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.
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.
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.
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.
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)
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