| Objective To study the clinical characteristics, pathological features, treatment and prognosis of intermediate trophoblastic tumor (ITT), and investigate the relevant research progress of the disease.Methods All the cases of gestational trophoblastic diseases admitted to Qilu Hospital of Shandong University during the period of 2006 to 2015 were collected. Among these cases, a retrospective analysis of nine patients diagnosed with ITT and confirmed by pathological examinations was performed. The clinical data analyzed by us included age, chief complaint, times of gravidity and parity, menstrual history, history of ectopic pregnancy, molar pregnancy and abortion, interval from last time of gravidity, preoperative β-HCG level, preoperative ultrasound and CT/MRI examination of pelvic cavity, preoperative chest X-ray/CT scan, selection of operation, postoperative pathological diagnosis, postoperative chemotherapy and follow-up record. We also reviewed relevant literatures and summarized the clinical and pathological features, treatment and prognostic factors of the disease.Results Nine patients were diagnosed with ITT among 492 cases of gestational trophoblastic diseases admitted to Qilu Hospital of Shandong University in the decade, including eight cases of placental site trophoblastic tumor (PSTT) and one epithelial trophoblastic tumor (ETT). The age of nine patients ranged from 22 to 51, all in reproductive stage, with an average age of 33.1 years old. The last gestation included abortion, term labor and hydatidiform mole. The interval between last gestation and diagnosis of ITT ranged between 5 to 32 months (data of one patient absent), with an average interval of 13.88 months. The most common chief complaint was abnormal vaginal bleeding (6/9), but intrauterine occupational disease found by examinations (1/9) and elevated level of serum β-HCG (2/9) could also be the chief complaint. The level of serum β-HCG ranged from 0.1 to 18550 mIU/ml, with an average level of 16076.9 mIU/ml. Only one patient showed a normal level of serum β-HCG, while 88.9％(8/9) of the serum β-HCG level were elevated. Imaging findings have nonspecific appearances. Preoperative ultrasound reports of eight patients were reviewed. Five of them showed enlarged uterus and three showed normal size. The eight ultrasound reports all presented abnormal intrauterine echo with blood flow signal. Preoperative pelvic MRI/CT scan was performed on six patients, all showing myometrial invasion of intrauterine tumor, with enlarged lymph nodes in pelvic cavity in one PSTT case. But there were no enlarged lymph nodes during operation, and didn’t do lymphadenectomy. The results of the chest X-ray/CT scan were normal in six cases, metastatic tumor not excluded in two cases and metastatic nodules in one case. Seven patients received hysterectomy, one of which was complicated with cervical adenocarcinoma and underwent radical hysterectomy for cervical cancer. The other two received fertility-preserving treatment, including uterine curettage and lesion resection of the uterus respectively. Postoperative pathological examination showed that the tumor cells were all derived from intermediate trophoblast cells and more than 1/3 of the myometrium was involved in most cases. Immunohistochemical analysis indicated positive expression of HPL, focally positive expression of HCG, negative expression of P63 in PSTT, with positive rate of Ki67 expression below 25%. However, positive expression of CK18 and weakly positive expression of HPL were detected in ETT. Six of the nine patients accepted combination chemotherapy and the other three didn’t receive adjuvant therapy. The combination chemotherapy included PE, BEP, EMA and FEAV, ranging from two to four courses of therapy. The postoperative follow-up indicated that one of the nine patients had died of lung and brain metastasis respectively. Six of the other eight patients were alive during the follow-up interval with no recurrence while two loss of follow-up. One patient with fertility preservation has conceived a child.Conclusions Intermediate trophoblastic tumor is rare in clinical practice, accounting for 1.83％ of all the gestational trophoblastic diseases admitted to Qilu Hospital of Shandong University. The disease can cause a diversity of clinical manifestations, among which irregular vaginal bleeding is the most common symptom. It is prone to misdiagnosis clinically. The tumor cells are derived from intermediate trophoblast cells shown in the postoperative pathological examination and the immunohistochemical analysis of the disease has its own unique features. Hysterectomy is the usual choice of treatment. If there are high risk factors, the combination chemotherapy should be performed to improve the therapeutic effects. Once the extrauterine metastasis is found, or the interval from last gestation is long, with increased mitotic index in pathology, the patient may present with a poor prognosis. |
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