primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor with only about 30 reported cases in the english literature .
surgical exploration is needed for the diagnosis and treatment , and many authors recommend extensive excision including total hysterectomy and bilateral salpingo - oophorectomy with enucleation of retroperitoneal tumor1 - 9 ) .
adjuvant chemotherapy is sometimes administered following complete surgical excision4 , 6 , 7 , 10 , 11 ) . however , the most desirable treatment for this rare tumor is still controversial .
we report here on a patient who had tumor excision and adjuvant chemotherapy , and the patient is without evidence of recurrence 42 months after surgery .
a 32-year - old married woman was referred to the surgical unit because of a self - palpable mass in her left lower abdomen for over 1 month and the abdominal pain for the recent several days .
physical examination revealed a slightly tender , ill - defined mass about 10 cm in size over the left lower abdomen .
the laboratory tests , including the complete blood count , the chemistry profile , urinalysis and chest x - ray , were all within normal limits .
abdominopelvic computed tomography scanning revealed a huge unilocular cystic mass with an enhancing solid portion , and this was probably located in the retroperitoneal space ( figure 1a , 1b ) .
laparotomy revealed a grossly normal uterus , 2 ovaries , 2 fallopian tubes and an appendix .
there were no abnormal findings in the liver , stomach , small bowel , large bowel and spleen .
a large , encapsulated cystic mass about 10 cm in diameter was found in the retroperitoneum .
the descending colon was displaced medially to the mass , and no ascites was found .
the inner surface of the cyst was smooth except for three solid mural nodules that were 0.8 , 1 and 1.5 cm in diameter , respectively .
microscopically , the cyst was lined by atypical columnar cells of mucinous cystadenocarcinoma ( figure 2a , 2b ) .
the solid mural nodules showed highly pleomorphic spindle cells and anaplastic giant cells with numerous atypical mitosis , and there were intervening atypical glands of adenocarcinoma ( figure 3a ) .
the spindle and giant cells were positive for cytokeratin , vimentin and smooth muscle actin , but negative for s-100 protein and desmin ( figure 3b , 3c ) .
the carcinoma cells were positive for cytokeratin and they were negative for vimentin , smooth muscle actin , s-100 protein and desmin .
the diagnosis of the mass was primary retroperitoneal mucinous cystadenocarcinoma with mural nodules of sarcomatoid change .
the serum tumor markers were not checked before tumor excision . at 10 days after operation ,
the serum concentration of ca125 was elevated ( 76.95 iu / ml ) , which decreased to within normal limits 3 months later , and the serum concentration of cea was normal .
adjuvant chemotherapy with six courses of cyclophosphamide 600 mg / m and cisplatin 60 mg / m was administered to the patient .
there was no evidence of recurrence on the follow - up abdominopelvic computed tomography 42 months after the operation .
the first case was described in 1977 by roth et al.12 ) , and about 30 cases have been reported in the english medical literature to date .
several hypotheses have been suggested to explain the histogenesis of primary retroperitoneal mucinous cystic neoplasm , and they are heterotopic or supernumerary ovary11 , 12 ) , retroperitoneal teratoma13 ) , intestinal duplication and coelomic metaplasia . to date , the hypothesis that has gained increasing support is coelomic metaplasia , that is , retroperitoneal mucinous cystadenocarcinomas arise from invaginations of the peritoneal mesothelium , with subsequent mucinous metaplasia1 , 3 , 4 , 5 , 12 , 14 - 16 ) .
the age at diagnosis ranges from 17 to 86 years and only 2 of the reported cases were male15 ) .
the patients usually visit the hospital with a complaint of abdominal pain or a palpable mass .
radiologic tests including ultrasonography , computed tomography or magnetic resonance imaging are used to localize the tumor and evaluate its nature .
the papillary nodules demonstrated within a cyst by ultrasonography or computed tomography have been reported to suggest malignancy16 ) .
however , it is often difficult to differentiate a benign from a malignant neoplasm , or even to determine the origin site of the tumor with using the preoperative radiologic images7 ) .
motoyama et al . suggested that the presence of glandular epithelial cells and high levels of cea in the cystic fluid was very useful for making the diagnosis15 ) .
the immunohistochemical findings of this neoplasm resemble those of its ovarian counterpart ; thus , a large number of authors recommend management according to the treatment protocol for ovarian neoplasms , including the staging procedure3 , 7 ) .
although almost all the cases , except the one reported by uematsu16 ) were diagnosed in the early stage , there have been many relapses cases in the literature6 , 10 - 14 ) .
roth et al . reported on a patient who had undergone only tumor excision and he died of spreading disease 6 months postoperatively12 ) .
two patients received tumor excision and adjuvant chemotherapy , but one patient had a paraovarian recurrence 21 months after surgery10 ) and another patient died of disease spread 4 months after surgery11 ) .
the patient with histologically borderline malignancy and who was reported on by banerjee underwent tumor excision , left salpingo - oophorectomy and descending colon resection , and mediastinal metastasis developed 4 years later14 ) .
mikami et al . noted a patient who experienced rupture of the tumor capsule during the operation with subsequent peritoneal tumor implantation , and the patient died 18 months after the initial surgery6 ) .
for the korean case reported on by song et al . , the tumor was removed laparoscopically after cyst aspiration .
five of the reported recurrent cases did n't undergo extensive surgical excision , including hysterectomy and bilateral salpingo - oophorectomy 10 - 14 ) .
hysterectomy and bilateral salpingo - oophorectomy were conducted after tumor excision in about half of the reported cases1 - 9 ) , and these case were alive without evidence of recurrence 3 to 36 months postoperatively , except for one case6 ) .
so , extensive exploratory laparotomy , including removal of both ovaries and the uterus with extirpation of the retroperitoneal tumor , may improve the prognosis and prevent recurrence1 , 2 , 5 , 11 , 17 ) .
the resected genital organs in several reports showed no evidence of tumor involvement or infiltration1 - 5 , 7 - 9 ) .
the only three of all the reported cases showed long - term survival ( > 5 years ) of 6 years , 5 years and 5 years , respectively , and these cases had not undergone hysterectomy and bilateral salpingo - oophorectomy16 , 18 , 19 ) .
law et al . recommended only tumor excision for young women who have no evidence of disease dissemination and who hope to stay fertile19 ) .
six cases , including the present case , have reported tumor that had one or more mural nodules6 , 8 , 9 , 11 , 12 ) , and these nodules satisfied the diagnostic criteria presented by baergen .
mikami et al . reported a patient with primary retroperitoneal mucinous cystadenocarcinoma with a mural nodule , and this nodule was composed of pleomorphic sarcomatoid cells with mitotic figures6 ) .
the authors commented that intraoperative rupture of the cyst in this case may have resulted in its peritoneal dissemination and recurrence .
two patients with mural nodules died of disease at 4 months and 6 months postoperatively , respectively11 , 12 ) .
cases with a mural nodule may have an aggressive prognosis like ovarian cancers with a mural nodule ; therefore , all mural nodules warrant careful histological assessment and careful postoperative follow - up6 , 8) . although our patient was diagnosed as having a neoplasm with mural nodules of sarcomatoid change , she is doing well without evidence of recurrence 42 months postoperatively .
cystic tumor rupture during operation may occur , and malignant cells may exist in the spilled fluid ; this results in peritoneal dissemination . among the patients with cystic rupture4 , 6 ) , one patient died 18 months postoperatively6 ) .
in contrast , another patient reported by tenti et al . was free of tumor 33 months postoperatively , and that patients underwent adjuvant chemotherapy4 ) . in the literatures ,
adjuvant chemotherapy was performed in 5 patients after tumor resection4 , 6 , 7 , 10 , 11 ) .
one of them underwent cytoxan chemotherapy for 21 months , but paraovarian recurrence developed10 ) .
2 other patients died 4 months and 18 months after surgery , respectively , of widespread metastasis6 , 11 ) .
the advantage of adjuvant chemotherapy has not been proved , and tumor might be hard to cure when it recurs , but patients may have benefit from chemotherapy if the cyst ruptures or if extracystic extension is present16 ) . in the present case , tumor resection and adjuvant chemotherapy were performed .
capsular rupture did not occur , but 3 mural nodules of sarcomatoid change were observed . the patient has no evidence of recurrence on follow - up computed tomography and according to the level of tumor marker .
we recommend careful histologic examination of the excised specimen and if possible , aggressive management with extensive surgical excision and adjuvant chemotherapy to prevent recurrence , and consideration must be given to the patient 's age , the extent of disease and the histologic findings .
primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor and in all reported cases , the duration of follow - up was not more than 5 years .
long - term follow - up is essential to evaluate the course and prognosis of this disease .
 primary retroperitoneal mucinous cystadenocarcinoma is a rare tumor . 
 only about 30 such cases have been reported in the worldwide literature , and a few korean cases have been reported . 
 the pathogenesis is not clear , and coelomic metaplasia of the retroperitoneal mesothelium has gained wide support . 
 there is no consensus on the appropriate treatment , but surgical exploration is needed for the diagnosis and treatment , and adjuvant chemotherapy may be recommended following complete surgical excision . 
 the long - term prognosis has not been established.we report here on a 32-year - old woman who was diagnosed as having a retroperitoneal mucinous cystadenocarcinoma with mural nodules of sarcomatoid change . 
 tumor excision and adjuvant chemotherapy were done and the patient is doing well without any evidence of recurrence at 42 months postoperatively . 
INTRODUCTION
CASE REPORT
DISCUSSION