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Year : 2019  |  Volume : 4  |  Issue : 2  |  Page : 53-55

Symptomatic Müllerian cyst in the retroperitoneum of an adult male: Case report and review of the literature

Division of Urology, Department of Surgery, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria

Date of Submission26-May-2019
Date of Decision21-Jun-2019
Date of Acceptance13-Sep-2019
Date of Web Publication20-Jan-2020

Correspondence Address:
Musliu Adetola Tolani
Division of Urology, Department of Surgery, Ahmadu Bello University/Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/archms.archms_19_19

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Retroperitoneal cysts of Müllerian origin are quite uncommon, and most of the cases reported in the literature are in females. This report details the case of a 20-year-old male who presented with recurrent right flank pain and right flank swelling. Imaging investigations revealed a simple cyst posterior to the right kidney. He subsequently had retroperitoneal exploration and excision of an ovoid cystic mass which was histologically confirmed as a Müllerian cyst. Thus, Müllerian cyst should be kept in mind in the differential diagnosis of retroperitoneal cysts.

Keywords: Müllerian cyst, retroperitoneal, symptomatic

How to cite this article:
Tolani MA, Bello A, Ahmed M, Maitama HY. Symptomatic Müllerian cyst in the retroperitoneum of an adult male: Case report and review of the literature. Arch Med Surg 2019;4:53-5

How to cite this URL:
Tolani MA, Bello A, Ahmed M, Maitama HY. Symptomatic Müllerian cyst in the retroperitoneum of an adult male: Case report and review of the literature. Arch Med Surg [serial online] 2019 [cited 2024 Feb 25];4:53-5. Available from: https://www.archms.org/text.asp?2019/4/2/53/276182

  Introduction Top

Retroperitoneal cyst refers to cyst lying in the retroperitoneal fatty tissues which have no apparent connections with any adult anatomical structure except by areolar tissue.[1] It is rare, occurring in 1/5750–1/250,000 people.[2] Müllerian cyst, a subtype of urogenital cyst just like other developmental retroperitoneal cyst, is characterized by distinct etiology and morphology.[3],[4],[5] This case report reviews the presentation and management of a 20-year-old male with Müllerian cyst located in the retroperitoneum. Following the first report in a male by Alzaraa et al. in 2008, examined literature has not reported a similar case in the retroperitoneum of an adult male.[3]

  Case Report Top

A 20-year-old male presented with a 1-year history of recurrent, dull right flank pain of insidious onset, severe enough to disrupt his daily activities. There were associated anorexia and weight loss. There was no hematuria or any gastrointestinal symptom. There were no contact with a chronically coughing adult, no family history of renal mass, and no previous history of abdominal surgery. Examination revealed an asthenic young man with a tender, mobile, retroperitoneal swelling having tympanitic percussion note over it on the right flank. Abdominal ultrasound showed an ovoid-shaped, thin-walled, anechoic cyst in the right lumbar region inferior to the right kidney and anterior to the right psoas muscle with a volume of 22.28 ml [Figure 1]. Abdominopelvic computed tomography scan showed a nonenhancing, small, hypodense (HU-14) lesion posteroinferior to but clearly distinct from the right kidney [Figure 2].
Figure 1: Abdominal ultrasound scan showing a retroperitoneal cystic mass (black arrow) inferior to the right kidney

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Figure 2: Abdominal contrast computed tomography-scan showing a nonenhancing, hypodense, retroperitoneal mass, posteroinferior to the right kidney (white arrow)

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The patient had open exploration through right transcostal flank approach and complete excision of a right retroperitoneal cyst (8 cm × 6 cm × 6 cm) which was not adherent to adjacent structures [Figure 3]. Gross examination revealed an ovoid cystic mass weighing 20 grams which was unilocular. Histology revealed a cyst lined by simple columnar cells with multiple intracytoplasmic vacuoles and contained serous fluid and was reported as a Müllerian cyst. At the time of this write-up, the patient was 6 months on follow-up with no sign of a recurrence.
Figure 3: Retroperitoneal exploration via flank approach showing the cyst (black arrow)

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  Discussion Top

The occurrence of Müllerian cyst in the retroperitoneum is rare.[5] Most of the cases of the cyst located at this site were documented in females.[6] [Table 1] details previously reported cases of Müllerian cyst located in the retroperitoneum found in the English literature. It could arise there as a result of the existence of an aberrant retroperitoneal Müllerian duct fragment or from the metaplastic transformation of a peritoneal lining to a tubal-type epithelium and its subsequent invagination into retroperitoneal tissue and from ectopic ovarian tissue or by reverse menstruation or pelvic instrumentation-associated endometrial tissue implantation into the retroperitoneum.[7],[8] Our study reported the occurrence of Müllerian cyst in the retroperitoneum of a male patient in the second decade of life. Although retroperitoneal Müllerian cyst has a slight female preponderance, Alzaraa et al. also reported its occurrence in a male patient in the sixth decade of life.[3],[8] Thus, these cysts could be clinically detected at any age.
Table 1: Clinicopathological features of previously reported cases of Müllerian cyst located in the retroperitoneum found in the English literature*

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Fat aromatization in obesity and hormonal therapy can provide endogenous and exogenous estrogen stimulus which promotes the proliferation of epithelial cells of Müllerian origin.[5] This could result in the growth and subsequent clinical manifestation of the cyst.[2],[9] Steinberg et al. and Lee et al. identified these risk factors in the cases reported in their publication.[5],[10] However, the index patient did not present with the above risk factors.

Despite the tendency of this cyst to increase in volume, the potential space within the retroperitoneum has the capacity to accommodate it, limiting the early presentation of the pathology with an abdominal swelling.[2] However, other symptoms could occur from infection, hemorrhage, or rupture of the cyst; from pressure or displacement of the ureter, small bowel, and large bowel by the cyst; and from adhesions around the renal pelvis.[4],[5],[7] The index patient presented with right flank pain which could be due to complications of the cyst.

Radiological investigations confirm the retroperitoneal location and fluid-filled nature of the swelling and its relationship to adjacent organs and other important radiologic features.[3],[7],[11] This can help limit the large number of potential differential diagnoses of retroperitoneal Müllerian cyst.[12] Previous reports have highlighted the use of abdominal ultrasound and computed tomography scan in the investigation of this pathology.[2],[3],[7] These are in consonance with the imaging modality used in the current case. The presence of a unilocular or multilocular cyst with thin walls and clear fluid content raises the suspicion for the diagnosis of a Müllerian cyst on imaging.[11] However, there is an overlap in the morphology of most developmental retroperitoneal cysts, and exact preoperative diagnosis of a specific cyst subtype is possible in only 25% of cases.[2],[13]

The decision on the treatment of retroperitoneal cyst depends on the presence of symptoms, the risk of complications, and the likelihood of malignant transformation.[12] Complete excision of the cyst through an open, laparoscopic, or retroperitoneoscopic approach remains the optimal treatment of this lesion.[7],[12] The index patient had open complete excision of the cyst. Other options of management include aspiration of the cyst, external marsupialization, and internal drainage.[14]

Histopathological examination of retroperitoneal cysts is important.[12] Similar to the findings in our study, the most common epithelial cell lining of the cyst wall is cuboidal or low columnar epithelium.[15] This epithelium is similar to the lining epithelium of Fallopian tubes.[14] However, some cyst walls have been reported to have smooth muscle and loose fibrous connective tissue.[15]

Recurrence of retroperitoneal Müllerian cyst may occur following conservative treatment or incomplete removal of a cyst wall.[6] Lee et al. reported that this risk appeared to be low with complete cyst excision.[5] Our patient is presently 6 months postcomplete excision with no sign of a recurrence.

  Conclusion Top

Although there are diverse subtypes of retroperitoneal cysts, Müllerian cyst should always be kept in mind as a differential diagnosis at all stages of evaluation. Optimal treatment involves complete cyst excision often with a good prognosis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Handfield-Jones RM. Retroperitoneal cysts: Their pathology, diagnosis, and treatment. Br J Surg 1924;12:119-34.  Back to cited text no. 1
Guile MW, Fagan M, Simopolous A, Ellerkman M. Retroperitoneal cyst of müllerian origin: A case report and review of the literature. J Pelvic Med Surg 2007;13:149-52.  Back to cited text no. 2
Alzaraa A, Mousa H, Dickens P, Allen J, Benhamida A. Idiopathic benign retroperitoneal cyst: A case report. J Med Case Rep 2008;2:43.  Back to cited text no. 3
McClellan DS, Brasch J, Rifkin H. Large retroperitoneal cysts in children and adolescents. J Urol 1981;126:815-6.  Back to cited text no. 4
Lee J, Song SY, Park CS, Kim B. Müllerian cysts of the mesentery and retroperitoneum: A case report and literature review. Pathol Int 1998;48:902-6.  Back to cited text no. 5
Shayan H, Owen D, Warnock G. Surgical images: Soft tissue. Müllerian cyst of the upper abdomen: A lesion mimicking pancreatic cystadenoma. Can J Surg 2004;47:369-71.  Back to cited text no. 6
Yohendran J, Dias MM, Eckstein R, Wilson T. Benign retroperitoneal cyst of mullerian type. Asian J Surg 2004;27:333-5.  Back to cited text no. 7
de Peralta MN, Delahoussaye PM, Tornos CS, Silva EG. Benign retroperitoneal cysts of müllerian type: A clinicopathologic study of three cases and review of the literature. Int J Gynecol Pathol 1994;13:273-8.  Back to cited text no. 8
Konishi E, Nakashima Y, Iwasaki T. Immunohistochemical analysis of retroperitoneal müllerian cyst. Hum Pathol 2003;34:194-8.  Back to cited text no. 9
Steinberg L, Rothman D, Drey NW. Müllerian cyst of the retroperitoneum. Am J Obstet Gynecol 1970;107:963-4.  Back to cited text no. 10
Yang DM, Jung DH, Kim H, Kang JH, Kim SH, Kim JH, et al. Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review. Radiographics 2004;24:1353-65.  Back to cited text no. 11
Renzulli P, Candinas D. Symptomatic retroperitoneal cyst: A diagnostic challenge. Ann R Coll Surg Engl 2009;91:W9-11.  Back to cited text no. 12
Madakshira MG, Singh V. A curious case of mullerian origin retroperitoneal cyst. J Case Rep 2016;6:331-3.  Back to cited text no. 13
Rainy A, Majbar MA, Alaoui M, Raiss M, Sabbah F, Hrora A, et al. Retroperitoneal mullerian cyst: A case report. J Med Surg Res 2015;2:163-5.  Back to cited text no. 14
Schuhrke TD, Kaplan GW. Prostatic utricle cysts (müllerian duct cysts). J Urol 1978;119:765-7.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]

This article has been cited by
Nayeemunnisa Nayeemunnisa, G. Ravi
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