Home > F. Pathology by regions > Abdomen > Retroperitoneum > retroperitoneal mucinous cystadenoma

retroperitoneal mucinous cystadenoma

Thursday 2 October 2008

Primary retroperitoneal mucinous cystadenomas (RMCs) are very rare, even though mucinous cystadenomas are frequent ovarian tumors. Like most retroperitoneal masses, they cause symptoms when growing large enough to exert pressure or obstructive effect on adjacent organs. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established.

The most common type of retroperitoneal mucinous tumors is the RMC, which shares a histological similarity to ovarian mucinous cystadenomas but can arise at any location in the retroperitoneum without attachment to the ovary.

Histogenesis

Because of the limited number of reported cases, the biological behavior and histogenesis of such tumors remain speculative. Laboratory studies and imaging methods cannot achieve an accurate preoperative diagnosis.

The histogenesis of primary RMCs remains unclear. Three main theories have been proposed to explain the histogenic origin of mucinous cystadenomas in the retroperitoneum.

These tumors can arise from teratoma, heterotopic ovarian tissue, or mucinous metaplasia of the mesothelial lining cells.

Primary RMCs occur exclusively in women, although three cases of RMC in men have been reported in the literature. Some authors questioned the diagnosis of these lesions as benign because all three patients died of the disease.

Furthermore, Subramony et al. reported that the estrogen receptor is positive in stromal cells of a RMC, which could explain the exclusive occurrence of these tumors in women.

Literature review

In a literature review using Medline starting in 1970 (Reference), a total of 19 cases of primary RMCs in the English literature have been found. Based on these cases, including the present one, it was found that all cases were women, with an age range of 14 to 85 years. The size of reported tumors ranged from 7 cm to 30 cm. There was no relationship between the age of patients and the size of tumors. The symptoms were nonspecific and most of the patients complained of asymptomatic mass or abdominal discomfort. The preoperative diagnosis was mesenteric cyst in 4 cases, ovarian cyst in 3 cases, and retroperitoneal cystic tumor in one case. Interestingly, preoperative diagnosis of renal cyst was considered in 2 cases. Serum levels of tumor markers were normal in 4 cases. However, two cases demonstrated a slight elevation of CA199 and CA125 levels, respectively. There was no evidence of recurrence after surgical management in 9 patients.

Preoperative diagnosis of primary RMCs is reportedly very difficult due to a lack of pathognomonic clinical features. Based on the review of cases reported in the English literature, most patients presented with asymptomatic mass and vague abdominal discomfort.

Reported tumors were relatively large, which may be large enough to evoke clinical symptoms or perceived by the patients. However, no cases examined presented with severe abdominal pain. Laboratory studies, including serum tumor markers and cytology study of cystic fluid are not helpful in making diagnosis of the tumors.

However, Motoyama et al. reported that measurement of CEA level in the cystic fluid may be useful in making the diagnosis. With regard to the imaging characteristics of RMCs, these tumors usually manifest as homogenous unilocular cystic masses at CT of the abdomen.

Furthermore, displacement of colon, kidney or ureter may suggest the retroperitoneal location of tumors.

Managment

As for the management of primary RMCs, complete surgical excision is recommended to eliminate the risk of infection, recurrence, and malignant degeneration, although these tumors seem to behave in a benign fashion with no recurrences after surgical removal, as demonstrated in our study. Exploratory laparotomy with complete enucleation of the cyst is traditionally indicated, although successful laparoscopic excision of a primary RMC has been reported.

References

- Primary retroperitoneal mucinous cystadenoma: Report of a case and review of the literatureSheng-Lei Yan, Ho Lin, Chien-Long Kuo, Hurng-Sheng Wu, Ming-Ho Huang, and Yueh-Tsung Lee. World J Gastroenterol. 2008 October 7; 14(37): 5769–5772. (Free)

- Tamura T, Yamataka A, Murakami T, Okada Y, Kobayashi H, Ohshiro K, Lane GJ, Miyano T. Primary mucinous cystadenoma arising from behind the posterior peritoneum of the descending colon in a child: a case report.Asian J Surg. 2003 Oct;26(4):237-9. PMID: 14530114

- Lai EC, Chung KM, Lau WY. Primary retroperitoneal mucinous cystadenoma. ANZ J Surg. 2006;76:537.

- Arribas D, Cay A, Latorre A, Cordoba E, Martinez F, Lagos J. Retroperitoneal mucinous cystadenoma. Arch Gynecol Obstet. 2004;270:292–293.

- Isse K, Harada K, Suzuki Y, Ishiguro K, Sasaki M, Kajiura S, Nakanuma Y. Retroperitoneal mucinous cystadenoma: report of two cases and review of the literature. Pathol Int. 2004;54:132–138.

- Subramony C, Habibpour S, Hashimoto LA. Retroperitoneal mucinous cystadenoma. Arch Pathol Lab Med. 2001;125:691–694.

- Pennell TC, Gusdon JP Jr. Retroperitoneal mucinous cystadenoma. Am J Obstet Gynecol. 1989;160:1229–1231.

- Williams PP, Gall SA, Prem KA. Ectopic mucinous cystadenoma. A case report. Obstet Gynecol. 1971;38:831–837.

- Banerjee R, Gough J. Cystic mucinous tumours of the mesentery and retroperitoneum: report of three cases. Histopathology. 1988;12:527–532.

- Balat O, Aydin A, Sirikci A, Kutlar I, Aksoy F. Huge primary mucinous cystadenoma of the retroperitoneum mimicking a left ovarian tumor. Eur J Gynaecol Oncol. 2001;22:454–455.

- Chen JS, Lee WJ, Chang YJ, Wu MZ, Chiu KM. Laparoscopic resection of a primary retroperitoneal mucinous cystadenoma: report of a case. Surg Today. 1998;28:343–345.

- Hansmann G, Budd J. Massive Unattached Retroperitoneal Tumors: An Explanation of Unattached Retroperitonel Tumors Based on Remnants of the Embryonic Urogenital Apparatus. Am J Pathol. 1931;7:631–674.

- de Peralta MN, Delahoussaye PM, Tornos CS, Silva EG. Benign retroperitoneal cysts of mullerian type: a clinicopathologic study of three cases and review of the literature. Int J Gynecol Pathol. 1994;13:273–278.

- Rothacker D, Knolle J, Stiller D, Borchard F. Primary retroperitoneal mucinous cystadenomas with gastric epithelial differentiation. Pathol Res Pract. 1993;189:1195–1204.

- Bortolozzi G, Grasso A, Zasso B. Mucinous cystadenoma of the retroperitoneum. A case report and review. Eur J Gynaecol Oncol. 1995;16:65–68.

- Yunoki Y, Oshima Y, Murakami I, Takeuchi H, Yasui Y, Tanakaya K, Konaga E. Primary retroperitoneal mucinous cystadenoma. Acta Obstet Gynecol Scand. 1998;77:357–358.

- Tamura T, Yamataka A, Murakami T, Okada Y, Kobayashi H, Ohshiro K, Lane GJ, Miyano T. Primary mucinous cystadenoma arising from behind the posterior peritoneum of the descending colon in a child: a case report. Asian J Surg. 2003;26:237–239.

- Min BW, Kim JM, Um JW, Lee ES, Son GS, Kim SJ, Moon HY. The first case of primary retroperitoneal mucinous cystadenoma in Korea: a case report. Korean J Intern Med. 2004;19:282–284.

- Sheen-Chen SM, Eng HL. Retroperitoneal mucinous cystadenoma. Dig Dis Sci. 2006;51:752–753.

- Kehagias DT, Karvounis EE, Fotopoulos A, Gouliamos AD. Retroperitoneal mucinous cystadenoma. Eur J Obstet Gynecol Reprod Biol. 1999;82:213–215.

- Ginsburg G, Fraser J, Saltzman B. Retroperitoneal mucinous cystadenoma presenting as a renal cyst. J Urol. 1997;158:2232.

- Yang DM, Jung DH, Kim H, Kang JH, Kim SH, Kim JH, Hwang HY. Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review. Radiographics. 2004;24:1353–1365.

- Motoyama T, Chida T, Fujiwara T, Watanabe H. Mucinous cystic tumor of the retroperitoneum. A report of two cases. Acta Cytol. 1994;38:261–266.