A case of primary retroperitoneal mucinous cystadenocarcinoma treated with fertility-sparing surgery

Abstract

Primary retroperitoneal mucinous cystadenocarcinoma is an extremely rare tumor. Preoperative diagnosis is very diffi cult and the treatment remains controversial. A 37-year-old Korean woman (gravida 0) presented with a huge abdominal mass. Computed tomography scan revealed an 18 × 11 cm sized unilocular cyst with irregular wall thickening and solid component at right adnexa. Serum CA 19-9 was slightly elevated (37.05 U/mL). At laparotomy, a huge right retroperitoneal cystic tumor originating from right paracolic gutter was found. Frozen section of the cystic tumor revealed a mucinous cystadenocarcinoma. Because the patient wished to remain fertile, fertility sparing surgery was performed. Microscopically, no evidence of metastasis was found and no further treatment was given. Six months after surgery, she has no evidence of recurrence. Fertility-sparing surgery should be considered for women with primary retroperitoneal mucinous cystadenocarcinoma, who wish to remain fertile. Copyright © 2012. Korean Society of Obstetrics and GynecologyPrimary retroperitoneal mucinous cystadenocarcinoma is an extremely rare tumor. To date, about 50 cases have been reported [1]. The histogenesis of this unusual neoplasm is not clear. In the majority of cases, preoperative diagnosis is not possible because radiologic study such as ultrasonography or computed tomography (CT) scan is not able to distinguish the exact origin of the lesion. Due to its rarity, the treatment of primary retroperitoneal mucinous cystadenocarcinoma remains controversial. Most of these cases were treated with radical surgery, including total hysterectomy and bilateral salpingo-oophorectomy with enucleation of retroperitoneal tumor [2]. But, fertility-sparing surgery also have been adopted for the treatment of primary retroperitoneal mucinous cystadenocarcinoma and showed good prognosis [1,2]. Here, we report on a case of primary retroperitoneal mucinous cystadenocarcinoma treated with fertility-sparing surgery. Case ReportA 37-year-old Korean woman (gravida 0) presented with a huge abdominal mass. The patient reported that she had had abdominal discomfort since 1 month ago and visited local clinic. On abdominal ultrasonography, a huge abdominal mass was detected and she was referred to department of gynecology. Her previous menstrual history was regular and past medical history was noncontributory. On physical examination, her abdomen was markedly distended. CT scan revealed an 18×21 cm sized unilocular cyst with irregular wall thickening and solid component at right adnexa (Fig. 1). Neither enlarged regional lymph node nor ascites was found. Hydronephrosis of right kidney and multiple uterine myomas were found. Laboratory analyses showed normal blood counts and nor-CASE REPORT Korean J Obstet Gynecol 2012;55(6):424-428 http://dx