Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Placental site trophoblastic tumor (PSTT) is a challenging rare variant of gestational trophoblastic disease (GTD) with variable characteristics. Historically, it was first described in 1895 and was considered a benign lesion until Scully and Young recognized its malignant potential in 1981. Current knowledge related to PSTT is largely based on the experience of handling this disease in established trophoblastic disease centers and on the experience of authors who reported small series or singular cases. In contrast to other forms of GTD, it arises from the implantation-site intermediate trophoblast, produces less beta-hCG and is less sensitive to chemotherapy. More than half of the patients present with disease confined to the uterus, whereas the remainder present with disease extension beyond the uterus. Because of the relative insensitivity to chemotherapy, simple hysterectomy is the mainstay of treatment. While the outcome of patients with disease confined to the uterus is usually excellent, most patients with disease extension beyond the uterus experience progression of disease and die despite surgery and aggressive chemotherapy. Other important adverse prognostic factors are interval from antecedent pregnancy > 2 years, age > 40 years and mitotic count > 5 mf/10 HPF Although the ideal chemotherapy regimen for PSTT has yet not been established, it seems that the EP/EMA regimen is the most effective first-line chemotherapy available to date for metastatic and relapsing PSTT. Although PSTT produces less hCG than choriocarcinoma, beta-hCG is still the best available serum marker to follow the disease and treatment course of PSTT.
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PMID:Placental site trophoblastic tumor--a challenging rare entity. 1729 May 81

Placental site trophoblastic tumor (PSTT) is a rare variant of gestational trophoblastic disease that originates from the implantation site intermediate trophoblast. We report four patients with PSTT and review pertinent literature. Three patients presented with disease confined to the uterus and one patient with disease extension beyond the uterus. Antecedent pregnancy was full-term pregnancy in three patients and termination of a 21-week pregnancy in one patient. Interval from the antecedent pregnancy was <1 year in three patients and 13 years in one patient. Primary treatment was simple hysterectomy in three patients and radical hysterectomy in one patient. Overall, three patients received chemotherapy; one had EP/EMA as adjuvant chemotherapy, one had EMA/CO for rising levels of serum beta-hCG and one had BEP then VIP for recurrent disease. The three patients with disease confined to the uterus have remained after treatment alive and with no evidence of disease, whereas the one patient with disease extension beyond the uterus died of disease despite surgery and aggressive chemotherapy. It is concluded that disease extension beyond the uterus is the most important adverse prognostic factor. Other adverse prognostic factors are interval from antecedent pregnancy >2 years, age >40 years, and mitotic count >5 mitotic figures/10 high-power fields. Because of the relative insensitivity to chemotherapy, hysterectomy is the mainstay of treatment. EP/EMA seems to be the most effective first-line chemotherapy available to date for metastatic and relapsing PSTT.
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PMID:Placental site trophoblastic tumor: report of four cases and review of literature. 1729 Dec 63

Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic disease (GTD) that originates from the implantation site intermediate trophoblast. It accounts for about 1% of all GTDs, with an estimated incidence of 1 per 100,000 pregnancies. Most patients are in their thirties and the prevailing presenting symptom is abnormal vaginal bleeding. More than half of the patients present with disease limited to the uterus and the remainder present with disease extension beyond the uterus. The overall mortality rate is 25%. The most important adverse prognostic factor is disease extension beyond the uterus. Other adverse prognostic factors are interval from antecedent pregnancy > 2 years, mitotic count > 5 mitotic figures/10 high-power fields, and age > 40 years. Since PSTT is less sensitive to chemotherapy than GTDs originating from cytotrophoblast and syncytiotrophoblast (hydatidiform mole, invasive mole, and choriocarcinoma), hysterectomy is the mainstay of treatment. Systemic multi-agent chemotherapy is administered in the presence of disease extension beyond the uterus and considered in the presence of other adverse prognostic factors. The EP/EMA regimen seems to be the most effective chemotherapy available to date for PSTT. Although PSTT produces less human chorionic gonadotropin (hCG) than choriocarcinoma, beta-hCG is still the best available serum marker for monitoring the response to treatment and for follow-up.
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PMID:[Placental site trophoblastic tumor]. 1729 52

Placental site trophoblastic tumor (PSTT) is a rare neoplasm that rises from intermediate trophoblasts and commonly presents with low and variable concentration of HCG immunoactivity in serum, which can be difficult to differentiate from early stage choriocarcinoma/gestational trophoblastic neoplasm (GTN) or quiescent gestational trophoblastic disease. PSTT can occur after a normal pregnancy, spontaneous abortion, termination of pregnancy, ectopic or molar pregnancy. There is a wide clinical spectrum of presentation and behavior ranging from a benign condition to an aggressive disease with fatal outcome. Nontrophoblastic malignancies such as germ cell tumors or other tumors secreting low HCG must also be considered in the differential diagnosis. Because treatments for these conditions are different, a means of differentiating PSTT from other diagnoses is important. Surgery is the cornerstone of treatment. Chemotherapeutic regimen should be EMA/CO for first line chemotherapy; EMA/EP should be used in EMA/CO refractory cases. This article reviews the literatures on this rare but fatal disease.
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PMID:Placental site trophoblastic tumor. 1770 27

This study was designed to analyze the outcomes of chemotherapy for high-risk gestational trophoblastic neoplasia (GTN) with EMA-CO regimen as primary and secondary protocol in China. Fifty-four patients with high-risk GTN received 292 EMA/CO treatment cycles between 1996 and 2005. Forty-five patients were primarily treated with EMA-CO, and nine were secondarily treated after failure to other combination chemotherapy. Adjuvant surgery and radiotherapy were used in the selected patients. Response, survival and related risk factors, as well as chemotherapy complications, were retrospectively analyzed. Thirty-five of forty-five patients (77.8%) receiving EMA-CO as first-line treatment achieved complete remission, and 77.8% (7/9) as secondary treatment. The overall survival rate was 87.0% in all high-risk GTN patients, with 93.3% (42/45) as primary therapy and 55.6% (5/9) as secondary therapy. The survival rates were significantly different between two groups (chi(2)= 6.434, P =0.011). Univariate analysis showed that the metastatic site and the number of metastatic organs were significant risk factors, but binomial distribution logistic regression analysis revealed that only the number of metastatic organs was an independent risk factor for the survival rate. No life-threatening toxicity and secondary malignancy were found. EMA-EP regimen was used for ten patients who were resistant to EMA-CO and three who relapsed after EMA-CO. Of those, 11 patients (84.6%) achieved complete remission. We conclude that EMA-CO regimen is an effective and safe primary therapy for high-risk GTN, but not an appropriate second-line protocol. The number of metastatic organs is an independent prognostic factor for the patient with high-risk GTN. EMA-EP regimen is a highly effective salvage therapy for those failing to EMA-CO.
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PMID:EMA-CO chemotherapy for high-risk gestational trophoblastic neoplasia: a clinical analysis of 54 patients. 1771 44

Placental site trophoblastic tumor is a rare form of gestational trophoblastic disease, derived from invasive implantation site (intermediate) trophoblastic cells. It is frequently resistant to chemotherapy. Patients with metastases, however, frequently have progressive disease and die despite surgery and multiagent chemotherapy. In this case, a 24-year-old woman was referred because of intermittent vaginal bleeding episodes for 5 months following delivery. Multiple metastases in lungs, liver, kidneys, breast, pancreas, and adrenal and thyroid glands were detected. Combination therapy including surgery and multiagent chemotherapy was planned. Hysterectomy and pelvic lymph node dissection were performed. All metastatic lesions disappeared with EMA-CO treatment. However four courses of BEP regimen, salvage therapy, was performed for plateauing hCG level. Surgery and multiagent chemotherapy seem mainstay of treatment of cases having multiple metastases of PSTTs.
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PMID:Placental site trophoblastic tumor with multiple metastases and complete response to salvage BEP regimen: a case report and review of the literature. 1850 43

Studies in Turkey are hospital based, and there is a wide variation in reported incidences that necessitate population based-studies to determine the real incidence of gestational trophoblastic diseases (GTDs). According to hospital-based studies, the frequency of GTD is generally very high and there are also regional differences. Epidemiologic study was performed to determine the frequency of hydatidiform mole (HM) in a rural part of Turkey. According to this study, the frequency of HM was lower than the frequencies reported by most of the hospital-based studies. In a national study, an inquiry form related to the approach to GTD was sent to obstetrics and gynecology departments. According to this study, a clinical classification system was used for gestational trophoblastic neoplasia by 60% of the hospitals. Methotrexate was the single-agent chemotherapy used most frequently. With regard to first-line combined chemotherapy, MAC (methotrexate, actinomycin, chlorambucil) was the preferred combination. EMA-CO (etoposide, methotrexate, actinomycin D, cyclophosphamide, Oncovin) was the most commonly used second-line chemotherapeutic regimen. From more recent studies, EMA-CO is the first-line combination chemotherapy. There is no national registry system for GTD in Turkey. There appears to be a need to conduct properly designed community-based studies with well-established case registry system in Turkey.
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PMID:Regional perspectives on gestational trophoblastic disease in Turkey. 1877 31

Patient with malignant Gestational Trophoblastic Neoplasm (GTN) was treated by mean of MTX-FA, MAC, EMA-CO and EMA-EP. Changes in serum human chorionic gonadotropine (beta hCG) levels and changes in ultrasonographic findings were checked weekly. Finally transabdominal hysterectomy with ovaries conservation was done and polychemotherapy administrated after the operation until three consecutive serum chorionic gonadotropine values were negative. This is a case report of Invasive mole in 32 years old patient without possibility to preserve reproductive health. GTN developed two months after spontaneous abortion in 13th week gestation. No changes in uterine structure were found during the first ultrasonographic examination. Three months after abortion and one month after GTN confirmed, massive destruction of lateral uterine wall was detected during transvaginal Doppler ultrasound examination. Resistance index of 0,366 was significantly lower than normal, with hypervascularisation in affected tissue. Serum beta hCG confirmed poor effect of polychemotherapy treatment and decision for operative treatment was made. Hystological findings after the operation confirmed malignant GTN- invasive mole. Specific changes in ultrasonographic picture could have an impact in therapy making decision and could not be refereed without the most relevant parameter such is serum human chorionic gonadotropine.
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PMID:Invasive mole--case report of massive uterine destruction. 1912 11

Placental site trophoblastic tumor (PSTT) is a rare type of gestational trophoblastic disease. There is a wide clinical spectrum of presentation and behavior ranging from a benign condition to an aggressive disease with a fatal outcome. PSTT limited to the uterus is in a good prognosis group, but PSTT with metastasis is a lethal disease. We document a case of PSTT with multiple metastases and extremely poor prognosis. A 36-year-old woman had abnormal irregular vaginal bleeding 14 months after her third pregnancy and delivery. The mitotic count of the tumor cells was quite high (23/10 high-power fields). It would have been difficult to remove the tumor by surgery because of the tumor size and its invasion, so we suggested chemotherapy. We treated her with EMA/CO (etoposide, methotrexate, actinomycin-D, cyclophosphamide, vincristine) as a first-line regimen. During the sixth cycle of EMA/CO, the disease became drug-resistant and she died 8 months after the first symptom. This was a rare case among documented patients with PSTT with metastasis, with the patient having short-term survival (<1 year). We conclude that a high mitotic count and atypical undifferentiated pathological features are significant poor prognostic factors for survival in PSTT.
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PMID:Placental site trophoblastic tumor (PSTT) with multiple metastases and extremely poor prognosis. 1985 56

A 53-year-old woman with choriocarcinoma (high-risk gestational trophoblastic disease: FIGO score 17) was treated with paclitaxel (175 mg/m(2)) and carboplatin (AUC=5). The patient was treated with an EMA/CO regimen as initial chemotherapy, but she developed EMA/CO-induced interstitial lung disease after the 3rd course of treatment. After high-dose steroid therapy, she received combination chemotherapy with paclitaxel and carboplatin. Her hCG-/b dropped to <0.1 ng/mL after 11 courses of the chemotherapy. A paclitaxel+carboplatin regimen is potentially effective for high-risk GTD, but a more effective combination or schedule with a platinum-taxane regimen should be explored.
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PMID:[A case of metastatic choriocarcinoma responding to combination chemotherapy with paclitaxel and carboplatin]. 2049 35


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