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 .
 we report two cases of uterine carcinosarcoma associated with alpha - fetoprotein ( afp)-producing hepatoid adenocarcinoma . 
 samples were obtained from two women aged 63 and 82 years . 
 serum afp levels of the two samples were 10,131 and 401 ng / ml , respectively . 
 histologically , in both cases the tumor cells were composed of hepatoid adenocarcinoma component and sarcoma component including rhabdomyosarcoma . 
 immunohistochemical analyses revealed that afp was expressed in the cytoplasm of the carcinomatous component . 
 after surgery , the patients received six courses of carboplatin / paclitaxel chemotherapy , and the serum levels of afp decreased to normal range . 
 the first patient is alive and well at the 2-year follow - up , while the second patient died of disease 1 year after initial operative treatment . 
 this is , to our knowledge , the second report of carcinosarcoma of the uterine corpus with afp - producing hepatoid adenocarcinoma , as proven by immunohistochemical analyses . 
Introduction
Case Reports
Case 1
Case 2
Discussion