Metastatic placental site trophoblastic tumor. Report of a case with complete response to chemotherapy

BACKGROUND: Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic neoplasia, commonly insensitive to chemotherapeutic agents. CASE: We report on long-term remission in a patient with metastatic PSTT after etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine combination chemotherapy. The 27-year-old patient with metastatic lung PSTT was alive, without evidence of disease, > 40 months after treatment. CONCLUSION: Treatment with multiagent chemotherapy can produce long-term remission, even in patients with metastatic PSTT.     

Chemoresistant placental site trophoblastic tumor with hilar lymph node metastasis: an unusual site of involvement

BACKGROUND: Placental site trophoblastic tumor (PSTT) is an uncommon variant of gestational trophoblastic diseases. In most cases, disease is confined to the uterus and treated with a simple hysterectomy. However, 30% of these patients will present with metastatic disease. Patients with metastases frequently have progression of disease and die despite aggressive multiagent chemotherapy. CASE: We present a case of 33-year-old female with PSTT and metastases to the hilar lymph nodes of the right lung. Primary surgical treatment consisting of abdominal hysterectomy and unilateral salpingo-oophorectomy was followed by six cycles of EMA/CO (etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine) chemotherapy. After the completion of chemotherapy, betahCG titers stayed within normal range, but a repeated CT scan of chest revealed enlargement of the hilar lymph nodes. The patient underwent right thoracotomy with hilar lymph nodes resection. The resected nodules were pathologically consistent with primary PSTT. CONCLUSION: In this case report, we have determined a PSTT with hilar region metastasis other than parenchyma of lung and confirmed the chemoresistant nature of tumor with the guidance of the previous reports.     

Placental site trophoblastic tumor presenting as subaponeurotic metastasis

Cases of metastatic placental site trophoblastic tumor (PSTT) have a very poor prognosis because these tumors tend to be less sensitive to chemotherapy than other types of gestational trophoblastic disease. We describe the case of a 25-year-old woman who presented with occipital tumor and abnormal vaginal bleeding. Hysterectomy, bilateral salpingo-oophorectomy, and occipital tumor removal revealed a primary PSTT in the uterus, with ovarian and occipital subaponeurotic metastases. She received etoposide, methotrexate, actinomycin-D/cyclophosphamide, vincristine chemotherapy and had a complete clinical remission. Fifteen months later, she had a recurrent subaponeurotic occipital tumor invading the cranium and underwent tumor removal along with cranial bone followed by local irradiation. She was then treated with etoposide, cis-platinum/etoposide, methotrexate, actinomycin-D chemotherapy and again had a remission for 5 months. The patient, however, had a left parietal subaponeurotic tumor, invading the dura mater, and received local irradiation. Soon after, she developed left orbital bone metastasis, treated by local irradiation. These bone metastases responded to the radiation completely. However, multiple organ metastases were found, and she died of the disease. This represents the first case of PSTT with initial subaponeurotic metastasis in a living patient. New modalities of treatment for high-risk or metastatic PSTT need to be developed.     

Prolonged remission of recurrent, metastatic placental site trophoblastic tumor after chemotherapy

BACKGROUND: Placental site trophoblastic tumor (PSTT) is a form of gestational trophoblastic neoplasm that is frequently resistant to chemotherapy. In most cases disease is confined to the uterus and can be cured by curettage or simple hysterectomy. Patients with metastases, however, frequently have progression of disease and die despite aggressive multiagent chemotherapy. CASE: A 31-year-old woman was found on review of uterine curettings to have a PSTT. Imaging studies revealed multiple lung lesions, a liver lesion, and an enlarged irregular uterus. Hysterectomy and staging surgery revealed a large tumor in the endometrial cavity and multiple metastases. She was treated with etoposide-methotrexate-dactinomycin and cyclophosphamide-vincristine and had a complete clinical remission. Six months later, however, she had a recurrence. She was then treated with six cycles of etoposide-methotrexate-dactinomycin and etoposide-cisplatin. Three years after completion of the second regimen she is without evidence of disease. CONCLUSION: Treatment with multiagent chemotherapy can produce long-term remission, even in patients with recurrent, metastatic PSTT. Addition of platinum may be helpful in patients who have recurred or progressed after treatment with non-platinum-containing regimens.     

Placental site trophoblastic tumour: clinical features and management

OBJECTIVE: To describe the clinical features, treatment and outcome of all consecutive patients with placental site trophoblastic tumour (PSTT) treated at the Sheffield Trophoblast Centre and to compare these findings to other reports. METHOD: All cases of PSTT on the Sheffield Trophoblastic Tumour Centre database from 1984 to 2004 were reviewed. Data obtained included age at diagnosis, antecedent pregnancy (AP), interval from antecedent pregnancy until diagnosis, presenting features, presenting serum human chorionic gonadotrophin hormone (hCG) level, number and sites of metastases, treatment received, outcome and follow-up. RESULTS: Seventeen patients with PSTT were identified from the database which incorporates a total of 7489 cases of trophoblastic disease. Fourteen (70.6%) were more than 30 years old at presentation; 5 were over 40. The median interval from pregnancy to diagnosis was 18 months (range 6 months to 22 years). The outcome of antecedent pregnancy was a female in 11 out of the 13 patients where the sex was known. Eleven (70.6%) of patients presented with irregular vaginal bleeding, with or without a preceding period of amenorrhoea. All 8 patients with non-metastatic (Stage I) disease were alive and well after hysterectomy (6), chemotherapy alone (1) or hysterectomy and chemotherapy (1) whereas only 4 of 9 patients with metastatic (Stage III/IV) disease were alive and well after treatment with chemotherapy and hysterectomy. CONCLUSION: PSTT is rare and accounts for 0.23% cases of gestational trophoblastic disease referred to this centre. It has a variety of presenting features and its course is unpredictable. Metastatic involvement and antecedent pregnancy interval greater than 4 years are poor prognostic factors. Hysterectomy is the primary mode of treatment in the majority of cases. However, chemotherapy can still play a major role when curative surgery is not feasible.     

EMA/EP方案治疗耐药性滋养细胞肿瘤疗效的初步分析

目的 分析足叶乙甙 甲氨蝶呤 放线菌素D／足叶乙甙 顺铂(EMA／EP)方案治疗耐药性恶性滋养细胞肿瘤的疗效。方法 回顾性分析了15例耐药性恶性滋养细胞肿瘤患者,采用EMA／EP方案化疗,部分患者辅以手术或超选择性动脉插管化疗,观察其疗效及毒副反应。结果 15例患者化疗的平均疗程数为6．2个,化疗后11例获完全缓解(73％),3例部分缓解(20％),1例无效(7％)。其中,3例转移性胎盘部位滋养细胞肿瘤(PslTr)经EMA／EP方案治疗后均完全缓解。该方案的主要毒副反应为骨髓抑制及消化道反应。结论 EMA／EP是治疗耐药性恶性滋养细胞肿瘤患者的有效方案。对于转移性PSTT患者EMA／EP可作为首选化疗方案。     

Placental site trophoblastic tumor presenting with scalp metastases

Placental site trophoblastic tumor (PSTT) usually presents with vaginal bleeding or amenorrhea and an enlarged uterus. Metastasis to the skin as the presenting sign, or as a metastatic site, has not been previously reported with PSTT. We report a case of PSTT in which the presenting sign was scalp metastases and the only other disease was a small focus in the uterus. The patient responded to multi-agent chemotherapy and repeated skin resection at the local site. She received 16 alternating cycles of etoposide-methotrexate-actinomycin D and cytoxan-oncovin (EMA/CO) and is currently without evidence of disease. Clinicians caring for reproductive age women should remain aware that gestational trophoblastic disease (GTD) may present in an unusual manner.     

EMA/EP chemotherapy for chemorefractory gestational trophoblastic tumor

OBJECTIVE: To evaluate the results of etoposide/ methotrexate/actinomycin D/etoposide/cisplatin (EMA/ EP) chemotherapy in patients with chemorefractory gestational trophoblastic tumor (GTT). STUDY DESIGN: Fifteen patients with chemorefractory GTT were treated with EMA/EP. RESULTS: Twelve of the 15 cases were choriocarcinoma, and the last 3 were metastatic placental site trophoblastic tumor (PSTT): International Federation of Gynecology and Obstetrics (FIGO) stage I, 2 cases; stage III, 10 cases; stage IV, 3 cases. Seven cases have FIGO score 7-10; the scores of the remaining 8 cases were > 10. Fifteen patients received a total of 93 cycles of the study regimen. The median number of courses for each patient was 6.2. Eleven cases (73.3%) achieved complete remission, while 3 (20%) had partial remission; 1 case (6.7%) showed no response. The main complications of EMA/EP chemotherapy were myelosuppression and gastrointestinal symptoms. CONCLUSION: The EMA/EP regimen is effective for chemorefractory GTT, and the chemotherapeutic results can be improved when combined with surgery and arterial infusion chemotherapy in selected patients. The EMA/EP regimen should be considered for primary management of metastatic PSTT.     

Failure of high-dose chemotherapy with peripheral blood stem cell support for refractory placental site trophoblastic tumor

Placental site trophoblastic tumor (PSTT) is a rare form of gestational trophoblastic disease. The metastatic and refractory cases have a very poor prognosis. To our knowledge, this is the first report of the application of high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) for the treatment of refractory metastatic PSTT. A 36-year-old woman had a metastatic PSTT refractory to several lines of chemotherapy. She was treated with high dose of carboplatin and etoposide with autologous PBSCT. She showed only a temporary response to high-dose chemotherapy with PBSCT support and died of disease.     

Clinical and pathologic characteristics and prognosis of placental site trophoblastic tumor

OBJECTIVE: To analyze the clinical and pathologic characteristics of placental site trophoblastic tumor (PSTT) cases and to discuss the diagnosis, treatment and prognosis of PSTT. STUDY DESIGN: The clinical and pathologic data on 11 patients with PSTT at Peking Union Medical College Hospital (PUMCH) from 2000 to 2005 were analyzed retrospectively using SPSS 11.0 software (Chicago, Illinois). RESULTS: Between 2000 and 2005, 635 patients with gestational trophoblastic neoplasms were treated at PUMCH, 11 with PSTT (1.73%). The mean age was 36 years. The antecedent pregnancy was molar in 5 cases (45.5%), full-term delivery in 4 cases (36.4%) and missed abortion in 2 cases (18.2%). The mean interval from the antecedent pregnancy to diagnosis was 16 months. The most common presentations were vaginal bleeding (72.7%) and amenorrhea (63.6%). All patients were pathologically diagnosed, in most cases with human placental lactogen immunohistochemical stain. Chemotherapy and hysterectomy were performed on all patients. Nine complete remissions and 1 partial remission were attained after therapy. CONCLUSION: Pathologic diagnosis of PSTT was the gold standard. Multidrug chemotherapy combined with hysterectomy was effective in metastasis cases. (J Re-     

Successful management of metastatic placental site trophoblastic tumor with multiple pulmonary resections

INTRODUCTION: Placental site trophoblastic tumor (PSTT) is an uncommon variant of gestational trophoblastic disease. Most of these tumors are confined to the uterus and treated with a simple hysterectomy. However, 30% of these patients will present with metastatic disease. These patients are typically treated with a hysterectomy followed by adjuvant multiagent chemotherapy. Unfortunately, PSTT is relatively resistant to chemotherapy when compared to other forms of gestational trophoblastic disease. Consequently, these patients have a poor prognosis. CASE: We present a case report of a 26-year-old female with multiple metastatic lesions to the lungs unresponsive to chemotherapy who was managed with multiple pulmonary resections. She has remained clinically free of disease at 28 months of follow up. CONCLUSION: A patient with metastatic PSTT was successfully managed with radical surgical resection of chemotherapy-resistant sites.     

胎盘部位滋养细胞肿瘤的诊治

胎盘部位滋养细胞肿瘤是一种较少见的妊娠滋养细胞肿瘤,其最常见的临床表现为停经和阴道流血,确诊必须以组织病理学诊断为依据,免疫组化检查在其诊断与鉴别诊断中具有重要意义。其对化疗的敏感性不如其他类型滋养细胞肿瘤,手术是首选的治疗方法,对有不良预后因素的患者还应多药联合化疗进行综合治疗。对于年轻、有生育要求、且子宫病灶局限的病例可以考虑采用保留生育功能治疗。     

Placental site trophoblastic tumor: Can it be treated by chemotherapy alonewithout surgery?

Nam J-H, Kim J-H, Park Y, Huh J, Kim Y-M, Kim Y-M, Mok J-E. Placental sitetrophoblastic tumor: Can it be treated by chemotherapy alone without surgery? Int J Gynecol Cancer 1997; 7 : 381–387.
Placental site trophoblastic tumor (PSTT) is a rare form of gestationaltrophoblastic disease and the mainstay of treatment is surgical removal of thetumor by hysterectomy.However, if retention of fertility is desired, several authors have suggestedthat conservative management can be adopted. This report presents twoadditional cases of PSTT treatedby chemotherapy alone following endometrial curettage without definitivesurgery. One patient, a 33 year-old gravida one, para one female with nometastatic lesion, was treatedwith four cycles of MTX-CF and exhibited successful remission for 13 monthsuntil the time of this report. The other patient, a 25 year-old gravida two,para one female withmultiple metastatic nodules in lung and brain, was managed by five cycles ofEMACO regimen and whole-brain irradiation of 4,000 cGy. Metastatic nodulesdisappeared and serumbeta-hCG level was normalized after completion of therapy for 11 months throughthe time of this report. We provide a report of these two cases combined with a review of theliterature on the issue of conservative treatment of PSTT by chemotherapyalone.     

Metastatic placental site trophoblastic tumor: a case report

Placental site trophoblastic tumor (PSTT) is very rare. It is an unusual variant of gestational trophoblastic neoplasia usually confined to the uterus, although 10% of patients have metastases. The clinical behaviour of PSTT varies and despite knowledge of its histology, diagnosis of this rare form of trophoblastic disease and prediction of its biological behaviour remains difficult due to only a few cases reported in literature.     

Placental site trophoblastic tumor--a challenging rare entity

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.     

Resolution of pulmonary metastases with chemotherapy in a patient with placental site trophoblastic tumor

Placental site trophoblastic tumor (PSTT), a rare variant of gestational trophoblastic disease, was first described in 1976. PSTT is usually seen in young women, generally treated by hysterectomy, and is associated with a 20% fatality rate. The development of metastases secondary to PSTT is associated with an extremely poor prognosis. Metastatic PSTT has generally been resistant to chemotherapy although one complete and some partial responses have been noted previously. We report a case of a complete chemotherapeutic response in a patient with pulmonary metastases.     

Case of PSTT treated with chemotherapy followed by open uterine tumor resection to preserve fertility

BACKGROUND: Placental site trophoblastic tumor (PSTT) is a rare variant of gestational trophoblastic malignancy, usually seen in young women with a 20% fatality rate. The hysterectomy is general for PSTT, but hysterectomy is undesirable for patients who wish to remain fertile. Recent advancement of chemotherapy and tumor detection and assessment technologies should allow removal of tumor from the uterus by conservative surgery, without losing fertility, although very few cases have been reported to date. This report describes a young PSTT patient treated by combination chemotherapy and open uterine surgery, which resulted in an early restoration of the menstrual cycle and apparent preservation of fertility. CASE: A 26-year-old secundigravida primipara woman presented with a case of PSTT which was diagnosed 4 months after a spontaneous abortion. The tumor was confined to the uterus. Two courses of EMA/CO chemotherapy resulted in a remarkable reduction of the tumor mass, but low levels of serum beta-hCG persisted. After precise evaluation of the residual tumor by MRI and hysteroscopy, the anterior wall of the uterus was opened to resect the tumor in the posterior myometrium. An argon beam coagulator was used to evaporate the myometrium tissue surrounding the lesion. One week later, the patient had normal menstruation. MRI taken 2 weeks after the operation detected no tumor in the uterus nor uterine deformation. Serum beta-hCG was reduced below the level of detection. CONCLUSIONS: Open uterine resection of PSTT tumor following appropriate chemotherapy could achieve long-term remission and save fertility of young patients who wish to avoid hysterectomy for future pregnancy.     

25 years' experience in the treatment of gestational trophoblastic neoplasia in Hungary

OBJECTIVE: To review our clinical experience in the treatment of gestational trophoblastic neoplasia (GTN) over the past 25 years in our national trophoblastic disease center. STUDY DESIGN: Between January 1, 1977, and December 31, 2001, we treated 355 patients with GTN. The patients were between 14 and 53 years of age, with an average of 28.3. Primary chemotherapy was selected based on the patient's stage of gestational trophoblastic tumor (GTT) and prognostic score. RESULTS: We found metastases in 49.3% (175 of 355) of our patients. Of 173 patients, 162 (93.2%) achieved remission as a result of methotrexate therapy. In 11 patients (6.8%) complete remission was achieved by combination chemotherapy, in some cases assisted by operation. Of 68 patients, 63 (92.6%) achieved remission as a result of actinomycin D therapy, and 5 (7.4%) achieved complete remission by combination chemotherapy. Chemotherapy, surgical intervention or other supplementary treatments resulted in 100% successful therapy in cases of nonmetastatic and low-risk metastatic disease. CONCLUSION: According to our experience, methotrexate/folinic acid or actinomycin D should be the primary treatment in patients with nonmetastatic or low-risk metastatic GTN. Patients with resistance to single-agent chemotherapy regularly achieve remission with combination chemotherapy.     

Tumor trofoblástico del lecho placentario: reporte de un caso

Placental site trophoblastic tumour (PSTT) is a very rare pathology that is part of the group of gestational trophoblastic neoplasia (GTN). It generally develops after a nonmolar abortion or term pregnancy from the pathological proliferation of extravillous intermediate trophoblast that can occur months or years after gestation. Its usual clinical presentation is abnormal uterine bleeding, amenorrhoea, and low levels of β-hCG, in contrast to choriocarcinoma or invasive mole. The treatment of choice is hysterectomy along with pelvic lymphadenectomy. It is particularly resistant to chemotherapy and 30% of patients present metastatic disease on diagnosis. A clinical case of PSTT diagnosed at our centre in July 2018 in a patient with infertility who, after 3 spontaneous abortions, debuted with low levels of β-hCG but on a plateau and with an upward trend. The diagnostic and therapeutic approach she received is addressed, as well as the evolution to date.