hepatoid adenocarcinomas are tumors that arise outside the liver but which resemble hepatic tissue and produce alpha - fetoprotein ( afp ) . hepatoid adenocarcinomas have been identified in many sites , including the stomach , lung , rectum , renal pelvis , ovary , uterus , and vagina . until now , only one case of hepatoid adenocarcinoma of uterine corpus carcinosarcoma and elevated serum afp has been reported . we report two cases of afp - producing hepatoid adenocarcinomas of uterine corpus carcinosarcoma with rhabdoid differentiation , and discuss the pathologic features and clinical significance of this pattern of differentiation in uterine corpus tumors . a 63-year - old multiparous woman , gravida 4 , para 3 , complained of abnormal vaginal bleeding of 2 months ' duration . vaginal ultrasonogram and magnetic resonance imaging showed a polypoid mass of 14  10 cm in size in the uterus , possibly representing a carcinosarcoma or leiomyosarcoma . the patient underwent a total abdominal hysterectomy , bilateral salpingo - oophorectomy and pelvic lymphadenectomy . the resected enlarged uterus , weighing 560 g and measuring 14  12  7 cm , had a smooth glistening surface . the polypoid tumor originated from the uterine cavity when the anterior wall of the uterus was opened . microscopic examination showed that the polypoid tumor was a mixture of carcinomatous and sarcomatous components ( fig . the tumor had invaded the uterine cervix , but no metastases were found in the dissected lymph nodes . the large pleomorphic cells were positive for desmin , actin , cd10 , myod1 , and myoglobin and could be regarded as being rhabdomyosarcoma . the tumor was histopathologically diagnosed as heterologous uterine corpus carcinosarcoma associated with afp - producing hepatoid adenocarcinoma . postoperatively , the patient received six courses of chemotherapy with paclitaxel ( 175 mg / m ) and carboplatin ( auc 5 ) . after the third course of chemotherapy , the patient 's serum afp level decreased to 4.5 ng / ml . the patient is alive with no evidence of recurrence or increase in the serum afp level in the 2 years since treatment . an 82-year - old woman , gravida 0 , para 0 , she had undergone surgery due to breast cancer at the age of 68 . on pelvic examination , portio vaginalis could not be detected , so the endometrial pap smear could not be done . magnetic resonance imaging revealed an enhanced mass , 8 cm in size , in the uterine corpus cavity , possibly representing malignant tumor . the serum afp level was elevated ( 401 ng / ml ) , but no elevation of other markers , such as carcinoembryonic antigen or carbohydrate antigen 125 , was observed . a total abdominal hysterectomy with bilateral salpingo - oophorectomy and pelvic washings was carried out . a partially necrotic polyp , 8 cm in size , was present within the endometrial cavity , which infiltrated into the myometrium for about two - thirds of its thickness but did not invade the cervix . the carcinomatous component was an endometrioid adenocarcinoma with trabecular hepatoid cells , mostly showing hepatocellular carcinoma - like proliferation . immunohistochemistry showed focal positivity of tumor cells with desmin , myod1 , and cd10 . on pathologic examination , the tumor was interpreted as being a heterologous uterine corpus carcinosarcoma associated with afp - producing hepatoid adenocarcinoma . we did not carry out any adjuvant chemotherapy or irradiation because of the patient 's advanced age , in addition , she and her family refused these therapies . two months after the surgery , the patient 's serum afp level decreased to a normal level . however , multiple lung metastases with a high - serum afp level reappeared and the patient died 1 year after the operation . a 63-year - old multiparous woman , gravida 4 , para 3 , complained of abnormal vaginal bleeding of 2 months ' duration . vaginal ultrasonogram and magnetic resonance imaging showed a polypoid mass of 14  10 cm in size in the uterus , possibly representing a carcinosarcoma or leiomyosarcoma . the patient underwent a total abdominal hysterectomy , bilateral salpingo - oophorectomy and pelvic lymphadenectomy . the resected enlarged uterus , weighing 560 g and measuring 14  12  7 cm , had a smooth glistening surface . the polypoid tumor originated from the uterine cavity when the anterior wall of the uterus was opened . microscopic examination showed that the polypoid tumor was a mixture of carcinomatous and sarcomatous components ( fig . the tumor had invaded the uterine cervix , but no metastases were found in the dissected lymph nodes . the large pleomorphic cells were positive for desmin , actin , cd10 , myod1 , and myoglobin and could be regarded as being rhabdomyosarcoma . the tumor was histopathologically diagnosed as heterologous uterine corpus carcinosarcoma associated with afp - producing hepatoid adenocarcinoma . postoperatively , the patient received six courses of chemotherapy with paclitaxel ( 175 mg / m ) and carboplatin ( auc 5 ) . after the third course of chemotherapy , the patient 's serum afp level decreased to 4.5 ng / ml . the patient is alive with no evidence of recurrence or increase in the serum afp level in the 2 years since treatment . an 82-year - old woman , gravida 0 , para 0 , was referred to our hospital with vaginal bleeding of 2 months ' duration . she had undergone surgery due to breast cancer at the age of 68 . on pelvic examination , portio vaginalis could not be detected , so the endometrial pap smear could not be done . magnetic resonance imaging revealed an enhanced mass , 8 cm in size , in the uterine corpus cavity , possibly representing malignant tumor . the serum afp level was elevated ( 401 ng / ml ) , but no elevation of other markers , such as carcinoembryonic antigen or carbohydrate antigen 125 , was observed . a total abdominal hysterectomy with bilateral salpingo - oophorectomy and pelvic washings was carried out . a partially necrotic polyp , 8 cm in size , was present within the endometrial cavity , which infiltrated into the myometrium for about two - thirds of its thickness but did not invade the cervix . the carcinomatous component was an endometrioid adenocarcinoma with trabecular hepatoid cells , mostly showing hepatocellular carcinoma - like proliferation . on pathologic examination , the tumor was interpreted as being a heterologous uterine corpus carcinosarcoma associated with afp - producing hepatoid adenocarcinoma . we did not carry out any adjuvant chemotherapy or irradiation because of the patient 's advanced age , in addition , she and her family refused these therapies . two months after the surgery , the patient 's serum afp level decreased to a normal level . however , multiple lung metastases with a high - serum afp level reappeared and the patient died 1 year after the operation . they defined hepatoid adenocarcinoma as afp - producing carcinoma with a mixture of tubular or papillary adenocarcinoma , with abundant , eosinophilic cytoplasm and centrally located nuclei . hepatoid adenocarcinoma has mostly been found in the stomach , but also occurs in many other organs , including the uterus and ovaries . afp - producing tumors other than hepatoma and germ cell tumors have been widely reported , especially in hepatoid adenocarcinoma . in uterine corpus carcinosarcoma six cases of hepatoid adenocarcinoma of the uterine corpus have been reported in the literature . four cases were categorized as endometrial carcinoma [ 2 , 3 , 4 , 5 ] and two as carcinosarcoma [ 6 , 7 ] . microscopically , in both our cases , the tumor cells were composed of hepatoid adenocarcinoma and sarcoma components including rhabdomyosarcoma . our two cases are also examples of hepatoid adenocarcinomas of uterine corpus carcinosarcoma , because of the close resemblance of the neoplastic cells to hepatoid cells and because of the afp production . in the present cases judging from the changes in serum afp values , the afp content of the tumor and the immunohistochemical localization of afp in the tumor tissue , afp was produced by the tumor . in our cases , a close correlation was noted between shrinkage of the pelvic tumor and declining afp levels . the world health organization classification of uterine mesenchymal tumors places rhabdomyosarcoma in the category of mesenchymal tumors composed of heterologous elements . we consider that immunohistochemical support is necessary for a definitive diagnosis of rhabdomyosarcoma . in our cases , desmin was positive . it is a muscle marker and does not help distinguish between a rhabdomyosarcoma and a leiomyosarcoma . this provides unequivocal evidence that the two neoplasms in this report exhibit skeletal muscle differentiation . to date , four cases of uterine carcinosarcoma with rhabdoid features have been reported [ 6 , 9 , 10 ] . all four patients were initially treated surgically with subsequent chemotherapy . in the three cases where clinical follow - up was available , two patients died of disease . carcinosarcoma of the uterine corpus is a rare and aggressive neoplasm that contains both carcinomatous and sarcomatous histologic elements . carcinosarcoma of the uterine corpus is characterized by a high incidence of advanced stage at diagnosis and high recurrence rates after local therapy . when advanced or recurrent disease is diagnosed , controlling it is difficult and chemotherapy is minimally successful . some drugs that have been examined as single - agent therapy options are as follows : adriamycin , ifosfamide , cisplatin , and paclitaxel . paclitaxel is a novel antineoplastic agent derived from the bark of the pacific yew tree , taxus brevifolia . the gynecologic oncology group has tested paclitaxel in a variety of studies and has reported activity in ovarian cancer and adenocarcinoma of the endometrium , as well as in squamous and nonsquamous cancers of the cervix . the combination of paclitaxel and carboplatin demonstrated higher activity than that found in previous studies in patients with advanced or recurrent uterine carcinosarcoma . in our first case , adjuvant chemotherapy comprising paclitaxel and carboplatin was administered after surgery , and 2 years after the end of treatment , the patient is still disease - free . we described two cases of carcinosarcoma of the uterine corpus with afp - producing hepatoid adenocarcinoma . further studies with clinical follow - up are necessary to determine the best treatment modalities .