This analysis aimed to assess the effects of current management on clinical outcomes and to identify potential prognostic indicators in ett. Epithelioid trophoblastic tumor ett is a term proposed for an unusual variant of trophoblastic tumor that is closely related to choriocarcinoma but shows monomorphic growth of highly atypical. These tumours are rare, and they appear when cells in the womb start to proliferate uncontrollably. Epithelioid trophoblastic tumor is rare and there is little information available in the literature regarding its appearance on ultrasound imaging. Epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Epithelioid trophoblastic tumor ett is a relatively recently described entity, distinct from placental site trophoblastic tumor pstt and choriocarcinoma cc. The objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor ett. Gtd is rare, affecting about one in every 1,000 pregnant women in the u. Gestational trophoblastic tumor genetic and rare diseases.
Epithelioid trophoblastic tumor ett is a term proposed for an unusual variant of trophoblastic tumor that is closely related to choriocarcinoma but shows monomorphic growth of highly atypical trophoblastic cells instead of the typical dimorphic pattern of choriocarcinoma. Other than choriocarcinoma, these tumors are very rare in the testis but have been reported on occasion in association with other germ cell tumors. The patients with this neoplasm were in the reproductive age group and presented with abnormal vaginal bleeding. Update on the pathology of gestational trophoblastic. A surgical approach is recommended for ett patients with earlystage disease. Update on the pathology of gestational trophoblastic disease. Epithelioid trophoblastic tumors journal of cancer. Gestational trophoblastic neoplasia gtn is a type of gestational trophoblastic disease gtd that is almost always malignant. It is pearshaped, hollow, and located in a womans pelvis between her bladder and rectum. Primary epithelioid trophoblastic tumor of the lung the. All three patients were of reproductive age and presented with vaginal bleeding and mild elevation of human chorionic gonadotropin hcg. It derives from intermediate trophoblastic cells of chorion laeve, while placentalsite trophoblastic tumor. Mixed germ cell tumor with embryonal carcinoma, choriocarcinoma, and epithelioid trophoblastic tumor in the ovary of a cynomolgus monkey y. Epithelioid trophoblastic tumor found on hysteroscopy zhang.
Placental site trophoblastic tumors and epithelioid. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor. Mitotic count of 610hpf is an unfavorable prognostic factor. Placental site pstt and epithelioid trophoblastic tumor ett are rare types of gestational trophoblastic neoplasia gtn that arise from intermediate trophoblast. Original article extrauterine epithelioid trophoblastic tumor. Emaco for highrisk gestational trophoblastic neoplasia. Epithelioid trophoblastic tumors etts are resistant to chemotherapy. Gestational trophoblastic disease gtd is a group of rare diseases in which abnormal trophoblast cells grow inside the uterus after conception. Objectives epithelioid trophoblastic tumor ett is very rare. The tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. Cases of ett present as abnormal vaginal bleeding in women of reproductive age.
Ett patients with advancedstage disease or an interval of. Pdf epithelioid trophoblastic tumor alexandros rouvas and. Original article extrauterine epithelioid trophoblastic tumor of the vagina. Seven cases of epithelioid trophoblastic tumor treated in the womens hospital of zhejiang university from 2004 september to 2008 december were retrospectively analyzed.
A 36yearold woman presented with a history of prolonged menstrual period and increased menstrual volume of 4 months. Due to the rarity of disease, epithelioid trophoblastic tumor remains a diagnostic challenge despite intense research e. Gestational trophoblastic disease gtd affects the uterus. Tumors of trophoblastic derivation in testis show morphologic spectrum analogous to the trophoblastic tumors of the female genital tract including choriocarcinoma, epithelioid trophoblastic tumor ett and placental site trophoblastic tumor pstt. Placental site trophoblastic tumor and epithelioid trophoblastic tumor are rare gestational trophoblastic neoplasias. Epithelioid trophoblastic tumor is a malignancy derived from the chorionic laevetype intermediate trophoblast with sufficient rarity that the vast majority of literature on the topic exists in the form of case reports and small series. This protocol is not required for accreditation purposes for the following. Epithelioid trophoblastic tumors etts are the rarest type of gestational trophoblastic neoplasias. Placental site trophoblastic tumor is composed of extravillous trophoblast of implantational type. Epithelioid trophoblastic tumor archives of pathology.
Ett can be a sequela of any gestational event and can present in both intrauterine and extrauterine sites. The tumor cells are proposed to be of chorionic type intermediate trophoblastic cell lineage due to their. This report describes the clinicopathologic and immunohistochemical features of 14 cases of epithelioid trophoblastic tumor ett, a distinctive but rare gestational trophoblastic tumor. Initially described as atypical choriocarcinoma, shih and kurman first reported the clinicopathological features of ett in 1998 3, and the world health organization 4. Epithelioid trophoblastic tumor found on hysteroscopy a 36yearold woman presented with a history of prolonged menstrual period and increased menstrual volume of 4 months. Ultrasonography showed inhomogeneous echo measuring 2. Mr imaging of uterine epithelioid trophoblastic tumor. These tumors arise from various subtypes of placental trophoblast, and each has a distinct pathobiology attributable to the proliferative ability of its constituent trophoblast. Epithelioid trophoblastic tumor is a rare neoplasm and represents the most recent addition to the gestational trophoblastic tumor category. Classically, it is regarded as a wellcircumscribed tumor with an expansile growth pattern that occurs in. Gestational trophoblastic tumors include 3 distinct entities. The uterus is a part of a womans reproductive system. The uterus is also known as the womb, where a fetus, or unborn baby, grows when a woman is pregnant.
Misdiagnosis delays effective treatment and affects the survival. We report the first case of epithelioid trophoblastic tumor ett presenting as primary metastasis to the spine. Ett is currently regarded as an unusual type of trophoblastic tumor, distinct from choriocarcinoma and placental site trophoblastic tumor. Metastatic spread to areas such as the lung and spine has been described previously. Epithelioid trophoblastic tumor, the most recent addition to gestational trophoblastic diseases, is a rare but distinctive proliferative lesion of the putative chorionictype intermediate. Chen, coexisting epithelioid trophoblastic tumor and placental site trophoblastic tumor of the uterus following a term pregnancy. Given that this cell of origin is different from other forms of gtn, it is not surprising that the clinical presentation, tumor marker profile, and treatment paradigm for pstt and ett are quite different as well. Epithelioid trophoblastic tumor ett is a rare type of gestational trophoblastic disease and only 25 cases have been reported so far.
It is a trophoblastic tumor of neoplastic chorionic type associated with the intermediate trophoblast. Extrauterine epithelioid trophoblastic tumor in hysterectomized woman. Intermediate trophoblastic cells can be villous or extravillous in form of implantationsite and chorionic leave type. In the absence of tissue for a definitive histopathologic diagnosis, disease diagnosed as a result of persistent elevation of human chorionic gonadotropin hcg. Placental site trophoblastic tumor pstt and epithelioid trophoblastic tumor ett are the rarest gestational trophoblastic neoplasias, developing from intermediate trophoblast of the implantation site and chorion leave, respectively. Protocol for the examination of specimens from patients. Epithelioid trophoblastic tumor ett is an extremely rare potentially highrisk gestational trophoblastic neoplasia. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational trophoblastic. Epithelioid trophoblastic tumors etts are the rarest type of gestational trophoblastic.
Epithelioid trophoblastic tumor ett is a relatively recently described entity, distinct from placental site trophoblastic tumor pstt and choriocarcinoma cc, with a resemblance to squamous cell carcinoma. Patients diagnosed at early stage should be treated surgically with adjuvant chemotherapy reserved for high risk cases. Epithelioid trophoblastic tumor ett is an extremely rare form of gestational trophoblastic neoplasia gtn. Procedure biopsy curettage primary resection specimen with no residual cancer eg, following neoadjuvant therapy. Epithelioid trophoblastic tumor of the lung urabe 2007. Epithelioid trophoblastic tumor ett is a rare gestational trophoblastic neoplasm of chorionic type intermediate trophoblasts, and it is most frequently located in the lower uterine segment and endocervix.
Cases of ett present as abnormal vaginal bleeding in women of reproductive age, with low human chorionic gonadotropin hcg levels. Gestational trophoblastic tumors gtt are malignant forms of gestational trophoblastic disease. Gestational trophoblastic neoplasia gtn encompasses a spectrum of histologic entities including invasive mole, choriocarcinoma, placental site trophoblastic tumor, and the extremely rare epithelioid trophoblastic tumor. Pdf epithelioid trophoblastic tumour ett is an unusual type of trophoblastic tumour, which can cause difficulties in diagnosis and as a. Gestational trophoblastic disease johns hopkins medicine. Here we report on the clinicopathologic and immunohistochemical features of three cases of epithelioid trophoblastic tumor. This rare tumor is composed of epithelioid cells with large atypical nuclei and moderate to abundant clear to eosinophilic cytoplasm, resulting in a carcinomalike appearance. Thisunique typeoftumor developsin intermediate trophoblastic cells. Synchronous choriocarcinoma and epithelioid trophoblastic tumor concurring at the cesarean scar. Epithelioid trophoblastic tumor ett is an extremely rare subtype of gestational trophoblastic neoplasia gtn, accounting for only 1.
Gestational trophoblastic disease gtd is a term used for a group of pregnancyrelated tumours. It was first proposed by mazur and kurman in 1994 as an unusual type of trophoblastic tumor that is distinct from placental site trophoblastic tumor and choriocarcinoma and has features resembling carcinoma. Signs and symptoms of gestational trophoblastic disease. Pdf epithelioid trophoblastic tumor ett is a rare gestational trophoblastic neoplasm of chorionictype intermediate trophoblasts, and it is most. Ett used to be called atypical choriocarcinoma because the cells look like choriocarcinoma cells under the microscope, but it is now thought to be a separate disease.
Epithelioid trophoblastic tumor ett is an extremely rare type of gestational trophoblastic disease that can be hard to diagnose. Hydatidiform mole hm is the most common type of gtd. Primary epithelioid trophoblastic tumor of the lung. Epithelioid trophoblastic tumor ett is a recently described neoplastic proliferation of intermediate trophoblast that. Methods we applied a literature search using pubmed to analyze the clinical data of 78. To describe a case of epithelioid trophoblastic tumor ett in a postmenopausal woman, which had several peculiar features that differentiate it from previously reported etts. Epithelioid trophoblastic tumor generally originates in the uterus, and abnormal vaginal bleeding is the characteristic presentation. Epithelioid trophoblastic tumor ett is a gestational trophoblastic disease with about 110 case reports in the literature. Epithelioid trophoblastic tumor most commonly appears in women of reproductive age, with a mean age at presentation of 38 years. Epithelioid trophoblastic tumor ett is a recently described neoplastic proliferation of intermediate trophoblast that is thought by some investigators to be. We investigated the clinical features, treatments, outcomes, and prognostic factors in patients with ett, and explored potential therapeutic targets. Stage i disease is treated with hysterectomy, while metastatic disease is treated with surgery and chemotherapy.
While some gtd tumors are malignant cancerous or have the potential to turn cancerous, the majority are benign. Ovarian epithelioid trophoblastic tumor in a cynomolgus monkey. Placental site trophoblastic tumor and epithelioid. Trophoblastic tumor an overview sciencedirect topics. Gestational trophoblastic disease is the name given to a group of tumors that form during abnormal pregnancies. Accurate diagnosis is important for clinical management of the patient.
Gestational trophoblastic neoplasia gtn gestational neoplasms include. Gestational trophoblastic disease gtd describes a spectrum of neoplastic conditions derived from the placenta, including hydatidiform moles, postmolar gestational trophoblastic neoplasia gtn, gestational choriocarcinoma, placental site trophoblastic tumor pstt, and epithelioid trophoblastic tumor ett. Etts exhibit slow growth and remain confined to the uterus for extended periods of time. In contrast to other forms of gestational trophoblastic diseases, approximately 50% of reported epithelioid trophoblastic tumors arose from the. Synchronous choriocarcinoma and epithelioid trophoblastic.
An unusual malignancy of ovary humera mahmood1, mohammad faheem 1, mohammad tahir2, sarosh arif, sana mahmood and javaid irfan1 abstract epithelioid trophoblastic tumor ett is a distinct but rare variety of gestational trophoblastic tumors. Epithelioid trophoblastic tumor found on hysteroscopy. Case report epithelioid trophoblastic tumor after induced abortion. Mar 01, 2011 the tumor always follows pregnancy, most often molar pregnancy hydatidiform mole. For example, if it has spread to the lung, the patient might cough or be short of breath. Due to the epithelialgrowth pattern with geographic necrosis exhibited by the neoplastic. The most common symptom of an epithelioid trophoblastic tumor ett is vaginal bleeding. Sonographic appearance of gestational trophoblastic. Pathology outlines epithelioid trophoblastic tumor. Jan 20, 2010 the objective of this study is to evaluate the clinicopathological features and immunohistochemical characteristics of epithelioid trophoblastic tumor ett. Gestational trophoblastic disease gtd includes complete and partial hydatiform mole, invasive mole, choriocarcinoma, placental site trophoblastic tumour and.
Epithelioid trophoblastic tumor is a very rare tumor, which is grouped into a spectrum of gestational trophoblastic disease, along with hydatidiform moles, invasive mole, and choriocarcinoma 2. Pdf epithelioid trophoblastic tumor alexandros rouvas. Pstt and ett share some clinicalpathological features, such as slow growth rates, early stage at presentation, relatively low. Epithelioid trophoblastic tumor ett is a rare trophoblastic tumor originating from chorionictype inter mediate trophoblasts its. Gestational trophoblastic neoplasms gtn are a group of neoplasms from fetal trophoblastic cells including choriocarcinoma cc, epithelioid trophoblastic tumors ett, and placental site trophoblastic tumors pstt 1. Its most striking feature is the fact that the growth pattern mimics that of a carcinoma. Ovarian epithelioid trophoblastic tumor in a cynomolgus. The cells that form gestational trophoblastic tumours are called trophoblasts and come from tissue that grows to form the placenta during pregnancy. Ett of the uterus is a rare form of trophoblastic tumor with only 100 cases distinguished until now. Pdf mr imaging of uterine epithelioid trophoblastic tumor.
Protocol for the examination of specimens from patients with. The number of mitotic figures varies, but at least a low level of mitotic activity is present. Epithelioid trophoblastic tumors the most common symptom of an epithelioid trophoblastic tumor ett is vaginal bleeding. Clinical and imaging features1 gestational trophoblastic disease gtd is a spectrum of both benign and malignant gestational tumors, including hydatidiform mole complete and partial, invasive mole, choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor. Pdf epithelioid trophoblastic tumor of the endocervix. Sonographic appearance of gestational trophoblastic disease. Dec 02, 2012 epithelioid trophoblastic tumor ett is a rare gestational trophoblastic tumor.
435 117 1209 501 24 1045 131 299 161 702 1285 935 53 756 881 1570 226 1278 122 837 1501 1507 679 878 813 490 553 191 1363 876 949 1207 602 1044 1322 1308 1320 1288 9 302 402 1347 1392 1193 992 20 1026 168 373 660