Primary retroperitoneal mucinous cystadenoma - case report

INTRODUCTION

The primary retroperitoneal cystadenoma is very rare tumor, described by Handfield-Jones in 1924 and observed almost exclusively in women¹. The symptoms are usually nonspecific, hampering its differential diagnosis with other retroperitoneal masses and makes them with imaging and surgical approach for diagnosis and treatment².

The present report is of one case of a primary benign retroperitoneal mucinous cystadenoma.

CASE REPORT

Woman 51 year old referred abdominal pain since one year ago, located on the right flank and radiating to the lumbar region, with progressive worsening. Physical examination revealed a palpable mass in the right flank, painless. Was submitted to ultrasound examination which identified a bulky abdominal cystic lesion. Computed tomography (Figures 1 and 2) revealed homogeneous retroperitoneal cystic lesion measuring 15x12, 5x5, 5 cm and medially displacing the ascending colon, suggesting the diagnosis of cystic lymphangioma. The patient underwent exploratory laparotomy (Figures 3 and 4), which identified massive retroperitoneal cystic lesion which was dissected from adjacent structures with ease, allowing complete resection. The pancreas and ovaries showed no alterations or contiguity with the injury. No complications were observed during the postoperative course. Pathological examination of the surgical specimen revealed retroperitoneal mucinous cystadenoma benign (Figures 5 and 6).

Figure 1
Computed tomography of the abdomen: shows a cystic image of regular walls without mural nodules or vegetations

Figure 2
Computed tomography of the abdomen: shows massive retroperitoneal cystic lesion occupying the right flank and iliac fossa

Figure 3
Intraoperative aspect: bulky retroperitoneal cystic lesion medially displacing the ascending colon

Figure 4
Surgical aspect: cystic lesion fully and completely resected

Figure 5
The cyst wall (hematoxylin and eosin, 100x)

Figure 6
Wall of the cyst: goblet cells without tissue invasion, characterizing benign cyst (hematoxylin and eosin - 400x)

DISCUSSION

Most patients have a palpable mass, asymptomatic or accompanied by mild abdominal pain that may be associated with nonspecific gastrointestinal complaints². The differential diagnosis must be made with cystic lymphangioma, cystic teratoma, cystic neoplasms of the pancreas and ovary. The assessed by CT or MRI identifies retroperitoneal cystic lesion, but does not define its exact nature³; so, confirmation of the diagnosis can be established only after histological examination of the surgical specimen. Thus, the surgical approach is indicated for proper evaluation of the topography and resection of the lesion.

Histologically primary retroperitoneal mucinous cystadenoma can be classified in three types: benign, borderline and malignant⁴. Benign, the most common, with no recurrence after surgical resection; borderline, with adjacent proliferative columnar epithelium and small malignant potential; malignant, which can be recurrent and metastatic⁵. It shares histological similarities with ovarian mucinous cystadenoma and can be located anywhere in the retroperitoneum without connections to the ovary. The histogenesis remains incompletely understood. However, there are two main assumptions. According to the first, as there is similarity with ovarian mucinous cystadenoma, it is possible originated from an ectopic ovarian tissue⁶; however, ovarian tissue was found in the cyst wall only in few cases⁷ and have been described in men⁸. The second hypothesis suggests that they originate from an invagination of multipotent mesothelial cells with subsequent mucinous metaplasia of the mesothelial cells⁶.

Complete surgical resection of the lesion, as well as allowing adequate diagnostic evaluation, represents the best treatment⁹.

REFERENCES

• ¹
  Calo PG, Congiou A, Ferreli C, Nikolosi A, Tarquin A. Primary retroperitoneal tumors. Our experience. Minerva Chirurgica 1994;49:43-9.
• ²
  Roma AA, Malpica A: Primary retroperitoneal mucinous tumors: a clinicopathologic study of 18 cases. AmJ Surg Pathol 2009;33:526-33.
• ³
  Falidas E, Konstandoudakis S, Vlachos K, Archontovasilis F, Mathioulakis S, Boutzouvis S et al. Primary retroperitoneal mucinous cystadenoma of borderline malignancy in a male patient. Case report and review of the literature. World Journal of Surgical Oncology 2011;9:98.
• ⁴
  Navin P, Meshkat B, McHugh S, Beegan C, Leen E, Prins H, Aly S. Primary retroperitoneal mucinous cystadenoma - A case study and review of the literature. International Journal of Surgery Case Reports 3 2012;486-488.
• ⁵
  Min BW, Kim JM, Um JW, Lee ES, Son GS. Kim SJ et al. The First Case of a Retroperitoenal Mucinous Cystadenoma in Korea: A Case Report. The Korean Journal of Internal Medicine 2004;19:282-284.
• ⁶
  Matsubara M, Shiozawa T, Tachibana R, Hondo T, Osasda K, Kawaguchi K, et al. Primary retroperitoneal mucinous cystadenoma of borderline malignancy: a case report and review of the literature. International Journal of Gynecological Pathology 2005;24:218-23.
• ⁷
  Pennell TC, Gusdon Jr JP. Retroperitoneal mucinous cystadenoma. American Journal of Obstetrics and Gynecology 1989;160:1229-31.
• ⁸
  Lai KKT, Chan YYR, Chin ACW, Ng WF, Huang YHH, Mak YLM, et al. Primary retroperitoneal mucinous cystadenoma in a 52-year-old man. Journal of Hong Kong College Of Radiologists 2004;7:223-5.
• ⁹
  Tapper EB, Shrewsberry AB, Oprea G, Majmudar B. A unique benign mucinous cystadenoma of the retroperitoneum: a case report and review of the literature. Archives of Gynecology and Obstetrics 2010;281:167-9.

• Financial source: none

Publication Dates

• Publication in this collection
  Jul-Sep 2014

History

• Received
  17 June 2013
• Accepted
  13 May 2014