there are a wide range of differential diagnoses including lymphangioma, cystic teratoma, lymphocele, urinoma and cystic mesothelioma [32]. In our case, because of a history of lung hydatid cyst, a second localization in the retroperitoneum was suspected. Ultrasonography is not specific [17].

The diagnostic value of computed tomography and magnetic resonance imaging scan is similar to differentiate between a cystic teratoma and cystadenoma through detection of calcification within the cyst and the description of the tumor in relation to soft tissue and radiological evidence of its origin [33]. No blood profile abnormalities are specific to PRMC; previous cases have demonstrated an increase in carcinoembryonic antigen (CEA) and CA 19-9 [34] [35] [36]. Surgery is the preferred treatment for most cystic retroperitoneal masses [37] by a complete enucleation [38].


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Table 1: Cases of primary retroperitoneal mucinous cystadenoma of benign type reported in the literature. [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25]




Conclusion

We present a rare case of primary retroperitoneal mucinous cystadenoma of benign type, the fourth in a male patient. Several hypotheses may explain its histogenesis. Surgical excision is advised for diagnostic assessment and management. Owing to the small number of reported cases and the insufficient surveillance data, prognosis of recurrence is unknown.


Acknowledgements

We are grateful to Dr. Faouzi Mosbah for his comments on the manuscript.


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Author Contributions
Malek Barka – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Faouzi Mallat – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellrctual content, Final approval of the version to be published
Mohamed Ben Mabrouk – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellrctual content, Final approval of the version to be published
Chaima Mrad – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Khaled Ben Ahmed – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Wissem Hmida – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Ghassen Tlili – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Sidiya Ould Chavey – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Sarra Mestiri – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Sonia Ziadi – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Faouzi Mosbah – Substantial contributions to conception and design, Acquisition of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2015 Malek Barka et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.